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Table of contents

1. Immunodeficiency states in children. Clinic, diagnosis, treatment (Primary immunodeficiencies. Secondary immunodeficiency conditions. Principles of treatment of immunopathological syndromes)
2. Vegetative-vascular dystonia. Arterial hypertension. Clinic, diagnosis, treatment (Vegetative-vascular dystonia. Arterial hypertension)
3. Cardiomyopathy in children. Clinic, diagnosis, treatment (Dilated cardiomyopathy. Hypertrophic cardiomyopathy. Restrictive cardiomyopathy)
4. Diseases of the pericardium in children. Clinic, diagnosis, treatment (Congenital pericardial defects. Pericarditis. Pericardial tumors)
5. Chronic heart failure in children. Clinic, diagnosis, treatment (Clinical manifestations. Clinical manifestations of chronic heart failure)
6. Cardiac arrhythmias in children. Clinic, diagnosis, treatment (Dysfunction of automaticity function. Disorders of conduction function)
7. Systemic vasculitis in children. Clinic, diagnosis, treatment (Microscopic polyangiitis. Churg-Strauss syndrome. Wegener's granulomatosis. Behçet's disease. Polyarteritis nodosa)
8. Renal failure. Clinic, diagnosis, treatment (Acute renal failure. Chronic renal failure (CRF))
9. Differential diagnosis of diffuse connective tissue diseases in children. Clinic, diagnosis, treatment (Systemic lupus erythematosus. Scleroderma (Morphea) localized. Dermatomyositis)
10. Chronic diseases of the colon in children. Clinic, diagnosis, treatment (Chronic constipation. Irritable bowel syndrome. Diverticular disease. Organic disorders of the colon)
11. Modern problems of dysbacteriosis in children. Clinic, diagnosis, treatment
12. Malabsorption syndrome in children. Clinic, diagnosis, treatment (Enzymopathy. Endocrine pancreatic insufficiency. Insufficiency of bile acids. Stomach dysfunction. Impaired motility. Pathology of the intestinal mucosa)
13. Differential diagnosis of liver diseases in children. Clinic, diagnosis, treatment
14. Medical illness in children
15. Helminthiasis in children. Clinic, diagnosis, treatment, prevention (Ascariasis. Alveococcosis. Hookworm disease (hookworm and necatoriasis). Diphyllobothriasis. Opisthorchiasis. Teniosis. Trichocephalosis. Fascioliasis. Echinococcosis. Enterobiasis)
16. Rheumatism in children and adolescents. Clinic, diagnosis, treatment
17. Broncho-obstructive syndrome. Clinic, diagnosis, treatment. Respiratory failure. Clinic, diagnosis, treatment (Acute bronchitis. Respiratory failure)
18. Congenital and hereditary lung diseases (Agenesis, aplasia and hypoplasia of the lungs. Polycystic lung disease. Congenital lobar emphysema. Williams-Campbell syndrome. Tracheobronchomegaly. Mounier-Kuhn syndrome. Primary ciliary dyskinesia (fixed cilia syndrome) and Kartagener syndrome. Idiopathic diffuse pulmonary fibrosis (Hammen-Rich syndrome, idiopathic fibrosing alveolitis - ELISA). Primary pulmonary hypertension (AERSA ​​syndrome). Idiopathic pulmonary hemosiderosis (Zelen-Gellerstedt syndrome). Goodpasture syndrome. Connective tissue pathology. Alveolar microlithiasis. Alveolar proteinosis. Lung lesions due to deficiency of a - protease inhibitor. Cystic fibrosis)
19. Respiratory diseases (Acute bronchitis. Clinic, diagnosis, treatment, prevention. Chronic bronchitis. Clinic, diagnosis, treatment, prevention)

LECTURE No. 1. Immunodeficiency conditions in children. Clinic, diagnosis, treatment

Immunity is a way to ensure and maintain antigenic homeostasis.

Immunodeficiencies - a decrease in the functional activity of the main components of the immune system, leading to a violation of the antigenic homeostasis of the body and, above all, to a decrease in the body's ability to defend itself against microbes, manifested in an increased infectious morbidity.

Classification of immunodeficiency states:

1) primary immunodeficiencies;

2) secondary immunodeficiencies.

1. Primary immunodeficiencies

Primary immunodeficiencies are genetically determined monogenic diseases transmitted as an autosomal recessive or X-linked trait. There is also an autosomal dominant type of inheritance.

Primary immunodeficiencies are congenital disorders of the immune system with defects in one or more of its components (cellular or humoral immunity, phagocytosis, complement system).

Classification of primary immunodeficiency states:

1) pathology of the humoral link of immunity, i.e., insufficiency in the production of antibodies;

2) pathology of the cellular link of immunity mediated by T-lymphocytes;

3) combined forms (SCID) of humoral and lymphocytic deficiency.

Clinical picture. The clinical picture of immunodeficiency states has common features.

1. Recurrent and chronic infections of the respiratory tract, paranasal sinuses, skin, mucous membranes, gastrointestinal tract, caused by opportunistic infections, protozoa, fungi, tending to generalize, septicemia and torpid to the usual reaction.

2. Hematological deficits: leukocytopenia, thrombocytopenia, anemia.

3. Autoimmune disorders: arthritis, scleroderma, chronic active hepatitis, thyroiditis.

4. Sometimes IDS are combined with allergic reactions in the form of eczema, Quincke's edema.

5. Tumors and lymphoproliferative diseases are more common in IDS.

6. Often IDS are combined with malformations.

7. Patients with IDS have digestive disorders, diarrheal syndrome, malabsorption syndrome.

8. Patients with IDS have unusual reactions to vaccination.

9. Reticular dysgenesis (defect of maturation of lymphoid and myeloid cells).

10. Swiss type SCID (T- and B-lymphocytosis with a-globulinemia).

11. Omenn's syndrome (SCID with eosinophilia and T-cell infiltration of a number of organs).

12. X-linked SCID (low block of T-cell maturation in combination with normal B-cell differentiation).

13. Autosomal recessive SCID.

Severe combined immune deficiency manifests itself in the 1st month of life: symptoms of a severe multi-organ infectious process with malnutrition. On the part of the respiratory system - bronchopulmonary lesion. From the gastrointestinal tract - diarrhea.

Candidiasis. Thymic dysplasia. Immunologically, a defect in stem cell differentiation with profound immunity disorders (immunological silence syndrome) is assumed. Laboratory examination revealed lymphopenia (especially T-lymphocytes), a decrease and disturbance in the ratio of components in the T- and B-systems. Death occurs in the 1st month or 1-2 years of life.

Syndrome of reticular dyskinesia. Clinically - the death of the fetus or child immediately after birth. Immunologically - a defect in the maturation of all lymphoid and myeloid cells in the bone marrow.

"Swiss type" Clinical manifestations - from the 1st month of life, delayed weight gain, candidiasis, prolonged recurrent pneumonia, rashes. Hypoplasia of the thymus and lymph nodes. Immunologically - T- and B-alymphocytosis with agammaglobulinemia (with the exception of IgG in infants). Laboratory testing revealed a decrease in adenosine deaminase levels. Death occurs before the age of 2 years.

Louis-Barr syndrome (ataxia-telangiectasia). Clinical manifestations are characterized by ataxia. Often misdiagnosed as cerebral palsy. In the future - repeated infections of the respiratory system, sinusitis, skin and eye telangiectasia. Hypoplasia of the thymus, cerebellar degeneration. Immunologically - deficiency of T-lymphocytes (especially T-helpers) and immunoglobulins (especially IgA and IgE). Death of a patient under the age of 2 years.

Wiskott-Aldrich syndrome. Clinical manifestations: boys have frequent infections, hemorrhages, eczema, and neoplasms from birth. Immunologically - T-lymphocyte deficiency, isolated IgM deficiency. Laboratory tests revealed thrombocytopenia (maybe isolated). Severity and prognosis are variable (death may occur up to 10 years).

DiGeorge syndrome. Clinical manifestations in the form of prolonged and inflammatory processes in the lungs, dermatitis, heart and vascular defects, hypoparathyroidism (tetany, hypocalcemia, convulsions). Absence or hypoplasia of the thymus. Immunologically - a defect in the development of T cells at the level of T-lymphocyte precursors. In a laboratory study, a decrease in the level of immunoglobulins with a normal or increased number of B cells. The severity of immunodeficiency usually decreases over time.

Nezelof's syndrome. Clinical manifestations - from birth, purulent infections, sepsis. Atrophy of the thymus and lymph nodes. Immunologically - a sharp decrease in the level of B-lymphocytes, with a normal content of Ig in the blood. Death in the first months of life.

X-linked agammaglobulinemia (Bruton's disease). Clinical manifestations are characterized by a predisposition to purulent infections (increased susceptibility to echo and enteroviruses - vaccine polio and chronic echovirus infection). Hypoplasia of lymphoid tissue (absence of tonsils, small lymph nodes). Immunologically - the T-link is preserved, the block is at the level of formation of early B-lymphocytes. Laboratory tests revealed a sufficient number of B-cell precursors in the bone marrow and agammaglobulinemia in the blood. Males with hypogammaglobulinemia should be evaluated for the presence of a mutation in the bruton-tyrosine kinase gene.

Common variable immune deficiency. Clinically, XLA is more severe, there are signs of combined immunodeficiency, and oncological complications. Pyogenic infections, giardiasis, mycobacteriosis, gastrointestinal disorders; autoimmune disorders, including hematological ones. Immunologically - low Ig levels, the number of B cells is normal or reduced. As it progresses, B cells may disappear from the peripheral blood. The most common types are selective hyper-IgM syndrome (type I) and IgA deficiency (type IV). Hyper-IgM syndrome has at least two forms - X-linked and autosomal recessive.

Diagnostics. Diagnosis of primary immunodeficiency states is as follows:

1) selection of children at risk of primary CHD (attention must be paid):

a) the pedigree of the child, indicating in it cases of death of children in the family at an early age from inflammatory diseases;

b) the development of vaccination, repeated, chronic, multifocal and unusually ongoing infections, parasitic and fungal diseases;

c) the presence in the pedigree of autoimmune, allergic and tumor processes, hemopathy and pathology associated with sex;

d) the presence of associated syndromes (lagging behind in physical development, endocrinopathy, skin and neurological manifestations, etc.);

2) when assessing the immune system of children, it is necessary to take into account:

a) negative Mantoux tests after vaccination and BCG revaccination;

b) thymus dysplasia in young and middle-aged children;

c) the absence of an increase in regional lymph nodes in response to the inflammatory process;

d) hypoplasia of the tonsils or, on the contrary, a pronounced hypertrophy of the tonsil tissue and lymph nodes in combination with recurrent inflammatory processes;

3) assessment of routine laboratory tests:

a) detection in a clinical blood test: hemolytic or hypoplastic anemia, neutropenia, thrombocytopenia, absolute lymphopenia (less than 1000 in 1 mm3), absence of plasma cells in response to an acute infection - all this may indicate a syndrome of insufficiency in T- and B -system;

b) analysis of the proteinogram - detection of hypoproteinemia and hypoalbuminemia, low levels of B - and especially g-globulins - makes it possible to roughly but reliably judge the state of some immune functions;

4) identification of bright clinical non-immunological markers:

a) ataxia and bulbar telangiectasias - with Louis-Barr syndrome;

b) malformations of the main vessels and convulsions against the background of hypocalcemia - with Di-George's syndrome.

Treatment. Principles of therapy for primary immunodeficiency states:

1) hospitalization for in-depth immunological and molecular studies and the choice of the method of therapy;

2) adequate replacement immunotherapy, which allows many patients to lead a normal life;

3) bone marrow transplantation - a radical and almost routine treatment for many forms of IDS;

4) refusal to consider a patient with congenital IDS as therapeutically unpromising.

Scheme of modern therapy of primary IDS.

1. Infection control - in some cases, lifelong antibacterial and antifungal therapy.

2. Replacement immunotherapy with drugs containing antibodies: native plasma (cryopreserved or fresh); immunoglobulins for enteral (CIP - a complex immunoglobulin preparation for oral administration, containing 50% Ig G and 25% each - Ig M and Ig A), intramuscularly (IGVM) and intravenously (IGVI) administration. IVIG approved for use in the Russian Federation (intraglobin F, intraglobin human normal, Biaven V.I., vigam-liquid, vigam-S, octagam, sandoglobulin, pentaglobin). Doses for IDS: 100-400 mg / kg (5% solution 2-8 ml / kg) for 1 injection 1 time per day, 1 time in 1-4 weeks.

Clinical indications for the choice of IVIG: primary and secondary IDS, immunopathological diseases (thrombocytopenic purpura, Kawasaki disease). Sepsis and severe bacterial infections. Prevention of infections in IDS.

3. Replacement therapy by other means: in case of deficiency of adenosine deaminase - injections of polyethylene glycol; with a deficiency of the C1 inhibitor - the introduction of recombinant C1 ING.

4. Activation of the T- and B-systems of immunity - is effective only in secondary IDS without defects of congenital origin.

5. Bone marrow transplantation is indicated for many and especially for combined IDS (SCID, Wiskott-Aldrich syndrome).

6. Gene therapy - adenosine deaminase transplantation (performed on several patients in Europe and the USA).

2. Secondary immunodeficiency states

Secondary immunodeficiency states are characterized by a violation of humoral and cellular immunity, the synthesis of complement components, the absence or decrease in the activity of cytotoxic lymphocytes and macrophages. In childhood, they lead to the breakdown of post-vaccination immunity and the ineffectiveness of vaccination programs.

Secondary immunodeficiency states are disorders of the immune system that develop in the postneonatal period in children or adults and are not the result of genetic defects. Causes leading to the development of secondary immunodeficiency states: nutritional deficiencies, chronic viral and bacterial infections, chemo- and corticosteroid therapy, irrational use of drugs, age-related atrophy of the thymus, exposure to radiation, unbalanced diet, poor-quality drinking water, extensive surgical operations, excessive physical activity , multiple injuries, stress, exposure to pesticides, other environmental factors.

Classification. Classification of secondary immunodeficiency states.

1. Systemic, developing as a result of damage to immunogenesis (with radiation, toxic, infectious and stress lesions).

2. Local, characterized by regional damage to immunocompetent cells (local disorders of the immune apparatus of mucous membranes, skin and other tissues, developed as a result of local inflammatory, atrophic and hypoxic disorders).

Diseases accompanied by secondary immunodeficiency conditions.

1. Infectious diseases: protozoal and helminthic diseases; bacterial, viral and fungal infections.

2. Nutritional disorders: exhaustion, cachexia, malabsorption syndrome, etc.

3. Exogenous and endogenous intoxications - with renal and hepatic insufficiency, with poisoning, etc.

4. Tumors of lymphoreticular tissue (lymphocytic leukemia, thymoma, granulomatosis and other neoplasms).

5. Metabolic diseases (diabetes mellitus).

6. Protein loss due to intestinal diseases, nephrotic syndrome, burn disease, etc.

7. The action of various types of radiation.

8. Strong prolonged stress.

9. The action of drugs.

10. Blockade by immune complexes and antibodies of lymphocytes in allergic and autoimmune diseases.

Treatment.

Replacement therapy with various immune drugs (immunoglobulins; antitoxic, anti-influenza and anti-staphylococcal serums). Correction of the effector link with pharmacological drugs (decaris, diucefon, imuran, cyclophosphamide, etc.), hormones and mediators of the immune system (leukocyte interferons, thymus preparations - thymosin, thymalin, T-activin). Removal of inhibitory factors that bind antibodies and block the effect of immunocorrection (hemosorption, plasmapheresis, hemodialysis, lymphpheresis).

1. In violation of the T-cell link: T-activin, thymogen, interferon, interleukin-2, levamisole, cimetidine, azimixon, imutiol, isoprecasin, etc.

2. For a defect in the macrophage link: MDP (muramyl dipeptide), lentinan, zymosan, aubzidan, peptolak, lactolene, interferon-A, prodigiosan, etc.

3. In case of insufficiency or defects in B-cell immunity - activation of T-helpers and macrophages.

4. With a defect in the complement system - plasma transfusion

5. For immunodeficiencies resulting from radiotherapy and chemotherapy, agents for stimulating the bone marrow: granulocyte-macrophage colony-stimulating factor (GM-CSF), granulocyte colony-stimulating factor (G-CSF), interleukin-1 and interleukin-3.

Correction of transient forms of secondary immunodeficiency states.

1. Achievement of the period of remission:

1) antigenic loads are excluded (dissociation of the patient from the team);

2) sanitation of foci of infections in the family;

3) reducing the influence of other social factors;

4) it is necessary to exclude contact with allergens and vaccines;

5) therapeutic measures are organized on an outpatient basis;

6) detoxification and enteral sorption are carried out;

7) complexes of vitamins, microelements and various antioxidants are used;

8) individual anti-inflammatory measures (if necessary - antibacterial drugs);

9) restoration of the functions of the gastrointestinal tract.

2. When the activity of pathological processes subsides:

1) immunotropic drugs containing factors of the thymus and bone marrow (taktavin, thymalin, thymogen, myelopid) are prescribed;

2) the choice of a specific drug is based on tests for determining the sensitivity of lymphocytes to drugs;

3) other drugs of this plan are prescribed only after the previous stage of rehabilitation of patients;

4) with an individual choice of a drug, the achievement of a positive result of therapy becomes natural;

5) it becomes possible to prevent acute and exacerbations of chronic diseases.

3. Treatment of the underlying pathology:

1) in case of neurological pathology - drugs that improve microcirculation and metabolic processes in the central nervous system, antihypertensive and diuretic drugs;

2) with primary vegetovascular dystonia - psychotherapy of family members; decrease in the predominant activity of the departments of the ANS;

3) for metabolic and constitutional disorders - membrane stabilizing agents, diet;

4) in chronic infectious processes - antibacterial, antiviral, antifungal and antiparasitic drugs; inducers of nonspecific defense of the body;

5) for all patients - drugs that improve metabolic processes.

3. Principles of therapy for immunopathological syndromes

With the syndrome of violation of anti-infectious protection - adequate antibacterial, antiviral, antifungal and antiparasitic therapy; immunomodulatory drugs; rational mode of antigenic loads, including vaccination.

Treatment of allergy syndrome - separation from causally significant allergens, elimination of metabolic products, enzyme replacement therapy, hyposensitizing effects, immunomodulatory measures according to an individual program. In the treatment of diseases associated with the development of autoimmune syndrome, antibacterial therapy is futile; cytostatics, extracorporeal treatment methods, and intravenous administration of immunoglobulin-containing drugs are indicated.

In the stage of subsidence of the inflammatory process - immunomodulatory therapy and drugs containing cytokines - leukinferon, interferon, reaferon, etc. In all cases - sanitation of foci of chronic infections.

LECTURE No. 2. Vegetative-vascular dystonia. Arterial hypertension. Clinic, diagnosis, treatment

1. Vegetative-vascular dystonia

The sympathetic division of the autonomic nervous system regulates predominantly adaptive-trophic processes in situations requiring intense mental and physical activity. The parasympathetic division of the autonomic nervous system manifests its main function outside the period of intense activity of the body, mainly during the "rest" period, and regulates anabolic processes, the insular apparatus, digestive functions, emptying of hollow organs, and helps to maintain a constant homeostasis. Autonomic reactivity is a change in autonomic reactions to internal and external stimuli. Irritants can be pharmacological preparations (mezaton, adrenaline, etc.), as well as physical effects (cold, heat, pressure on reflexogenic zones, etc.). There are 3 variants of vegetative reactivity:

1) normal (sympathicotonic);

2) hypersympathicotonic;

3) sympathicotonic.

Autonomic support is the maintenance of an optimal level of functioning of the autonomic nervous system, ensuring adequate functioning of various organs and systems under stress conditions. Vegetative support in practical work is assessed using the clinoorthostatic test (COT). Autonomic-vascular dysfunction is caused by a violation of the neurohumoral regulation of autonomic functions, which most often can appear with neuroses, physical inactivity, endocrine pathology in the prepubertal period and during menopause. But depending on the etiology and manifestations of VSD, pathogenetic disorders are distinguished at any level: cortical, hypothalamic, with a predominance of the parasympathetic division of the autonomic nervous system or the sympathetic division of the autonomic nervous system.

Determination of the variant of vegetative dystonia depending on the results of assessing the initial vegetative tone and COP. Clinical manifestations in some patients include fatigue, irritability, sleep disturbances, decreased pain sensitivity with various senestopathies. Signs of autonomic dysfunction may include palpitations with a tendency to sinus bradycardia or tachycardia; supraventricular extrasystole, paroxysmal tachycardia. Pathological vasomotor reactions can be manifested by a feeling of hot flashes, cold, decreased or increased blood pressure, pallor of the skin or hyperemia of the skin, general or local sweating, impaired secretory function, motor dysfunction of the gastrointestinal tract, and impaired sexual function. In the presence of neurovegetative imbalance, the activity of the parasympathetic nerves predominates, which is expressed by bradycardia, hyperemia of the skin, increased peristalsis of the stomach and intestines, a positive symptom of persistent red dermographism, and a decrease in pulse.

Treatment. Principles of treatment of vegetative dystonia.

1. Pathogenetic therapy, symptomatic therapy.

2. Long-term treatment to restore balance between the divisions of the autonomic nervous system, this requires more time than to form an imbalance between them.

3. An integrated approach, including various types of effects on the body.

4. Selectivity of therapy depending on the variant of vegetative dystonia, both in permanent (permanent) and crisis (paroxysmal) course.

The main sedatives in the treatment of vegetative dystopia in children.

1. Means of plant origin (valerian, motherwort, St. John's wort, viburnum, mint, oregano, sweet clover, lemon balm).

2. Tranquilizers (seduxen, tazepam, elenium, meproman).

3. Antipsychotics (sanopax, teralen, frenolon).

Non-drug therapy includes: proper organization of work and rest; maintaining a daily routine; physical education classes; balanced diet; psychotherapy; hydrotherapy and balneotherapy; physiotherapy; massage; acupuncture (according to indications). Types of sports for vegetative dystonia in children (recreational swimming, cycling, race walking, skiing, skating). Hydrotherapy depending on the type of vegetative dystonia, physiotherapy. Gymnastics, jumping, tennis, boxing, and weightlifting are not recommended. For vagotonia, showers (circular, contrast, needle, jet, Charcot shower), baths (oxygen, pearl, salt-pine) are recommended. For sympathicotonia, showers (fine, rain, circular) and baths (coniferous, sage-green) are recommended.

Stimulating and tonic herbal products (ginseng, lemongrass, eleutherococcus, green tea, licorice root). Nootropic drugs used in the complex treatment of autonomic disorders in children (piracetam, pyriditol, aminalon, glycine, glutamic acid, acepheren).

2. Arterial hypertension

Arterial hypertension is an increase in blood pressure from the mouth of the aorta to the arterioles, inclusive.

Classifications of arterial hypertension: primary arterial hypertension and secondary arterial hypertension.

Etiology, pathogenesis. Etiopathogenesis of arterial hypertension.

1. Etiological factors: psycho-emotional effects, brain hypoxia, age-related neuroendocrine restructuring, perinatal disorders, salt overload.

2. First-line predisposing factors: hyperreactivity of nerve centers regulating blood pressure; dysfunction of norepinephrine deposympathetic structures. Borderline arterial hypertension develops.

3. Second-line predisposing factors: weakening of hypertensive renal function, disturbances of the renin-angiotensin-2-aldosterone pressor system, changes in cell membranes.

The development of hypertension in various forms

Before prepubertal age, an increase in blood pressure is observed more often in diseases of the kidneys, endocrine pathology, coarctation of the aorta, pheochromocytoma, etc.

Classification. Classification of blood pressure levels and severity of arterial hypertension

1st degree. Systolic - 140-159 mm Hg. Art., diastolic - 90-99 mm Hg. Art.

Borderline degree: systolic - 140-149 mm Hg. Art., diastolic - 90-94 mm Hg. Art.

2st degree. Systolic - 160-179 mm Hg. Art., diastolic - 100-109 mm Hg. Art.

3rd degree. Systolic - more than 180 mm Hg. Art., diastolic - more than 110 mm Hg. Art.

Classification according to M. Ya. Studennikov.

1. Vascular vegetative dystonia of the hypertonic type.

2. Hypertension.

3. Symptomatic (secondary) hypertension.

Clinical manifestations. Often detected by chance, with diseases of the urinary system, the numbers of both maximum and minimum pressure usually increase. Hypertension with coarctation of the aorta is diagnosed by low pressure in the lower extremities and the presence of systolic murmur. Pheochromocytoma is characterized by high blood pressure crises and painful headaches; the diagnosis is established when an increased content of catecholamines is detected in the urine and blood. In prepubertal and pubertal age, hypertensive conditions occur with vegetative-vascular dystonia. Hypertension is unstable, pressure fluctuates throughout the day, and a close connection with emotional factors can be noted. There are complaints of poor health, irritability, easy fatigue, pain in the heart, feeling of heat, etc. An objective examination revealed tachycardia, inadequate response to physical activity, and autonomic lability.

Treatment. Treatment of arterial hypertension in vegetative-vascular dystonia: sedative therapy is indicated - bromine with valerian, seduxen, normalization of the daily routine, mandatory stay in the fresh air, children are shown moderate physical activity and sports with gradually increasing loads. For arterial hypertension, according to indications, diuretics, ACE inhibitors, β2-blockers, L-blockers, adrenergic blockers, and calcium channel blockers can be used.

Prevention: the correct mode of the day, nutrition, physical education and sports, a sufficiently long sleep.

LECTURE No. 3. Cardiomyopathies in children. Clinic, diagnosis, treatment

Classification of cardiomyopathies:

1) dilated (DCMP);

2) hypertrophic (HCMP);

3) restrictive (RCMP);

4) arrhythmogenic right ventricle (AKMP).

1. Dilated cardiomyopathy

Dilated cardiomyopathy is a sharp expansion of the cavity of the ventricles, especially the left one.

Clinical manifestations. At any age, in any gender (more often in men), signs of heart failure (up to total), decreased blood pressure, expansion of the boundaries of the heart, cardiomegaly. Auscultation: deafness of the first tone at the apex, bifurcation, gallop rhythm. Respiratory organs: tympanitis or dullness on the right, fine bubble moist rales on the left.

Diagnostics. ECG-tachycardia, arrhythmia, appearance of the R wave and ("-") T wave; FCG - I tone weakened, systolic, protodiastolic murmur; EchoCG - dilatation of all parts of the heart, EchoCG - dilatation of the left ventricular cavity (LVDC = 56 mm), decreased myocardial contractility (ejection factor 0,34), EchoCG - symmetrical myocardial hypertrophy Tzsp = 28, mitral regurgitation.

Treatment.

Principles of treatment.

I. Conservative.

1. ACE inhibitors (capoten, enalapril, renitek).

2. Angiotensin-2 receptor blockers (Cozaan, Diovan).

3. Diuretics.

4. β-blockers (carvediol).

5. Antiaggregants, anticoagulants. II. Surgical.

2. Hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy is expressed by hypertrophy of the left ventricle and interventricular septum.

Clinical manifestations. More often in boys, there are familial forms, shortness of breath, pain in the heart, expansion of the borders of the heart, weakening of the first tone at the apex, emphasis of the second tone over the pulmonary artery, variable systolic-diastolic murmur along the left edge of the sternum, development of cardiovascular failure of the left ventricular type.

Diagnostics. ECG - signs of hypertrophy of the left atrium and left ventricle; Q wave changed in the resp., V4, echocardiography - thickening of the interventricular septum, a decrease in the volume of the left ventricle. X-ray examination of the chest - depends on the size of the heart, the displacement of the mitral valve forward.

Carnitine CMP.

Ventricular hypertrophy: KDDlzh = 65 mm, atriomegaly, EF = 0,2, mitral regurgitation of the 2nd-3rd degree. Treatment. Surgical - septal myectomy.

1. Valve prosthetics - two-chamber constant stimulation.

2. Conservative:

1) limitation of physical activity;

2) diet with restriction of salt and water;

3) β-blockers;

4) Ca-channel blockers;

5) antiarrhythmics of other groups;

6) ACE inhibitors.

Cardiac glycosides are contraindicated in HCM.

3. Restrictive cardiomyopathy

Restrictive cardiomyopathy is associated with a decrease in the extensibility of the walls of the ventricles with the manifestation of signs of hypodiastole and symptoms of stagnation in the systemic and pulmonary circulation.

Primary myocardial (isolated myocardial damage, similar to DCM).

Endomyocardial (thickening of the endocardium and infiltrative necrotic and infiltrative changes in the myocardium).

1. Lefler's hypereosinophilic parietal fibroplastic endocarditis.

2. Endomyocardial fibrosis (Davis' disease). Stages.

I. Necrotic.

II. thrombotic.

III. Fibrotic: on the ECG - a decrease in the voltage of the teeth, a violation of the processes of conduction and excitation, a change in the final part of the ventricular complex.

Ultrasound reveals dilatation of the cavities of the heart, a decrease in myocardial contractility. On the radiograph, the enlarged size of the heart or its departments is determined.

Arrhythmogenic cardiomyopathy of the right ventricle. Clinic: ventricular extrasystoles, paroxysmal tachycardia.

Cardiomyopathy in mitochondrial pathology. These include the following:

1) Kearns-Sayre syndrome;

2) MELAS syndrome;

3) MERRF syndrome;

4) Barth's syndrome;

5) carnitine CMP;

6) histiocytic cardiomyopathy;

7) CMP with a deficiency of the P-complex of the chain of respiratory enzymes.

Criteria for cardiomyopathy in mitochondrial pathology.

1. Extracardiac:

1) infantile somatotype (3-5 centile);

2) muscle weakness;

3) decreased tolerance to physical activity;

4) visual impairment (ptosis), hearing;

5) stroke-like episodes;

6) periodic neutropenia;

7) persistent enlargement of the liver;

8) high levels of lactate and pyruvate;

9) increased excretion of organic acids;

10) decrease in blood carnitine content - acidosis.

2. Cardiac:

1) disorders of the conducting system of the heart are malignant, ventricular arrhythmias are typical for young children;

2) a combination of HCM, DCM, fibroelastosis;

3) detection of HCM at an early age;

4) family nature of the disease;

5) giant T waves on the ECG in the left chest leads.

Kearns-Sayre syndrome. Debut by 20-30 years. Symptoms: KMP with the development of complete atrioventricular blockade, the formation of HCM and DCMP is possible, ophthalmoplegia with ptosis, retinopathy, delayed physical and sexual development, valgus deviation of the foot, cerebellar ataxia.

MELAS syndrome (mitochondrial myopathy-encephalopathy-lactate acidosis, stroke-like episodes). Debut between 6 and 10 years. Symptoms: convulsions, headache, vomiting, anorexia, dementia.

MERRF syndrome (myoclonus epilepsy and cerebral infarction, RRF fibers). Debut - from 3 to 63 years. Symptoms: myoclonus epilepsy, ataxia, dementia (due to multiple brain infarcts), hearing loss, muscle weakness, HCM.

Bart's syndrome. Debut in 5-7 months of life. Symptoms: weight and height by 3-5 centiles, growth retardation; lag of bone age by 1-2 years; skeletal myopathy; neutropenia; GKMP, DKMP.

Carnitine CMP. Debut in 3-5 months. Often sudden death due to metabolic stress. Symptoms: myocardial hypertrophy with dilatation of the left ventricular cavity, endocardial fibroelastosis, ECG giant T waves (above R) in the left chest leads.

Histiocytic cardiomyopathy (deficiency of cytochrome-B). Debut - in 3 weeks - 1 year. More often in girls. Despite treatment, they die. Symptoms: tachyarrhythmias, ventricular fibrillation, myocardial hypertrophy with dilatation of the left ventricular cavity, cardiac fibroelastosis.

CMP with deficiency of the P-complex of the respiratory enzyme chain. Debut - after 9 years. Symptoms: ophthalmoplegia, encephalomyopathy, ataxia, myoclonic jerks, secondary carnitine deficiency, lactic acidosis, HCM, DCM, ACM.

LECTURE No. 4. Pericardial diseases in children. Clinic, diagnosis, treatment

Clinical and morphological classification of pericardial lesions (A. A. Terke, Z. M. Volynsky, E. E. Gogin).

1. Malformations of the pericardium (anomalies):

1) the defects are complete;

2) partial defects;

3) pericardial lacing defects (diverticula and cysts).

