Free technical library

Faculty pediatrics. Cheat sheet: briefly, the most important

Directory / Lecture notes, cheat sheets

Comments on the article

1. Types of diathesis

The constitution is a set of relatively stable morphological and functional properties of a person, due to heredity, age and long-term intense environmental influences, which determines the functionality and reactivity of the body.

Diathesis is a genetically determined feature of the organism, which determines the originality of its adaptive reactions and predisposes to a certain group of diseases. Diathesis is not a disease, but a predisposition that, under certain environmental conditions, can transform into a disease.

Allergic diathesis is an anomaly of the constitution, characterized by the predisposition of the body to allergic reactions and inflammatory diseases. Allergic diathesis is a common anomaly of the constitution. Allergic diathesis is detected at the age of 3-6 months and persists for 1-2 years and disappears in most children in the future. The following types of allergic diathesis according to I. M. Vorontsov are distinguished: atopic diathesis, autoimmune, infectious-allergic.

Atopic diathesis - diathesis, manifested by excessive synthesis of immunoglobulin E, an increase in the number of T-helpers, an imbalance in the production of interleukins, a deficiency of total and secretory immunoglobulin A, and a lack of phagocytic activity of neutrophils and macrophages. At the same time, in the history of the child's parents, there are positive allergological data on the maternal and paternal lines.

Autoimmune diathesis - diathesis, in which there is an increased sensitivity of the skin to UV radiation, a significant increase in the level of g-globulins in the blood, the frequent detection of LE cells, antinuclear factors, polyclonal activation of B-lymphocytes, as well as T-helpers with a decrease in the activity of T-suppressors, an increase in the level of immunoglobulins M in the blood in a state of complete clinical well-being.

Infectious-allergic diathesis - diathesis, in which there are long periods of increased ESR and subfebrile temperature, after acute respiratory viral infections and diseases of the nasopharynx.

Clinical manifestations

In the first year of life, children with allergic diathesis have signs of atopic dermatitis. Children have increased nervous excitability, irritability, reduced appetite, disturbed sleep. With a deficiency of immunoglobulin A, chronic foci of infection develop, an increase in peripheral lymph nodes, spleen, prolonged subfebrile conditions, and a protracted course of infectious diseases.

Diagnosis

It is established on the basis of anamnestic data and should not be used as a nosological one.

Prevention

It should be comprehensive and begin in the antenatal period with the exclusion of allergic foods and drugs from the diet of a pregnant woman.

2. Exudative catarrhal diathesis

Exudative-catarrhal diathesis is a peculiar state of reactivity in young children, characterized by a tendency to recurrent infiltrative-desquamous lesions of the skin and mucous membranes, the development of pseudo-allergic reactions and a protracted course of inflammatory processes, lymphoid hyperplasia, and lability of water-salt metabolism.

Etiology and pathogenesis have not been fully studied, but a special role is played by hereditary predisposition, as well as the influence of environmental factors.

Clinical manifestations. Such children are characterized by a large body weight at birth (especially if the child is the first in the family); diaper rash that appears early and persists for a long time under conditions of proper care; disappearing and reappearing seborrhea of ​​the scalp; as well as an increase in body weight that is significantly higher than the age norm, which can fluctuate under the influence of adverse conditions. It is possible to determine positive skin tests for exogenous allergens, even before various clinical manifestations of allergic diathesis. An objective examination of the child draws attention to a puffy pale face, a decrease in tissue turgor (pastose type), thinness or overweight, geographical tongue, abdominal pain, flatulence. In the first year of life, children have increased nervous excitability, irritability, sleep disorders appear, appetite is reduced, children become capricious and nervous. Children do not tolerate heavy physical exertion. Often, children with immunoglobulin A deficiency develop chronic foci of infection, enlarged peripheral lymph nodes, spleen, prolonged subfebrile conditions appear, and infectious diseases become protracted. Only in infants can one find dirty gray or brown seborrheic scales, resembling a cap or shell on the scalp (gneiss), most often they are located on the crown and crown. The course of gneiss is usually favorable, but in some children it turns into seborrheic eczema (redness, swelling, weeping, increased crusting), which is observed in older children. In children of the older age group, eczema can transform into neurodermatitis.

The diagnosis is established on the basis of the anamnesis and clinical data. Differential diagnosis is carried out with erythroderma, true eczema, dermatitis, immunodeficiency, psoriasis, and malabsorption syndrome.

Treatment. There is no special diet in the treatment of exudative-catarrhal diathesis. Rational nutrition is needed. Breastfeeding is best continued. In the mother's diet, it is necessary to reduce the amount of fats, carbohydrates, salt, and, if possible, allergens. Used in the treatment of drugs that help reduce itching and permeability of the vascular wall, sedatives: 3-5% sodium bromide solution, diphenhydramine or antihistamines at an age dosage. Vitamins B5, B6, B12, B15, A, C are also prescribed in therapeutic doses for at least 3-4 weeks.

3. Lymphatic diathesis

Lymphatic diathesis (lymphatic-hypoplastic) is a hereditary insufficiency of the lymphatic system associated with a decrease in the function of the thymus gland as the main organ that controls the maturation of lymphocytes. The disease is manifested by a generalized persistent enlargement of the lymph nodes, dysfunction of the endocrine system (decrease in the function of the adrenal glands, sympathoadrenal system, etc.), a tendency to allergic and hyperergic reactions, as well as to infectious diseases. One of the manifestations of lymphatic diathesis may be a violation of local immunity of the mucous membranes, reduced production of secretory immunoglobulins.

Clinical manifestations. Dysplastic physique in the form of a short torso, several elongated limbs. The skin is pale, the skin fold is flabby, the muscles are poorly developed, its tone is lowered, and the tissues are pasty. Lymph nodes are enlarged, adenoids and tonsils are hyperplastic, loose. Adenoids after adenotomy tend to re-grow. An x-ray examination in 70% of cases reveals an enlarged thymus gland, which can cause a violation of bronchial conduction. There is a tendency to arterial hypotension. In the blood, a slight leukocytosis, lymphocytosis, monocytosis, anemia, neutropenia.

The diagnosis is established on the basis of the anamnesis, the characteristic appearance of the child, when hyperplasia of the lymph nodes and thymus gland is detected. Differential diagnosis is carried out with immunodeficiency states.

Treatment. Compliance with the daily routine, sufficient exposure to fresh air, hardening procedures, massage, gymnastics, physiotherapy, vitamin therapy. Appointment of adaptogens and agents that stimulate the body's defenses and adrenal function (dibazole, metacil, aloe, eleutherococcus, ginseng). Periodically prescribe vitamin therapy, calcium preparations. With persistent viral infection and the presence of foci of chronic infection, a course of viferon is prescribed. Adenoid growths should be removed surgically only in the complete absence of nasal breathing, with frequent relapses of inflammation of the respiratory system.

Preventive vaccinations for exudative-catarrhal diathesis are done at the usual time with preliminary preparation.

Prevention. Rational nutrition of a pregnant woman and proper feeding of a child adequate to age. It is necessary to observe the daily routine: walking, hardening, rest, massage, gymnastics. Mandatory use of adaptogen plants (Eleutherococcus, etc.) in combination with vitamins in separate courses for 2 weeks.

The prognosis, subject to therapeutic and preventive measures, is favorable.

4. Nervous-arthritic diathesis

Nervous-arthritic diathesis is characterized by increased nervous excitability, a tendency to ketoacidosis, and further predisposition to the development of obesity, interstitial nephritis, urolithiasis, atherosclerosis, diabetes mellitus, and gout. Violation of uric acid metabolism is the leading, but not the only laboratory marker.

Etiology. In the formation of the disease, on the one hand, the inheritance of pathological properties of metabolism is involved, on the other hand, food in the family, regime, environment.

Pathogenesis. The following disorders are of particular importance:

1) a high level of excitability at any level of reception;

2) violation of the metabolism of purines with an increase in their concentration in the blood and urine;

3) low acetylating ability of the liver and other, undeciphered mitochondrial defects.

Clinical manifestations. Already in infancy, increased nervous excitability is noted, which increases even more over time. Mental development is ahead of age norms: children are inquisitive, lively, remember what they heard or read. Sometimes these children have night terrors, tics, choreo-like seizures, and emotional lability. Periodically or suddenly after a short malaise, attacks of headache, nausea, vomiting, abdominal pain, the smell of acetone from the mouth may occur, an acetone crisis develops. During the crisis in the blood test, the level of ketone bodies, ammonia, uric acid is increased, and the pH decreases.

The diagnosis is established on the basis of anamnesis, clinical and laboratory data.