2. Pericarditis:

1) acute (dry fibrinous, exudative);

2) chronic (duration - 3 months);

3) adhesive (asymptomatic, squeezing, with dysfunction of the cardiovascular system, but without signs of cardiac compression);

4) exudative (with moderate dysfunction of the cardiovascular system, but without cicatricial compression of the heart);

5) exudative-squeezing.

3. Tumors of the pericardium (malformations of the pericardium):

1) pericardial defects (complete and partial);

2) pericardial lacing defects.

Embryogenesis of these anomalies is associated with disturbances in the formation of the pericardium in the early stages of development (the first 6 weeks).

1. Congenital defects of the pericardium

Congenital defects of the pericardium include the following.

1. Partial left-sided absence of the pericardium 70%. It is complicated by the formation of a hernia, infringement of the heart at the site of the defect. There are chest pains, shortness of breath, fainting, or sudden death. Surgical treatment - pericardioplasty.

2. The complete absence of the pericardium is manifested by the symptom of "free heart": pain in the region of the heart, shortness of breath, palpitations, sometimes fainting; with percussion, unusual mobility of the heart, on the left side it shifts to the axillary line, and when the head is lowered it moves up.

3. Partial right-sided absence of the pericardium 17%.

4. Congenital lacing of the pericardium.

5. Pericardial cysts (tender, thin-walled formations, not soldered to the surrounding tissues and filled with a clear liquid - "spring water", but it can be bloody (in case of injury) and purulent (in case of inflammation).

6. Pericardial diverticula - communications with the pericardial cavity, can be wide - resembling the finger of a rubber glove, or narrow, resembling a cyst communicating with the pericardium. Cysts and diverticula of the pericardium in childhood are asymptomatic. Sometimes there may be pain and shortness of breath. For partial defects of the pericardium, there are no direct clinical and radiological signs, they are found in concomitant diseases.

Pericardial cysts. Pericardial cysts are divided into pseudocysts and encysted and multilocular pericardial effusions (the appearance of which is due to rheumatic pericarditis, bacterial infection (especially tuberculosis), trauma and surgery); echinococcal cysts (occur after rupture of such cysts in the liver and lungs).

Clinic: in most cases, cysts are not clinically manifested and are detected incidentally on radiography as homogeneous radiopaque oval-shaped formations, usually in the right cardiodiaphragmatic angle. Complaints of discomfort in the chest, shortness of breath, cough or palpitations due to cardiac compression. Percutaneous aspiration and ethanol sclerosing are used to treat congenital and inflammatory cysts. Surgical excision of echinococcal cysts is not recommended.

2. Pericarditis

Pericarditis is an inflammation of the visceral and parietal sheet, it can be fibrinous, purulent, hemorrhagic, serous.

Etiology. Viral diseases, severe septic, more often staphylococcal, processes, rheumatism, diffuse connective tissue diseases.

Pathogenesis. The pathogenesis of an allergic or autoimmune nature, with infectious pericarditis, the infection is a trigger, and direct damage to the membranes of the heart by bacterial or other agents is also not excluded.

Clinical manifestations. Acute serous-fibrinous pericarditis is manifested by the main symptoms - acute pain in the region of the heart, radiating to the shoulder and epigastric region and passing in an upright position and when bending forward. It is associated with damage to the pleural and diaphragmatic pericardium. Sometimes pain in the abdomen, simulating an acute abdomen Pericardial friction rub - determined during systole and diastole, heard during systole and diastole, aggravated in an upright position. Often not constant. Other symptoms: high fever, tachycardia, tachypnea.

Acute exudative pericarditis develops when the inflammatory process of the heart membrane is accompanied by a total lesion.

Clinic: the apex beat of the heart is displaced up and inside from the lower left border of dullness. The boundaries of the heart change depending on the position of the patient's body: vertically, the dullness zone in the 2nd and 3rd intercostal spaces is reduced by 2-4 cm on each side, and dullness in the lower intercostal space expands by the same distance. The heart sounds in the lower left sections are weakened. X-ray picture: early signs and accumulation of exudate, changes in the cardiac shadow, chronic triangular pericardial effusions. A spherical shadow indicates an active process with a rapid increase in the volume of effusion. On echocardiography, the fluid layer anterior and posterior to the heart contour is confidently visualized as an anechoic space. Often there are also fibrous deposits in the form of heterogeneous shadows and thickening of the sheets of the pericardium, and with large effusions, fluctuations of the heart inside the stretched pericardial sac are characteristic.

Chronic exudative pericarditis. The clinical picture depends on the rate of exudate accumulation. Usually the general condition worsens sharply, shortness of breath appears, dull pains in the region of the heart, the patient takes a forced position, the apex beat is weakened, the heart sounds are sharply muffled ECG: lowering of the teeth, negative T waves, shift of the ST interval. X-ray examination: expansion of the shadow of the heart, which takes a triangular or trapezoidal shape.

Chronic adhesive (adhesive, constrictive) pericarditis. The pericardium thickens and both of its leaves, visceral and parietal, grow together both with each other and with the underlying myocardium.

The onset is gradual, edematous syndrome develops, protein-losing enteropathy appears, leading to hypoalbuminemia, followed by an increase in edematous syndrome, the development of hepatomegaly, ascites, and pronounced edema of the extremities. The pulse is small, blood pressure with a small amplitude. Heart sounds are weakened, gallop rhythm.

Diagnostics. Diagnosis of acute pericarditis: during auscultation, pericardial friction noise (one-, two- and three-phase).

ECG

Stage I: ST-segment concave in anterior and posterior leads, PR segment deviations opposite P-wave polarity.

Early stage II: ST connection returns to baseline, PR interval deviation persists.

Late stage II: T waves gradually flatten and begin to invert.

Stage III: Generalized T-wave inversion.

Stage IV: restoration of the original ECG characteristics observed before the development of pericarditis.

Echo-CG: effusion types BD.

Signs of cardiac tamponade

Blood tests:

1) determination of ESR, the level of C-reactive protein and lactate dehydrogenase, the number of leukocytes (markers of inflammation);

2) determination of the level of troponin I and MB-fraction of creatine phosphokinase (markers of myocardial damage).

Chest x-ray - the image of the heart may vary from normal to the appearance of a "water bottle" silhouette. In the course of this study, concomitant diseases of the lungs and mediastinal organs can be detected. Diagnostic interventions that are mandatory for cardiac tamponade are a class I indication; at the discretion of the doctor with large or recurrent effusions or with insufficient information content of the previous examination - an indication of class Pa; and for small effusions, a class IIb indication.

Pericardiocentesis with drainage of the pericardial cavity: the results of polymerase chain reaction and histochemical analysis allow us to determine the etiopathogenesis of pericarditis (infectious or tumor).

Diagnostic interventions that are used at the discretion of the doctor or in case of insufficient information content of the previous examination are an indication of class Pa.

Computed tomography: effusions, condition of the peri- and epicardium.

With magnetic resonance imaging: effusions, the state of the peri- and epicardium. With pericardioscopy, a biopsy of the pericardium is performed with the establishment of the etiology of pericarditis.

Diagnosis of constrictive pericarditis. The clinical picture is manifested by signs of severe chronic systemic venous congestion caused by low cardiac output: swelling of the jugular veins, arterial hypotension with low pulse pressure, increased abdominal volume, peripheral edema and muscle weakness. ECG - the results are either normal, or there is a decrease in the amplitude of the QRS complex, generalized inversion (or flattening) of the T wave, changes in the electrical activity of the left atrium, atrial fibrillation, atrioventricular block, intraventricular conduction disorders, and in rare cases, pseudo-infarction changes. An X-ray examination of the chest reveals pericardial calcification and pleural effusion. Echo-CG reveals thickening of the pericardium and its calcification, as well as indirect signs of constriction: an increase in the PP and LA with a normal ventricular configuration and preserved systolic function; early paradoxical movement of the IVS (sign of “diastolic retraction and plateau”); flattening of the waves of the posterior wall of the LV; no increase in LV size after the early rapid filling phase; the inferior vena cava and hepatic veins are dilated and their sizes change little depending on the phases of the respiratory cycle. Limitation of LV and RV filling; when assessing blood flow through the atrioventricular valves, differences in filling levels during inspiration and expiration exceed 25%. Doppler - Echo-CG is determined by measuring the thickness of the pericardium. When using esophageal echo-CG, thickening and/or calcification of the pericardium, a cylindrical configuration of one or both ventricles, narrowing of one or both atrioventricular grooves, signs of congestion in the vena cava, and an increase in one or both atria are determined. Computed tomography and/or magnetic resonance imaging determines the sign of “diastolic retraction and plateau” on the pressure curve in the RV and/or LV. Equalization of end-diastolic pressure in the LV and RV in the range < 5 mm Hg. Art. Angiography of the RV and/or LV reveals a decrease in the size of the RV and LV, and an increase in the size of the RA and LA. During diastole, after the early rapid filling phase, there is no further increase in ventricular size (a sign of “diastolic retraction and plateau”). Angiography of the coronary arteries is indicated for all patients over 35 years of age, as well as at any age if there is a history of indications for irradiation of the mediastinal area.

Treatment. Therapeutic actions: general measures, suppression of the inflammatory response, etiotropic treatment, unloading therapy, symptomatic therapy.

Implementation of the tasks of complex therapy:

1) bed rest;

2) good nutrition;

3) NSAIDs;

4) glucocorticosteroids;

5) broad-spectrum antibiotics;

6) pericardiocentesis;

7) diuretics;

8) relief of pain syndrome;

9) correction of hemorrhagic syndrome;

10) if conservative therapy is ineffective - pericardiectomy.

3. Tumors of the pericardium

Primary pericardial tumors are less common than cardiac tumors.

Clinical manifestations. Clinically, they are manifested by symptoms of hemorrhagic or sero-fibrous pericarditis, sometimes with suppuration. The diagnosis of a tumor of pericarditis is established by cytological examination of the punctate of the contents of the pericardial cavity, the introduction of carbon dioxide into the cavity of the pericardium, histological examination of the biopsy of the pericardium or angiocardiography.

Treatment. Treatment of malignant tumors of the heart is most often symptomatic. The most common are radiotherapy and chemotherapy.

LECTURE No. 5. Chronic heart failure in children. Clinic, diagnosis, treatment

Heart failure is a condition in which the heart, despite sufficient blood flow, does not provide the body's need for blood supply. Causes of chronic circulatory failure: direct effect on the myocardium (toxic, infectious, traumatic), cardiovascular diseases.

Classification. Classification of chronic heart failure (according to Strazhesko-Vasilenko). I stage. Compensated. III stage. Decompensated-reversible. IB stage. Decompensated-slightly reversible. III stage. Terminal.

International classification of chronic heart failure.

I functional class.

II functional class.

III functional class.

IV functional class.

Pathogenesis. The pathogenesis of chronic heart failure is manifested by a decrease or increase in blood supply, blood flow and / or pressure in the central or peripheral parts of the blood circulation. These changes occur as a mechanical consequence of a violation of the pumping function of the heart and as a result of the inadequacy of adaptive reactions. These reactions include tachy- and bradycardia, changes in vascular peripheral and pulmonary resistance, redistribution of blood supply, hypertrophy and expansion of individual chambers of the heart, fluid retention, sodium. Hemodynamic disorders lead to pathological changes in the heart, blood vessels and other organs and systems.

Clinical manifestations.

clinical forms.

1. Congestive left ventricular failure occurs more often with mitral valve disease. An increase in pressure in the pulmonary veins leads to the filling of the left ventricle and the preservation of cardiac output. Congestive changes in the lungs disrupt the function of external respiration and are a factor that aggravates the patient's condition with congestive left ventricular failure. Clinical manifestations: shortness of breath, orthopnea, auscultation shows signs of stagnation in the lungs (dry rales below the level of the shoulder blades, migrating moist rales) and radiological changes, cardiac asthma and pulmonary edema, secondary pulmonary hypertension, tachycardia.

2. Left ventricular failure is characteristic of aortic disease, coronary artery disease, arterial hypertension. Clinical manifestations: cerebrovascular insufficiency, manifested by dizziness, darkening of the eyes, syncope, coronary insufficiency and echocardiographic signs of low output. In severe cases, Cheyne-Stokes respiration, presystolic gallop rhythm (pathological IV tone), congestive left ventricular failure appear.

3. Congestive right ventricular failure manifests itself with mitral, tricuspid disease or constrictive pericarditis. More often it is associated with congestive left ventricular failure. Clinical manifestations: swelling of the neck veins, increased venous pressure, acrocyanosis, enlarged liver, peripheral and cavitary edema.

4. Right ventricular failure is observed with pulmonary artery stenosis and pulmonary hypertension.

Clinical manifestations of chronic heart failure.

Stage I of chronic heart failure (I f. to.).

Complaints of weakness. On physical examination, the skin is pale. Signs of heart failure only with great physical exertion: shortness of breath, tachycardia. Hemodynamics is not disturbed.

Stage IIA of chronic heart failure (II f. to.) Complaints: sleep disturbance, increased fatigue. Signs of heart failure at rest:

1) left ventricular heart failure, shortness of breath (no cough), tachycardia;

2) right ventricular heart failure enlargement of the liver and its soreness, pastosity in the evening on the lower extremities (no edema).

11B stage of chronic heart failure (II f. to.) Complaints: irritability, tearfulness. All signs of heart failure at rest: icterus, cyanosis of the skin, pronounced LVHF and PVHF, decreased diuresis, expansion of the boundaries of the heart, muffled tones, arrhythmia.

Stage III of chronic heart failure (IV f.c.) Cachexic circulation, emaciation, light tan skin. Edema-dystrophic blood circulation (thirst, edema, cavitary edema (pulmonary edema)). The progression of chronic heart failure is manifested by oliguria and hepatosplenomegaly.

Treatment.

Principles of treatment.

1. Cardiac glycosides.

2. Diuretics.

3. ACE inhibitors

4. β-blockers.

Tactics of treatment of chronic heart failure. Stage I - (I f. to.) basic therapy of the underlying disease. IIA stage (II f. to.) - diuretics.

CB stage (III stage) - diuretics, cardiac glycosides. Stage III (IV f.k.) - diuretics, cardiac glycosides, peripheral vasodilators.

In stage I, it is necessary to observe the regime of work and rest, moderate physical exercises. In severe stages, physical activity should be limited, bed rest, semi-bed rest is prescribed. A complete, easily digestible food rich in proteins, vitamins, potassium. With a tendency to fluid retention and with arterial hypertension, a moderate restriction of sodium chloride is indicated. With massive edema, a short-term strict salt-free diet is prescribed. Cardiac glycosides are not prescribed for obstructive hypertrophic cardiomyopathy, severe hypokalemia, hyperkalemia, hypercalcemia, atrioventricular heart block, sick sinus syndrome, ventricular extrasystoles, and paroxysms of ventricular tachycardia. Cardiac glycosides are prescribed in doses close to the maximum tolerated. First, a saturating dose is used, then the daily dose is reduced by 1,5-2 times. With glycoside intoxication, unithiol is prescribed (5% solution of 5-20 ml IV, then IM 5 ml 3-4 times a day). According to the indications, antiarrhythmic therapy is carried out. The patient and his relatives should be familiarized with the individual treatment regimen with cardiac glycosides and with the clinical signs of their overdose. Digoxin is prescribed 2 times a day in tablets of 0,00025 g or parenterally 0,5-1,5 ml of a 0,025% solution (saturation period), then 0,25-0,75 mg (maintenance dose) per day . The use of digoxin cardiac glycoside requires special care. The selection of the dosage of cardiac glycosides should be done in a hospital. Diuretics are used for edema, liver enlargement, congestive changes in the lungs. Use the minimum effective dose during treatment with cardiac glycosides. The treatment regimen is individual, which is corrected during the course of treatment. Complications of diuretic therapy - hypokalemia, hyponatremia, hypocalcemia (loop diuretics), hypochloremic alkalosis, dehydration and hypovolemia. Hypotaazid is used in tablets of 0,025 g, loop diuretic furosemide or lasix in tablets of 0,04 g or parenterally. Peripheral vasodilators are prescribed in severe cases with the ineffectiveness of cardiac glycosides and diuretics. With stenosis (mitral, aortic), as well as with systolic (blood pressure is reduced from 100 mm Hg. Art. and below) should not be used. Predominantly venous dilators, nitropreparations lower the filling pressure of the ventricles in congestive insufficiency. The arteriolar dilator hydralazine 0,025 g is prescribed 2-3 tablets 3-4 times a day, as well as the calcium antagonist nifedipine, Corinfar. Venuloarteriolar vasodilators: captopril at a daily dose of 0,075-0,15 g. The use of venulo-arteriolo-dilators together is used in severe, refractory to cardiac glycosides and diuretics heart failure with significant dilatation of the left ventricle. Potassium preparations can be administered together with cardiac glycosides, diuretics and steroid hormones. Potassium preparations are prescribed for ventricular extrasystoles, hypokalemia, tachycardia refractory to cardiac glycosides, and flatulence in seriously ill patients. It is necessary to ensure the need for potassium through a diet (prunes, dried apricots, apricots, peach, apricot, plum juice with pulp). Potassium chloride is usually poorly tolerated by patients; appoint inside only in a 10% solution of 1 tbsp. l.

LECTURE No. 6. Heart rhythm disturbances in children. Clinic, diagnosis, treatment

Arrhythmias are heart rhythm and conduction disorders that occur with congenital heart defects, acquired heart diseases, dysfunction of the central and autonomic nervous system.

Classification of cardiac arrhythmias.

1. Violation of the function of automatism - consists in changing the number of impulses rotating in the sinus node (violation of the formation of an excitation impulse):

1) nomotopic rhythm disturbances (disturbances in the formation of an impulse in the sinus node - sinus arrhythmia, sinus tachycardia, sinus bradycardia;

2) heterotopic rhythm disturbances (the impulse originates outside the sinus node).

2. Passive ectopic rhythms:

1) nodal rhythm;

2) migration of the pacemaker (from the sinus node to the atrioventricular connection).

3. Active ectopic rhythms:

1) extrasystole;

2) paroxysmal tachycardia;

3) atrial and ventricular fibrillation;

4) flutter of the atria and ventricles.

4. Disorders of the conduction function (blockade), slowdown, complete delay in the conduction of excitation through the conduction system:

a) sinoauricular blockade;

b) atrioventricular blockade;

c) intra-atrial blockade;

d) intraventricular blockade;

e) blockade of the legs of the bundle of His.

1. Violation of the function of automatism

Nomotopic arrhythmias. Sinus arrhythmia is manifested in the intermittent increase and decrease in heart rate. Patients do not present complaints. Sinus arrhythmia is often associated with respiratory phases and can occur in healthy children. Respiratory arrhythmia occurs when the heart rate increases on inspiration and the heart rate decreases on expiration. It is caused on inspiration by a reflex decrease in the tone of the vagus nerve, on expiration - an increase in the tone of the vagus. On the ECG, a change in heart rhythms (RR or P-P intervals of various durations due to an increase in diastolic T-P intervals).

Sinus bradycardia - a decrease in the number of heart contractions. It is caused by an increased influence on the sinus node of the parasympathetic nervous system or a decreased influence of the sympathetic nervous system. It occurs in healthy child athletes, with vegetative-vascular dystonia of the vagotonic type, with rheumatism, hypothyroidism, traumatic brain injury, brain tumors, and some infectious diseases. The patients do not make any complaints. On the ECG, the atrial and ventricular complexes are not changed, the RR (cardiac cycle), T-P (cardiac diastole) intervals are prolonged, and the duration of the P-Q interval is slightly increased.

Sinus tachycardia - an increase in the number of heart contractions. Associated with the effect on the sinus node of biologically active substances that increase its excitability, or an increase in the tone of the sympathetic nervous system, or a decrease in the tone of the vagal nerve. Sinus tachycardia appears during physical and emotional stress, increased body temperature, organic heart disease, various infections and intoxications, and thyrotoxicosis. On the ECG, the atrial and ventricular complexes are not changed, the RR (cardiac cycle), T-P (cardiac diastole) intervals are shortened.

Heterotopic arrhythmias. Nodal rhythm - an increase in the automatic function of the atrioventricular node and a decrease in the automatic ability of the sinus node due to functional or organic changes. No complaints, sometimes complaints of pulsation in the neck, which is noted with simultaneous contraction of the atria and ventricles. With auscultation of the heart, an increase in 1 tone is determined. On the ECG - a negative P wave precedes the QRS complex, the RR interval is shortened.

There is a periodic change in rhythm from the sinus to the atrioventricular node. In this case, the heart is excited under the influence of impulses emanating alternately from the sinus node, then from the atrial conduction system, then from the atrioventricular connection, and again the pacemaker migrates in the same sequence. There are no complaints, no objective changes. The clinical picture boils down to the underlying disease (rheumatism, intoxication). On the ECG, the shape, amplitude, position of the P wave changes, as well as the duration of the P-Q interval, which becomes shorter when moving to the atrioventricular node.

Extrasystole - premature contraction of the whole heart or a separate part of it, arising under the influence of an additional focus of excitation emanating from the sinus node Causes: inflammatory, dystrophic, degenerative, toxic, mechanical damage and neurogenic disorders. Depending on the place of origin, there are ventricular, atrial, atrioventricular. Extrasystoles can be single, multiple, can occur after each contraction in a certain sequence (bigemia) or after two contractions (trigemia). Extrasystoles arising in various ectopic centers are called polytopic. Complaints are often not presented, sometimes there are unpleasant sensations in the region of the heart (fading, stopping, a strong push). With auscultation of the heart, additional pulse beats, additional heart sounds are noted. With atrial extrasystole, excitation from the ectopic focus occurs earlier than monotopic excitation, and after premature contraction of the heart, a long incomplete compensatory pause occurs. On the ECG - a deformed P wave is premature or superimposed on the previous P wave, shortening of the R-P interval, the QRS complex is not changed, the T-P interval is moderately increased.

With atrioventricular extrasystoles, the impulse comes from the Aschoff-Tavara node, spreads to the atria retrogradely, from the bottom up, and the excitation of the ventricles is normal. On the ECG - a negative P wave in a different location in relation to the QRS complex, or before the complex, or merges with it, or comes after it, the shape of the QRS complex is not changed, the T-P interval is equal to two normal heart beats (complete compensatory pause). With ventricular extrasystoles, the sequence of excitation of the heart changes, the impulse that occurs in the ventricles does not propagate retrograde, and the atria are not excited. The excitation of the ventricles occurs alternately, and non-simultaneously, as in the norm, which depends on the localization of the ectopic focus.

On the ECG, ventricular extrasystoles appear:

1) premature occurrence of the QRS complex without the preceding P wave;

2) QRS complex with high voltage, broadened, split, serrated, transitional T wave without S-T interval;

3) the discoordinate direction of the T wave in relation to the maximum tooth of the QRS complex of the extrasystole;

4) prolongation of the compensatory pause after the extrasystole; the distance between two RR intervals, including the extrasystole, is equal to two normal cycles. There are right and left ventricular extrasystoles: with right ventricular extrasystoles in lead 1 the largest R wave of the QRS complex is, the extrasystoles are directed upward, and in lead 3 the largest is the S wave directed downward.

With the left ventricular type, in lead 1 the largest S wave of the QRS complex of extrasystoles is directed downward, in lead 3 the largest R wave is directed upward. The origin of functional extrasystole is due to a violation of extracardiac, often autonomic, regulation.

The main signs of a functional extrasystole (most common in prepubertal and pubertal age):

1) labile during the day, changes with a change in body position, with physical activity;

2) children show signs of vegetative-vascular dystonia, foci of chronic infection, endocrine disorders;

3) when using special research methods, violations of myocardial contractility are not detected; a clinoorthostatic test, a test with dosed physical activity, pharmacological tests with ECG registration testify in favor of functional extrasystole. The origin of organic extrasystole occurs as a result of damage to the myocardium or the conduction system of the heart. The main signs of organic extrasystole:

1) permanent character;

2) the general condition is usually disturbed and there are signs of organic damage to the heart (rheumatism, non-rheumatic carditis, congenital heart defects).

Paroxysmal tachycardia is an attack of a sharp increase in heart rate, 2-3 times higher than the normalization of the rhythm, which occurs in the presence of an ectopic center capable of generating impulses of high frequency. Complaints in older children about discomfort in the heart, a feeling of tension in the neck, dizziness, fainting, pain in the epigastric region, abdomen. In young children, paroxysmal tachycardia is accompanied by convulsive and dyspeptic symptoms. On physical examination, shortness of breath, cyanosis, pulsation of the veins, congestion in the lungs, enlargement of the liver, the pulse cannot be counted, low filling, and a decrease in blood pressure.

There are atrial, atrioventricular, and ventricular forms of paroxysmal tachycardia. An ECG with atrial paroxysmal tachycardia reveals a long row of atrial extrasystoles with a sharp shortening of the T-P interval, layering of the P wave on the T wave with its deformation, the QRS complex is not changed or moderately deformed, atrioventricular paroxysmal tachycardia is characterized by multiple repetitions of atrioventricular extrasystoles having negative P waves, or their displacement onto the QRS complex, or merging with the T wave. The ventricular form of paroxysmal tachycardia on the ECG is a deformed, dilated QRS complex. Atrial P waves appear regularly and overlap the ventricular complex of the extrasystole.

Atrial fibrillation is a violation of the proper activity of the atria due to the appearance of one or more foci of excitation in the atria. Complaints about deterioration in health, feelings of fear, anxiety. During auscultation, there is a different sonority of tones, a random alternation of short and long pauses, the number of ventricular contractions depends on the form of atrial fibrillation, there is a pulse deficiency (during auscultation, the number of heart contractions is greater than pulse waves). On the ECG, the P wave is absent and is slowed down by waves of various sizes and shapes. The QRS complex is not changed, the S-T interval is below the isoelectric line, the T wave and the isoelectric line are deformed by flicker waves.

2. Disorders of the conduction function

Blockades are manifested by slowing down (incomplete blockade) or complete cessation (complete blockade) of the conduction of impulses from the sinus node to the terminal branches of the conduction system of the heart.

Classification.

1. Sinoauricular blockade.

2. Intra-atrial blockade.

3. Atrioventricular blockade degree).

4. Intraventricular blockade (blockade of the legs of the bundle of His). Sinoauricular blockade is a violation of the conduction of excitation from the sinus node to the atrial myocardium.

Causes: autonomic dysfunction with vagotonia, sinus node immaturity in newborns, hyperkalemia, drug intoxication, degenerative and inflammatory changes in the sinus node and myocardium. There are no complaints. Periodic dropouts of individual contractions of the heart (complete RR cycles) appear on the ECG, and a pause equal to the double RR interval is recorded in their place.

Intra-atrial blockade is a violation of the conduction of an impulse along the interatrial pathways, as a result of which the synchronism of the activity of both atria is disturbed. Occurs in diseases with atrial enlargement in rheumatism, cardiomyopathy, heart defects. On the ECG - a change in the amplitude and duration of the P wave, which can be split, bifurcated in lead I, double-humped in leads 1, 2 and 5.

Atrioventricular blockade degree) appears as a result of a slowdown or complete cessation of the conduction of excitation from the atria to the ventricles.

Blockade classification:

1) incomplete (I, II degree);

2) complete (III degree);

3) functional, congenital, acquired. Causes of vegetative-vascular dystonia according to the vagotonic type:

1) congenital malformations of the conducting system of the heart, combined with congenital heart defects;

2) rheumatism;

3) tachyarrhythmias;

4) progressive muscular dystrophy;

5) injuries;

6) embolism;

7) collagenosis;

8) drug intoxication (digoxin). Atrioventricular blockade of the XNUMXst degree is manifested by increased fatigue, dizziness, pain in the heart, with an objective examination, there is an expansion of the borders of the heart to the left, with auscultation of the heart, muffled tone I at the apex. On the FVD, a pronounced atrial tone is noted, the first tone is split or bifurcated.

Second degree atrioventricular block manifests itself in the form of a pause that occurs due to loss of heart contractions. On the ECG, the P-Q interval increases, the QRST complex periodically falls out, followed by a normal P-Q interval, which gradually lengthens and ends with the QRST loss.

Atrioventricular blockade of the III degree, when conduction disturbances register independent contractions of the atria and ventricles. The atria contract in a frequent rhythm under the influence of impulses from the sinus node, the ventricles contract in a rare rhythm under the influence of impulses from automatic centers of the II and III order. Complete atrioventricular block is more often congenital and is clinically manifested by bradycardia, clapping I tone at the apex of the heart, hypertrophy of the right and left parts of the heart due to hemodynamic disturbances. The ECG shows the appearance of positive P waves that are not associated with the QRS complex and located at different distances from it, the RR intervals are constant, and the P-P intervals are shorter than the RR intervals and change in the presence of sinus arrhythmia. Intraventricular blockade (blockade of the legs of the bundle of His) occurs as a result of an anatomical break (malformation, inflammation, sclerosis) or a functional block (supraventricular tachyarrhythmia, extrasystole). leg. As a result, the ventricles contract non-simultaneously. Patients do not complain. On auscultation of the heart, deafness of tones and often their splitting is heard (gallop rhythm due to non-simultaneous contraction of the right and left ventricles).

The following changes are recorded on the ECG.

1. High widened, deformed, serrated QRS complex preceded by a normal P wave with a normal or slightly prolonged P-Q interval.

2. The QRS complex and broadened T wave in each lead have a discordant direction.

With blockade of the left leg of the bundle of His in lead I, the direction of the QRS complex is characteristic upward, and with blockade of the right leg, the QRS complex in lead I is directed downward. Blockade of the terminal branches of the conduction system occurs with severe myocardial damage (diffuse myocarditis, myocardiosclerosis). The ECG with this disease reveals a low voltage of the teeth of the complex and their broadening. The T wave is flattened or negative. Intraventricular block combined with a shortened atrioventricular interval, called Wolff-Parkinson-Watt syndrome, is more common in children with rheumatism, but can also be in healthy children. No complaints, no clinical manifestations. On the ECG, the P-Q interval is shortened, the QRS complex is elongated and notched.

Treatment.

In the absence of vital indications, antiarrhythmic drugs should not be used. Of particular importance from anti-arrhythmic drugs are: 10% solution of calcium chloride, 1 hour, des., Art. l. depending on age 3-4 times a day, novocainamide 0,1-0,5 g 2-3 times a day, /? - blockers. During an attack of paroxysmal tachycardia, means of mechanical excitation of the vagus nerve are used (pressure on the sinuses of the carotid artery, eyeballs, inducing a gag reflex by pressing on the root of the tongue), Isoptin is used intravenously at a dose of 0,3-0,4 ml for newborns up to 1 year - 0,4-0,8 ml, 1-5 years - 0,8-1,2 ml, 5-10 years - 1,2-1,6 ml, 10 years and older - 1,6-2,0 ml and digitalis preparations.

LECTURE No. 7. Systemic vasculitis in children. Clinic, diagnosis, treatment

Systemic vasculitis is a heterogeneous group of diseases with primary inflammatory and necrotic changes in the vascular wall.

Etiology of systemic vasculitis: viruses (cytomegaloviruses, hepatitis virus), bacteria (streptococci, staphylococci, salmonella), parasitic diseases (ascaris, filariotosis). Classification of vasculitis.

1. Primary vasculitis with the formation of granulomas (giant cell arteritis, Takayasu arteritis, Wegener's granulomatosis, Churg-Strauss syndrome) and without the formation of granulomas (polyarteritis nodosa, Kawasaki disease, microscopic polyangitis).

2. Secondary vasculitis in rheumatic diseases (aortitis in rheumatoid arthritis, rheumatoid vasculitis, vasculitis in rheumatic diseases), infections (aortitis in syphilis, hepatitis B, HIV infection).

1. Microscopic polyangiitis

Microscopic polyangiitis (microscopic polyarteritis) is a necrotizing vasculitis with few or no immune deposits, predominantly affecting small vessels.

Clinic. The clinic is dominated by symptoms of glomerulonephritis and pulmonary capillaritis.

Signs: early development of renal failure due to the presence of extracapillary glomerulonephritis (in urine tests, hematuria, moderate proteinuria). Absence of arterial hypertension. Pulmonary syndrome: damage to the upper respiratory tract - necrotic lesion of the nasal mucosa (necrotic rhinitis), which does not lead to destructive processes that deform the nose, in the lower respiratory tract - pronounced pulmonary hemorrhagic processes (hemorrhagic capillaritis, pulmonary bleeding).