Differential diagnosis. It should be distinguished from neurosis, rheumatism, diabetes, etc.

Treatment. Rational regimen and diet, hardening, walking, physical education. Exclusion of mental stress, limit TV viewing. With the initial symptoms of an acetone crisis or its precursors, sweet tea, fruit juices, alkaline mineral waters are given to drink. It is advisable to hospitalize the child in a hospital. They do a cleansing enema, gastric lavage is done to better remove ketone bodies from the intestines. Apply Essentiale or vitamin B₁₂. With acetonemic vomiting, treatment is aimed at combating acytosis (removal and elimination of ketone bodies): a solution of 5% glucose, 0,9% sodium chloride solution is injected. At a blood pH below 7,2, a 4% solution of sodium bicarbonate is administered.

Prevention. Compliance with the daily routine of the child, rational nutrition, protecting the child from mental stress.

5. Rickets

Rickets is a disease of infants and young children with a disorder of bone formation and insufficiency of bone mineralization. The cause of rickets is a deficiency of vitamin D and its active metabolites during the most intensive growth of the body.

Clinical manifestations. According to severity, the following degrees of rickets are distinguished.

I degree - mild: small changes appear on the part of the nervous and muscular systems, there are no residual changes.

II degree - moderate severity: there are pronounced changes in the bone, muscle, nervous and hematopoietic systems, there are moderately pronounced changes in the internal organs and a violation of their function, a slight increase in the size of the liver and spleen, anemia.

III degree - severe: pronounced disorders of the central nervous, skeletal, muscular systems and internal organs.

According to the nature of the course, acute, subacute, recurrent rickets are distinguished. During the course of the disease, periods are distinguished: initial, peak, convalescence, residual effects. The initial period is more often noted on the 2-3rd month, but it can also occur throughout the first year of life. On the part of the autonomic nervous system - sweating, nape baldness, on the part of the nervous system - anxiety, irritability, tearfulness, disturbing sleep, muscular dystonia; unsharp softening of the edges of the large fontanel and bones along the swept and lambdoid sutures.

differential diagnosis.

It is carried out with a number of rickets-like pathologies of a hereditary nature - phosphate diabetes, renal tubular acidosis, Debre de Toni - Fanconi syndrome, as well as with congenital dislocation of the femur, chondrodystrophy, osteopathy in chronic renal failure, congenital bone fragility.

Treatment. In the diet of the child, it is necessary to introduce fruits and vegetables in a timely manner. Complementary foods should contain a sufficient amount of vitamins, salts, and foods containing natural vitamin D3 are also needed. It is important to normalize the daily regimen with sufficient exposure to fresh air, conduct massage courses, and gymnastics. At the initial clinical manifestations, full-term children should be prescribed vitamin D2 preparations at 300-800 IU / day, for a course of 400-000 IU; during the peak of the disease with moderate and severe rickets, it is recommended to prescribe 600-000 IU / day in 10-000 doses, for a course of therapy 16-000 IU.

During treatment, it is necessary to control the sensitivity of the child's body to vitamin D using Sulkovich's tests to prevent hypervitaminosis. It is recommended to use ultraviolet therapy, which has a beneficial effect in the initial period of the development of the disease and in the subacute course of rickets in young children. Massage and gymnastics are used at any time, but not in acute cases.

6. Hypotrophy

Hypotrophy is a chronic eating disorder that manifests itself in varying degrees of weight loss.

Hypotrophy classification:

1) congenital (prenatal);

2) acquired (postnatal).

Congenital malnutrition is most often caused by maternal diseases or associated with intrauterine hypoxia, infectious lesions of the fetus, genomic and chromosomal mutations. The causes of acquired malnutrition can be exogenous and endogenous.

The pathogenesis of malnutrition is based on a decrease in the utilization of nutrients with a violation of the processes of digestion, absorption and assimilation under the influence of various factors.

Clinical manifestations. Allocate I, II, III severity of malnutrition.

Hypotrophy of the 20st degree is manifested by weight loss of no more than XNUMX% of the weight due to age. There is pallor of the skin and mucous membranes. The subcutaneous fat layer on the abdomen becomes thinner, turgor and tissue elasticity decrease. The growth of the child does not lag behind the norm.