2. Churg-Strauss syndrome

Churg-Strauss syndrome is a granulomatous inflammation involving the respiratory tract associated with asthma and eosinophilia, and necrotizing vasculitis affecting small to medium vessels.

Clinic. Bronchial asthma for several years. Increased levels of eosinophils in the peripheral blood and their migration into tissues (chronic eosinophilic infiltration of the lungs). Severe attacks of asthma + signs of systemic vasculitis (fever, myalgia, arthralgia, weight loss). Damage to the central nervous system (peripheral neuropathy, hemorrhagic stroke). Kidney damage. Damage to the gastrointestinal tract (ischemia and perforation of the wall of the stomach or intestines). Skin lesions (painful purpura mainly on the lower extremities).

Diagnosis criteria. Asthma, eosinophilia > 10%, changes in the paranasal sinuses, sinusitis, mono- or polyneuropathy, variable (volatile) pulmonary infiltrates, extravascular tissue eosinophilia. Laboratory criteria: hypereosinophilia, increased immunoglobulin E, C-reactive protein, accelerated ESR.

Treatment. Glucocorticoids, cytostatics, plasmapheresis.

3. Wegener's granulomatosis

Wegener's granulomatosis is a granulomatous inflammation of the respiratory tract, necrotizing vasculitis of small and medium-sized vessels, usually associated with necrotizing glomerulonephritis.

There are 3 forms: localized, transitional, generalized. Laboratory criteria: ESR acceleration, leukocytosis, hypergammaglobulinemia.

Criteria for diagnosis. The criteria for diagnosis are as follows.

1. Ulcerative-necrotic lesion of the upper respiratory tract.

2. Progressive areas of necrosis of ENT organs with cartilage destruction.

3. Involvement of the eye orbit.

4. A large number of eosinophils, granulomas in the biopsy of the nasal mucosa, septum, palate.

Treatment. Glucocorticoids, cytostatics, NSAIDs, antiaggregants, anticoagulants.

4. Behçet's disease

Behçet's disease is a vasculitis that affects small to medium-sized arteries and presents as a clinical triad of recurrent aphthous stomatitis, genital ulcerative necrotic changes, and eye involvement.

Diagnostic criteria. Recurrent oral ulcer (at least 3 times during the year): minor aphthae, major aphthae, herpetiform rash. Also 2 of the following signs: recurrent genital ulcers, eye lesions (anterior posterior uveitis). Skin lesions (erythema nodosum, pseudofolliculitis, papulopustular inflammation, acneiform nodules). Formation of pustules at injection sites.

5. Polyarteritis nodosa

Polyarteritis nodosa is a necrotizing inflammation of the arteries of medium and small caliber without the formation of granulomas.

Clinic.

clinical options.

1. Thromboangiitis (juvenile) - damage to the vessels of the skin.

2. Visceral (classic):

1) damage to the vessels of the lungs;

2) damage to the vessels of the gastrointestinal tract;

3) damage to the vessels of the kidneys;

4) damage to the vessels of the heart.

diagnostic criteria. Weight loss by more than 4 kg, livedo reticularis on the skin, myalgia, weakness, decreased blood pressure (especially diastolic), arteriographic vascular changes (aneurysm, occlusion). Biopsy - necrotizing vasculitis.

Treatment. Glucocorticoids, cytostatics, symptomatic therapy (angioprotectors, antihypertensives, anticoagulants). Hemorrhagic vasculitis is a systemic inflammatory disease, Ig A, deposits, causing damage mainly to capillaries, arterioles and venules of the skin, joints, abdominal cavity and kidneys, which is based on multiple thrombovasculitis. It is more common in children 4-11 years old and is a classic immunocomplex disease.

clinical syndromes. Skin syndrome - maculopapular rashes on the lower extremities with the transition to the buttocks. stomach.

Articular syndrome - polyarthralgia, arthritis of large joints without deformation.

Abdominal syndrome - sharp spastic pains in the abdomen, aggravated after eating; nausea, vomiting.

Renal syndrome - micro-, macrohematuria, severe capillary-toxic glomerulonephritis.

CNS damage - headaches, strokes, subdural hematomas, subdural hematomas.

diagnostic criteria. Palpable purpura, age of onset of the disease is younger than 20 years, bouts of abdominal pain, hematuria, granulocytic infiltration in the walls of arterioles and venules in the biopsy.

Treatment. Exclusion of provoking factors, sanitation of foci of infection, treatment of the underlying disease, anticoagulants and antiplatelet agents, anti-inflammatory therapy, local treatment. Takayasu arteritis is a disease from the SV group, characterized by granulomatous inflammation of the aorta and its branches with the development of panarteritis; more often manifests itself in women 10-20 years old, chronic inflammation with destructive changes in the walls of large arteries, the aorta and its branches, which is accompanied by stenosis and ischemia of the blood supplying organs.

Localization of the process (IV anatomical type).

1. Aortic arch (I type).

2. Region of the descending abdominal aorta (type II).

3. Mixed forms (type III).

4. Involvement of the pulmonary artery and its branches (type IV). Clinical syndromes. Clinical syndromes in the acute phase of inflammation: general inflammatory, cardiovascular, peripheral blood flow insufficiency syndrome, cerebrovascular, pulmonary, arterial hypertension syndrome.

Criteria for diagnosis. The onset of the disease is before the age of 40, a decrease in the pulse on the brachial artery, the difference in blood pressure in the arms is more than 10 mm Hg. Art., noise heard over the subclavian artery and in the aorta, intermittent claudication, changes in the arteriogram.

Treatment. Conservative (glucocorticoids, NSAIDs, aminoquinoline drugs, cytostatics, treatment of concomitant pathology - tuberculosis, anticoagulants and antiplatelet agents, a-blockers).

Surgical (excision of the occlusal part). Excision of the occlusal part of the vessel.

LECTURE No. 8. Kidney failure. Clinic, diagnosis, treatment

The main functions of the kidneys are the removal of metabolic products, maintaining a constant water-electrolyte composition and acid-base state, carried out by renal blood flow, glomerular filtration and tubules (reabsorption, secretion, concentration ability).

Renal failure - this syndrome develops with severe violations of the renal processes, leads to a disorder of hemostasis, is characterized by azotemia, a violation of the water-electrolyte composition and the acid-base state of the body.

1. Acute renal failure

Acute renal failure occurs suddenly due to acute, most often reversible, kidney disease. Causes of acute renal failure:

1) violation of renal hemodynamics (shock, collapse, etc.);

2) exogenous intoxication: bites of poisonous snakes, insects, medicines, poisons that are used in the national economy, everyday life, medicines;

3) infectious diseases (hemorrhagic fever with renal syndrome and leptospirosis);

4) acute kidney disease (acute glomerulonephritis and acute pyelonephritis);

5) urinary tract obstruction;

6) arenal condition (trauma or removal of a single kidney).

Renal failure is characterized by changes in homeostatic constants (pH, osmolarity, etc.) as a result of significant impairment of renal function and is an outcome or complication of diseases conventionally divided into renal (glomerulonephritis, pyelonephritis), prerenal (hypovolemia, dehydration, disseminated intravascular coagulation) and postrenal (obstructive uropathy). ) Acute renal failure is characterized by a sudden disruption of homeostasis (hyperazotemia, electrolyte disturbances, acidosis) due to an acute disturbance of the basic functions of the kidneys (nitrogen excretion, regulation of metabolic balance, water-electrolyte balance). Acute renal failure can develop in diseases manifested by hypotension and hypovolemia (shock, burn, etc.) with a subsequent decrease in renal blood flow; DIC syndrome in septic shock, HUS; with glomerulonephritis, pyelonephritis, with cortical necrosis of the kidneys in newborns, with difficulty in the outflow of urine from the kidneys. There are 4 periods of surge arrest:

1) initial period;

2) oligoanuric period;

3) polyuric period;

4) recovery period.

Clinic. The initial period is characterized by symptoms of the underlying disease (poisoning, painful shock, anaphylactic or bacterial), hemolysis, acute poisoning, and infectious disease. On the first day, you can detect a decrease in diuresis (less than 500 ml/day), i.e., a period of oliguria, anuria begins, and homeostasis changes. In plasma, the level of creatinine, urea, residual nitrogen, sulfates, phosphates, magnesium, potassium is increased, the level of sodium, chlorine, and calcium is decreased. Adynamia, loss of appetite, nausea, vomiting appear; in the first days, oliguria and anuria can be observed. With increasing azotemia, the level of urea increases daily by 0,5 g/l; with increasing acidosis, overhydration and ectrolyte disorders, muscle twitching, lethargy, drowsiness are observed, shortness of breath appears due to acidosis, pulmonary edema, the early stage of which is determined by x-ray. Characteristic changes in the cardiovascular system: tachycardia, expansion of the borders of the heart, dull heart sounds appear on auscultation, systolic murmur at the apex, pericardial friction noise. Heart rhythm disturbances develop as a result of hyperkalemia, which can cause death. With hyperkalemia, the ECG shows a tall, pointed T wave, the QRS complex is widened, and the R wave is reduced. Heart block and ventricular fibrillation can lead to cardiac arrest. Anemia develops during all periods of acute renal failure. The period of oliguria and anuria is characterized by the appearance of leukocytosis. There may be complaints of abdominal pain, liver enlargement, and symptoms of acute uremia. Death in acute renal failure develops against the background of uremic coma, changes in hemodynamics, and sepsis. In acute renal failure, hypoisosthenuria appears.

The oligoanuric period is manifested by a rapid (within several hours) decrease in diuresis to 100-300 ml/day with a low specific gravity of urine of no more than 1012, lasting 8-10 days, a gradual increase in weakness, anorexia, nausea, vomiting, itching of the skin. With unlimited administration of fluid and salt, hypervolemia and hypertension occur; Peripheral edema and pulmonary edema may occur. Hyperazotemia quickly increases (up to 5-15 mmol/day urea, creatinine more than 2 mmol/l), severe acidosis, hyperkalemia (up to 9 mmol/l), hyponatremia (below 115 mmol/l) cause uremic coma. Hemorrhages, gastrointestinal bleeding, decrease in hemoglobin, leukocytes to 2,0 x 109/l. The color of urine is red due to gross hematuria, proteinuria is usually small - up to 9%. Clinical improvement occurs gradually: the level of azotemia decreases and homeostasis is restored. During an attack of polyuria, hypokalemia (less than 3,8 mmol/l) may develop, and the ECG shows characteristic changes (decrease in T wave voltage, U wave, extrasystole). With the normalization of residual nitrogen in the blood, homeostasis is restored, a period of glomerular filtration and concentration develops, kidney function is preserved, and in case of chronic failure, the course becomes chronic, with associated pyelonephritis playing a special role. The recovery period lasts about 1 year and is manifested by the gradual restoration of renal functions.

In the treatment of peritoneal dialysis and hemodialysis in the complex therapy of acute renal failure, mortality decreased to 20-30%, the outcome in chronic renal failure is rarely noted, as well as the development of acute renal failure against the background of chronic renal failure.

Prerenal acute renal failure Etiology. Dehydration, hypovolemia, and hemodynamic disturbances lead to impaired renal blood flow. Surveys.

1. When taking an anamnesis and physical examination, signs of dehydration, hypovolemia, shock, and a decrease in cardiac output can be detected.

2. Blood pressure and central venous pressure are measured, a urinary catheter is inserted in order to assess diuresis, which indicates severe oliguria, you can also obtain urine for a general urine test to determine osmolarity, sodium, potassium, and creatinine levels. After this, the catheter must be removed.

The diagnosis of prerenal acute renal failure is established on the basis of a sodium level in the urine of less than 15 mEq/L and an excreted sodium fraction (ESF) of less than 1%. EF# = (urine Na+ / plasma Na+) / (urine creatinine / plasma creatinine) x 100%. Renal insufficiency index (RII) < 1%, RFI = urine Na + / (urine creatinine / plasma creatinine) x 100%. Urine urea/plasma urea ratio > 10, urine creatinine/plasma creatinine > 40, urine osmolarity > 500 mOsmol/kg. The potassium level in the urine is at least 40 mEq/L. Rehydration therapy increases diuresis and blood volume. GFR increases with improved cardiac performance.

Treatment of prerenal AKI is aimed at restoring perfusion and renal function.

1. Vein catheterization is prescribed for the administration of drugs. Sometimes CVP monitoring is needed.

2. Restore BCC.

3. If, after restoration of bcc, oliguria and anuria persist, prescribe mannitol - a 20% solution at a dose of 0,5 g/kg, intravenously for 10-20 minutes, and subsequently diuresis should increase by 6 ml/kg, if this does not occur, the administration of mannitol is stopped.

4. After the restoration of the BCC, a test dose of furosemide is administered, 1 mg/kg intravenously.

5. If significant oliguria or anuria persists, parenchymal or postrenal AKI should be ruled out.

Renal (parenchymal) acute renal failure

Etiology. A history of prolonged marked reduction in renal perfusion suggests acute tubular necrosis. Other causes of parenchymal acute renal failure include glomerulonephritis, malignant hypertension, hemolytic uremic syndrome, urate nephropathy and vasculitis.

Examination and diagnosis. First, prerenal and postrenal causes of acute renal failure are excluded.

1. Before carrying out invasive diagnostic interventions, the patient's condition must be stabilized.

2. Assess kidney function.

Parenchymal acute renal failure is characterized by the following symptoms:

1) urine creatinine/blood creatinine ratio < 20;

2) urine osmolarity below 350 mosmol/kg;

3) urinary sodium level above 40 mEq/l, EFN > 3%, PPI > 1%;

4) Renal scintigraphy evaluates renal blood flow, kidney function, also using this method, cortical necrosis of the kidneys can be excluded, ultrasound can exclude urinary tract obstruction.

Treatment. If severe oliguria or anuria is due to kidney damage, remove the urinary catheter immediately. Weigh the patient 2 times a day. Measure the volume of injected and excreted fluid. With water-electrolyte balance in the absence of edema and hyperhydration, the amount of fluid and electrolytes administered is calculated along with diuresis and latent water loss. The calorie content of food should be maximum; parenteral nutrition is used only when the usual is impossible. With parenteral nutrition, 10-15% glucose can be injected into the patient's peripheral vein, and up to 30% into the central vein. After the restoration of diuresis, the loss of water and electrolytes in the urine must be replaced with infusion solutions. Loss of potassium is replaced until plasma levels return to normal.

1. If the plasma potassium level is 5,5-7,0 mEq/L, it is necessary to administer sodium polystyrene sulfonate in sorbitol solution 1 g/kg orally, administered every 4-6 hours until the plasma potassium level decreases. When potassium is excreted, sodium is excreted and may develop hypernatremia.

2. When the plasma potassium level is more than 7 mEq / l, characteristic changes appear on the ECG, immediately take the following measures, monitoring the ECG:

1) a 10% solution of calcium gluconate is administered at a dose of 0,5-1 ml / kg IV for 5-10 minutes;

2) sodium bicarbonate is administered at a dose of 2 meq/kg intravenously by bolus over 5-10 minutes.

3. If hyperkalemia persists, insulin is prescribed at 0,1 IU/kg, administered intravenously with 25% glucose, 0,5 g/kg (2 ml/kg), for 30 minutes.

It is necessary to monitor blood glucose levels using the express method and prepare everything for hemodialysis. Emergency hemodialysis is indicated if plasma potassium levels are greater than 7,5 mEq/L and previous interventions are ineffective. Acidosis usually improves with glucose administration. Bicarbonate, citrate, lactate can be prescribed for administration at a dose of 1-3 mEq/L. But you need to remember that 1 mEq/L contains 1 mEq/L of sodium and potassium. Treatment of severe acidosis is difficult due to overhydration; hemodialysis is indicated. Diuretics are not used for anuria.

Postrenal acute renal failure

Etiology. Urinary tract obstruction develops with congenital valve anomalies, with structural disorders of the urethra, with hematuria, tumor, or retroperitoneal fibrosis.

Examination and diagnostics. Obstruction of the urinary tract is established on the basis of anamnesis (congenital anomalies in the development of the urinary tract, genital organs, injuries of the lower abdomen); palpated volumetric formation in the lateral parts of the abdomen, overflowing bladder. Anuria may indicate bilateral ureteral obstruction. Carry out ultrasound and scintigraphy of the kidneys. If these methods cannot be carried out, determine the level of creatinine in serum, less than 5 mg% - excretory urography is indicated. It is necessary: ​​to eliminate dehydration and introduce a minimum amount of low-osmolar contrast agent, consult a urologist, in case of anuria, obstruction of the urinary tract, perform cystoscopy and retrograde pyelography.

Treatment. Pathogenetic therapy is prescribed based on the cause of acute renal failure. Plasmapheresis should be performed, the volume of which can be determined by the severity of the patient’s condition and the degree of intoxication. In case of hemodynamic disorders, anti-shock therapy is prescribed, replacement of blood loss with transfusion of blood components, blood substitutes (100-400 mg of prednisolone is administered intravenously). In case of hypotension (after replenishment of blood loss), an intravenous drip of 1 ml of 0,2% solution of norepinephrine in 200 ml of isotonic sodium chloride solution is prescribed. In case of poisoning, measures are used to remove poison from the body. With large intravascular hemodialysis, if the hematocrit is below 20%, a replacement transfusion of blood or plasma is performed. If the cause is bacterial shock, then antishock therapy and antibiotics are prescribed. In the initial period of oligurianuria, furosemide is prescribed intravenously to stimulate diuresis, 160 mg 4 times a day.

Further therapy should be aimed at regulating homeostasis. Assign a diet with limited intake of protein and potassium, but with sufficient calories from carbohydrates and fats. The amount of fluid injected should be more than diuresis, the amount of water lost with vomiting and diarrhea, no more than 500 ml, this volume should include 400 ml of a 20% glucose solution with 20 units of insulin, with hyperkalemia, 10- 20 ml of a 10% solution of calcium gluconate, also in / in drip 200 ml of a 5% solution of sodium bicarbonate (after establishing the degree of acidosis and under the control of blood pH). Indications for hemodialysis and peritoneal dialysis: if the level of urea in plasma is more than 2 g / l, potassium - more than 6,5 mmol / l; if there is decompensated metabolic acidosis; if there are clinical manifestations of acute uremia.

Contraindications are cerebral hemorrhage, gastric bleeding, intestinal bleeding, severe hemodynamic disturbances, and decreased blood pressure. A contraindication to peritoneal dialysis is a recent operation on the abdominal organs, or adhesions in the abdominal cavity. Treatment includes surgery or urinary diversion. Obstruction of the lower urinary tract is identified and eliminated by catheterization of the bladder, obstruction of the ureters is detected by ultrasound. After restoration of patency of the urinary tract, polyuria develops, which leads to dehydration; in these cases, 0,45% NaCl is administered.

2. Chronic renal failure (CRF)

Chronic renal failure gradually develops as a result of progressive irreversible loss of functioning parenchyma.

Diagnosed in children with diseases of the urinary system, if they persist for 3-6 months and reduce glomerular filtration less than 20 ml / min, increase the level of serum creatinine, urea. Over 50 diseases are manifested by kidney damage and lead to chronic renal failure, which is characterized by progression and irreversibility.

Etiology. Causes of chronic renal failure: chronic pyelonephritis, chronic glomerulonephritis. hereditary nephritis, nephritis in systemic diseases, nephroangiosclerosis, polycystic kidney disease, diabetic glomerulonephrosis, amyloidosis of the kidneys, urological diseases The pathogenetic mechanism of CRF is a progressive decrease in the number of active nephrons, which leads to a decrease in the efficiency of renal processes and to impaired renal function. Before chronic kidney failure develops, chronic kidney disease can last from 2 years or more. They go through several stages, when glomerular filtration and tubular reabsorption are at a normal level, the underlying disease is at a stage that is not accompanied by impaired renal processes. Over time, glomerular filtration becomes below normal, the ability of the kidneys to concentrate urine decreases, and the disease passes into the stage of impaired renal processes. At this stage, homeostasis is preserved and there is no renal failure yet. If the number of active nephrons and the glomerular filtration rate is below 50 ml / min, the level of creatinine in the blood plasma is more than 0,02 g / l and urea is more than 0,5 / g / l, conservative treatment of chronic renal failure is required at this stage. When filtration is below 10 ml/min, azotemia and other disturbances of homeostasis grow and the end stage of chronic renal failure occurs, which requires the use of dialysis. The cause of development is acquired and hereditary diseases of the urinary system, factors leading to the development of acute renal failure and chronic renal failure. With progressive kidney disease, they gradually decrease in size and become sclerotic. Morphological changes appear in the form of sclerosed glomeruli and tubules with hypertrophied glomeruli and dilated tubules, with areas of interstitial tissue fibrosis. In infants, chronic renal failure progresses against the background of structural, functional immaturity of the kidneys, with urolithiasis, with destruction of the kidneys, hydronephrosis, pyelonephritis.

1. With sclerosis of 75-80% of nephrons, others lose their ability to further hypertrophy, which causes minimal reserve capacity, clinically manifested by a decrease in tolerance to the intake of potassium, sodium, decompensation of chronic renal failure.

2. Clinical signs of chronic renal failure: decrease in excretory and other renal functions, activation of secondary factors aimed at compensating primary disorders (calcium removal from bones to compensate for acidosis), as well as damage to other organs (pericarditis, etc.), in conditions of change homeostatic constants (acidosis, hyperazotemia, etc.).

Clinic. Complaints of fatigue, decreased performance, headache, loss of appetite. CRF is characterized by the gradual development of weakness, pallor of the skin, anorexia. Sometimes an unpleasant taste in the mouth is noted, nausea and vomiting appear. The skin is pale, the skin is dry, flabby.

Muscle tone is reduced, small muscle twitching, tremor of fingers and hands are observed. There are pains in the bones and joints. Anemia develops, leukocytosis and bleeding appear. Arterial hypertension develops with underlying kidney disease. The boundaries of the heart are expanded, with auscultation, heart sounds are muffled, characteristic changes on the ECG (sometimes they are associated with dyskalemia). Conservative therapy regulates homeostasis, the general condition of the patient is satisfactory, but physical exertion, mental stress, errors in diet, infection, surgery can lead to deterioration of kidney function and the appearance of uremic symptoms. Arterial pressure is normal in the initial and polyuric stages. arterial hypertension appears in the oligoanuric and uremic stage. In the polyuric stage of chronic renal failure (diuresis reaches 2-3 l / day), which can last for years, hyperazotemia is moderately expressed, glomerular filtration is 20-30 ml / min. the relative density of urine is lower than the relative density of blood plasma (1010-1012). With congenital nephropathy (proteinuria up to 1 g / day), proteinuria, hematuria, leukocyturia appear. In the oligoanuric stage, the patient's condition deteriorates sharply, which is due to the addition of hemorrhagic syndrome, cardiovascular insufficiency. When glomerular filtration is below 10 ml/min, conservative therapy is performed, homeostasis is impossible. For the terminal stage of chronic renal failure, emotional lability is characteristic (apathy is replaced by excitement), disruption of night sleep, lethargy, and inappropriate behavior. Puffy face, gray-yellow color, skin itching, scratching on the skin, hair dull, brittle, dystrophy, hypothermia is characteristic. Decreased appetite Voice hoarse. An ammonia smell appears from the mouth, aphthous stomatitis develops. Coated tongue, vomiting, regurgitation, sometimes diarrhea, fetid, dark stools. Anemia, hemorrhagic syndrome, muscle twitches appear. With prolonged uremia, pain in the arms and legs, bone fragility appear, which can be explained by uremic nephropathy and renal osteodystrophy. Uremic intoxication can be complicated by pericarditis, pleurisy, ascites, encephalopathy, and uremic coma. In children with chronic renal failure, symptoms of rickets (bone and muscle pain, bone deformities, growth retardation) are observed, which is associated with insufficient production of the biologically active metabolite of vitamin D.

During this period, anemia, hyperkalemia, and impaired renal function by osmotic dilution increase, which leads to the development of hypovolemia with inadequate fluid administration.

Treatment. Treatment of chronic renal failure in conjunction with treatment of the underlying kidney disease that leads to kidney failure. In the initial stage, when there is no violation of renal processes, etiotropic and pathogenetic therapy is prescribed, which will cure the patient and prevent the development of renal failure or lead to remission and a slow course of the disease. At the stage of impaired renal processes, pathogenetic therapy is carried out with symptomatic methods of treatment (antihypertensive drugs, antibacterial treatment, protein restriction in the daily diet, spa treatment, etc.).

Conservative treatment of CRF is aimed at restoring homeostasis, reducing azotemia, and reducing symptoms of uremia. The glomerular filtration rate is below 50 ml / min, the level of creatinine in the blood is above 0,02 g / l - it is necessary to reduce the amount of protein consumed to 30-40 g / day. The diet should be high in calories and contain essential amino acids (potato-egg diet without meat and fish). Food is prepared with a limited (up to 2-3 g) amount of table salt. To reduce the level of phosphates in the blood, almagel is used in 1-2 tsp. 4 times a day. During treatment, it is necessary to control the level of calcium and phosphorus in the blood. With acidosis, depending on the degree, 100-200 ml of a 5% sodium bicarbonate solution is injected intravenously. With a decrease in diuresis, lasix is ​​prescribed in doses (up to 1 g / day) that provide polyuria. Antihypertensive drugs are prescribed to lower blood pressure. Anemia is treated with iron supplements. With a hematocrit of 25% and below, transfusions of erythrocyte mass by fractional administration are indicated. Antibiotics and their chemotherapeutic drugs in chronic renal failure are used carefully: doses are reduced by 2-3 times. Derivatives of nitrofurans in chronic renal failure are contraindicated. In heart failure and chronic renal failure, cardiac glycosides are used carefully in reduced doses, especially with hypokalemia. Hemodialysis may be indicated for exacerbation of renal failure, after the exacerbation subsides. When the patient's condition improves, conservative therapy is carried out. Plasmapheresis courses give a good effect in chronic renal failure. In the terminal stage, the patient is transferred to hemodialysis. Regular hemodialysis is used when creatinine clearance is below 10 ml/min and plasma levels are above 0,1 g/l. CRF must be differentiated from acute renal failure, which is distinguished by a sudden onset with an oligoanuric stage and reverse development, from neurohypophyseal diabetes insipidus, the difference is that there is no hyperazotemia and other signs of chronic renal failure, from anemic syndrome and other diseases (hypoplastic anemia, etc.), in which there are no symptoms of CRF.

Treatment is aimed at reducing hyperazotemia and correcting water and electrolyte metabolic disorders. Basic principles, as in the treatment of acute renal failure. The "dialysis-kidney transplantation" program remains the most promising in the treatment of children with CRF, which helps patients return to normal life. Indications for the implementation of the program are the lack of effect of conservative therapy, an increase in serum creatinine to 0,6 mmol/l (6 mg%) and potassium in the blood over 7 mmol/l.

Forecast. Hemodialysis and kidney transplantation change the fate of patients with chronic renal failure, prolong life and achieve rehabilitation. The selection of patients for these types of treatment is carried out by specialists from the centers of hemodialysis and organ transplantation.

LECTURE No. 9. Differential diagnosis of diffuse connective tissue diseases in children. Clinic, diagnosis, treatment

Classification of rheumatic diseases.

1. Rheumatism.

2. Juvenile rheumatoid arthritis.

3. Ankylosing spondylitis.

4. Other spondyloarthropathies.

5. Systemic lupus erythematosus.

6. Vasculitis:

1) hemorrhagic vasculitis (Sheklein-Genoch);

2) periarteritis nodosa (polyarteritis in young children, Kawasaki disease, Wegener's disease);

3) Takayasu's arteritis.

7. Dermatomyositis.

8. Scleroderma.

9. Difficult to classify rheumatic syndromes.

10. Various diseases associated with rheumatic symptoms and signs in children:

1) benign rheumatoid nodules;

2) erythema nodosum;

3) Lyme disease;

4) sarcoidosis;

5) Steven-Johnson syndrome;

6) Goodpasture's syndrome;

7) symptoms of fibrositis and fibromyalgia;

8) Behçet's syndrome;

9) Sjögren's syndrome.

11. Non-rheumatic diseases, clinically similar to rheumatic ones.

Diffuse connective tissue diseases (DBST, collagenoses) are an immunopathological lesion of connective tissue with systemic damage to blood vessels and various organs, with a progressive course.

In the etiology of DBST, 3 leading factors can be distinguished.

1st factor - genetic predisposition, which is confirmed by epidemiological studies, illness of close relatives, twins and detection of markers of certain diseases according to the HLA system.

The 2nd factor is a trigger mechanism, which is slow-reacting RNA-containing viruses (retroviruses), and a chronic persistent infection transmitted transplacentally.

3rd factor - resolving: stressful situation, hyperinsolation, hypothermia.

With all DBST, the degree of disease activity is determined; the nature of the flow; the presence of visceral lesions: the functional ability of the patient according to the state of the organs and systems involved in the process; the nature of the complications. In the DBST prodrome there are a number of common features that combine various nosological forms: unmotivated weakness, physical inactivity, loss of appetite and body weight; fever resistant to antibiotics, non-steroidal anti-inflammatory drugs and responsive to GC treatment; damage to the skin and mucous membranes; articular syndrome; lymphadenopathy; enlargement of the liver and spleen; persistent mono- or polyviscerites. In laboratory diagnostics, for most DBST (except for SJS), the following are typical: high acute phase indicators (ESR, sialic acids, CRP, etc.); some are more specific (LE cells, HLA, etc.).

In the DBST group, 4 "large collagenoses" are distinguished.

1. Systemic lupus erythematosus (SLE).

2. Systemic scleroderma (SSD).

3. Progressive sclerosis.

4. Dermatomyositis (DM).

1. Systemic lupus erythematosus

Systemic lupus erythematosus is a chronic polysyndromic disease of connective tissue and blood vessels that develops due to genetically determined imperfection of immunoregulatory processes.

Etiology. The importance of a viral infection against the background of genetically determined immunity disorders is assumed.

Pathogenesis: circulating antibodies are formed, of which antinuclear antibodies are of great diagnostic and pathogenetic importance; the formation of circulating immune complexes that are deposited on the basement membranes of various organs, causing their damage and inflammation. Hyperreactivity of humoral immunity is associated with impaired cellular immunoregulation. Recently, importance has been attached to hyperestrogenemia, accompanied by a decrease in clearance. Family genetic predisposition, girls and women get sick more often. Provocative factors: insolation, pregnancy, abortion, childbirth, the onset of menstrual function, infections (especially in adolescents), drug or post-vaccination reactions.

Clinic. The disease begins gradually, with recurrent polyarthritis, asthenia. Less often there is an acute onset (high fever, dermatitis, acute polyarthritis). In the future, a relapsing course is noted. Polyarthritis, polyarthralgia - an early symptom of the disease. The defeat of the small joints of the hands, wrist, ankle, less often - the knee joints. Erythematous rashes on the skin of the face in the form of a "butterfly", in the upper half of the chest in the form of a decollete, on the extremities are also a characteristic sign of systemic lupus erythematosus. Polyserositis, dermatitis, polyarthritis - a diagnostic triad. Characterized by damage to the cardiovascular system with the development of pericarditis, which is joined by myocarditis. Libman-Sachs verrucous endocarditis with damage to the mitral, aortic and tricuspid valves is relatively common. Damage to the vessels with the development of Raynaud's syndrome (long before the typical picture of the disease), damage to small and large vessels with corresponding clinical symptoms. Lung damage may be associated with the underlying disease in the form of lupus pneumonitis, manifested by coughing, shortness of breath, with auscultation in the lower parts of the lungs, moist rales. An x-ray examination can detect an enhanced pulmonary pattern and deformation of the pulmonary pattern in the basal sections of the lungs, focal-like shadows are detected. When examining the gastrointestinal tract, aphthous stomatitis is detected, dyspeptic syndrome and anorexia develop. Painful abdominal syndrome develops when the peritoneum is involved in the pathological process, mesenteric and splenic vasculitis develops, and segmental ileitis develops. The defeat of the reticuloendothelial system is manifested in the form of an increase in all groups of lymph nodes, an increase in the liver, spleen. Lupus hepatitis develops very rarely, but an enlarged liver can be detected with heart failure, pancarditis, severe effusion pericarditis, with the development of fatty liver. Lupus diffuse glomerulonephritis (lupus nephritis) develops in some patients during the period of generalization of the process. You can meet different variants of kidney damage: urinary syndrome, nephritic syndrome, nephrotic syndrome. When recognizing lupus nephritis, a puncture biopsy with immunomorphological and electron microscopic studies of a kidney biopsy is of great importance. The development of renal pathology with recurrent articular syndromes, fever, elevated ESR, which requires the exclusion of lupus nephritis. It must be remembered that every fifth patient with nephrotic syndrome may have systemic lupus erythematosus and damage to the neuropsychic sphere in patients can develop in all phases of the disease. At the initial stage, asthenovegetative syndrome is diagnosed, then characteristic signs of damage to the central and peripheral nervous system appear in the form of encephalitis, polyneuritis, myelitis. Very rarely, epileptiform seizures can develop. Perhaps the development of hallucinations (auditory or visual), delusional states, etc.

Treatment. Non-steroidal anti-inflammatory drugs and aminoquinoline derivatives are shown. Non-steroidal anti-inflammatory drugs are prescribed for articular syndrome. Immunosuppressants, B vitamins, ascorbic acid during the spring-autumn course. Patients are prescribed treatment in local-type sanatoriums (cardiological, rheumatological). Climatobalneological, physiotherapeutic treatment is contraindicated, since ultraviolet irradiation, insolation and hydrotherapy can exacerbate the disease.

2. Scleroderma (Morphea) localized

This is a chronic systemic disease of the connective tissue, small vessels with widespread fibro-sclerotic changes in the skin and stroma of internal organs and symptoms of obliterating endarteritis in the form of systemic Raynaud's syndrome.

Major criteria: scleroderma-like skin changes proximal to the major finger joints. Small Criteria:

1) sclerodactyly;

2) dimpled scars or loss of soft tissue substance of the fingertips, hands and/or feet;

3) bilateral basal pulmonary fibrosis. The diagnosis of scleroderma is considered reliable if either a major criterion or at least two minor criteria are present. Etiology. The etiology is unknown.

Provoking factors are cooling, trauma, infection, vaccination, etc.

Pathogenesis. Of particular importance is the violation of collagen metabolism, which is associated with functional hyperreactivity of fibroblasts and smooth muscle cells of the vascular wall. Also, a factor in pathogenesis is a violation of microcirculation associated with damage to the vascular wall and a change in the intravascular aggregate properties of the blood.

Systemic scleroderma is a collagen disease that is associated with increased collagen formation and fibrosis, functionally defective fibroblasts and other collagen-forming cells. The predisposition is family-genetic, women are ill 3 times more often than men.

Clinic. The clinical picture usually begins with Raynaud's syndrome (vasomotor disorders), trophic disorders and persistent arthralgia, weight loss, fever, asthenia. Systemic scleroderma begins with a single symptom and quickly acquires the features of a multisyndromic disease. Skin lesions are a characteristic symptom of the disease. The most common is dense edema, followed by thickening and atrophy of the skin. More often, changes are localized on the skin of the face, limbs, very rarely the skin of the entire body becomes dense. At the same time, focal or widespread pigmentation develops with areas of depigmentation and telangiectasia. Characteristic ulcers and pustules on the fingertips do not heal for a long time, very painful, there is a deformation of the nails, hair loss up to baldness. Fibrosing interstitial myositis often develops. Muscular syndrome manifests itself in the form of myalgia, characterized by progressive compaction, then muscle atrophy, and a decrease in muscle strength. And rarely there is acute polymyositis with pain, swelling of the muscles. Fibrosing changes in the muscles may be accompanied by fibrosis of the tendons, which leads to muscle-tendon contractures. The damage to the joints is mainly associated with the pathological process of the peri-articular tissue (skin, tendons, articular bags, muscles). Arthralgias are accompanied by a pronounced deformity of the joint due to proliferative changes in the periarticular tissue; X-ray examination does not reveal significant destruction. A special diagnostic sign is osteolysis of the terminal, as well as in severe cases, middle phalanges of the fingers, very rarely of the legs. The deposition of calcium salts in the subcutaneous tissue is more often localized in the areas of the fingers, periarticular tissues, which is expressed in the form of painful uneven formations that can spontaneously open with the deposition of crumbly calcareous masses. The defeat of the cardiovascular system is observed in almost all patients, the myocardium and endocardium are affected. Sclerodermic cardiosclerosis is clinically characterized by pain in the region of the heart, shortness of breath, extrasystole. muffled tones, systolic murmur at the apex, expansion of the borders of the heart to the left. An X-ray examination reveals a weakening of the pulsation, smoothness of the contours of the heart, X-ray kymography can reveal silent zones in areas of large-focal cardiosclerosis, and in more severe cases, a heart aneurysm may form due to the replacement of muscle tissue with fibrous tissue. On the ECG, a decrease in voltage is recorded, conduction disturbance up to atrioventricular blockade, an infarction-like ECG is recorded with the development of massive foci of fibrosis in the myocardium. When the process is localized in the endocardium, the development of scleroderma heart disease and damage to the parietal endocardium is possible. The mitral valve is usually affected. Scleroderma is characterized by a benign course. With the defeat of small arteries, arterioles, the following peripheral symptoms of scleroderma Raynaud's syndrome, gangrene of the fingers are detected. Damage to the vessels of internal organs leads to severe visceral pathology of hemorrhage, ischemic, even necrotic changes with a clinical picture of visceritis (decay of lung tissue, "true scleroderma kidney"), with vascular pathology is determined by the speed of the process, its severity and outcome of the disease. At the same time, it is also possible to damage large vessels with a clinic of thromboangiitis obliterans, it is possible to identify ischemic symptoms, namely gangrene of the fingers, toes, migrating thrombophlebitis with trophic ulcers in the feet, legs, etc. n Lung damage in the form of diffuse or focal pneumofibrosis, accompanied by emphysema and bronchiectasis. Complaints of shortness of breath. difficulty in deep breathing, hard breathing, wheezing is heard on auscultation of the lungs, on percussion a boxed tone of percussion sound, on respiratory function, a decrease in lung capacity to 40-60% of the proper one, bilateral amplification and deformation of the lung pattern, it is sometimes possible to detect a fine-mesh structure in the form of "honeycombs" ", an x-ray examination of the chest reveals signs that are characteristic of scleroderma pneumofibrosis. Kidney damage is more often manifested by focal nephritis and diffuse glomerulonephritis with hypertension and renal failure may develop. With the rapid progression of systemic scleroderma, "true scleroderma kidney" sometimes develops, leading to focal necrosis of the cortex and the development of renal failure. With damage to the esophagus, the clinical picture is in the form of dysphagia, expansion and weakening of peristalsis, rigidity of the walls with a slowdown in the passage of barium during X-ray examination, which is often observed and is of great diagnostic value. In connection with the affected vessels, ulceration, hemorrhage, ischemic necrosis and bleeding in the digestive tract may develop. Damage to the nervous system is clinically manifested by polyneuritis, autonomic instability (impaired sweating, thermoregulation, vasomotor skin reactions), emotional lability, irritability, tearfulness, suspiciousness, and insomnia. Sometimes, in rare cases, a picture of encephalitis or psychosis appears. Perhaps the development of a clinical picture of sclerosis of cerebral vessels in connection with their scleroderma lesions, even in young people. A lesion of the reticuloendothelial system is revealed in the form of polyadenia, and in some patients hepatosplenomegaly and damage to the endocrine system in the form of a pathology of one or another endocrine gland. Systemic scleroderma often has a chronic course, and the disease lasts for several decades with minimal activity of the process and the gradual spread of the lesion to various internal organs, the functions of which are not disturbed for a long time. These patients suffer mainly from diseases of the skin and joints with trophic disorders. In chronic scleroderma, CRST is isolated - a syndrome that is manifested by calcification, Raynaud's syndrome, sclerodactyly, telangiectasia, characterized by a long benign course with an extremely slow development of visceral pathology. In subacute course, systemic scleroderma begins with arthralgia, weight loss, visceral pathology rapidly increases, and the disease begins to progress steadily, acquiring a widespread pathological process in many organs and systems. The death of such patients occurs within 1-2 years from the onset of the disease.

1. Plaque form - swelling, erythema, violations of the skin pattern and pigmentation with a lilac-pink "corolla", induration, fibrosis, atrophy.

2. Keloid form - dense strands resembling a keloid scar.

3. Linear form - a lesion along the neurovascular bundle, affecting the fascia, muscles and bones; areas of lipodystrophy, amyotrophy, shortening of the tendons and dysplasia of the limb.

4. Deep subcutaneous nodular form - localized on the thighs and buttocks with the involvement of the fascia. Isolated nodules along tendons resembling rheumatoid and transforming calcifications.

5. Atypical forms - idiopathic atrophoderma (bluish-violet spots on the body without previous seals); Buschke's scleredema is a pseudoscleroderma disease (dense swelling of the dermis and subcutaneous tissue).

Diagnostics. Diagnosis based on clinical and laboratory data: usually there is a moderate normo- or hypochromic anemia, moderate leukocytosis and eosinophilia, transient thrombocytopenia. ESR is normal or moderately elevated in chronic course and significantly increased to 50-60 mm / h, with subacute.

Treatment. The principles of treatment are as follows.

1. Antifibrotic agents (D-penicillamine, diucifone, colchicine, enzymatic preparations, dimethyl sulfoxide).

2. The use of NSAIDs.

3. Treatment with immunosuppressive agents (suppresses the autoimmune inflammatory process in the connective tissue, inhibits excessive fibrosis); administration of glucocorticoids.

4. The use of antihypertensive and microcirculation-improving agents.

5. Local therapy, massage, exercise therapy.

6. Symptomatic treatment for lesions of the digestive system.

7. Spa treatment.

3. Dermatomyositis

Dermatomyositis is a systemic disease of the skeletal, smooth muscles and skin.

Etiology. The etiology is not known. Assume a viral (Coxsackie B2) etiology of dermatomyositis. Provoking factors are insolation, cooling, trauma, pregnancy, drug intolerance, vaccination, stressful situations.

Pathogenesis. Various immunopathological disorders.

Clinic. The disease begins with an acute or subacute course, with a muscular syndrome in the form of myasthenia gravis, myalgia, arthralgia also develops, fever appears, with skin lesions, the clinical picture is in the form of dense, widespread edema.

Skin lesions are diverse, more often purple paraorbital erythema with or without edema, erythema over the extensor surfaces of the joint, sometimes with atrophic scars. Damage to skeletal muscles is observed in the form of myalgia during movement and at rest, manifested in the form of pain on pressure and increasing weakness. The muscles of the shoulder and pelvic girdle are compacted, enlarged, active movements are significantly impaired, the patient cannot sit down on his own, raise his head from the pillow and keep it sitting or standing, and raise his limbs. With a large common process, patients are completely immobilized, and in severe cases they are in a state of complete prostration. With damage to the skeletal muscles, weakness, pain, swelling, muscle thickening, muscle wasting, calcification appear. Visceral-muscular syndrome - damage to symmetrical, predominantly proximal, muscle groups, facial muscles leads to a mask-like face, with damage to the pharyngeal muscles, dysphagia develops, and if the intercostal muscles and muscles of the diaphragm are damaged, breathing is disturbed and a decrease in lung capacity, hypoventilation. The oculomotor muscles may be affected with the development of diplopia, strabismus, bilateral ptosis of the eyelids, etc. Polyarthralgia during movement is manifested by limited joint mobility up to the development of ankylosis, mostly due to muscle damage. There is an inflammatory or dystrophic myocardial lesion, which is manifested by persistent tachycardia and pulse lability, expansion of the borders of the heart to the left, with auscultation muffled heart sounds, systolic murmur at the apex, arterial hypotension. Lung damage is associated with the underlying disease, more often it is due to infection, to which patients are predisposed due to hypoventilation of the lungs. The gastrointestinal tract is also involved in the process, anorexia, abdominal pain are noted, symptoms of gastroenterocolitis appear, hypotension of the upper third of the esophagus. Damage to the peripheral and central nervous system.

Diagnostics. Criteria for the diagnosis of dermatomyositis are as follows.

1. Proximal muscle weakness for at least one month.

2. Myalgia within one month in the absence of sensitivity disorders.

3. The ratio of the concentration of creatine in the urine to the sum of the concentrations of creatine and creatinine in the urine, exceeding 40%.

4. A significant increase in the blood level of creatine phosphokinase or transaminases in the absence of creatine phosphokinase or transaminases in the absence of other reasons.

5. Degenerative changes in muscle fibers on biopsy, 4 signs - reliable diagnosis, 3 signs - probable

diagnosis, 2 signs - a possible diagnosis.

Diagnosis: based on clinical and laboratory data, moderate leukocytosis with pronounced eosinophilia, a moderate increase in ESR, and hypergammaglobulinemia are usually observed.

The following studies are of great diagnostic value: a biochemical study of blood and urine, muscle biopsy, especially in subacute and chronic course (thickened muscle fibers are found with transverse striation, fragmentation and dystrophy up to necrosis, a significant cellular reaction appears in the form of an accumulation of leukocytes, plasma cells etc.).

Treatment. In acute and subacute course, glucocorticoids are indicated in large daily doses (prednisolone).

After achieving the effect, the dose of corticosteroids is reduced very slowly (half a tablet every 7-10 days), to a maintenance dose, against the background of delagil (0,25 g), plaquenil (0,2 g), 1 tablet after dinner.

With the development of stable remission, glucocorticoids can be completely canceled. In complex treatment, vitamins of group B, ascorbic acid are prescribed. With severe muscle fatigue, prozerin and its analogues are prescribed in normal doses, ATP.

With early treatment with adequate doses of corticosteroids in patients with acute dermatomyositis, a stable recovery occurs. In subacute cases, remission can be achieved with a maintenance dose of glucocorticoids. In chronic dermatomyositis, the disease becomes undulating.

LECTURE No. 10. Chronic diseases of the colon in children. Clinic, diagnosis, treatment

Chronic nonspecific diseases of the colon.

1. Functional disorders:

1) chronic constipation;

2) irritable bowel syndrome;

3) diverticular disease.

2. Organic disorders:

1) chronic colitis;

2) nonspecific ulcerative colitis;

3) Crohn's disease;

4) intestinal amyloidosis.

Classification of functional bowel disorders.

1. Intestinal upset:

1) irritable bowel syndrome;

2) functional bloating;

3) functional constipation;

4) functional diarrhea;

5) unidentified functional disorders.

2. Functional abdominal pain:

1) functional abdominal pain syndrome;

2) functional pain of an unknown nature.

3. Functional disorders of the gastrointestinal tract in children:

1) functional dyspepsia;

2) irritable bowel syndrome;

3) functional abdominal pain;

4) abdominal migraine;

5) aerophagy.

1. Chronic constipation

Etiology. Causes of development: improper diet, suppression of the urge to defecate, medicinal substances, local organic causes.

Clinic. The frequency of stool becomes less than 3 times a week, the consistency of feces becomes more dense ("sheep feces"), there are difficulties associated with emptying the intestine. Chronic constipation is divided into:

1) functional, which are divided into alimentary, dyskinetic, psychoneurogenic, endocrine, inflammatory;

2) organic.

Functional chronic constipation includes cases of rare bowel movements, when it is not possible to identify any organic cause of this condition.

Diagnostics. Diagnosis of chronic constipation is as follows.

1. High proctolonoscopy.

2. Proctosigmoidoscopy in combination with irrigoscopy.

3. Fractional X-ray injection of barium through the small intestine.

Treatment. Diet therapy for chronic constipation.

1. Black and white bread with bran, legumes; cereals: oatmeal, buckwheat and barley.

2. Meat with a lot of connective tissue.

3. Raw vegetables and fruits, dried fruits.

4. Pickles, sweet kissels and compotes.

5. Dairy products. Water, fruit juices, mineral waters (Essentuki No. 4 and 17).

Diet therapy for constipation in infancy.

1. Fruit juices, vegetable purees.

2. Cabbage and beetroot juices, prune puree.

3. A mixture of "Fris" (for regurgitation, constipation, intestinal colic).

4. A mixture of "Semperbifidus" (lactolactulose), etc. Diet therapy for constipation in other age groups.

1. Wheat bran.

2. Seaweed (kelp).

3. Thermal contrast liquids for drinking.

4. Biologically active additives (with dietary fiber).

Principles of treatment.

1. Laxative antraglycosides (leaves of senna, rhubarb; yew-sem, senadexin); synthetic (phenolphthalein, bisacodyl); lactulose (normaze, portalak), hilak-forte.

2. Motility regulators (raglan, cerucal, motilium, coordi-nax).

3. For pain, a group of reserpine, antispasmodics. Anti-inflammatory in microclysters.

4. Phytotherapy.

5. Physiotherapy.

6. Therapeutic exercise.

7. Psychotherapy.

2. Irritable Bowel Syndrome

Irritable bowel syndrome is pain associated with the small intestine and violations of its function in the absence of morphological changes.

Clinic. Characteristic complaints associated with a violation of the regularity of bowel movements, which exist for more than 3 months (or recur); stool changes, flatulence and flatulence, occupying more than 25% of the time of the day.

Clinical symptoms: violation of the regularity of bowel movements (mainly in the form of constipation, diarrhea). Bloating, bloating, flatulence. Difficult localized abdominal pain, varying in intensity. Feeling of incomplete emptying of the bowels after a bowel movement.

Diagnostics. Ultrasound of the abdominal organs, endoscopic examination, combined endoscopic and X-ray examination of the colon.

Treatment. Treatment for irritable bowel syndrome is as follows.

1. Motility regulators: antispasmodics - pinaverium bromide (dicetel); forlax.

2. Enveloping and adsorbing: smectite.

3. Normalization of microflora: probiotics and prebiotics; intestinal antiseptics (intetriks, metronidazole).

4. Psychotherapy: psychotropic drugs, autogenic training, exercise therapy with the participation of a psychotherapist.

3. Diverticular disease

Diverticular disease - is based on visible pathological changes in the colon.

Diverticula are formed as a result of weakening of the intestinal wall at the passage of blood vessels.

Clinic. With uncomplicated diverticulitis - symptoms of IBS.

In acute diverticulitis, symptoms of peritoneal irritation; fever, leukocytosis; acute cramping, often left-sided, abdominal pain; sometimes blood impurities in the stool.

Diagnostics. X-ray contrast study (irrigoscopy), endoscopy with biopsy, ultrasound of the abdominal organs, computed tomography.

Treatment. Treatment of diverticular disease is as follows.

I stage - a diet with ballast substances, swelling agents, treatment of constipation.

Stage II - restriction of food intake, parenteral nutrition, antispasmodics and antibiotics; then - a diet rich in ballast substances, swelling agents.

Stage II diverticulitis recurrent and resistant to therapy, as well as stage III and IV diverticulitis with local complications - surgery.

4. Organic disorders of the colon

Chronic colitis. Classification of chronic colitis.

1. By process localization:

1) right-handed;

2) left-sided;

3) transverse;

4) total.

2. Process phase:

1) exacerbation;

2) remission.

3. Type of intestinal dyskinesia:

1) hypomotor;

2) hypermotor;

3) mixed.

Diagnosis of chronic colitis.

1. Sigmoidoscopy (catarrhal or catarrhal-follicular proctosigmoiditis).

2. Irrigography, irrigoscopy (thickening and expansion of folds and their discontinuity; smoothness of haustra; narrowing of the intestine),

3. Colonofibroscopy (hyperemia, edema and hypertrophy of the mucosal folds, extended vascular pattern; the mucosa, as a rule, is devoid of luster, may be whitish).

4. Morphology (dystrophic changes, plethora of blood vessels and hemorrhages, edema, etc.).

Treatment of chronic colitis.

1. Diet number 4 (for 3-5 days), then diet number 4b.

2. Anti-inflammatory: sulfonamides; azo compounds (sulfasalazine), salofalk and salozinal; intetrix; 5-NOC (nitroxoline), nicodin.

3. Correction of intestinal dysbiosis.

4. Antispasmodic and myotropic (atropine, no-shpa, papaverine, etc.).

5. Vitamin therapy.

6. Phytotherapy.

7. Physiotherapy.

8. Local treatment.

9. Therapeutic exercise.

10. Mineral waters.

Nonspecific ulcerative colitis. Nonspecific ulcerative colitis is an autoimmune inflammatory-dystrophic lesion of the colon mucosa with the development of hemorrhages and erosions, the formation of extraintestinal manifestations of the disease and local and systemic complications.

The main reasons for the development of NUC:

1) viral or bacterial;

2) milk intolerance;

3) emotional stress reactions;

4) violation of the biocenosis of the intestine and the environment;

5) hereditary predisposition;

6) immunological changes and allergic reactions to food products (most often to cow's milk). Diagnosis of nonspecific ulcerative colitis. In the study of blood (anemia, accelerated ESR, leukocytosis, hypoproteinemia, dysproteinemia).

Coprogram (mucus, leukocytes, erythrocytes, sometimes feces looks like "raspberry jelly").

Bacteriological examination of feces (manifestations of intestinal dysbacteriosis).

Sigmoidoscopy (hyperemia, edema, bleeding, erosion, ulcers, mucus, fibrin, pus).

Irrigoscopy, irrigography (diffuse granularity of the mucosa, absence of gaustra; serrated contours of the colon, filling defects).

Endoscopy for NUC: active stage - redness, loss of vascular pattern; granularity of the mucous membrane; vulnerability on contact, petechiae, bleeding; mucus, pus; ulceration of the mucous membrane, flat, confluent, superficial; pseudopolyps (inflammatory, not tumorous); continuous spread from the rectum in the proximal direction; "relapsing ileitis". The inactive stage is a pale, atrophic mucosa.

Morphological picture of UC: continuous infiltration of polymorphonuclear leukocytes, limited by the mucous membrane. Crypt abscesses. Reducing the number of goblet cells.

X-ray picture in UC: the mucous membrane is covered with granulations, "acicularity" (spicules). Ulcerations, "button ulcers". Pseudopolyps. Loss of haustration, "garden hose phenomenon". Minor erosions against the background of remodeled mucosal relief in ulcerative proctosigmoiditis. Serrated contours of the large intestine due to many edges that form ulcers in ulcerative colitis. There are a large number of ulcers in the relief in the transverse colon. Fringing Contours of the large intestine in ulcerative colitis Symptom "cobblestones" Pseudopolyposis in the left half of the intestine Pseudopolyposis in total ulcerative colitis.

Treatment. Crohn's disease is a granulomatous inflammation of any part of the digestive tract with the development of mucosal ulceration, narrowing of the lumen, fistulas, and extraintestinal manifestations of the disease.

Treatment of UC and Crohn's disease.

1. Rational diet: frequent, fractional meals.

2. Basic drug therapy: azo compounds (sulfasalazine, salazopyrin, salazopyridazine; salofalk, salozinal); angioprotectors (trental, parmidin); multivitamins. Crohn's disease (azathioprine, cyclosporine, methotrexate).

3. Corticosteroids (prednisolone). For Crohn's disease (bu-desonide).

4. Eubiotics (intetrix, trichopolum, ercefuril, enterol, etc.).

5. Treatment of intestinal dysbacteriosis.

6. Infusion therapy. Hemosorption and plasmapheresis.

7. Anabolic hormones (nerabol, retabolil, etc.) - according to strict indications.

8. Treatment of anemia: iron preparations (ferrumlek, ectofer, etc.) parenterally.

9. Antihistamines (diazolin, suprastin, tavegil, etc.).

10. Normalization of bowel function antispasmodics and analgesics (papaverine, no-shpa, halidor); enzymes (pancreatin, panzinorm, digestal, mezim-forte); imodium; fight against constipation (bran, vaseline oil).

11. Sedatives (relanium, valerian root decoction, motherwort tincture, seduxen).

12. Dimephosphone (membrane stabilizing, immunomodulatory, bactericidal action).

13. Sandostatin is an analogue of somatostatin.

14. Herbal medicine (chamomile, calendula, St. John's wort, gray alder, motherwort, mint, plantain).

15. Physiotherapy.

16. Local treatment.

17. Surgical treatment (subtotal one- or two-stage colectomy).

Amyloidosis of the colon. Colon amyloidosis is a partial or complete paralytic ileus.

Gastrointestinal bleeding. Ulcers of the intestinal mucosa. Malabsorption Syndrome.

Treatment for amyloidosis of the colon.

1. Derivatives of 4-aminoquinoline (chloroquine, delagil, plac-venil); corticosteroids (prednisolone); immunocorrectors (T- and B-activin, levamisole).

2. Means of stimulation of amyloid resorption: ascorbic acid, anabolic hormones.

3. Colchicine, dimethyl sulfoxide, prednisolone.

In secondary amyloidosis, the underlying disease should be treated first.

LECTURE No. 11. Modern problems of dysbiosis in children. Clinic, diagnosis, treatment

Three phases of microbial colonization of the gastrointestinal tract in a child:

1) the first - aseptic, lasting from 10 to 20 hours;

2) the second - the initial colonization by microorganisms, the duration is from 2 to 4 days, depending on external environmental factors, the nature of nutrition and the time of breastfeeding;

3) the third - stabilization of microflora (up to 1 month). The intestinal microflora is a complex, dynamically balanced ecosystem. Contains over 500 different types of bacteria. 1 g of feces contains more than 1011 bacteria. The intestinal microflora is subject to frequent disturbances under the influence of adverse factors. Most of the intestinal microorganisms are the so-called obligate (indigenous) microflora (bifidobacteria, lactobacilli, non-pathogenic Escherichia coli, etc.). 92-95% of the intestinal microflora consists of obligate anaerobes.

Facultative (UP and saprophytic) microflora is unstable, has no significant biological functions, is represented by bacteria - citrobacters, micrococci, proteus, yeast-like fungi, clostridia. The composition of the intestinal microflora is quite individual and is formed in the first days of a child's life. An important factor in the formation of normal intestinal microflora is natural feeding. Women's milk contains a large number of substances that contribute to the colonization of the intestine by certain types of microorganisms in certain quantities (staphylococci, etc.). The composition of the intestinal flora of a child after two years is practically the same as that of an adult: the majority are anaerobes, which are difficult to cultivate. The density of bacteria in the stomach, jejunum, ileum and colon is 1, 10, 100 and 1 thousand, respectively, in 000 ml of intestinal contents.

Functions of the intestinal microflora.

1. Protective:

1) a barrier against microbial contamination (acidic environment, colony competition);

2) a decrease in the permeability of the mucous membrane for macromolecules.

2. Immune:

1) synthesis of immune defense factors (lysozyme, complement, properdin);

2) stimulation of the maturation of the lymphoid apparatus of the intestine and the synthesis of Ig;

3) stimulation of maturation of phagocytes.

3. Metabolic:

1) synthesis of B vitamins;

2) iron metabolism;

3) the exchange of bile acids.

4. Digestive:

1) the breakdown of carbohydrates;

2) synthesis of enzymes;

3) parietal digestion;

4) regulation of absorption;

5) stimulation of gastrointestinal motility.

Bifidobacteria inhibit the growth of potential pathogens. Restore normal intestinal flora during antibiotic therapy. They produce B vitamins and folic acid. Reduce the level of urea in the blood. They act as immunomodulators. Reduce the level of cholesterol in the blood. Intestinal dysbiosis is always secondary. It occurs as a result of changes in the internal environment of the intestine, and a direct effect on the intestinal microflora. It can lead to damage to the intestinal epithelium, disruption of the processes of digestion and absorption, aggravating the already existing adverse changes in the gastrointestinal tract. Changes in the quantitative and qualitative composition of microflora in various parts of the intestine. The appearance of facultative strains that are not part of the resident microflora: Proteus, Morganella, Klebsiella, Enterobacter, Citrobacter, Hafnia, E. coli with enzymatic deficiency and hemolytic properties, Pseudomonas. Factors affecting the state of the gastrointestinal mucosa: diseases that change the internal environment of the intestine (oxygen content, enzyme composition). Antibiotic therapy (rational and irrational).

Functional disorders of intestinal motility.

1. Immunodeficiency states.

2. The influence of the nature of food on intestinal dysbiosis: food rich in carbohydrates stimulates the bifidoflora and leads to an increase in the bacterial mass of the large intestine. Fatty food inhibits bifidobacteria and enterococci, and stimulates the reproduction of bacteroids.

3. Protein diet practically does not affect the spectrum and quantity of intestinal bacteria.

Causes of microbiocenosis changes.

1. Lesions of the gastrointestinal tract of an infectious and non-infectious nature.

2. Acute infections of extraintestinal localization.

3. Chronic inflammatory and allergic diseases

4. Leukemias and other malignant processes.

5. Post-radiation syndrome.

6. The use of cytostatics and antibiotics. Classification of dysbacteriosis.

Stage I - a decrease in the number and level of activity of bifidobacteria and lactobacilli. Aerobic flora changes.

Stage II - an increase or a sharp decrease in E. coli. The emergence of defective strains of E. coli and atypical species of enterobacteria.

Stage III - high titers of associations of opportunistic microflora. A sharp decrease in bifido- and lactobacilli or suppression of their activity.

Stage IV - a sharp decrease in bifidobacteria and lactobacilli or suppression of their activity. Pronounced imbalance of microflora High titers of bacteria of the genus Proteus, Pseudomonas aeruginosa. clostridia.

The clinical picture of intestinal dysbiosis: liquid or unstable stools with an admixture of mucus, greens, or particles of undigested food. Less often - constipation, regurgitation, vomiting, flatulence, rumbling along the intestinal loops. Abdominal pains (intermittent) Thrush, coated tongue; hyperemia around the anus. Anorexia, poor weight gain. Clinical forms of dysbacteriosis.

1. Compensated (latent compensated): violation of the normal composition of the microflora. There are no clinical symptoms.

2. Subcompensated (local subcompensated): violation of the normal composition of the microflora, symptoms of intestinal inflammation with intoxication and bacteremia.

3. Decompensated (generalized decompensated): the appearance of metastatic inflammatory foci, intoxication and bacteremia, the development of sepsis and septicemia.

Examination plan and diagnosis of dysbacteriosis: hemogram, extended coprogram, cytocoprogram, bacteriological examination of feces, pH of feces; sugar curve, trypsin activity, biochemical blood test; elimination tests (exclusion of gluten, dairy products, sweet vegetables and fruits); determination of sweat chlorides, ultrasound of the abdominal organs.

Indications for the study of intestinal microflora (for dysbacteriosis):

1) intestinal dysfunction after the use of antibacterial drugs;

2) prolonged period of convalescence after OKA;

3) chronic intestinal disorders without isolation of pathogenic bacteria;

4) food allergy;

5) chronic diseases of the gastrointestinal tract in combination with flatulence, diarrhea and constipation;

6) irritable bowel syndrome. Stages of correction of dysbacteriosis:

1) diet therapy;

2) normalization of intestinal microflora;

3) adsorption and excretion of toxic products from the intestine;

4) restoration of normal digestion;

5) relief of intestinal motility disorders;

6) correction of the immune status;

7) normalization of metabolism.

Diet therapy for persistent constipation and the absence of severe pain.

Diet number 3 - stimulating intestinal motility.

1. Low-fat boiled meat, fish; buckwheat, rice or oatmeal; unleavened cottage cheese, white stale bread, pasta and vermicelli.

2. Adding bran to food, wholemeal bread.

3. Fruit juices, honey, sweet dishes, carbonated drinks, vegetables, salty foods, cold foods, white wines and highly mineralized mineral waters (Arzni, Yessentuki No. 17).

Diet therapy for diarrhea. Recommended.

1. White stale bread and crackers from it, dry biscuits.

2. Slimy rice and oatmeal soups; rice, oatmeal and semolina porridge; fresh cottage cheese, dishes from boiled vegetables; omelettes, soft-boiled egg; jelly, mousses and juices from quince, pear, dogwood, carrot, black currant and blueberry.

3. Warm and hot dishes, strong tea, cocoa, natural red wines.

Forbidden: fatty foods, raw fruits, wheat milk and barley porridge.

Limited: vegetable fiber and sugar (no more than 40 g per day).

Diet therapy for fermentative dyspepsia (pronounced flatulence; profuse, frothy, sour stools). Recommended.