With malnutrition of the II degree, the loss of body weight is 25-30% when compared with the age norm. The subcutaneous layer remains only on the face, especially thinned on the abdomen and limbs. The skin is dry, pale, flabby, easily folded, hanging down in some places, tissue turgor is reduced. There is a lag in growth, a decrease in appetite, the child becomes weak, irritable, noisy, whiny, loses previously acquired skills, thermoregulation is disturbed, and therefore there is a rapid cooling or overheating. The chair is unstable.

With malnutrition of the III degree, the loss of body weight is more than 30% of that due to age. An increase in body weight does not occur, the child is severely stunted. Outwardly - an extreme degree of exhaustion, the child resembles a skeleton covered with skin. The skin is pale gray, dry, the limbs are cold, the subcutaneous fat layer is completely absent. The skin fold does not straighten, there is no elasticity. The mucous membranes are pale, dry, in the mouth there are elements of candidal stomatitis (thrush). Breathing is shallow, heart sounds are muffled, blood pressure is reduced. The body temperature is lowered, periodic rises to subfebrile figures can be recorded, there is no difference between the axillary and rectal temperatures. Infectious processes proceed with few symptoms. Often there are signs of subacute rickets. The abdomen is distended, distended, or bowel loops are contoured. The stool is unstable, often constipation, alternating with soapy-calcareous stools.

Treatment of patients should be comprehensive and include measures that are aimed at eliminating or correcting particularly significant factors, diet therapy, the appointment of restorative procedures, enzymes, symptomatic agents, vitamin therapy, and the elimination of foci of infection.

7. Vitamin A deficiency

Hypovitaminosis is a group of diseases that are caused by a deficiency in the body of one or more vitamins.

There are primary hypovitaminosis (exogenous, which are caused by a deficiency in the intake of vitamins from food) and secondary (endogenous, which are associated with a change in the absorption of vitamins in the gastrointestinal tract or their absorption, as well as an increased need for vitamins in the treatment of certain antibiotics).

Vitamin A deficiency (retinol deficiency) develops with a lack of vitamin A and carotene in food, a violation of its absorption in the intestine and the synthesis of vitamin A from carotene in the body. Vitamin A is found in a large number of different animal products (butter, egg yolk, liver, some fish and marine animals); plant foods contain carotene and are provitamin A, from which vitamin A is formed in the body, the need for an adult is 1,5 mg (5000 IU). Vitamin A normalizes metabolism, promotes the growth and development of the body, affects the physiological functions of the epithelium of the skin and mucous membranes, sebaceous, sweat, lacrimal glands, and the organ of vision.

Clinical manifestations in the form of hemeralopia (night or "chicken" blindness due to degenerative changes in the retina and optic nerves), xerophthalmia (dryness of the conjunctiva, the formation of whitish opaque plaques on it), keratomalacia (corneal ulceration), hyperkeratosis (dystrophic changes in the epithelium of the skin, mucous membranes and skin glands, dryness, peeling and pallor of the skin, keratinization of hair follicles; atrophy of sweat and sebaceous glands, etc.), a tendency to pustular skin diseases, infectious lesions of the respiratory system, gastrointestinal tract, urination , general malaise, weakness. In children, there is a delay in growth and development, neurological disorders.

The diagnosis is established by studying the anamnesis, clinical and laboratory data. In a biochemical blood test, the concentration of retinol, with a lack of vitamin A is below 100 μg / l, carotene is below 200 μg / l, an ophthalmological examination can determine the time of dark adaptation.

Treatment. Good nutrition with the mandatory inclusion of foods rich in retinol and carotene, prescribe retinol preparations depending on the severity of vitamin deficiency for 2-4 weeks under close medical supervision, since large doses and uncontrolled treatment can cause hypervitaminosis A. With hemeralopia, xerophthalmia and retinitis pigmentosa at the same time prescribed riboflavin.

Prevention. A varied diet with the inclusion of foods rich in retinol and carotene, with a forced monotonous diet, retinol is additionally prescribed in 1-2 tablets (3300-6600 IU).