1. Increase in the amount of boiled protein products (meat, fish).

2. Non-concentrated broths, fish soup, scrambled eggs, boiled or baked potatoes.

3. Use of cloves, bay leaf, pepper. Forbidden: honey, jam, sweets, watermelons, bananas, grapes, milk and vegetable fiber - for 1-2 weeks.

Diet therapy for putrefactive dyspepsia (frequent headaches, signs of intoxication, moderate flatulence, spasms and pain in the distal intestines). Recommended.

1. The first 2 days - hunger (broth of wild rose, slightly sweet tea),

2. From the 3rd day - dry biscuits, white bread crackers. From the 5-6th day, rice porridge on the water and sour-milk products.

3. In the future - a vegetable diet. Forbidden: protein foods and coarse fiber. Restricted: fats.

Antibacterial drugs: do not disturb the balance of the microbial flora in the intestine - intetrix - a combination of 3 antiseptics from the group of 8-hydroxyquinolones, effective against pathogenic bacteria and fungi. Nifuroxazide is effective against Gram-positive cocci and Gram-negative (Salmonella, Shigella and Proteus) bacteria. Enterosediv contains streptomycin, bacitracin, etc. Dpendan containing furazolidone and metronidazole.

Biologically active substances are used to improve the function and regulation of the microbiocenosis of the gastrointestinal tract, prevention and treatment: dietary supplements, functional nutrition, probiotics, prebiotics, synbiotics, bacteriophages, biotherapeutic agents.

Dietary supplements - natural nutrients: vitamins, minerals, proteins, enzymes, herbal products.

Requirements for dietary supplements that must contain one or more nutritional components: vitamins, minerals, herbs or other plants, amino acids. Not intended to be used as the main ordinary food or the sole source of nutrition. They are used only as an addition to the main diet in order to increase the daily intake of certain nutritional components. Functional nutrition should be understood as ready-to-sell food products in which biological products have been added. Functional nutrition is defined as a modified food product that provides health benefits more optimally than the original product. Functional nutrition includes antioxidants, carotenoids, digestive enzymes, yoghurts and dairy products fortified with pro- and prebiotics. Mixtures containing pre- and probiotics (sour-milk products, NAN fermented milk with bifidobacteria, NAN 6-12 months with bifidobacteria). Mixtures with the addition of thickening polysaccharides (for example, Frisovoy). Acidobif is a food supplement for children over 1 year old with lactase deficiency, milk protein intolerance and intestinal dysbacteriosis, containing lacto- and bifidobacteria. Eugalan forte is a food supplement for adults and children over 3 years old, containing bifidobacteria and lactulose.

Probiotics are preparations based on microorganisms (lyophilized powders containing bifidobacteria and lactobacilli). Probiotics are drugs and food products that contain substances of microbial and non-microbial origin. With a natural route of administration, they have a beneficial effect on the physiological functions and biochemical reactions of the body through the optimization of its microecological status. Probiotics (eubiotics) - live, weakened strains of microorganisms: more often bifidobacteria, less often - yeast, which, based on the term "probiotic", refer to the normal inhabitants of the intestines of a healthy person. Microorganisms that make up probiotics are contained in large quantities, are non-pathogenic, non-toxic, and remain viable when passing through the gastrointestinal tract and during storage. Modern requirements for probiotics: natural origin resistance to the action of gastric acid and bile, the ability to colonize the intestines, antagonism to pathogenic bacteria. Proven clinical effect. Indications for the use of probiotics: diarrhea associated with taking antibiotics, infectious diarrhea, prevention of diarrhea, irritable bowel syndrome, gastrointestinal syndrome with allergies, inflammatory diseases.

Probiotics - preparations, mono- and polycomponent preparations: bifidumbacterin, lactobacterin, bifikol, colibacterin, primadofilus, floradophilus, bifinorm, subamin, bifi-din, bifilin, biobacton, bifilong, linex, biofructolact, bactisubtil. Combined preparations: bifiform, bifi-zil, acipol, bifacid, bifidumbacterin-forte, fermented milk eubiotics bifidok, bifikefir, lactofidus.

Prebiotics are non-digestible food ingredients that selectively stimulate the growth and metabolic activity of one or more groups of bacteria (lactobacteria, bifidobacteria) in the colon. In order for a food component to be classified as a prebiotic, it must not be hydrolyzed by enzymes and absorbed in the upper gastrointestinal tract. The prebiotic must be a selective substrate for bifidobacteria and lactobacilli. Substance prebiotics: fructose-oligosaccharides, galacto-oligosaccharides (in dairy products, corn flakes, cereals, bread, onions, field chicory, garlic, bananas, and many others).

Galacto-oligosaccharides are found in breast and cow's milk. Inulin is found in the tubers and roots of dahlias, artichokes, and dandelions.

Lactulose is a synthetic disaccharide not found in nature.

Prebiotics = drugs: hilak-forte (concentrate of metabolic products of normal microflora) - inhibits the growth of pathogens and restores the biological environment. Calcium pantothenate helps to increase the biomass of bifidobacteria. Pamba (para-aminobenzoic acid) promotes the growth of normal microflora. Lysozyme has mucolytic and bifidogenic properties and is active against Gram-positive cocci.

Synbiotics are a mixture of probiotics and prebiotics that improve the survival and establishment of live bacterial supplements in the intestines and selectively stimulate the growth and activation of the metabolism of indigenous bacteria. Examples of synbiotics: bifidumbacterin-forte, bifilis, lactofidus, nutrolin B, vita and other biotherapeutic agents contain substances and (or) live microorganisms that have therapeutic properties. Microorganisms used as biotherapeutic agents include: Lactobacillus acidophilus, L. plantarum, L. casei, L. bulgaricus; Bifidobacterium longum, Enterococcus faecium. Probiotic preparations are not considered as therapeutic agents until clinical studies have proven their effectiveness. Biotherapeutic agents - drugs bifidumbacterin, lactobacterin, coli-bacterin, primadofilus, enterol, bactisubtil. Hilak forte - drops, calcium pantothenate and pamba.

Enterosorbents - smecta, bilignin, tannacomp, polysorb, polyphepan, espumizan. Assign for the correction of compensated dysbacteriosis. Children from birth can be prescribed probiotics containing bifidumbacteria. Clinical efficacy is assessed 10 days after the end of therapy, microbiological - after 1 month. The number of bifid therapy courses is 1-3. With a decrease in the number of lactobacilli, linex, acipol and lactobacterin, acilact are used. Acid-forming preparations are recommended - prebiotics that do not contain live bacteria: normase or hilak-forte (together with bifid preparations). You can use lysozyme, bifiliz. Correction of sub-compensated dysbacteriosis involves the appointment of 3-4 courses of combined multicomponent preparations - bifido- and lacto-containing probiotics. Combination therapy with normase or hilak-forte is recommended only if the content of lactobacilli is normal. In addition to lysozyme, CIP (complex immunoglobulin preparation) can be used. In the absence of a sufficient effect, chlorophyllipt can be used; sporobacterin, biosporin (or their analogue - bactisubtil). Correction of decompensated dysbacteriosis is a short-term (3-7 days) appointment of sorbents (carbolene, carbolong, vaulen, microsorb P, polyphepan, lignin, smecta). Use of 3-6 courses of bifido- and lactose-containing probiotics. With developed immunodeficiency, immune preparations (interferon or reaferon, leukinferon, levamisole, etc.), enzyme preparations (mezim-forte, panzinorm, festal, pancitrate, creon) are prescribed for 1-3 weeks. With septic manifestations - the use of antibacterial therapy against the background of probiotics (with selective contamination - non-absorbable drugs, with secondary extraintestinal foci - resorptive drugs) - ercefuril, furazolidone aminoglycosides, erythromycin, macrolides, metronidazole; antifungal drugs.

LECTURE No. 12. Malabsorption syndrome in children. Clinic, diagnosis, treatment

Enteropathy is a pathological condition resulting in a deficiency or dysfunction of certain intestinal enzymes, due to the absence, deficiency or violation of the structure of certain intestinal enzymes that provide digestive processes.

Absorption of carbohydrates: food carbohydrates consist of disaccharides:

1) sucrose (regular sugar = fructose + glucose), lactose (milk sugar = galactose + glucose);

2) monosaccharides: glucose and fructose;

3) vegetable starches (a polysaccharide consisting of glucose molecules).

The enterocyte is unable to transport carbohydrates more than the monosaccharide. Therefore, carbohydrates must be broken down before absorption. Malabsorption syndrome - a syndrome of impaired intestinal absorption, malabsorption syndrome can lead to: lack of pancreatic enzymes - violation of the abdominal phase of digestion; cholestasis, intestinal dysbiosis - a violation of the metabolism of bile acids, lipid transport to a violation of the biliary phase of digestion; deficiency of cavity enzymes (disaccharidases, peptidases, etc.) leads to disruption of membrane digestion; atrophy of the mucous membrane of the small intestine - to a violation of absorption, i.e., the cellular phase of digestion, while membrane digestion also suffers; pathology of the intestinal lymph flow, mesenteric circulation to - deterioration of the further transport of absorbed substances - the outflow phase suffers; accelerated passage of food through the intestines - to a violation of all types of digestion.

1. Enzymopathy

Enzymopathy is a pathological condition caused by the absence, deficiency or violation of the structure of certain cellular enzymes that provide digestive processes.

The clinical picture is due to impaired absorption through the mucous membrane of the small intestine of one or more nutrients. Clinical manifestations of malabsorption syndrome in the form of diarrhea, weight loss, protein deficiency, signs of hypovitaminosis.

Malabsorption syndrome can be primary (hereditary) or secondary (acquired). Classification. pathogenic classification.

1. Exocrine pancreatic insufficiency:

1) cystic fibrosis of the pancreas;

2) chronic malnutrition with protein and calorie deficiency;

3) Shwachman-Diamond syndrome;

4) chronic pancreatitis with exocrine insufficiency;

5) specific enzyme defects (lipase, trypsinogen).

2. Insufficiency of bile acids:

1) obstruction of the biliary tract (biliary atresia, cholelithiasis, cancer of the pancreatic head);

2) resection of the ileum;

3) cirrhosis of the liver, chronic hepatitis (decreased secretion);

4) dysbacteriosis.

3. Violations of the functions of the stomach:

1) postgastrectomy syndrome;

2) vagotomy;

3) pernicious anemia caused by vitamin B12 deficiency

4. Dysmotility:

1) hyperthyroidism;

2) diabetes mellitus;

3) scleroderma; 4 amyloidosis.

5. Pathology of the intestinal mucosa:

1) celiac disease;

2) lactase deficiency;

3) sucrase and isomaltase deficiency (combined);

4) exudative enteropathy;

5) intolerance to cow's milk protein (and/or soy);

6) insufficiency of enterokinase;

7) abetalipoproteinemia (Bassen-Kornzweig syndrome);

8) impaired transport of amino acids (tryptophan, methionine, lysine, etc.);

9) vitamin B12 malabsorption (transcobalamin-II deficiency);

10) congenital disorders of folic acid absorption processes;

11) chlorine-losing diarrhea;

12) vitamin D-dependent rickets;

13) enteropathic acrodermatitis;

14) Menkes syndrome (curly hair syndrome);

15) Crohn's disease;

16) malabsorption after suffering enteritis;

17) tropical sprue;

18) Whipple's disease;

19) chronic infections (immunodeficiency), in particular giardiasis;

20) primary immune deficiency (Wiskott-Aldrich syndrome);

21) congenital short intestine;

22) short bowel syndrome after resection (resection of the proximal portion of the small intestine, resection of the ileum, resection of the ileocecal region);

23) eosinophilic gastroenteritis.

Etiology. The etiology in each case is different (absence or reduced activity of lactase, a-glucosidase, enterokinase). Disaccharidase deficiency (sugarase, lactase, isomaltase), true celiac disease (gliadin intolerance), enterokinase deficiency, intolerance to monosaccharides (glucose, fructose, galactose), malabsorption of amino acids (cystinuria, Hartnup's disease, etc.) ), malabsorption of vitamin B12 and folic acid, etc. Secondary, or acquired, malabsorption occurs in many chronic diseases of the stomach and intestines (pancreatitis, hepatitis, dysbacteriosis, dyskinesia, Crohn's disease, etc.).

Clinic. The clinical picture in children is dominated by chronic diarrhea with a high content of lipids in the feces. Dystrophy gradually develops, children lag behind in growth. Clinical manifestations of vitamin deficiency, disturbances in water and electrolyte balance (dry skin, seizures, glossitis, hypokalemia, hyponatremia, hypocalcemia, etc.) are added. Pancreatic digestion deficiency syndrome is characterized (pancreatic steatorrhea): fat, amylorrhea (extracellular starch). For the syndrome, disturbances in the flow of bile are typical (hepatogenic steatorrhea): steatorrhea (with a predominance of fatty acids against the background of a smaller amount of neutral fat in the complete absence of soaps), creatorrhea is possible (altered muscle fibers prevail).

Intestinal diarrhea is characterized by steatorrhea, which is predominantly soaps and fatty acids. Diagnostics. Methods of paraclinical examination.

1. General blood analysis.

2. General urine analysis.

3. Feces for Giardia, helminth eggs, scraping for enterobiasis.

4. Coprogram (extended, expanded): starch, neutral fat, fatty acids, connective tissue, iodine-philic microflora are absent in normal feces.

5. Biochemical study of blood serum: proteinogram, functional liver tests (ALT, AST, alkaline phosphatase, bilirubin, cholesterol), K, Fe, Ca, P, folic acid, vitamin B12, carotene).

6. Sugar curve - a flat sugar curve indicates diffuse mucosal damage. It should be noted that the study of glucose is carried out on an empty stomach, after 15-30, 60-120 minutes.

7. d-xylose test. d-xylose is a pentose absorbed passively through intact mucosa. Excreted by the kidneys. In malabsorption, most of the xylose is lost in the stool and does not reach the circulatory system. At least 30% is excreted in the urine, i.e. more than 1,25 g of the administered per os (5 g), (PS According to studies, this test has a direct correlation with the glucose load test. Considering the complexity of the d-xylose test) .

8. Load tests with lactose, maltose, sucrose, starch. With a lactose load, an increase in the blood lactose cleavage product after oral administration of 50 g of lactose by less than 20% indicates lactase deficiency.

9. Chromatographic identification of carbohydrates, amino acids contained in urine.

10. Hydrogen breath test: Determination of the amount of hydrogen in exhaled air after loading with sugars at a dose of 2 g/kg (maximum up to 50 g). The test is based on the fact that if sugar is not adsorbed in the upper parts of the small intestine, it reaches the distal parts, where intestinal bacteria act on it to produce hydrogen. The latter is quickly absorbed and exhaled in measurable quantities. An increased amount of exhaled hydrogen (more than 20 g/million during the first 2 hours) is considered a pathology. (PS In patients taking AB, and in approximately 2% of healthy individuals, hydrogen-producing intestinal flora is absent).

11. Sowing feces for dysbacteriosis.

12. Research of pancreatic enzymes in blood and urine.

13. Ultrasound of the pancreas, liver, gallbladder.

14. Determination of chlorine (Cl) in sweat fluid. If necessary, molecular genetic testing for cystic fibrosis.

15. X-ray of the gastrointestinal tract - information about the time of passage of barium through the intestines, damage to the mucous membrane, the presence of a stricture or tumor.

16. Gastroduodenoscopy with biopsy. A biopsy of the small intestine helps in the diagnosis of celiac disease, but it is not indicative of many other diseases.

17. Colonoileoscopy with biopsy.

2. Endocrine pancreatic insufficiency

Cystofibrosis of the pancreas (cystic fibrosis) is a hereditary disease with a recessive type of inheritance. Frequency in population 2-8: 100 population. In patients with cystic fibrosis, the chloride channel on the apical part of the cell membrane "does not work", leading to a disruption in the release of chlorine from the cell, which contributes to an increased escape of sodium ions from the lumen into the cell, followed by the aqueous component of the intercellular space. The consequence is a thickening of the secretions of the glands of external secretion (bronchopulmonary system, pancreas, salivary glands, gonads). The respiratory system and pancreas are most commonly affected. The disease is manifested by respiratory and intestinal syndromes already in the first year of life. The degree of interest of systems and organs is different. Paraclinical markers are: coprogram - the presence of a large amount of neutral fat and almost always its predominance over muscle fibers and polysaccharides.

Ultrasound of the pancreas - diffuse compaction of the parenchyma, and with age, a decrease in the size of the pancreas.

High sweat chlorides (60,0 mmol/l or more), repeatedly positive (at least 3 times).

Molecular genetic testing. But it should be noted that the negative results of this study do not exclude the diagnosis of cystic fibrosis.

Shwachman-Diamond syndrome (congenital hypoplasia of the pancreas, combined with neutropenia, short stature and anomaly of the bones).

Diagnosis based on the syndrome of pancreatic insufficiency in the first year of life, neutropenia, normal sweat chlorides.

Chronic pancreatitis with exocrine insufficiency - causes can be past diseases, including mumps, trauma, drugs, toxins, diseases and abnormalities of the bile and pancreatic ducts, systemic diseases.

Specific enzyme defects (lipase, trypsinogen).

1. Isolated deficiency of pancreatic lipase (Shedon-Rey syndrome) is manifested by fatty diarrhea due to neutral fats. Children have a good appetite, adequately develop physically and mentally.

Diagnosis: based on fatty stools, neutral fat in feces, normal sugar curve, d-xylose test, sharp decrease or absence of lipase in pancreatic juice, no morphological changes in the pancreas, normal sweat chlorides.

Treatment: replacement therapy.

2. Isolated trypsin deficiency occurs with a frequency of 1: 10; the type of inheritance is autosomal recessive.

Clinically manifested shortly after birth with mushy or watery, fetid stools against the background of natural feeding, poor development and increasing malnutrition. A large amount of protein and fats is found in the feces. Treatment: replacement therapy.

3. Amylase deficiency - diagnosis based on clinical manifestations, watery, sour-smelling stools. Coprogram (starch). Decreased or absent amylase activity. Effect on the background of an elimination (starch-free) diet.

3. Bile acid deficiency

Obstruction of the biliary tract (atresia of the biliary tract, cholelithiasis, cancer of the head of the pancreas), resection of the ileum, dysbacteriosis, cirrhosis of the liver, chronic hepatitis (decrease in secretion). In a coprological study, the predominance of fatty acids against the background of a smaller amount of neutral fat in the complete absence of soaps; Creatorrhoea is possible - altered muscle fibers predominate.

4. Disorders of the functions of the stomach

Postgastrectomy syndrome, vagotomy, pernicious anemia caused by vitamin B12 deficiency.

5. Dysmotility

Hyperthyroidism, increased thyroid hormone levels, increased motility, reduced food transit time, decreased digestion and absorption of fats, diarrhea and steatorrhea. Diabetes Mellitus: Diarrhea is secondary and is associated with diabetic neuropathy.

Syndrome mechanisms:

1) violation of the exocrine function of the pancreas, steatorrhea;

2) decreased motility of the antrum of the stomach, impaired emulsification of fats, steatorrhea;

3) violation of the autonomic vegetative regulation of the intestine, intestinal stasis, increased reproduction of bacteria, deconjugation of bile acids, malabsorption of fats. Scleroderma, decreased motility of the small intestine associated with a decrease in the number of smooth muscle cells of the intestinal wall, increased reproduction of bacteria, deconjugation of bile acids, malabsorption of fats. Amyloidosis, deposition of amyloid protein in many organs, including the smooth muscle tissue of the intestinal wall, decreased motility, increased multiplication of bacteria, deconjugation of bile acids, malabsorption of fats.

6. Pathology of the intestinal mucosa

Celiac disease (gluten enteropathy, celiac disease, celiac sprue, non-tropical sprue).

Causes of development: congenital chronic disease of the small intestine, caused by the absence or decrease in the activity of peptidases of the brush border of the small intestine, which break down gliadin, an integral part of the protein of various cereals (wheat, rye, barley, oats).

Diagnosis of malabsorption: subtotal or total atrophy of the small intestine mucosa, clinical effect of a gluten-free diet, improvement in absorption and x-ray data when gluten is excluded from the diet, improvement in the morphology of the small intestine from the use of a gluten-free diet, recurrence of morphological disorders after stopping the gluten-free diet.

X-ray signs: dilatation of the intestine is the most constant and important symptom, it is especially pronounced in the middle and distal jejunum. Dilatation of the intestine is explained by its hypotension. Expansion of the intestine is absent in pancreatic steatorrhea and is not due to increased fat content in the intestine.

Serological diagnostics is possible: determination of the content of antigliadin (AGA) antibodies, anti-endomysial (EMA) and antireticular (ARA) IgA antibodies in the blood serum. The presence of these antibodies is considered specific for the disease, and their study can be used to screen the next of kin of patients, and also to establish the frequency of celiac disease in the population.

In the expanded coprogram - the presence of neutral fat and especially fatty acids and soaps, a flat sugar curve with a load of glucose, indicating a violation of the absorption of di-and mono-substances.

The main method of treatment is a diet with the exclusion of all foods containing gluten. Of the cereals, only rice, buckwheat, corn, the preparation of cakes, pastries, cookies from soy, rice flour, and starch are allowed. The diet is prescribed even in the asymptomatic course of the disease and must be observed throughout the life of the patient. It must be remembered that even 100 mg of wheat flour taken daily causes pronounced changes in the morphological picture during a biopsy.

lactase deficiency. Lactase deficiency in children and adults has a heterogeneous molecular genetic nature.

There are two varieties of the enzyme: infant lactase and the more specific adult-type lactase. At the age of 3-5 years, the gene regulation of enzyme synthesis switches from the child to the adult type.

There are 4 forms of lactase deficiency:

1) primary hereditary lactase deficiency: alactasia with an autosomal recessive (possibly dominant) type of inheritance;

2) transient lactase deficiency in preterm infants;

3) hypolactasia of the adult type (persistence of the child form of lactose);

4) secondary lactase deficiency (with enteritis, food allergies, celiac disease, immunodeficiency states). Clinical manifestations in the form of diarrhea after taking milk. Diagnosis: diarrhea, in the coprogram pH < 5,0. If there is no violation of abdominal or membrane digestion, then other changes in the coprogram can not be found, glycemic curves are normal with a load of glucose, galactose, d-xylose, and flat with a load of lactose.

A biopsy of the small intestine - in individuals with a primary deficiency of morphological changes in the mucous membrane of the small intestine is usually not found, jejunoscopy - more often without features, a visual endoscopic picture of the mucosa - a positive effect on the abolition of dairy products, deterioration (diarrhea) after taking milk.

Treatment, if lactase deficiency occurs in a child who is breastfed, has 2 options.

1. If the child has clinical and laboratory signs of lactase deficiency, but is gaining weight, breastfeeding should be continued, but milk and dairy products, as well as beef, should be excluded from the mother's diet.

2. If, against the background of lactase deficiency, the child’s body weight does not increase or decreases with a sufficient amount of breast milk, and therapeutic measures do not give a positive effect, it is necessary to stop breastfeeding and switch to dairy-free or low-lactose formulas. With a correct diagnosis, the clinical effect is noted already in the first 2-3 days.

Sucrase and isomaltase deficiency. Diagnosis: recurrent diarrhea, vomiting after taking sucrose, delayed physical development, onset of the disease after transferring the child to mixed or artificial feeding, improvement in general condition after excluding sucrose and starch from the diet, flat glycemic curve after loading with sucrose, normal, as a rule, curve at glucose load, urine sugar chromatography, coprogram, as in lactase deficiency.

Treatment: elimination diet.

exudative enteropathy. Exudative enteropathy (proteolytic enteropathy) is characterized by increased secretion due to increased intestinal permeability of protein into the intestinal lumen from the blood and loss of protein with feces.

There are primary and secondary forms of exudative enteropathy.

Primary - a congenital generalized disease of the lymphatic system, selectively localized in the intestine. In foreign literature, they are characterized as an independent nosological unit.

Pathogenesis: increased extravasation of the protein occurs with an increase in pressure in the lymphatic vessels of the intestine.

Secondary causes of exudative enteropathy are celiac disease, gastroenteritis, cow's milk protein intolerance, and many other diseases.

Pathogenesis: increased permeability of intestinal membranes for macromolecules.

Clinic and diagnostics: edematous syndrome; blood serum decrease in the total level of protein, g-globulins, cholesterol: in the coprogram: as in celiac disease due to impaired absorption of fat and its transport (neutral fat, fatty acids, soaps); fluoroscopy of the intestine: radiological changes are associated with edema of the intestinal wall. The passage of the contrast medium is timely. With lymphangiectasia, the intestinal wall may have a cone-shaped thickening of circular folds in the form of a garland. In more severe cases, pseudopolyposis manifestations can be detected. The endoscopic picture is diverse: with preserved folding of the jejunal mucosa, its pale pink or pink color with a pronounced vascular pattern, sometimes combined with punctate hemorrhages, lymphofollicular hyperplasia, there is also free hyperplasia of the mucosa in the form of numerous bulges.

Biopsy: according to Waldmann, exudative enteropathy revealed changes in the lymphatic system of the mucous membrane and mesentery of the small intestine, which were called intestinal lymphangiectasia - enlargement of the lymphatic vessels, interstitial edema.

Treatment.

1. Low-fat diet, medium chain triglycerides are recommended because they are absorbed without the involvement of the intestinal lymphatic system and they can reduce pressure in the intestinal lymphatic vessels. Limit salt.

2. In / in the introduction of protein drugs.

3. Symptomatic therapy.

Intolerance to cow's milk protein. Cow's milk protein intolerance occurs most often in children, predominantly under the age of 2 years, in about 1 in 200 young children.

Pathogenesis: immune response to proteins, mainly /? - lactoglobulin (this particular protein is not found in human milk). Clinical manifestations in the form of diarrhea.

Diagnosis: acute symptoms should resolve within 48 hours, and chronic symptoms within 1 week after stopping milk feeding. With endoscopy and biopsy of the jejunum: changes develop that resemble cases of untreated celiac disease, although not so pronounced, an increased titer of antibodies to cow's milk protein.

Treatment: elimination of cow's milk, and if necessary, soy.

Enterokinase deficiency. Enterokinase activates pancreatic trypsinogen, converting it to the active enzyme trypsin. Due to the deficiency of enterokinase, the digestion of proteins in the intestine is disturbed. Occurs only in children.

Clinical manifestations: diarrhea, edema, hypoproteinemia, enterokinase activity in the duodenal contents is practically absent, while the activity of amylase and lipase is not changed.

Treatment: replacement therapy with pancreatic enzymes.

Abetolipoproteinemia. Abetolipoproteinemia (Bassen-Kornzweig syndrome) is a condition in which there is no apo-B protein necessary for the formation of chylomicrons and very low density lipoproteins, which leads to overflow of enterocytes with fats, and absorption of fats is impaired.

Clinical manifestations in the first year of life, developmental delay is detected. The feces are copious and discolored, the abdomen is distended. Mental development is somewhat behind. Characteristically, after the age of 10 years, the appearance of cerebellar symptoms. In adolescence, atypical retinitis pigmentosa develops. Diagnosis: detection of acanthocides in peripheral blood, hypocholesterolemia (200-800 mg/l), absence or minimal amount of β-, β-lipoproteins (β- or β-lipoproteinemia), pronounced accumulation of fasting lipids in the villi of enterocytes of the duodenal mucosa.

Treatment: restriction of long chain fats, vitamins A, D, E, K, consumption of medium chain triglycerides, Violation of amino acid transport.

Diagnosis: based on thin-layer chromatography of amino acids in urine, blood serum.

Vitamin B12 malabsorption (transcobalamin-P deficiency). Deficiency of transcobalamin-II, a protein necessary for intestinal transport, vitamin B12, leads to severe megaloblastic anemia, diarrhea, and vomiting.

Treatment: vitamin B12 1000 mcg / week for transcobalamin-II deficiency and 100 mcg / month for other diseases, Congenital disorders of folic acid absorption.

Clinic - megaloblastic anemia, decreased intelligence Chlorine-wasting diarrhea is a rare specific congenital disease caused by a defect in the transport of chlorides in the ileum. Clinical manifestations: debilitating diarrhea from the moment of birth as a result of the accumulation of chloride ions in the intestinal lumen.

Diagnosis: diarrhea, hypokalemia, hypochloremia, alkalosis. In other respects, the absorption function of the intestine is not disturbed. Treatment: additionally add potassium to the diet, limit the intake of chlorides.

Enteropathic acrodermatitis is caused by malabsorption of Zn.

Clinic: dermatitis (rash at the transition of the skin into the mucous membrane), there may be alopecia, diarrhea, developmental delay, a decrease in Zn in the blood serum, alkaline phosphatase activity.

Treatment: zinc sulfate 150 mg/day.

Menkes syndrome. Menkes syndrome (curly hair syndrome) is caused by impaired transport of Cu (copper). It is inherited in an autosomal recessive manner.

Clinic: growth retardation, hair anomaly, cerebellar degeneration.

Diagnosis: on the basis of the clinic and a decrease in Cu in the blood serum.

The prognosis is unfavorable.

Whipple syndrome. It almost never occurs in children.

Etiology: believe that rod-shaped microorganisms are affected, including the small intestine.

Clinic: fever, diarrhea, arthralgia, polyserositis.

Diagnosis: a biopsy in the duodenum reveals PAS-positive macrophages, and bacteria can be detected in the mucosa.

Diagnosis methods.

I stage. Identification of the syndrome of malabsorption.

According to clinical signs:

1) weight loss with normal or increased appetite - reduced absorption of fat, protein, carbohydrates;

2) plentiful fetid stools - reduced absorption of fat;

3) muscle weakness, edema - reduced absorption of proteins;

4) flatulence, rumbling in the stomach, copious discharge of gases - the digestion of carbohydrates by the intestinal microflora;

5) parasthesia, bone pain, tetany - decreased absorption of Ca and vitamin D;

6) muscle cramps - excessive loss of K and Mg;

7) hemorrhagic syndrome - decreased absorption of vitamin K;

8) glossitis, stomatitis, cheilitis - deficiency of vitamin B12, folic acid and other B vitamins;

9) acrodermatitis - Zn deficiency.

II stage. Exclusion of infectious genesis of enteropathy.

III stage. Establishment of the type (phase) of indigestion (abdominal, parietal-membrane):

1) in case of violation of abdominal digestion (preenteral mechanisms), identify its causes: dysfunction of the stomach, excretory pancreatic insufficiency, bile deficiency (reduced formation, impaired excretion or reabsorption in the intestine);

2) in case of violation of parietal digestion (enteral mechanisms), the following methods can be used: the timing (age of the patient) of the manifestation of the disease, the relationship of the manifestation of the disease with the characteristics of the child's nutrition, the relationship of deterioration of the stool with the characteristics of the child's nutrition. The prognosis for timely diagnosis and treatment is favorable.

Prevention consists in diet therapy with the exclusion of intolerable foods.

LECTURE No. 13. Differential diagnosis of liver diseases in children. Clinic, diagnosis, treatment

Stage I: inspection.

Anamnesis: the presence of liver damage is established. Laboratory methods: (AlAT, AsAT, alkaline phosphatase, proteinogram, thymol, prothrombin index, urine bilirubin and urobiligen) - diagnosis assumption. Hepatic parenchymal blood clearance with technetium is the only or early sign of the disease (hepatitis, cirrhosis, amyloidosis).

Ultrasound and scintigraphy of the liver with technetium (confirmation of damage, differentiation of focal and diffuse pathology, determination of focal localization).

Stage II: nosological diagnosis (laparoscopy, selective angiography, targeted liver biopsy, latex agglutination test for echinococcosis).

With focal lesions in the depths of the organ - celiacography, selective hepatography (for contrasting the arteries of the liver, portal veins and vessels of the spleen).

Needle biopsy (if a diffuse disease is suspected). Determination of mitochondrial antibodies to liver tissue.

Stage III: specification of the diagnosis (activity of the process, stage of the disease, complications).

Biochemical studies (cholestasis syndrome - increased levels of cholesterol and conjugated bilirubin, alkaline phosphatase activity; cytolysis syndrome - an increase in conjugated bilirubin, transaminase activity; hepatoprival syndrome - a decrease in cholesterol, albumin, procoagulants, fibrinogen levels; mesenchymal inflammatory syndrome - an increase in the content of g-globulins, indicators of diphenylamine and thymol samples, ESR index).

Immunological research.

Classification of hepatomegaly:

1) primary diseases of the hepatic parenchyma (hepatitis, pigmented hepatosis, liver cirrhosis, tumors);

2) metabolic disorders (fatty hepatosis, amyloidosis, hemosiderosis, glycogenosis, lipoidosis);

3) circulatory disorders (stagnation, heart attack in adults);

4) secondary infiltrative processes (acute and chronic infections and intoxications, blood diseases, collagenoses);

5) diseases of the biliary tract (cholangitis, disorders of the outflow of bile).

Idiopathic hemochromatosis - iron deposits in the liver, skin, heart, joints, glands. Autoimmune course of hepatitis, primary biliary cirrhosis - fibrosing alveolitis, thyroiditis, tubulointerstitial nephritis, joint damage.

With viral chronic hepatitis, there are, in addition, glomerulonephritis, polyneuropathy, pulmonary vasculitis, pulmonary granulomatosis, myocarditis, and systemic vasculitis.

Classification of chronic hepatitis.

1. Viral (B, C, D, E, F, G).

2. Autoimmune (Epstein-Barr viruses, cytomegaly, Coxsackie, herpes simplex).

3. Medicinal (tuberculostatics, phenothiazines, poisonous mushrooms, DDT and its analogues).

4. Cryptogenic (etiology not established, possibly viral). Cirrhosis of the liver in childhood is rare, but occupies a significant place among liver diseases in children.

Etiology: acute viral hepatitis (B, C, D), vascular disorders (Buddy-Chiari syndrome and disease), narrowing v. portae - congenital or due to inflammation.

Clinical manifestations: enlarged, bumpy, dense liver with necessarily enlarged spleen. Pronounced systemic disorders, complications (dilation of the veins of the esophagus, stomach, hemorrhoidal veins (collaterals between v. portae and vena cava), morphological - massive inflammation in the portal tracts and hexagonal lobules, nodes - regenerates, fibrosis, degeneration of hepatocytes in combination with their necrosis .

Fundamentals of therapy of chronic diffuse liver diseases.

One of the main mechanisms of liver cell destruction is excessive activation of lipid peroxidation (LPO) and depletion of the antioxidant defense system.

Pharmacological regulation of lipid peroxidation by hepatoprotectors and antioxidants is the most important direction in the treatment of chronic liver diseases.

For viral CKD, antiviral drugs: viferon (recombinant interferon-referon with the addition of antioxidants, interferon-intron A); It is possible with a preliminary short course of prednisolone.

Basic therapy of diffuse liver diseases in children. Basic (non-drug) therapy is traditional and is prescribed to all patients, regardless of the etiology of liver damage. It includes a protective regimen (limitation of physical activity, bed rest during periods of exacerbations), adequate medical nutrition (table 5, during exacerbations - 5a), a complex of multivitamins.

Nonspecific therapy should be as economical as possible, but sufficient. Not performed when the process is not active. Important - prevention of gastrointestinal dysfunction and intestinal autointoxication (appointment of enzymes, eubiotics, laxatives). Hepatoprotectors and antioxidants are used only in sick children with inflammatory activity and hyperenzymemia. Herbal preparations with membrane stabilizing, antitoxic and choleretic effects (karsil, legalon, hepatofalk, LIV-52, galstena, hepabene, tykveol, hofitol, heptral, silimar, tanacehol, etc.). Relief of cholestasis - adsorbents (cholestyramine, bilignin, polyfepam), heptral, ursodeoxycholic acid preparations (ursofalk, ursosan), hemo- and plasmasorption. With severe cytolysis and impaired protein-synthetic and detoxification functions of the liver - intravenous administration of detoxification agents (polyionic buffer solutions, 5% glucose solution), protein preparations (albumin, plasma, freshly heparinized blood, coagulation factors ), solutions of amino acids (alvezin, aminofuzin, hepasteril, aminosteril); methods of extracorporeal detoxification.

LECTURE No. 14. Drug-induced disease in children

Complications are divided into:

1) medication, which are divided into true side effects of drugs;

2) toxic effects of drugs;

3) complications associated with the sudden withdrawal of the drug;

4) individual intolerance to the drug.

Side effects of drugs: an undesirable effect of a drug, due to its structure and properties, which it has on the body along with its main actions. The toxic effects of drugs can be due to: overdose, rapid saturation of the body, rapid administration of medium and even minimal doses, insufficient excretion function of the body, impaired drug detoxification processes in the body (with primary liver failure).

Complications due to rapid withdrawal of drugs: withdrawal syndrome, withdrawal symptoms, exacerbation of those symptoms for which treatment was carried out. Individual intolerance to drugs is divided into unusual and perverted reaction. An unusual reaction of the body to normal doses of drugs that are harmless to most people. Individual intolerance is a disease of altered reactivity, a disease of the body. Individual intolerance includes idiosyncrasy, an allergic reaction. Idiosyncrasy is a genetically determined, idiosyncratic response to a particular drug when first taken. The cause of idiosyncrasy is an insufficient amount or low activity of enzymes (for example, a lack of the enzyme glucose phosphate DG in response to taking certain drugs, quinidine, CA drugs, aspirin, pyrazalones, antibiotics leads to the development of hemolytic anemia).

Factors in the development of drug disease are as follows.

1. Uncontrolled use of drugs by both doctors and patients themselves; the presence of the underlying disease changes the reactivity of the organism, and the altered reactivity manifests itself in unexpected effects when using drugs.

2. Polypharmacy, creating conditions for polyvalent sensitization; malnutrition during the use of drugs can change the reactivity of the body and the tolerance of drugs.

3. Age-related decrease in the participation of enzyme systems in the breakdown and neutralization of certain substances (higher sensitivity of children to barbiturates and salicylates, in the elderly - to SG).

4. Genetic conditionality of a number of drug-induced lesions.

5. The degree and rate of sensitization of the body partly depends on the routes of administration of drugs (local applications and inhalations lead to increased sensitivity of the body; with intravenous administration of drugs, sensitization of the body is less than with intramuscular and intramuscular injections).

Allergic reactions are the most common cause of intolerance to certain drugs.

Allergy is understood as an altered reactivity of the organism to the action of a given substance due to the hereditary high sensitivity of the organism.

A drug disease is one of the most significant clinical forms of an allergic reaction of the body to drugs.

Necessary steps for the development of drug allergies:

1) the transformation of the drug into a form that is able to interact with proteins;

2) the transformation of the drug into a form that can react with body proteins to form a complete antigen;

3) the body's immune response to this formed complex, which has become foreign, in the form of antibody synthesis through the formation of immunoglobulins.

Stages of allergic manifestations: preimmunological - is the formation of complete (complete) allergens (antigens). Immunological, when the antigen-antibody reaction occurs in the tissues of the shock organs. An antigen-antibody reaction is a specific reaction that is caused only by the introduction of a specific allergen.

Patochemical reaction - as a result of the formation of an antigen-antibody complex, biologically active substances (histamine, heparin, serotonin, etc.) are released, the reaction is nonspecific. The pathophysiological reaction is manifested by the action of biologically active substances on various organs and tissues. Classification of allergic reactions.

1. An immediate type reaction is associated with the presence of circulating antibodies in the blood. This reaction occurs 30-60 minutes after the administration of the drug and after that it is characterized by acute manifestations: local leukocytosis, eosinophilia in the blood test.

2. Delayed-type reaction due to the presence of antibodies in tissues and organs, accompanied by local lymphocytosis, occurs 1-2 days after taking the drug.

Classification of allergic reactions according to pathogenetic type.

1. True (allergic) reactions are divided into chimergic (B-dependent) and kitergic (T-dependent):

1) chimergic allergic reactions are caused by the reaction of an antigen with antibodies, the formation of which is associated with B-lymphocytes;

2) kytergic allergic reactions with allergen binding by sensitized lymphocytes.

2. False (pseudo-allergic, non-immunological) reactions - do not have an immunological stage in their development.

Classification of medicinal disease.

1. Acute forms: anaphylactic shock, bronchial asthma, angioedema, vasomotor rhinitis, acute hemolytic anemia.

2. Prolonged forms: serum sickness, Lyell's syndrome, drug-induced vasculitis, etc.

3. Light (itching, angioedema, urticaria), in which the symptoms disappear 3 days after the use of antihistamines; moderate severity (urticaria, eczematous dermatitis, erythema multiforme, fever up to 39 ° C, poly- or monoarthritis, toxic-allergic myocarditis). Symptoms disappear after 4-5 days, but require the appointment of GC in average doses of 20-40 mg.

4. Severe form (anaphylactic shock, exfoliative dermatitis, Lyell's syndrome), damage to internal organs (myocarditis with rhythm disorders, nephrotic syndrome). All symptoms disappear after 7-10 days of combined administration of GCs, immunomodulators and antihistamines.

Diagnosis of drug disease: carefully collected allergic anamnesis. It should be borne in mind that many patients do not take drugs that they use daily (sedatives, laxatives, analgesics, eye drops, nasal drops) as medicines.

The essence of elimination tests is the abolition of absolutely all drugs.

Allergic skin tests (in / to, scarification, application) give a sharply positive reaction with certain drug allergens.

Provocative tests (nasal, inhalation, conjunctival). Basophilic test. The hemagglucination reaction consists in agglutination of erythrocytes loaded with an allergen by the patient's serum. RBTL (lymphocyte blast transformation reaction) is used to diagnose a delayed-type allergic reaction. The patient's lymphocytes are mixed with a possible allergen. After many days of incubation, the degree of transformation of lymphocytes is assessed according to morphological criteria or DNA or RNA synthesis using the isotopic label of Lyell et al.

Treatment of drug disease: bed rest; diet is not irritating, with sufficient fluid intake; cancellation of all medications; desensitizing therapy (calcium chloride, antihistamines, calcium, glucocorticoids); specific hyposensitization is ineffective; not performed for pancytopenia; symptomatic therapy.

Treatment of anaphylactic shock.

1. S / c to enter 0,5-1 ml of 0,1% solution of adrenaline.

2. Prevention of aspiration of vomit.

3. Introduce 10,0 ml of a 10% solution of Ca chloride or 10,0 ml of a 10% solution of Ca gluconate intravenously.

4. In / in the stream, then drip 300-500 ml of a 5% glucose solution or saline + 0,5-1 ml of a 0,1% solution of adrenaline or 1,0 ml of a 1% solution of mezaton with HA.

5. With bronchospasm - 10 ml of a 2,4% solution of eufillin, novocaine blockade.

6. With laryngeal edema - tracheostomy, humidified oxygen

7. Antihistamines (suprastin 2% - 2,0, tave-gil 0,1% - 1,0, diphenhydramine 1% - 1,0).

8. Cardiac glycosides.

9. In anaphylactic shock of penicillin etiology - up to 1 million units of penicillinase, again - after 6-8 hours.

10. Resuscitation (artificial ventilation of the lungs, closed heart massage in case of cessation of respiratory and cardiac activity).

LECTURE No. 15. Helminthiasis in children. Clinic, diagnosis, treatment, prevention

Helminthiases are diseases that develop when parasitic helminth worms and their larvae are localized in the body. Classification of helminthiases:

1) according to the biological principle: nematodes (roundworms), cestodoses (tape), trematodes (flukes);

2) according to epidemiological: geohelminthiases, biohelminthiases, bontactic.

1. Ascariasis

The causative agent is a roundworm that parasitizes in the adult stage in the small intestine. The life span of Ascaris is about a year. In the migration stage (the first 6-8 weeks after infection), Ascaris larvae have a mechanical and sensitizing effect, causing eosinophilic infiltrates in the tissues of various organs and causing hemorrhages. In the intestinal phase (8 weeks after the lesion), adult roundworms cause toxic-allergic and neuroreflex reactions of the body, a variety of local mechanical effects.

Clinic. The migratory phase often proceeds under the guise of acute respiratory infections, bronchitis (malaise, dry cough or scanty sputum, subfebrile temperature, dry and moist rales in the lungs). Urticaria, vesicular rash on the hands and feet are possible, and volatile infiltrates in the lungs may occur.

In the intestinal phase, the gastrointestinal form is distinguished, manifested by salivation, nausea, loss of appetite, cramping pains around the navel, sometimes disorder of the stool and gastric secretion; hypotonic form, manifested by a decrease in blood pressure, weakness; neurological form, manifested by dizziness, headache, fatigue, sleep disturbance, vegetative-vascular disorders.

Complications. Ascariasis ileus, ascariasis appendicitis; perforative peritonitis; ascariasis of the liver with the occurrence of jaundice, subdiaphragmatic abscess; ascariasis of the pancreas with clinical manifestations of acute pancreatitis, creeping of ascaris into the respiratory tract with the development of asphyxia.

Diagnosis. Based on laboratory data, it is based on the detection of nematode larvae in sputum, antibodies in the blood, and the late intestinal phase of roundworm eggs in feces.

Treatment. To expel young individuals and adult roundworms, piperazine, levamisole, and combantrin are used. Piperazine is prescribed after meals 2 times a day, the interval between taking the drug is 2-3 hours, for 2 days, the recommended dose is 1,5-2 g per dose (3-4 g / day). Efficacy is enhanced when piperazine is taken after dinner before bed. Decaris (levamisole) is prescribed after a meal at a dose of 150 mg once, pyrantel is prescribed once after a meal at a rate of 10 mg/kg of body weight.

Oxygen treatment is carried out on an empty stomach or 3-4 hours after a meal, preferably in the morning, 2-3 days in a row.

Forecast and prevention. The prognosis in the absence of complications that require surgical treatment is favorable.

Prevention: mass examination of the population and treatment of all persons infested with ascariasis. Protection of the soil of kitchen gardens, orchards, berry fields from contamination with feces. Thorough washing and scalding vegetables and fruits with boiling water. Personal hygiene measures.

2. Alveococcosis

Etiology and pathogenesis. The causative agent is the larval stage of alveococcus. Human infection occurs after oncospheres enter the oral cavity through contact with contaminated skins of arctic foxes, dogs, foxes, through the water of stagnant reservoirs when eating wild berries collected in an epidemic area. The larvae usually accumulate in the liver, infiltrate and grow into the tissues, disrupting the blood supply to the organs, causing tissue degeneration and atrophy.

Clinic. For a long time remains asymptomatic, but there is a progressive enlargement of the liver, heaviness and pressure in the right hypochondrium appear, dull aching pain appears. A few years later, a tuberous and very dense liver can be palpated. Jaundice may develop, sometimes the spleen is enlarged. With the disintegration of the nodes, the body temperature rises, sweating is observed.

Diagnosis. Based on laboratory data, leukocytosis, eosinophilia, increased ESR, hyperproteinemia, hypergammaglobulinemia. Use serological reactions with alveococcal antigen. In order to clarify the localization of the process, X-ray and ultrasound examinations, liver scans, and computed tomography are used. Trial puncture is prohibited due to the risk of contamination of other organs. Differentiate from tumors, echinococcosis and cirrhosis of the liver.

Treatment. Treatment is surgical and symptomatic.

3. Ankylostomiasis (ankylostomiasis and necatoriasis)

Etiology, pathogenesis. The causative agents - hookworm and necator, parasitize in the human small intestine, more often in the duodenum. Infection occurs when the larvae penetrate the skin or when the larvae are swallowed with contaminated fruits, vegetables, and water. The larvae migrate through the systemic and pulmonary circulation for about 7-10 days. In the small intestine, they turn into sexually mature individuals and after 4-6 weeks begin to lay eggs. The life expectancy of ankylostomids ranges from a few months to 20 years. During the migration period, they can cause toxic-allergic reactions. Adult helminths are hematophagous. When fixing to the intestinal mucosa, they injure the mucosa and tissues, which leads to the formation of hemorrhage, cause bleeding, contribute to the development of anemia, maintain at a certain level the state of allergy, dyskinesia of the gastrointestinal tract and dyspepsia.

Clinic. Skin itching and burning, asthmatic phenomena, fever, eosinophilia in the blood test. In the late stage, nausea, salivation, abdominal pain, vomiting, bowel dysfunction (constipation or diarrhea), and bloating appear.

The diagnosis is confirmed by the detection of eggs in the feces, and sometimes in the duodenal contents.

Treatment. Deworming is carried out with combantrin or levamisole. With severe anemia (hemoglobin below 67 g / l), iron preparations, red blood cell transfusions are prescribed.

Forecast and prevention. The prognosis is favorable in most cases.

Prevention: in the foci of ankylostomiasis, you can not walk barefoot, lie on the ground without bedding. It is necessary to thoroughly wash and scald fruits, berries, vegetables with boiling water before eating them, you can not drink unboiled water.

4. Diphyllobothriasis

Etiology, pathogenesis. The causative agent is a wide tapeworm. The duration of its life is tens of years. Human infection with diphyllobothriasis occurs when eating fresh or lightly salted caviar and raw fish (pike, perch, omul, etc.). The tapeworm is attached to the intestinal mucosa with its bothria and injures it. A large accumulation of parasites can clog the intestinal lumen. The metabolic products of the helminth sensitize in the body.

Clinic. Characterized by nausea, weakness, dizziness, abdominal pain, unstable stool, excretion of scraps of strobilus during defecation.

The diagnosis is confirmed by the detection of lentelets eggs and strobila fragments in the feces.

Treatment. In case of severe anemia, before helminthization, vitamin B is prescribed at 300-500 mcg intramuscularly 2-3 times in 7 days for a month, hemostimulin, preparations containing iron, hematogen. For deworming, fenasal, male fern extract, and a decoction of pumpkin seeds are prescribed.

Forecast and prevention. The prognosis in the absence of complications is favorable.

Do not eat raw, poorly cooked or insufficiently dried and salted fish, as well as "live" pike caviar.

5. Opisthorchiasis

Etiology, pathogenesis. The causative agent is the cat fluke, which parasitizes the bile ducts of the liver, pancreas, and gallbladder in humans; it can also parasitize dogs and cats. The parasite can live in the human body for about 20-40 years. Human infection occurs when eating raw (frozen), lightly salted and insufficiently fried fish of carp species (ide, chebak, dace, etc.). Opisthorchises injure the mucous membranes of the pancreatic and bile ducts, which creates an obstruction to the outflow of bile and contributes to the occurrence of cystic enlargements and liver tumors. They have toxic and neuro-reflex effects.

Clinic. The incubation period is about 2 weeks. In the early period, there may be fever, pain in the muscles and joints, disorders of the gastrointestinal tract in the form of vomiting and diarrhea, pain and enlargement of the liver on palpation, less often - the spleen, leukocytosis and high eosinophilia in the blood test, allergic skin rashes. With the development of the chronic stage, the patient complains of pain in the epigastric region, right hypochondrium, pain attacks like gallbladder colic. Dizziness and the development of other dyspeptic disorders may appear. Muscle resistance in the right hypochondrium, liver enlargement, sclera icterus, enlargement of the gallbladder, symptoms of pancreatic damage are detected. Most often, with opisthorchiasis, the phenomena of cholecystitis, biliary dyskinesia, chronic pancreatitis and hepatitis develop, rarely symptoms of gastroduodenitis, enterocolitis. Opisthorchiasis may be asymptomatic.

The diagnosis is made upon detection of helminth eggs in the feces and duodenal contents.

Treatment. Deworming is carried out with mebendazole (ver-mox).

Prevention: it is imperative to explain to the population the danger of eating raw, thawed and frozen (stroganina), lightly salted and undercooked fish.

6. Teniasis

Etiology. The causative agent - tapeworm pork, can parasitize in humans in the sexually mature stage, in the larval stage, causes the disease cysticercosis. The adult helminth parasitizes in the small intestine for many years. Infection with human teniasis occurs when eating raw or half-cooked meat containing Finns.

The diagnosis is made on the basis of repeated stool examination to detect helminth segments from the perianal folds by scraping for tapeworm eggs.

Treatment. Vermox. Sometimes an essential extract of male fern and pumpkin seeds are used.

Prevention. You can not eat undercooked and undercooked pork.

7. Trichuriasis

Etiology, pathogenesis. The causative agent - whipworm, parasitizes in the human large intestine. The life span of the whipworm parasite is about 5 years. Vlasoglav injures the intestinal mucosa and is a hematophagus, which contributes to the inoculation of microflora, vlasoglav causes reflex reactions in other organs of the abdominal cavity. The products of their metabolism sensitize in the body.

Clinic. The patient is disturbed by salivation, a decrease (less often an increase) in appetite, pain appears in the right half of the abdomen and epigastric region, nausea, constipation or diarrhea, headache, restless sleep, dizziness, irritability are sometimes noted, moderate hypochromic anemia and slight leukocytosis. With a low intensity, whipworm invasion is not clinically manifested. The diagnosis is made when whipworm eggs are found in the feces. Treatment. Prescribe anthelmintic therapy (mebendazole and other drugs). Previously, the patient is given a cleansing enema.

Forecast. The prognosis is favorable.

8. Fascioliasis

Etiology and pathogenesis. The causative agent of fascioliasis is the liver and giant flukes. The main source of human infection are various farm animals. Infection of people often occurs in the warm season when fasciol larvae are swallowed with water, sorrel and any other herbs.

The life expectancy of helminths in the human body is about 10 years. Of particular importance is trauma and toxic-allergic damage to the hepatobiliary system. But it is possible to carry fasciols into other tissues and organs.

Clinic. The disease is determined by a blood test with eosinophilia, allergic manifestations, disorders of the liver and gallbladder, which resemble the symptoms of opisthorchiasis (jaundice, attacks of gallbladder colic are more common).

The diagnosis of the early stage of fascioliasis is difficult due to the fact that helminth eggs are released only 3-4 months after infection. Immunological methods can be used. In the late stage, the diagnosis is established on the basis of the detection of fasciol eggs in the duodenal contents and feces.

Treatment. Anthelmintic drugs are prescribed, and after deworming it is necessary to prescribe choleretic drugs for 1-2 months. Long-term medical examination of patients is carried out.

Forecast and prevention. The prognosis for treatment is favorable.

Prevention consists in the fact that it is necessary to prohibit the use of water from stagnant reservoirs, recommend thorough washing and scalding greens with boiling water.

9. Echinococcosis

Etiology. The causative agent of hydatous echinococcosis is the larval stage of a small cestode that has a scolex with 4 suckers and hooks and 3-4 proglotids filled with eggs. The larva is a single-chamber bubble, the wall of which consists of two layers of cells, outer and inner, which form small parietal protrusions. The bubble cavity is filled with liquid. Echinococcus eggs are highly stable in the external environment, withstand drying and exposure to low temperatures.

Epidemiology. It is widespread throughout the world, infection of the population is very widespread, shepherds, hunters and persons who have constant contact with the final owners of echinococcus are more likely to get sick. Reservoir and source of invasion: the final hosts are carnivores, domestic animals (dog, fox, wolf), in which a mature worm parasitizes in the intestines; its segments containing eggs are excreted with feces into the external environment. Intermediate hosts are herbivores and omnivores (sheep, goats, pigs, horses, rodents).

The mechanism of transmission of invasion: fecal-oral (as a result of ingestion of invasive echinococcus eggs upon contact with dogs, sheep, on the wool of which there may be helminth eggs), the route of transmission is food, water, household.

Pathogenesis. When a person swallows eggs of echinococcus in the stomach and intestines, they are released from the oncosphere, penetrate the intestinal wall into the bloodstream, then into the liver, where the larval stage of echinococcosis is formed. The growing bubble compresses the surrounding tissues, lung, bronchi, blood vessels and involves the pleura in the pathological process with the appearance of symptoms of volumetric formation. The death of the parasite leads to the attachment of a bacterial infection and the formation of a lung abscess.

Clinic. Pain in the chest of a different nature, dry cough, then with purulent sputum, hemoptysis, shortness of breath. If the bladder breaks into the bronchus, there is a strong cough, cyanosis, suffocation, the contents of the bladder can be found in the sputum. When echinococcal blisters fester, a lung abscess develops. With liver echinococcosis, patients lose their appetite, weakness, weight loss, headaches, decreased performance, a feeling of heaviness in the epigastrium appear. Pain in the right hypochondrium, liver enlargement, induration and tenderness on palpation, nausea, vomiting, upset stool. In rare cases, subectericity of the skin and the appearance of jaundice.

Diagnostics. Based on clinical and laboratory data using serological reactions (RSC, RNGA, latex agglutination reaction with antigen from echinococcal bladder fluid), additional research methods, chest X-ray, computed tomography of the lungs, ultrasound of the lungs.

Treatment. Usually by surgery.

Prevention. Prevention of infection of animals and humans, observance of personal hygiene rules, periodic helminthological examination of dogs and timely deworming of infected animals and humans. Information from medical and veterinary institutions is of particular importance.

10. Enterobiasis

Etiology. The causative agent is a female pinworm 9-12 cm long, males - 3-4 cm. Males die after fertilization, females come out of the anus and begin to lay eggs on the perianal region and perineum. Infection occurs as a result of ingestion of invasive eggs. Possible auto-invasion. In the upper small intestine, infective larvae leave the egg shells and reach sexual maturity in the large intestine. Pinworms stick to the intestinal mucosa and penetrate to the muscle layer, producing toxins.

Clinic. With a slight invasion, complaints may be absent. There is itching around the anus, scratching, infection, rapid stools with pathological impurities, symptoms of intoxication, vulvovaginitis in girls.

Diagnostics. Based on the detection of pinworm eggs in feces or by scraping on pinworm eggs. In the blood - eosinophilia.

Treatment. Mebendazole (Vermox) from 2 to 10 years 25-50 mg/kg once, pyrantel (Combantrin) - 10 mg/kg once after breakfast, chew, piperazine up to 1 year 0,2 x 2 times 5 days; 2-3 years - 0,3; 4-5 years - 0,5; 6-8 years - 0,5; 9-12 years - 1,0; 13-15 years old - 1,5.

Prevention. Compliance with personal hygiene.

LECTURE No. 16. Rheumatism in children and adolescents. Clinic, diagnosis, treatment

Rheumatism is a systemic inflammatory disease of the connective tissue with a characteristic lesion of the heart.

Etiology, pathogenesis. The main etiological factor in acute forms of the disease is group A b-hemolytic streptococcus. In patients with prolonged and continuously recurrent forms of rheumatic heart disease, it is often not possible to establish a connection between the disease and streptococcus. In the development of rheumatism, immune disorders are of particular importance.

It is assumed that sensitizing agents in the body (streptococcus, viruses, nonspecific antigens, etc.) can lead at the first stages to the development of immune inflammation in the heart, and then to a violation of the antigenic properties of its components with their transformation into autoantigens and the development of an autoimmune process. A special role in the development of rheumatism plays a genetic predisposition.

Classification. It is necessary to identify a previously inactive or active phase of the disease.

Activity can be minimal (I degree), medium (II degree) and maximum (III degree). To determine the degree of activity, the severity of clinical manifestations, as well as changes in laboratory parameters, are used.

Classification according to the localization of the activity of the rheumatic process (carditis, arthritis, chorea, etc.), the state of the blood circulation and the course of the disease.

Allocate an acute course of rheumatism, a subacute course, a protracted course, a continuously relapsing course and a latent course of the disease. The allocation of a latent course is justified only for the retrospective characteristics of rheumatism, the latent formation of heart disease, etc.

Clinic. Most often, the disease develops 1-3 weeks after suffering a sore throat, sometimes another infection. With relapses, this period may be shorter. Relapses of the disease often develop after any intercurrent diseases, surgical interventions, physical overload. A manifestation of rheumatism is a combination of acute migratory and completely reversible polyarthritis of large joints with moderately severe carditis. The onset of the disease is acute, stormy, rarely subacute. Polyarthritis develops rapidly, accompanied by remitting fever up to 38-40 0 C with daily fluctuations of 1-2 0 C, severe sweating, but more often without chills.

The first symptom of rheumatic polyarthritis is acute pain in the joints, increasing and intensifying with the slightest passive and active movements. The pain is accompanied by swelling of the soft tissues in the area of ​​​​the joints and at the same time an effusion appears in the joint cavity. The skin over the affected joint is hot, there is sharp pain on palpation of the joint, the range of motion is limited due to pain.

A characteristic feature is a symmetrical lesion of large joints - more often knee, wrist, ankle, elbow. The "volatility" of inflammatory changes is typical, manifested in the rapid and reverse development of arthritic manifestations in some joints and the same rapid increase in other joints. All articular changes disappear without a trace even without treatment, they last no more than 2-4 weeks.

Rheumatic myocarditis, if there is no concomitant defect, is not severe, with complaints of mild pain or unpleasant, vague sensations in the region of the heart, slight shortness of breath during exertion, rarely - complaints of interruptions in the work of the heart, palpitations. With percussion, the heart is of normal size or moderately enlarged to the left, with auscultation and FCG, a satisfactory sonority of tones is characteristic, a slight muffling of 1 tone, sometimes 3 tone is recorded, rarely 4 tone, soft muscle systolic murmur at the apex of the heart and projections of the mitral valve. Blood pressure is normal or moderately low. On the ECG - flattening, widening and serration of the P wave and the QRS complex, rarely there can be an elongation of the PQ interval for more than 0,2 s, in some patients a slight shift of the S-T interval downwards from the isoelectric line and a change in the T wave becomes low, negative, rarely biphasic (primarily in leads V1-V3). Rarely, extrasystoles, atrioventricular blockade of the 2nd or 3rd degree, intraventricular blockade, nodal rhythm appear.

Diffuse rheumatic myocarditis is manifested by significant inflammation of the myocardium with its pronounced edema and, consequently, impaired function. From the onset of the disease, the patient is worried about severe shortness of breath, which forces him to take the orthopnea position, there is constant pain in the cardiac region, and palpitations. Characterized by "pale cyanosis", swelling of the cervical veins. The heart is diffusely dilated, weak apex beat. The tones are sharply muffled, very often a clear III tone (protodiastolic gallop rhythm) and a distinct, but significantly soft systolic murmur are heard. The pulse quickens, weak filling. Arterial pressure is lowered. Venous pressure rises rapidly, but in combination with collapse also decreases. On the ECG, a decrease in the voltage of all teeth, flattening of the T wave, a change in the S-T interval, and atrioventricular blockade are recorded. The outcome of rheumatic myocarditis in the absence of adequate treatment may be myocardial cardiosclerosis, which often characterizes the degree of prevalence of myocarditis. With focal cardiosclerosis, myocardial functions are not disturbed. Diffuse myocarditis cardiosclerosis is characterized by signs of a decrease in the contractile function of the myocardium, which is manifested by a weakening of the apex beat, muffled tones (especially I), and systolic murmur. Rheumatic endocarditis, which is the cause of rheumatic heart disease, has very few clinical symptoms.

An essential symptom during auscultation is a clear systolic murmur with sufficient sonority of tones and the absence of signs of severe myocardial damage. In contrast to the murmur associated with myocarditis, endocardial murmur is coarse, but sometimes can have a musical tone. The sonority of endocardial murmur increases with a change in the patient's posture or after exercise.

Reliable signs of endocarditis are the variability of existing noises and especially the emergence of new ones with unchanged heart boundaries. Diastolic murmurs disappear easily and quickly, are sometimes heard at the very beginning of a rheumatic attack on the projection of the mitral valve, as well as on the vessels, can also be partly associated with endocarditis. Deep endocarditis of the leaflets or aortic valve in some patients is reflected on the echocardiogram: thickening of the leaflets, their shaggyness", multiple echoes from them. Pericarditis in the clinic of rheumatism is rare.

Dry pericarditis is clinically manifested by constant pain in the region of the heart and a pericardial friction rub, which is more often heard along the left edge of the sternum. The intensity of the noise during auscultation is different, often it is determined in both phases of the cardiac cycle. The ECG reveals an upward shift in the S-T interval in all leads at the very beginning of the disease. With further development, these intervals return to the isoelectric line, and biphasic or negative T waves are also formed simultaneously. Dry pericarditis itself is not capable of causing an increase in the heart.

Exudative pericarditis is a further stage in the development of dry pericarditis. The main first clinical sign of the appearance of effusion is the disappearance of pain due to the separation of the inflammatory sheets of the pericardium, accumulating exudate.

Clinical manifestations include shortness of breath, which worsens when the patient lies down. The area of ​​the heart with a large amount of exudate swells, the intercostal spaces are smoothed, the apex beat is not palpable. The heart is significantly enlarged and takes the shape of a trapezoid or round graphite. The pulsation of the contours during fluoroscopy is small. On auscultation, the tones and noises are dull (as there is effusion). The pulse is frequent, small in filling; blood pressure is reduced. Venous pressure is always increased, swelling of the cervical and peripheral veins appears. The electrocardiogram is the same as for dry pericarditis; an additional symptom may be a noticeable decrease in the voltage of the QRS complex. Echocardiography, which determines the presence of fluid in the heart sac, is of particular diagnostic importance. When the skin is affected, annular erythema is practically characteristic, which is pink ring-shaped elements that never itch and are located mainly on the skin of the inner surface of the arms and legs, as well as the abdomen, neck, and torso. It is found in only 1-2% of patients. The “rheumatic nodules” described in the old manuals are now almost never encountered. Erythema nodosum, hemorrhages, and urticaria are also not typical. With kidney damage, mild proteinuria and hematuria are detected (due to generalized vasculitis and damage to the renal glomeruli and tubules). Damage to the nervous system and sensory organs. Lesser chorea, the most typical “nervous form” of rheumatism, is observed mainly in children, especially girls. Minor chorea is characterized by a combination of emotional lability with muscle hypotonia and violent movements of the torso, facial muscles and limbs. Minor chorea occurs with relapses, but by the age of 17-18 it almost always ends. A feature of this form may be relatively minor damage to the heart, as well as slightly expressed laboratory indicators of the activity of rheumatism.

Diagnosis: based on anamnesis, clinical and laboratory data. In the blood test, neutrophilic leukocytosis with a shift to the left, thrombocytosis, an increase in ESR up to 40-60 mm/h. An increase in titers of antistreptococcal antibodies is characteristic: antistreptohyapuronidase and antistreptokinase more than 1: 300, antistreptolysin more than 1: 250. The height of titers of antistreptococcal antibodies and their dynamics does not show the degree of activity of rheumatism. In a biochemical study, an increase in the level of plasma fibrinogen above 4 g / l, globulins above 10%, g-globulins - above 20%, seromucoid - above 0,16 g / l, the appearance of C-reactive protein in the blood test. In many cases, biochemical indicators of activity are parallel to the ESR value. Allocate large diagnostic criteria for rheumatism: polyarthritis, carditis, annular erythema, chorea, rheumatic nodules. Allocate small diagnostic criteria for rheumatic fever, arthralgia, past rheumatic fever, the presence of rheumatic heart disease, elevated ESR, a positive reaction to C-reactive protein, prolongation of the P-Q interval on the ECG.

The diagnosis can be considered certain if the patient has two major diagnostic criteria and one minor diagnostic criterion, or one major and two minor diagnostic criteria, but only if both of the following evidence exist simultaneously, it is possible to judge a previous streptococcal infection: scarlet fever (which is an indisputable streptococcal disease); sowing group A streptococcus from the mucous membrane of the pharynx; increased titer of antistreptolysin O or other streptococcal antibodies.

Treatment. Stay in bed for 3 weeks or more. The diet shows the restriction of salt, carbohydrates, sufficient intake of proteins and vitamins. Exclusion of products that cause allergization. Antibacterial therapy of benzylpenicillin, sodium salt is used for 2 weeks, then long-acting drugs - bicillin-5, with intolerance to penicillins - replacement with cephalosporins, macrolides. Prescribe vitamin therapy, potassium preparations. Pathogenetic therapy: glucocorticoids, prednisolone. Non-steroidal anti-inflammatory drugs (indomethacin, voltaren). Aminoquinoline preparations (rezokhin, delagil) - with a sluggish, protracted and chronic course. Immunosuppressants are rarely used. Symptomatic therapy of heart failure is carried out. When indicated, diuretic therapy is prescribed. Antirheumatic drugs have practically no effect on the manifestation of chorea minor. In these cases, it is recommended to add luminal or other psychotropic drugs such as chlorpromazine or seduxen to ongoing therapy. Of great importance for the management of patients with minor chorea is a calm environment, a positive attitude of others, instilling confidence in the patient in full recovery. In necessary cases, it is required to take measures to prevent self-harm of the patient due to violent movements.

Treatment in a hospital is 1,5-2 months, then treatment in a local sanatorium for 2-3 months, where chronic foci of infection are treated and follow-up with a local pediatrician and cardio-rheumatologist.

Prevention: primary correct treatment of streptococcal infection, sanitation of foci of chronic infection, rational nutrition. Secondary prevention includes bicillin-drug prophylaxis for all patients, regardless of age and the presence or absence of heart disease, who have undergone a significant rheumatic process. The prognosis is favorable.

LECTURE No. 17. Broncho-obstructive syndrome. Clinic, diagnosis, treatment. Respiratory failure. Clinic, diagnosis, treatment

Broncho-obstructive syndrome is a clinical symptom complex observed in patients with a generalized obstruction of the bronchial tract, its leading manifestation is expiratory dyspnea, asthma attacks. Diseases associated with airway obstruction.

Major causes of airway obstruction in children.

1. Upper airway obstructions:

1) acquired:

a) allergic rhinitis;

b) nasal polyps;

c) hypertrophy of the tonsils;

d) inflammation of the epiglottis;

e) viral laryngotracheitis;

f) laryngospasm (with spasmophilia);

g) foreign body;

h) congenital stridor;

i) retraction of the tongue in an unconscious state; j) mechanical compression of the trachea and bronchi;

2) congenital:

a) thymomegaly;

b) enlarged lymph nodes;

c) tumor.

2. Obstruction of large intrathoracic airways:

1) narrowing of the lumen (developmental anomaly, tumor, scar, foreign body);

2) compression from the outside (tumor, abnormal vessel);

3) excessive collapse due to weakness of the cartilaginous rings and (or) the membranous part (tracheomalacia). III. Obstruction of the lower respiratory tract:

1) viral bronchiolitis;

2) bronchial asthma;

3) aspiration of vomit;

4) foreign bodies;

5) cystic fibrosis;

6) a₁ -antitrypsin deficiency. Mechanisms of disorders in obstructive syndrome.

1. Reversible:

1) inflammatory edema and mucosal infiltration and submucosal edema;

2) violation of mucociliary transport, obturation of the bronchial lumen with a viscous secret;

3) bronchospasm.

2. Irreversible:

1) fibroplastic changes in the walls of the bronchi.

2) stenosis, deformation and obliteration of the bronchial lumen.

3) expiratory collapse of the bronchi, the presence of emphysema.

Protective mechanisms of the respiratory apparatus.

1. Mechanical.

2. Biochemical.

3. Immunological.

Mechanical protective system of the breathing apparatus:

1) aerodynamic mechanism;

2) mucociliary escalator mechanism;

3) kinetic energy of exhaled air;

4) cough push, biochemical protective system of the respiratory apparatus;

5) bronchial secretion of the respiratory apparatus (sialomucins, fucomucins, glucosaminoglycans, etc.);

6) phospholipids of cell membranes of bronchi, alveoli, surfactant;

7) BAS (serotonin, histamine, etc.).

Immunological defense system of the respiratory apparatus.

1. Specific:

1) secretory IgA;

2) plasma IgM, G, E.

2. Non-specific:

1) alveolar macrophages;

2) lysozyme;

3) kallikrein;

4) lactoferrin;

5) interferon;

6) b-lysine.

1. Acute bronchitis

Acute bronchitis is a common disease: there are 1000-200 cases per 250 children of the first years of life.

Etiology. The vast majority of bronchitis are viral diseases. Respiratory syncytial virus - 50%, parainfluenza viruses - 21%, mycoplasma pneumoniae - 8,3%, cytomegalovirus - 6,3%, rhinoviruses - 4,2%, coronaviruses - 4,1%, echoviruses I serotypes - 2%, influenza A virus - 2%, adenoviruses - 2% Bacterial agents are rare or play no role in the etiology of bronchitis. The bacterial flora is more often found in "non-whistlers" than in "wheezers".

Clinic. Clinical symptoms of bronchial obstruction against the background of SARS in young children:

1) acute onset of the disease;

2) wheezing;

3) variability of dry and wet rales;

4) swelling of the chest;

5) shortness of breath (reaches 60-80 in 1 min);

6) retraction of the jugular fossa and intercostal spaces (hypoxemia);

7) low body temperature. In addition to the main symptoms, there may be:

1) rhinitis;

2) frequent painful cough;

3) swelling of the wings of the nose (hypoxemia);

4) refusal of the breast;

5) loss of appetite;

6) the presence of small crepitant rales, often diffuse;

7) stool disorder;

8) poor sleep;

9) cyanosis (hypoxemia);

10) apnea (hypoxemia).

Laboratory data. Blood test: red blood - no features, accelerated ESR, leukocytosis. X-ray data are characterized by an increase in the transparency of the lung fields, an increase in the anteroposterior diameter of the chest due to overflow of the lungs with air, emphysema, high standing of the dome of the diaphragm, and basal infiltration. In almost 1/3 of patients, scattered areas of seals are visible, which can be explained by the development of atelectasis in response to obstruction. In about 44% of cases, the radiological picture remains normal. Sowing discharge from the nose and trachea is the usual flora.

Virological examination - by the method of immunofluorescence, an increase in the titer of antibodies in the blood.

2. Respiratory failure

Respiratory (ventilatory-pulmonary) insufficiency is characterized by such disorders in which pulmonary gas exchange is impaired or occurs at the cost of excessive energy costs.

Types of respiratory failure:

1) ventilation;

2) distribution-diffusion (shunt-diffusion, hypoxemic);

3) mechanical.

Clinic.

I degree. Shortness of breath varies without the participation of auxiliary muscles in the act of breathing; at rest, as a rule, is absent. Perioral cyanosis, intermittent, aggravated by anxiety, disappearing when breathing 40-50% oxygen; pallor of the face. Arterial pressure is normal, rarely moderately elevated. The ratio of the pulse to the number of breaths is 3,5-2,5: 1; tachycardia. Behavior is restless or not disturbed.

II degree. Shortness of breath at rest is constant, with the participation of auxiliary muscles in the act of breathing, retraction of compliant places of the chest; it can also be with a predominance of inhalation or exhalation, i.e., wheezing, grunting exhalation. Perioral cyanosis of the face, hands is permanent, does not disappear when breathing 40-50% oxygen, but disappears in an oxygen tent; generalized pallor of the skin, sweating, pallor of the nail beds. Arterial pressure is increased. The ratio of the pulse to the number of breaths is 2-1,5: 1, tachycardia. Behavior: lethargy, somnolence, adynamia, followed by short periods of excitement; decrease in muscle tone.

III degree. Severe shortness of breath (respiratory rate - more than 150% of the norm); shallow breathing, intermittent bradypnea, respiratory desynchronization, paradoxical breathing. Decrease or absence of respiratory sounds on inspiration. Cyanosis is generalized; there is cyanosis of the mucous membranes, lips, does not go away when breathing 100% oxygen; generalized marbling or pallor of the skin with blue; sticky sweat. Arterial pressure is reduced. The ratio of pulse to number of breaths varies. Behavior: lethargy, somnolence, consciousness and reaction to pain are suppressed; muscular hypotension, coma; convulsions. Causes of acute respiratory failure in children.

1. Respiratory - acute bronchiolitis, pneumonia, acute laryngotracheitis, false croup, bronchial asthma, congenital malformations of the lungs.

2. Cardiovascular - congenital heart disease, heart failure, pulmonary edema, peripheral circulatory disorders.

3. Neuromuscular - encephalitis, intracranial hypertension, depression, poliomyelitis, tetanus, status epilepticus.

4. Injuries, burns, poisoning, surgical interventions on the brain, chest organs, poisoning with sleeping pills, narcotic, sedative drugs.

5. Renal failure.

Differential diagnosis. Acute bronchiolitis in children of the 1st year of life is carried out with bronchial asthma, bronchiolitis obliterans, congenital malformations of the vascular system and heart, congenital lobar emphysema, bronchopulmonary dysplasia, cystic fibrosis, foreign body, acute pneumonia.

Acute bronchiolitis in older children is carried out with allergic alveolitis, aspiration of foreign bodies, bronchial asthma, gastroesophageal reflux and aspiration of food into the respiratory tract, parasitic pneumonia. Obstructive syndrome is manifested by an increase in breathing up to 70 per 1 minute and above; anxiety of a child changing positions in search of the most comfortable; noticeable on the exhale tension of the intercostal muscles; the appearance of difficult inhalation with retraction of compliant places of the chest; central cyanosis (one of the signs is cyanosis of the tongue); decrease in PO2; an increase in PCO2.

Treatment. Treatment of obstructive syndrome: a constant supply of oxygen through a nasal catheter or nasal cannulas, the introduction of β-agonists in an aerosol (2 doses without a spacer, and preferably 4-5 doses through a spacer with a capacity of 0,7-1 l), parenterally or orally: salbutamol ( ventolin), terbutaline (bricanil), fenoterol (berotec), berodual (fenoterol + ipratropium bromide), orciprenaline (alupent, asthmapent). Together with a β-agonist, one of the corticosteroid drugs, prednisolone, is administered intramuscularly (6 mg/kg - at the rate of 10-12 mg/kg/day). If there is no effect from the introduction of β-agonists, aminofillin is used together with corticosteroids by intravenous drip (after a loading dose of 4-6 mg/kg, a constant infusion at a dose of 1 mg/kg/hour). In / in the infusion of fluid is carried out only if there are signs of dehydration. The effectiveness of therapeutic measures is judged by a decrease in the respiratory rate (by 15 or more per 1 minute), a decrease in intercostal retractions and the intensity of expiratory noises.

Indications for mechanical ventilation in obstructive syndrome:

1) weakening of respiratory sounds on inspiration;

2) preservation of cyanosis during breathing with 40% oxygen;

3) decrease in pain reaction;

4) PaO2 drop below 60 mm Hg. Art.;

5) an increase in PaCO2 above 55 mm Hg. Art.

Etiotropic therapy begins with the appointment of antiviral agents.

1. Chemotherapy - rimantadine (inhibits the specific reproduction of the virus at an early stage after penetration into the cell and before the start of RNA transcription) from the 1st year of life, a course of 4-5 days - arbidol (the same mechanism + interferon inducer), from the age of 6 age - 0,1 each, over 12 years old - 0,2, course - 3-5 days - amixin is used in children over 7 years old. With adenovirus infection, ointments are used locally (intranasally, on the conjunctiva): oxolinic ointment 1-2%, florenal 0,5%, bonafton 0,05%.

2. Interferons - native leukocyte interferon (1000 units / ml) 4-6 times a day in the nose - recombinant a-interferon (reoferon, gripferon) more active (10 units / ml) intranasally, viferon in the form of rectal suppositories.

3. Interferon inducers:

1) cycloferon (methylglucamine acridone acetate), neovir (cridanimod) - low molecular weight substances that promote the synthesis of endogenous b-, c-, and g-interferons;

2) amixin (tiloron) - ribomunil (in the acute stage of a respiratory disease, it is used according to the scheme (1 sachet of 0,75 mg or 3 tablets of 0,25 mg in the morning on an empty stomach for 4 days). Antipyretic drugs in pediatric practice are not used - amidipyrine, antipyrine , phenacetin, acetylsalicylic acid (aspirin).Currently, only paracetamol, ibuprofen are used as antipyretics in children, and also, when it is necessary to quickly reduce the temperature of the lytic mixture, they are injected intramuscularly at 0,5-1,0 ml 2,5, 50% solutions of chlorpromazine and promethazine (pipolphen) or, less desirable, analgin (0,1% solution, 0,2-10 ml / XNUMX kg of body weight. Symptomatic therapy: antitussive drugs are indicated only in cases when the disease is accompanied by an unproductive, painful, painful cough, leading to sleep disturbance, appetite and general exhaustion of the child.It is used in children of any age with laryngitis, acute bronchitis and other diseases accompanied by a painful, dry, obsessive cough. It is preferable to use non-narcotic antitussive drugs. Mucolytic drugs are used in diseases accompanied by a productive cough with thick, viscous, sputum that is difficult to separate. To improve its evacuation in acute bronchitis, it is better to use muco-regulators - carbocestein derivatives or mucolytic drugs with an expectorant effect. Mucolytic drugs should not be used with antitussive drugs. Expectorants are indicated if the cough is accompanied by thick, viscous sputum, but its separation is difficult. Antitussive drugs of central action.

1) narcotic: codeine (0,5 mg/kg 4-6 times a day);

2) non-narcotic: sinekod (butamirate), glauvent (glaucine hydrochloride), fervex for dry cough (also contains paracetamol and vitamin C).

Non-narcotic antitussive drugs of peripheral action: libexin (prenoxdiazine hydrochloride), levopront (levodropropizine).

Antitussive combination drugs: tussin-plus, stoptussin, broncholithin (glaucine, ephedrine, citric acid, basil oil).

mucolytic agents.

1. Actually mucolytic drugs:

1) proteolytic enzyme;

2) dornase (pulmozyme);

3) acetylcysteine ​​(ACC, mucobene);

4) carbocysteine ​​(bronkatar, mucodin, mucopront, fluvik).

2. Mucolytic drugs with expectorant effect:

1) bromhexine (bisolvon, broxin, solvin, phlegamine, fullpen);

2) ambroxol (ambrobene, ambrohexal, ambrolan, lazolvan, ambrosan).

3. Expectorant drugs:

1) broncholithin (glaucine, ephedrine, citric acid, basil oil);

2) glyceram (licorice);

3) Dr. MOM (licorice, basil, elecampane, aloe);

4) coldrex (terpinhydrate, paracetamol, vitamin C). Bronchodilators are used for obstructive

forms of bronchitis. Preference is given to sympathomimetic β-agonists in the form of an aerosol. B2-adrenergic agonists:

1) salbutamol (ventolin);

2) fenoterol (berotek);

3) salmeterol (long-acting);

4) formoterol (action begins quickly and lasts a long time).

The program "ARI in children: treatment and prevention" (2002) states that the use of EUFILLIN is less desirable due to possible side effects. Anti-inflammatory drugs. Inhaled glucocorticosteroids:

1) beclomethasone (aldecine, becotide, etc.);

2) budesonide (budesonide mite and forte, pulmicort);

3) flunisolide (ingacort);

4) fluticasone (flixotide).

Non-steroidal anti-inflammatory drugs Erespal (fenspiride) - counteracts bronchoconstriction and has an anti-inflammatory effect in the bronchi.

Indications: treatment of functional symptoms (cough and sputum) accompanying bronchopulmonary diseases. Antihistamines are prescribed when acute respiratory infections are accompanied by the appearance or intensification of allergic manifestations (blockers of histamine H1 receptors).

Preparations of the first generation: diazolin, diphenhydramine, pipolfen, suprastin, tavegil, fenistil.

II generation drugs: zyrtec, claritin, semprex, telfast, erius.

Immunotherapy.

1. Ribomunil is a ribosomal immunomodulator, which includes ribosomes of the main pathogens of infections of the ENT organs and respiratory organs, which have a vaccinating effect, and membrane proteoglycans that stimulate nonspecific resistance of the body.

2. Bronchomunal, IRS-19 - bacterial lysates, including bacteria of the main pneumotropic pathogens and having mainly an immunomodulatory effect.

3. Likopid - membrane fractions of the main bacteria that cause respiratory infections stimulate nonspecific resistance of the body, but do not contribute to the development of specific immunity against pathogens.

Indications for the appointment of ribomunil.

1. Inclusion in rehabilitation complexes:

1) recurrent diseases of ENT organs;

2) recurrent respiratory diseases;

3) frequently ill children.

2. Inclusion in the complex of etiopathogenetic therapy:

1) acute otitis;

2) acute sinusitis;

3) acute pharyngitis;

4) acute tonsillitis;

5) acute laryngotracheitis;

6) acute tracheobronchitis;

7) acute bronchitis;

8) pneumonia.

Immunoglobulins for intravenous administration, registered and approved for use in the Russian Federation.

1. Human immunoglobulins are normal (standard) for intravenous administration:

1) normal human immunoglobulin for intravenous administration (Imbio, Russia);

2) immunoglobulin (Biochemie GmbH, Austria);

3) intraglobin (Biotest Pharma GmbH, Germany);

4) octagam (Oktapharma AG, Switzerland);

5) sandoglobulin (Novartis Pharma services, Switzerland);

6) endobulin (Immuno AG, Austria);

7) Biaven V. I. (Pharma Biajini S. p. A, Italy);

8) wigam-liquid (Bio Products Laboratory, UK);

9) wigam-C (Bio Products Laboratory, UK).

2. Immunoglobulins for intravenous administration, enriched with antibodies of the IgM class - pentaglobin (Biotest Pharma GmbH, Germany).

Non-drug methods of treatment.

1. Exercise therapy.

2. Electrical procedures (UHF, microwave, diathermy) are indicated for sinusitis, lymphadenitis; in diseases of the chest organs, their effectiveness has not been proven, including electrophoresis of drugs.

3. Thermal and irritating treatments. Dry heat for sinusitis, lymphadenitis, wet compress for otitis media (subjective relief). Fat rubbing is not effective and should not be used. Mustard plasters, jars, hot patches and rubbing are painful, fraught with burns and allergic reactions.

Conditions that are not indications for the use of antibiotics in ARVI.

1. General disorders: body temperature less than 38 °C or more than 38 °C for less than 3 days, febrile convulsions, loss of appetite, headache, myalgia, herpetic eruptions.

2. Syndromes: rhinitis, nasopharyngitis, tonsillitis, laryngitis, bronchitis, tracheitis, conjunctivitis.

3. Respiratory syndromes: cough, hyperemia of the pharynx, hoarseness, scattered wheezing, airway obstruction, difficulty breathing.

Signs of a probable bacterial infection: body temperature above 38 °C from 3 days or more, asymmetry of wheezing on auscultation, chest indrawing, severe toxicosis, leukocytosis more than 15 and / or more than 000% of young forms of stab, accelerated ESR more than 5 mm / h, sore throat and raids (strep throat is possible), ear pain (acute otitis media), nasal congestion for 20 weeks or more (sinusitis), swollen lymph nodes (lymphadenitis), shortness of breath without obstruction (pneumonia). (See tables 2, 1)

Table 1

The choice of starting drug for community-acquired pneumonia

Table 2

Choice of starting antibiotic for nosocomial pneumonia

LECTURE No. 18. Congenital and hereditary lung diseases

A malformation is an anomaly in most cases of intrauterine development, resulting in gross changes in the structure and function of an organ or tissue.

Classification of malformations of the bronchopulmonary system.

1. Malformations associated with the underdevelopment of the organ as a whole or its anatomical, structural, tissue elements:

1) lung agenesis;

2) lung aplasia;

3) lung hypoplasia;

4) cystic hypoplasia (polycystic);

5) tracheobronchomegaly (Mounier-Kuhn syndrome);

6) Williams-Campbell syndrome;

7) congenital lobar emphysema.

2. Malformations associated with the presence of excessive dysembryogenetic formations:

1) accessory lung (lobe) with normal blood supply or with abnormal blood supply;

2) lung cyst with normal blood supply or with abnormal blood supply;

3) hamartoma and other tumor-like formations.

3. Unusual anatomical arrangement of lung structures, sometimes of clinical significance:

1) reverse arrangement of the lungs (Kartegener's syndrome);

2) mirror lung;

3) tracheal bronchus;

4) share of the unpaired vein.

4. Localized violations of the structure of the trachea and bronchi:

1) stenosis;

2) diverticula;

3) tracheoesophageal fistulas.

5. Anomalies of blood and lymphatic vessels:

1) stenosis of the pulmonary artery and its branches;

2) varicose pulmonary veins;

3) multiple arteriovenous fistulas without clear localization.

Hereditary diseases of the respiratory system, according to various authors, range from 5 to 35% of the total number of patients with nonspecific lung diseases.

Chronic lung diseases in children (S. Yu. Kaganov, 200311/).

1. Infectious and inflammatory diseases.

2. Congenital malformations of the bronchopulmonary system.

3. Hereditary lung diseases.

4. Lung lesions in other hereditary diseases.

5. Allergic diseases of the lungs.

Classification of COPD in children (E. V. Klimanskaya, 2001):

1) a common type of pathological changes that cause obstruction:

a) common malformations with insufficiency of the muscular-elastic and cartilaginous framework of the trachea and bronchi. Tracheobronchomalacia, tracheobronchomegaly (Mounier-Kuhn syndrome), Williams-Campbell syndrome;

b) a hereditary defect in the structure of the ciliary epithelium of the mucous membrane of the respiratory tract. Primary ciliary dyskinesia, immobile cilia syndrome, Kartagener's syndrome;

c) universal genetically determined exocrinopathy (abnormal viscosity of bronchial secretion). cystic fibrosis;

2) local type of changes causing obstruction (developmental defects):

a) tracheobronchial stenoses, fistulas, cysts;

b) cardiovascular anomalies with compression of the trachea, anomaly of the aorta (double arch) and pulmonary artery.

Acquired diseases:

1) a common type of pathological changes that cause obstruction:

a) allergic inflammation, bronchial asthma;

b) infectious inflammation;

2) recurrent and chronic obstructive bronchitis;

3) local type of pathological changes causing obstruction (mechanical factors);

4) foreign body, tumor, infectious granuloma, post-traumatic cicatricial stenosis.

Congenital malformations are persistent morphological changes in an organ or organism that go beyond variations in their structure and occur in utero as a result of developmental disorders of the embryo, fetus, or sometimes after the birth of a child as a result of a violation of the further formation of organs. The vast majority of malformations are associated with hereditary pathology.

Only 3-5% of all malformations are associated with the actions of teratogenic factors.

Stages of impaired embryonic development of the lung (Monaldi, 1959).

1. The first stage includes lung agenesis as a result of the absence of a primary bronchial kidney.

2. At the second stage, there is a violation of the development of the primary bronchial kidney, leading to underdevelopment of the main bronchus and aplasia of the lung. These defects occur on the 3-4th week of the embryonic period.

3. The third stage of the violation occurs on the 30-40th day of intrauterine development and is characterized by the presence of lung hypoplasia.

4. The fourth stage (II-V months of the intrauterine period) is determined by a violation of the development of small bronchi and leads to the occurrence of polycystic lung disease.

Diagnosis of congenital and hereditary lung diseases: it is believed that of the numerous pulmonary symptoms, cough, sputum, and hemoptysis have the greatest objective significance in the diagnosis of respiratory diseases.

Other important symptoms: shortness of breath, cyanosis, change in the shape of the chest (retraction, flattening, keeled bulging of the sternum), "drumsticks", "watch glasses", percussion: shortening of percussion sound, displacement of the heart towards the pathologically altered lung, auscultation: constancy of auscultatory pictures (weakened breathing, its absence, various wheezing).

Research methods.

1. X-ray examinations, bronchological examination (bronchoscopy, bronchography), angiography.

Indicated for suspected pulmonary separation and sequestration, as well as for detection of vascular changes (an anomaly of the aortic ring, hypoplasia, ectasia and atypical pulmonary artery origin).

2. Computed tomography.

3. Study of the cilia of the mucous membrane of the respiratory tract (electron microscopic examination; phase-contrast study).

4. Determination of mucociliary clearance (mucociliary cleansing system).

5. Immunological examination.

6. Sweat test.

7. Molecular genetic examination.

8. Functional methods for studying external respiration

9. Morphological research methods. Congenital malformations of the lungs.

1. Agenesis, aplasia and hypoplasia of the lungs.

2. Polycystic lungs.

3. Congenital lobar emphysema.

4. Williams-Campbell syndrome.

5. Tracheobronchomegaly (Mounier-Kuhn's syndrome).

6. Anomalies of branching of the bronchi.

Pulmonary agenesis is the absence of a lung along with the main bronchus.

Aplasia of the lung - the absence of a lung in the presence of a rudimentary main bronchus.

Hypoplasia of the lung - there are main and lobar bronchi, which end in a functionally imperfect rudiment, lung tissue is underdeveloped, agenesis, aplasia and hypoplasia of the lungs.

Clinical picture: cough, shortness of breath. Repeated pneumonia, bronchitis. Children are lagging behind in physical development. Deformation of the chest - retraction or flattening on the side of the defect. Children with lung hypoplasia have keeled bulging of the sternum (compensatory emphysema of the unaffected lung). The organs of the mediastinum are displaced towards the defect.

1. Agenesia, aplasia and hypoplasia of the lungs

On radiography, a decrease in the volume of the chest on the side of the defect, intense darkening in this area, high standing of the dome of the diaphragm. The spinal column is "bare" There may be a prolapse of a healthy lung into the other half of the chest with the formation of a "pulmonary hernia".

With bronchoscopy, the absence or rudiment of the main bronchus, narrowing of the lobar bronchi.

With bronchography, if there is agenesis and the absence of the main bronchus; if aplasia has a rudimentary bronchus, large bronchi are filled with hypoplasia, in the absence of small bronchial branches - agenesis, aplasia and hypoplasia of the lungs.

With agenesis and aplasia, conservative therapy aimed at suppressing bronchopulmonary infection.

With hypoplasia of the lung, surgical treatment is preferred.

2. Polycystic lung disease

Polycystic lung disease (cystic hypoplasia) is a malformation caused by antenatal underdevelopment of the lung parenchyma, vessels and bronchial tree with the formation of many cavities (cysts) distal to the subsegmental bronchi.

clinical picture. Cough, purulent sputum, sometimes hemoptysis. Almost from birth, a continuously relapsing course of inflammation in the bronchopulmonary system. Children are lagging behind in physical development, "drumsticks". Deformation of the chest on the side of the defect.

Diagnostics. With X-ray and tomography, cellular formations are detected.

Bronchography revealed multiple rounded cavities. Cystic formations are more often localized in the left lung or there is a bilateral lesion.

Computed tomography showed cystic formations, their predominant localization in the left lung.

Complications. Complications of polycystic lung disease: suppurative pulmonary processes, pneumothorax, pulmonary bleeding, amyloidosis (rarely).

Treatment. Surgical.

Contraindications: the prevalence of the process, severe manifestations of pulmonary heart disease.

3. Congenital lobar emphysema

Congenital lobar emphysema is characterized by stretching of the parenchyma of a lobe (less often a segment) due to partial obstruction of the draining bronchus.

Hypotheses of pathogenesis:

1) underdevelopment or absence of bronchial cartilage;

2) hypertrophy of the mucous membrane of the bronchi with the formation of folds, mucous plugs;

3) compression of the bronchus from the outside by bronchogenic cysts, abnormally located vessels (the favorite localization is the upper lobe of the left lung).

With X-ray and tomography, increased transparency of the affected part of the lung. The pulmonary pattern in this area is depleted or not traced at all. The diaphragm is flattened, its excursion is limited. The mediastinum is displaced towards the unaffected lung.

Bronchological examination is uninformative, and most importantly, unsafe for patients, as it can lead to rupture of the emphysematous swollen part of the lung.

Treatment. Surgical removal of the affected part of the lung.

4. Williams-Campbell Syndrome

Williams-Campbell syndrome is characterized by the complete absence or insufficient development of the cartilaginous rings of the bronchi of the 3rd-8th orders. An autosomal recessive inheritance of the defect is assumed.

clinical picture. Early onset of bronchopulmonary inflammation. Deformation and swelling of the chest. Shortness of breath, wheezing, cough with sputum, moist rales in the lungs. Deformation of the nails and terminal phalanges of the fingers in the form of "drumsticks". A sharp violation of respiratory function, the development of obstructive ventilation insufficiency.

Diagnostics. On radiographic examination, swelling of the lung tissue.

FVL: obstructive ventilation disorders. With bronchoscopy, the picture of bronchitis, prolapse of the bronchial walls is observed.

On bronchography, the presence of generalized and ballooning bronchiectasis with typical proximal localization; the lower lobes are predominantly affected.

The course is unfavorable; patients die from progressive pulmonary heart failure.

5. Tracheobronchomegaly

Tracheobronchomegaly is characterized by the expansion of the trachea and main bronchi. It is believed that the defect is based on a congenital defect in the elastic and muscle fibers in the wall of the bronchi and trachea. An autosomal recessive inheritance of the defect is assumed.

6. Mounier-Kuhn syndrome

clinical picture. From an early age, cough with sputum, repeated exacerbations of bronchopulmonary disease, an increase in respiratory failure during an exacerbation and with age. Deformation of the nail phalanges in the form of "drumsticks".

X-ray signs: deformation of the lung pattern with foci of compaction. Expansion of the lumen of the trachea and large bronchi. Bronchiectasis in the lower lobe segments.

With bronchoscopy, the expansion of the lumen of the trachea (bronchi), thickening of the walls with bulging into the lumen of the intercartilaginous spaces, pathological secretion.

Treatment. Treatment is conservative, aimed at combating bronchopulmonary infection.

Monogenic lung diseases:

1) primary ciliary dyskinesia and Kartagener's syndrome;

2) idiopathic diffuse pulmonary fibrosis (Hamman-Rich syndrome, idiopathic fibrosing alveolitis);

3) primary pulmonary hypertension (Aers syndrome);

4) idiopathic hemosiderosis of the lungs (Tselen-Gellerstedt syndrome);

5) Goodpasture's syndrome;

6) family spontaneous pneumothorax;

7) alveolar microlithiasis;

8) alveolar proteinosis;

9) cystic fibrosis;

10) a1-antitrypsin deficiency.

7. Primary ciliary dyskinesia (fixed cilia syndrome) and Kartagener's syndrome

It is based on a genetically determined defect in the structure of the ciliated epithelium of the respiratory tract mucosa.

The morphological essence of the defect in its classic version is the loss of dynein handles containing ATP, which ensures the movement of cilia.

Kartagener's syndrome is characterized by the following triad, which includes the reverse arrangement of internal organs, bronchiectasis and chronic sinusitis, rhinitis, otitis media. Pathogenesis:

1) a congenital defect of the cilia with a violation of their movement (slowdown, desynchronization);

2) decrease in mucociliary transport;

3) stagnation of the secret;

4) sinusitis, bronchitis (bronchiectasis, polycystosis, pneumosclerosis), otitis media, rhinitis.

clinical picture. Continuously recurrent broncho-pulmonary inflammation from the first days of life. Lagging behind in physical development, persistent cough with purulent sputum, moist rales in the lungs, changes in the shape of the nails and terminal phalanges of the fingers, chronic sinusitis.

Diagnostics. X-ray examination determines the deformation of the lung pattern, focal seals, bronchiectasis. Reverse arrangement of internal organs in Kartagener's syndrome.

With bronchoscopy, a chronic purulent-inflammatory process is determined, a mirror arrangement of the bronchi in Kartagener's syndrome.

Laboratory data: with electron microscopy, pathology in the structure of the ciliary apparatus.

Treatment is aimed at suppressing the inflammatory process in the lungs and nasopharynx; drainage therapy (postural drainage, exercise therapy, therapeutic bronchoscopy, inhalation of mucolytics), surgical treatment is usually ineffective.

8. Idiopathic diffuse pulmonary fibrosis (Hamman-Rich syndrome, idiopathic fibrosing alveolitis - ELISA)

Pathogenesis. The pathogenesis of ELISA is considered as an autoimmune process; rare in childhood, more common in humans

50-60 years.

clinical picture. Shortness of breath (mostly difficult to inhale), cough (dry, unproductive), inconsistency of shortness of breath with relatively small physical changes in the lungs, nail plates in the form of "drumsticks", sometimes hemoptysis, auscultatory few tiny crepitant moist rales ("cellophane crackling"), hypoxemia, hypercapnia.

An x-ray examination determines a diffuse enhancement of the pulmonary pattern, the presence of focal shadows; a symptom of "frosted glass" - a diffuse decrease in the transparency of the lung tissue.

With bronchography, narrowing of the bronchi, their deformation.

9. Primary pulmonary hypertension (AERS syndrome)

It is characterized by hypertrophy of the myocardium of the right ventricle, expansion of the trunk of the pulmonary artery. Morphologically, fibrosis and fibroelastosis of the intima, fibrinoid-necrotic arteritis of small branches of the pulmonary artery and thrombosis are detected. It is believed that the immediate cause is fibrosis and fibroelastosis of the muscular layer of the pulmonary arterioles, most likely associated with a genetically determined defect in smooth muscle fibers. Refers to diseases with an autosomal dominant type of inheritance. It is more common in young women and girls.

clinical picture. Shortness of breath, cyanosis, right ventricular hypertrophy, physical changes in the lungs are usually absent, "drumsticks".

Severe, rapidly progressive primary pulmonary hypertension, occurring with severe cyanosis, shortness of breath, polycythemia, severe hypertrophy of the right heart is defined as AERS syndrome (AERS).

Diagnostics. An x-ray examination determines a sharp expansion of the proximal pulmonary artery with a weakening of the pulmonary pattern in the peripheral sections of the lungs, an increase in the size of the right sections of the heart, an expansion of the roots of the lungs and their increased pulsation.

On the ECG: signs of a sharp overload of the right ventricle and its hypertrophy.

ECHO KG: expansion of the pulmonary artery, enlargement of the right ventricle, regurgitation of blood into the right ventricle.

Treatment. Calcium antagonists, a-blockers.

Forecast. Adverse; death from progressive right ventricular failure.

10. Idiopathic hemosiderosis of the lungs (Celena-Gellerstedt syndrome)

The disease is based on the formation of anti-pulmonary antibodies in response to exposure to a sensitizing agent. The resulting immune complexes are fixed on the basement membranes of the alveoli and pulmonary capillaries and cause damage to the lung tissue. An allergic reaction that unfolds on the territory of the shock organ causes damage to the pulmonary capillaries, diapedesis and destruction of erythrocytes, followed by the deposition of hemosiderin in the alveoli and alveolar septa.

11. Goodpasture's Syndrome

Goodpasture's syndrome is a combination of pulmonary hemosiderosis and glomerulonephritis, characterized by immunological damage to the basement membranes of the lungs and kidneys.

Pathogenesis. For various reasons, erythrocytes enter the lung tissue from the vessels, become autoAG; they produce autoAT; as a result of the AG-AT reaction, the breakdown of erythrocytes occurs. This hypothesis is based on the immunoallergic genesis of idiopathic pulmonary hemosiderosis. Refers to respiratory allergies, which can explain the cyclical course of the disease. The disease is predominantly of childhood.

Morphological picture: deposition of hemosiderin in the alveoli and interalveolar septa.

Clinical manifestations. Clinical picture: the onset of the disease may occur in children of the first years of life; the course is usually undulating: periods of crises are replaced by remission of varying durations. During crises, temperature rises, cough, shortness of breath, chest pain, hemoptysis (blood streaks, intense coloration of sputum, pulmonary hemorrhages are possible), anemia; hepatolienal syndrome; the cor pulmonale gradually forms.

Diagnostics. In the blood test, hypergammaglobulinemia, an increase in the level of the CEC, the detection of antipulmonary antibodies (sometimes), an increase in indirect bilirubin, hypochromic anemia, a decrease in serum iron.

X-ray examination during crises reveals multiple cloud-like shadows, usually bilateral; pneumosclerosis gradually develops.

Forecast. Patients die from pulmonary heart failure or pulmonary bleeding, spontaneous pneumothorax (due to thinning of the walls and rupture of subpleurally located emphysematous bullae). Refers to the number of hereditary diseases with an autosomal dominant type of inheritance (as an independent disease).

12. Pathology of connective tissue

May accompany hereditary connective tissue pathology (Marfan syndrome, Ehlers-Danlos syndrome, a1-antitrypsin deficiency).

clinical picture. Sudden sharp stabbing pain in the chest, aggravated by a deep breath, shortness of breath, percussion on the side of the lesion "box" sound, a sharp weakening of the breath sounds (auscultation), a shift in cardiac dullness in the opposite direction.

X-ray is determined by the presence of air in the pleural cavity, the collapse of the lung.

Treatment. Treatment for spontaneous pneumothorax is drainage of the pleural cavity with constant active aspiration (according to Belau).

13. Alveolar microlithiasis

It is characterized by the formation in the pulmonary alveoli of the smallest calculi, which consist of calcium carbonate and thiophosphates with a small admixture of iron salts and traces of magnesium. As a result of the deposition of calculi, an alveolar-capillary block occurs, ventilation-perfusion relations are disturbed. It is inherited in an autosomal recessive manner.

Pathogenesis. The formation of calculi in the alveoli is associated with impaired production of alveolar fluid, as well as a disorder in the exchange of carbonic acid - alveolar microlithiasis. The disease occurs in all age groups.

Clinical manifestations. The clinical picture is varied. A discrepancy between the poor clinical picture and radiological changes is characteristic.

There may be no symptoms at all; may be bothered by shortness of breath, cyanosis, decreased exercise tolerance. As the process progresses, signs of chronic pneumonia appear: cough, sputum, fever, “drumsticks” and cor pulmonale appear.

Diagnostics. X-ray examination reveals small diffuse shadows of stony density, located mainly in the lower and middle parts of the lungs; there is a thickening of the pleura (differentiated with tuberculosis).

FVD: restrictive respiratory disorders. In a lung biopsy, calcifications are found in the lumen of the alveoli, sometimes located in the lumen and wall of the bronchi. Treatment. Symptomatic.

Forecast. Adverse; death from pulmonary heart failure.

14. Alveolar proteinosis

Alveolar proteinosis is caused by the accumulation of a protein-lipoid substance in the alveoli. The histological picture is characterized by the presence in the alveolar lumen of a granular exudate with a PAS-positive reaction. It is transmitted in an autosomal recessive manner.

Pathogenesis. A genetic defect leading to the synthesis of a defective surfactant that does not have surface-active properties; this lipoprotein is characterized by a strong PAS-positive reaction; filling the alveoli with lipoprotein causes changes in lung function and the corresponding clinical symptoms: progressive dyspnea, cough, chest pain, hemoptysis; in the future, a cor pulmonale is formed with the corresponding symptoms.

Diagnostics. X-rays reveal bilateral small-focal (small-dotted) opacities that tend to merge, and later fibrotic changes are detected.

Biopsy: presence of PAS-positive substance (diagnosis confirmation).

Electron microscopy reveals surfactant in the form of lamellar bodies in the alveoli and alveolar macrophages.

Treatment. Therapeutic bronchoalveolar lavage; trypsin, chymotrypsin.

15. Lung lesions in a deficiency of a - protease inhibitor

Lung lesions in a1-protease inhibitor deficiency are characterized by a predominant lesion of the respiratory section of the lung tissue in the form of early-developing primary emphysema due to the action of non-inactivated proteases (trypsin, elastase, etc.) on the lung tissue. α1-antitrypsin deficiency is inherited in an autosomal recessive manner (a gene on the 14th chromosome).

Pathogenesis. Links of pathogenesis:

1) proteases, trypsin, chemotrypsin, elastase;

2) hereditary deficiency of α1-anti-titrypsin;

3) imbalance in the protease-antiprotease system during inflammation, trauma, burns towards an increase in proteases;

4) damage to elastin, collagen, proteoglycans;

5) destruction of the elastic fibers of the lung tissue;

6) depletion and rupture of the alveolar septa;

7) primary panlobular emphysema. Protease inhibitors are proteins that have the ability to inactivate proteolytic enzymes of endogenous and exogenous origin.

Clinic. Shortness of breath (main complaint), gradual weight loss, rare (dry) or absent cough, scanty sputum, barrel-shaped chest.

Diagnostics. When radiography is determined by an increase in the transparency of the lung fields, with the formation of giant bullae, there is no pulmonary pattern ("disappearing", "supertransparent" lung); the diaphragm is usually flattened, stands low, its mobility is sharply limited; heart shadow of small size - "drop-shaped".

Computed tomography reveals foci of bullous emphysema or giant bullae. Study of the content of aj-antitrypsin in blood serum (ELISA).

Treatment:

1) substitution therapy (intravenous administration of native c^-antitrypsin;

2) introduction of native human plasma;

3) introduction of contrical, gordox;

4) gene therapy: introduction of a gene using a retrovirus vector (in animals).

Forecast. The prognosis is ambiguous, most often doubtful.

16. Cystic fibrosis

Cystic fibrosis (cystic fibrosis of the pancreas) is characterized by a systemic lesion of the exocrine glands due to an increase in the viscosity of their secretion, which, in relation to the bronchopulmonary system, causes a sharp violation of the cleansing function of the bronchi and bronchial patency.

A frequent monogenic disease caused by a mutation in the cystic fibrosis gene, characterized by damage to the exocrine glands, vital organs and systems, and usually having a severe course and prognosis.

In most countries in Europe and North America, CF affects between 1:2000 and 1:4000 newborns. In Russia 1: 12 0 °C newborns.

It is inherited in an autosomal recessive manner, i.e. both parents must be carriers of the mutant gene. The probability of having a CF patient in such a family is 25%, 2-5% of the population are carriers of the CF gene.

The CF gene was isolated in 1989 and is located in the middle of the long arm of chromosome 7. To date, more than 1000 gene mutations have been identified. The most common mutation is del F 508 (53%). Mutations of the CF gene in the homozygous state lead to disruption of the synthesis of the protein that forms the chlorine channel in the membranes of epithelial cells, through which passive transport of chlorine ions occurs. This protein is called cystic fibrosis transmembrane conductance regulator (CFTR).

Pathogenesis. The pathogenesis lies in the fact that the secretion of the exocrine glands, due to dysfunction of the chlorine channel, becomes especially viscous, which explains most of the pathological processes underlying the pathogenesis of the disease.

Clinic. In the bronchopulmonary system, a viscous secret, accumulating in the lumen of the bronchi, leads to complete obstruction of small bronchioles. As a result of infection with pathogenic microflora, purulent inflammation develops. The most common pathogens are Staphylococcus aureus and Pseudomonas aeruginosa. The bronchial wall is destroyed. Formed bronchiectasis, cor pulmonale.

In patients with cystic fibrosis, the chlorine channel on the apical part of the cell membrane does not work, leading to a disruption in the release of chlorine from the cell, which contributes to an increased escape of sodium ions from the lumen into the cell, followed by the aqueous component of the intercellular space. The consequence is a thickening of the secretions of the glands of external secretion (bronchopulmonary system, pancreas, salivary glands, gonads).

The presence in the family of diseases of the lungs and intestines, stillbirths, spontaneous abortions. From birth - dry, hacking cough. Early onset of continuously recurrent bronchopulmonary inflammation. Exhaustion and lag in physical development. Respiratory failure. "Drumsticks".

Carinated protrusion of the sternum. FVD - persistent obstructive and restrictive disorders. Often sowing Pseudomonas. Pulmonary heart. Almost all patients have excretory pancreatic insufficiency.

Gastrointestinal injury in cystic fibrosis:

1) reflux esophagitis;

2) ulcerative esophagitis;

3) gastritis;

4) duodenitis;

5) biliary reflux;

6) gastric and duodenal ulcer;

7) coprostasis;

8) meconium ileus;

9) delayed evacuation of meconium;

10) fecal ileus;

11) intestinal invagination;

12) biliary cirrhosis;

13) portal hypertension;

14) acute pancreatitis;

15) fatty degeneration of the pancreas;

16) diabetes mellitus.

Diagnostics. Survey plan.

1. X-ray of the chest. X-ray signs: in the form of deformation of the bronchopulmonary pattern, atelectasis, pneumofibrosis, bronchiectasis.

Bronchoscopy reveals inflammatory changes and obstruction of the bronchi with purulent secretions.

2. X-ray of the paranasal sinuses.

3. Ultrasound of the pancreas.

4. Expanded coprogram (neutral fat).

5. Sweat test (sweat chlorides).

6. Molecular genetic examination.

7. Sputum culture (if possible).

8. Examination of respiratory function (after 6 years).

Laboratory data: an increase in the content of chlorides in sweat (repeatedly above 60,0 mmol / l). Identification of a mutant cystic fibrosis gene.

Search group to rule out cystic fibrosis. In infancy:

1) recurrent or chronic respiratory symptoms (cough, shortness of breath);

2) recurrent or chronic pneumonia;

3) lag in physical development;

4) unformed, profuse, oily and fetid stools;

5) chronic diarrhea;

6) prolonged neonatal jaundice;

7) salty skin taste;

8) heat stroke or dehydration in hot weather;

9) chronic hypoelectrolytemia;

10) family history data on the death of children in the first year of life or the presence of sibs with similar clinical manifestations;

11) hypoproteinemia/edema.

Search group for the exclusion of cystic fibrosis in preschool children:

1) persistent cough with or without purulent sputum;

2) diagnostically unclear recurrent or chronic shortness of breath;

3) retardation in body weight and height;

4) prolapse of the rectum;

5) invagination;

6) chronic diarrhea;

7) a symptom of "drum sticks";

8) salt crystals on the skin;

9) hypotonic dehydration;

10) hypoelectrolytemia and metabolic alkalosis;

11) hepatomegaly or diagnostically unclear liver dysfunction.

Search group to rule out cystic fibrosis in school-aged children:

1) chronic respiratory symptoms of unclear etiology;

2) pseudomonas aeruginosa in sputum;

3) chronic sinusitis;

4) nasal polyposis;

5) bronchiectasis;

6) a symptom of "drum sticks";

7) chronic diarrhea;

8) syndrome of distal intestinal obstruction;

9) pancreatitis;

10) prolapse of the rectum;

11) diabetes mellitus in combination with respiratory symptoms;

12) hepatomegaly;

13) liver disease of unknown etiology.

Search group for ruling out cystic fibrosis in adolescents and adults:

1) purulent lung disease of unclear etiology;

2) a symptom of "drum sticks";

3) pancreatitis;

4) syndrome of distal intestinal obstruction;

5) diabetes mellitus in combination with respiratory symptoms;

6) signs of liver cirrhosis and portal hypertension;

7) stunting;

8) delayed sexual development;

9) sterility with azoospermia in males;

10) reduced fertility in females. Treatment.

Goals of therapy for patients with cystic fibrosis.

1. Supporting the patient's lifestyle as close as possible to the life of healthy children.

2. Control of respiratory infections.

3. Ensuring adequate nutrition. Mandatory directions in treatment:

1) physiotherapy exercises (physiotherapy, kinesitherapy);

2) mucolytic therapy;

3) antimicrobial therapy;

4) enzyme therapy (pancreatic preparations);

5) vitamin therapy;

6) diet therapy;

7) treatment of complications;

8) kinesitherapy. Methods:

1) postural drainage;

2) percussion and vibration of the chest (clopmassage);

3) active breathing cycle;

4) autogenous drainage;

5) breathing exercises using flutter and PEP-mask.

Sports recommended for patients with cystic fibrosis swimming, running, cycling, skiing, badminton, tennis, horseback riding, yoga, wushu, volleyball, golf, hiking Sports prohibited for patients with cystic fibrosis: skating, weightlifting, football , boxing, hockey, diving, rugby, judo, basketball, motorsport.

Inhalation therapy (bronchodilators, mucolytics, antibiotics). Recommendations from the Cystic Fibrosis Center.

1. Take a bronchodilator (salbutamol, etc.) 5 minutes before inhalation.

2. Thoroughly blow your nose.

3. Take the correct position: sit straight, straighten your chest, shoulders and shoulder blades are lowered down.

4. Mucolytic inhalation (N-acetylcysteine, saline, etc.) 8-10 min.

5. Kinesitherapy: breathing exercises, drainage, exercise therapy.

6. Antibiotic inhalation and topical corticosteroid through spacer.

In the case of using pulmozyme, it is inhaled 30-40 minutes after inhalation of other drugs.

A stepwise approach to the treatment of cystic fibrosis. St. Aureus.

1. Antibiotics up to 2-4 months. per year, of which 1-2 courses in / in or / m (1-2 drugs).

2. PEP therapy. Pseudomonas aeruginosa.

1. Antibiotics - 2-4 IV courses for 14 days (2 drugs) Total antibiotics up to 4-6 months a year.

2. Hepatotropic drugs.

3. Bacterial preparations. Pseudomonas aeruginosa resist.

1. Antibiotics - 4-6 IV courses for 14-20 days (2-3 drugs).

2. Hepatotropic drugs.

3. Bacterial preparations.

4. Antimycotics in inhalations.

5. NSAIDs.

6. Hormonal preparations.

The life prognosis is due to respiratory disorders caused by chronic lung infection.

The progression of the bronchopulmonary process increases after the development of chronic Pseudomonas aeruginosa infection.

Various oral, inhaled, and intravenous antibiotic regimens currently in use can prevent or delay the development of chronic lower respiratory infections.

LECTURE No. 19. Respiratory diseases. Acute bronchitis. Clinic, diagnosis, treatment, prevention. Chronical bronchitis. Clinic, diagnosis, treatment, prevention

1. Acute bronchitis

Acute bronchitis is an acute diffuse inflammation of the tracheobronchial tree. Classification:

1) acute bronchitis (simple);

2) acute obstructive bronchitis;

3) acute bronchiolitis;

4) acute bronchiolitis obliterans;

5) recurrent bronchitis;

6) recurrent obstructive bronchitis;

7) chronic bronchitis;

8) chronic bronchitis with obliteration. Etiology. The disease is caused by viral infections (influenza viruses, parainfluenza, adenoviruses, respiratory syncytial, measles, whooping cough, etc.) and bacterial infections (staphylococci, streptococci, pneumococci, etc.); physical and chemical factors (cold, dry, hot air, nitrogen oxides, sulfur dioxide, etc.). Chilling, chronic focal infection of the nasopharyngeal region and impaired nasal breathing, deformity of the chest predispose to the disease.

Pathogenesis. The damaging agent enters the trachea and bronchi with inhaled air by the hematogenous and lymphogenous route. Acute inflammation of the bronchial tree is accompanied by a violation of bronchial patency of the edematous-inflammatory or bronchospastic mechanism. Characterized by hyperemia, swelling of the mucous membrane; on the wall of the bronchus and in its lumen is a mucous, mucopurulent or purulent secret; degenerative disorders of the ciliated epithelium develop. In severe forms of acute bronchitis, inflammation is localized not only on the mucous membrane, but also in the deep tissues of the bronchial wall.

Clinical signs. Clinical manifestations of bronchitis of infectious etiology begin with rhinitis, nasopharyngitis, moderate intoxication, fever, weakness, feeling of weakness, soreness behind the sternum, dry, turning into a wet cough. There are no auscultatory signs or hard breathing is determined over the lungs, dry rales are heard. There are no changes in peripheral blood. This course is observed more often with damage to the trachea and bronchi. In moderate bronchitis, general malaise, weakness are significantly expressed, a strong dry cough appears with difficulty breathing, shortness of breath, pain in the chest and in the abdominal wall, which is associated with muscle strain when coughing. Cough gradually becomes wet, sputum acquires a mucopurulent or purulent character. In the lungs during auscultation, hard breathing, dry and moist small bubbling rales are heard. Body temperature is subfebrile. There are no pronounced changes in peripheral blood. A severe course of the disease is observed with a predominant lesion of the bronchioles. Acute clinical manifestations of the disease begin to subside by the 4th day and, with a favorable outcome, almost completely disappear by the 7th day of the disease. Acute bronchitis with a violation of bronchial patency has a tendency to a protracted course and the transition to chronic bronchitis. Acute bronchitis of toxic-chemical etiology is severe. The disease begins with a painful cough, which is accompanied by the release of mucous or bloody sputum, bronchospasm quickly joins (against the background of an extended expiration during auscultation, dry wheezing can be heard), shortness of breath progresses (up to suffocation), symptoms of respiratory failure and hypoxemia increase. A chest x-ray can identify symptoms of acute pulmonary emphysema.

Diagnosis: based on clinical and laboratory data.

Treatment. Bed rest, plenty of warm drink with raspberries, honey, lime blossom. Assign antiviral and antibacterial therapy, vitamin therapy: ascorbic acid up to 1 g per day, vitamin A 3 mg 3 times a day. You can use cans on the chest, mustard plasters. With a strong dry cough - antitussive drugs: codeine, libexin, etc. With a wet cough - mucolytic drugs: bromhexine, ambrobene, etc. Inhalation of expectorants, mucolytics, heated mineral alkaline water, eucalyptus, anise oil using a steam inhaler is indicated. inhalation - 5 minutes 3-4 times a day for 3-5 days. Bronchospasm can be stopped with the appointment of aminophylline (0,25 g 3 times a day). Showing antihistamines, Prevention. Elimination of the etiological factor of acute bronchitis (hypothermia, chronic and focal infection in the respiratory tract, etc.).

2. Chronic bronchitis

Chronic bronchitis is a progressive diffuse inflammation of the bronchi, not associated with local or generalized lung damage, manifested by cough. You can talk about chronic bronchitis if the cough continues for 3 months in the 1st year - 2 years in a row.

Etiology. The disease is associated with prolonged irritation of the bronchi by various harmful factors (inhalation of air polluted with dust, smoke, carbon monoxide, sulfur dioxide, nitrogen oxides and other chemical compounds) and recurrent respiratory infection (a large role belongs to respiratory viruses, Pfeiffer's bacillus, pneumococci), less often occurs in cystic fibrosis. Predisposing factors - chronic inflammatory, suppurative processes in the lungs, chronic foci of infection and chronic diseases localized in the upper respiratory tract, decreased body reactivity, hereditary factors.

Pathogenesis. The main pathogenetic mechanism is hypertrophy and hyperfunction of the bronchial glands with increased secretion of mucus, with a decrease in serous secretion and a change in the composition of secretion, as well as an increase in acid mucopolysaccharides in it, which increases the viscosity of sputum. Under these conditions, the ciliated epithelium does not improve the emptying of the bronchial tree, usually the entire layer of secretion is renewed normally (partial clearance of the bronchi is possible only with coughing). Prolonged hyperfunction is characterized by depletion of the mucociliary apparatus of the bronchi, the development of dystrophy and atrophy of the epithelium. In case of violation of the drainage function of the bronchi, a bronchogenic infection occurs, the activity and relapses of which depend on the local immunity of the bronchi and the occurrence of secondary immunological deficiency. With the development of bronchial obstruction due to hyperplasia of the epithelium of the mucous glands, edema and inflammatory compaction of the bronchial wall, bronchial obstruction, excess viscous bronchial secretion, bronchospasm are observed. With obstruction of the small bronchi, overstretching of the alveoli on exhalation and a violation of the elastic structures of the alveolar walls and the appearance of hypoventilated or non-ventilated zones develop, and therefore the blood passing through them is not oxygenated and arterial hypoxemia develops. In response to alveolar hypoxia, a spasm of the pulmonary arterioles and an increase in total pulmonary and pulmonary arteriolar resistance develop; Pericapillary pulmonary hypertension develops. Chronic hypoxemia leads to an increase in blood viscosity, which is accompanied by metabolic acidosis, which further increases vasoconstriction in the pulmonary circulation. Inflammatory infiltration in large bronchi is superficial, and in medium and small bronchi, bronchioles - deep with the development of erosion and the formation of meso- and panbronchitis. The remission phase is manifested by a decrease in inflammation and a large decrease in exudation, proliferation of connective tissue and epithelium, especially with ulceration of the mucous membrane.

Clinical manifestations. The onset of the disease is gradual. The first and main symptom is a cough in the morning with sputum discharge, gradually the cough begins to occur at any time of the day, intensifies in cold weather and becomes constant over the years. The amount of sputum increases, the sputum becomes mucopurulent or purulent. Shortness of breath appears. With purulent bronchitis, purulent sputum may occasionally be released, but bronchial obstruction is not very pronounced. Obstructive chronic bronchitis is manifested by persistent obstructive disorders. Purulent-obstructive bronchitis is characterized by the release of purulent sputum and obstructive ventilation disorders. Frequent exacerbations during periods of cold damp weather: coughing increases, shortness of breath, the amount of sputum increases, malaise appears, fatigue. The body temperature is normal or subfebrile, hard breathing and dry rales over the entire lung surface can be determined.

Diagnostics. A slight leukocytosis with a stab-nuclear shift in the leukocyte formula is possible. With an exacerbation of purulent bronchitis, a slight change in the biochemical parameters of inflammation occurs (C-reactive protein, sialic acids, fibrogen, seromucoid, etc. increase). Sputum examination: macroscopic, cytological, biochemical. With a pronounced exacerbation, sputum acquires a purulent character: a large number of neutrophilic leukocytes, an increased content of acid mucopolysaccharides and DNA fibers, the nature of sputum, mainly neutrophilic leukocytes, an increase in the level of acid mucopolysaccharides and DNA fibers, which increase the viscosity of sputum, a decrease in the amount of lysozyme, etc. Bronchoscopy, which evaluates the endobronchial manifestations of the inflammatory process, the stages of development of the inflammatory process: catarrhal, purulent, atrophic, hypertrophic, hemorrhagic and its severity, but mainly to the level of subsegmental bronchi.

Differential diagnosis is carried out with chronic pneumonia, bronchial asthma, tuberculosis. Unlike chronic pneumonia, chronic bronchitis always develops with a gradual onset, with widespread bronchial obstruction and often emphysema, respiratory failure and pulmonary hypertension with the development of chronic cor pulmonale. In X-ray examination, the changes are also diffuse in nature: peribronchial sclerosis, increased transparency of the lung fields due to emphysema, expansion of the branches of the pulmonary artery. Chronic bronchitis differs from bronchial asthma in the absence of asthma attacks, with pulmonary tuberculosis it is associated with the presence or absence of symptoms of tuberculosis intoxication, Mycobacterium tuberculosis in sputum, the results of X-ray and bronchoscopic examination, tuberculin tests.

Treatment. In the phase of exacerbation of chronic bronchitis, therapy is aimed at eliminating the inflammatory process, improving bronchial patency, as well as restoring disturbed general and local immunological reactivity. Assign antibiotic therapy, which is selected taking into account the sensitivity of the sputum microflora, administered orally or parenterally, sometimes combined with intratracheal administration. Showing inhalation. Apply expectorant, mucolytic and bronchospasmolytic drugs, drink plenty of water to restore and improve bronchial patency. Phytotherapy using marshmallow root, coltsfoot leaves, plantain. Assign proteolytic enzymes (trypsin, chymotrypsin), which reduce the viscosity of sputum, but are now rarely used. Acetylcysteine ​​has the ability to break the disulfide bonds of mucus proteins and contributes to a strong and rapid liquefaction of sputum. Bronchial drainage improves with the use of mucoregulators that affect the secretion and production of glycoproteins in the bronchial epithelium (bromhexine). In case of insufficiency of bronchial drainage and existing symptoms of bronchial obstruction, bronchospasmolytic agents are added to the treatment: eufillin, anticholinergics (atropine in aerosols), adrenostimulants (ephedrine, salbutamol, berotek). In a hospital setting, intratracheal lavages for purulent bronchitis must be combined with sanitation bronchoscopy (3-4 sanitation bronchoscopy with a break of 3-7 days). When restoring the drainage function of the bronchi, physiotherapy exercises, chest massage, and physiotherapy are also used. With the development of allergic syndromes, calcium chloride and antihistamines are used; if there is no effect, a short course of glucocorticoids can be prescribed to relieve the allergic syndrome, but the daily dose should not be more than 30 mg. The danger of activation of infectious agents does not allow the use of glucocorticoids for a long time. In patients with chronic bronchitis, complicated respiratory failure and chronic cor pulmonale, the use of veroshpiron (up to 150-200 mg / day) is indicated.

The food of patients should be high-calorie, fortified. Apply ascorbic acid 1 g per day, nicotinic acid, B vitamins; if necessary, aloe, methyluracil. With the development of complications of such a disease as pulmonary and pulmonary heart failure, oxygen therapy, auxiliary artificial ventilation of the lungs are used.

Anti-relapse and maintenance therapy is prescribed in the phase of exacerbation subsidence, is carried out in local and climatic sanatoriums, this therapy is prescribed during medical examination. It is recommended to allocate 3 groups of dispensary patients.

1st group. It includes patients with cor pulmonale, with severe respiratory failure and other complications, with disability. Patients are prescribed maintenance therapy, which is carried out in a hospital or by a local doctor. Inspection of these patients is carried out at least once a month.

2nd group. It includes patients with frequent exacerbations of chronic bronchitis, as well as moderate respiratory dysfunction. Inspection of such patients is carried out by a pulmonologist 3-4 times a year, anti-relapse therapy is prescribed in autumn and spring, as well as in case of acute respiratory diseases. An effective method of administering drugs is the inhalation route; according to indications, it is necessary to sanitize the bronchial tree using intratracheal lavage, sanitation bronchoscopy. In case of active infection, antibiotics are prescribed.

3rd group. It includes patients in whom anti-relapse therapy led to a cessation of the process and the absence of relapses for 2 years. Such patients are shown preventive therapy, which includes funds aimed at improving bronchial drainage and increasing its reactivity.

Author: Pavlova N.V.

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