8. Vitamin B deficiency₁

Deficiency of vitamin B1 (thiamine) occurs when there is a deficiency of vitamin B1 in food, mainly in countries where polished rice predominates in the diet (in East and Southeast Asia), impaired absorption in the intestines and impaired absorption in severe intestinal diseases that occur with malabsorption, prolonged vomiting, diarrhea, etc. Predispose to the occurrence of the disease pregnancy, lactation, heavy physical exertion, feverish conditions, diabetes mellitus, thyrotoxicosis. The daily requirement of an adult for vitamin B is approximately 2 mg. Vitamin B is part of some enzymes that are involved in carbohydrate metabolism; in the human body, vitamin B is converted into cocarboxylase, which is a prosthetic group of enzymes involved in carbohydrate metabolism. With the development of a complete clinical picture of avitaminosis B, the concomitant lack of other B vitamins is of great importance.

Clinic. It is manifested by a common lesion of the peripheral nerves (polyneuritis), the cardiovascular system and edema. There are general weakness, fatigue, loss of appetite, shortness of breath, palpitations during exercise. Then symptoms of polyneuritis, paresthesia, decreased skin sensitivity in the legs, and then in other parts of the body, a feeling of heaviness and weakness in the lower extremities, lameness, fatigue when walking join. The calf muscles become hard, painful on palpation. The characteristic gait of patients with beriberi: they step on the heel and then on the outer edge of the foot, sparing the fingers. Then tendon reflexes fade, muscle atrophy develops. The activity of the cardiovascular system is disturbed, tachycardia appears, dystrophic changes in the heart muscle with circulatory failure, a decrease in blood pressure, mainly diastolic. There are symptoms of dystrophic lesions and disorders of the functioning of the organs of the gastrointestinal tract, visual disturbances, mental disorders. Depending on which symptoms prevail, there is an edematous form (characterized by pronounced lesions of the cardiovascular system and edema) and dry (with a predominant lesion of the nervous system), as well as an acute, malignant "lightning" form, often ending in death. It is very difficult to recognize beriberi disease in young children.

Treatment. Complete food rich in vitamin B. In severe and moderate cases - in the hospital. Bed mode. Vitamin B is used, 30-50 mg intramuscularly or s / c, then they are switched to oral administration; at the same time, nicotinic acid (25 mg), riboflavin (10-20 mg), vitamin B6 are prescribed.

Symptomatic therapy: cardiovascular agents, diuretics, strychnine injections (1: 1000, 1-1,5 mg) are used to increase the tone of the nervous system. With endogenous vitamin B deficiency caused by chronic bowel diseases - their treatment.

Prevention. A varied diet with the inclusion of foods rich in thiamine.

9. Deficiency of vitamins B2 and B6

Deficiency of vitamin B2 (riboflavin) occurs when there is a lack of vitamin B2 in food, when its absorption and assimilation is disturbed, or when it is highly destroyed in the body. Riboflavin is found in large quantities in animal and vegetable products. The daily requirement of an adult for riboflavin is 2-3 mg.

Clinic. It is manifested by a decrease in appetite, weight loss, headache, weakness, impaired twilight vision, degenerative changes in the skin and mucous membranes, a burning sensation of the skin and pain in the eyes, the appearance of conjunctivitis, angular and aphthous stomatitis, seborrheic dermatitis, especially pronounced on the wings of the nose, in the area of ​​nasolabial folds, on the ears, dry itchy dermatitis on the hands. With a long course of the disease, disorders of the nervous system appear, represented by paresthesia, increased tendon reflexes, ataxia, and hypochromic anemia. The course is chronic, with relapses in the spring and summer months.

Treatment. Riboflavin is administered orally at 10-30 mg for a course of 2-4 weeks. At the same time, other B vitamins are used. With endogenous deficiency - treatment of intestinal diseases, with existing malabsorption.

Deficiency of vitamin B6 (pyridoxine) is observed only in an endogenous form with suppression of the bacterial flora of the intestine, with prolonged use of antibiotics, sulfanilamide and anti-tuberculosis drugs, especially with an increased need for this vitamin during significant physical exertion, during pregnancy. Vitamin B6 is found in sufficient quantities in animal and vegetable products, especially in yeast. In the body, pyridoxine is converted to pyridoxal-5-phosphate and is part of the enzymes that are involved in the decarboxylation and transamination of amino acids, in the metabolism of histamine, and in fat metabolism. The daily requirement for pyridoxine in an adult is 2-2,5 mg.

Clinic. Irritability or lethargy, polyneuritis of the upper and lower extremities, paresthesia, insomnia, dyspeptic disorders, anorexia, stomatitis, seborrheic and desquamative dermatitis of the face or scalp, neck appear, hypochromic anemia develops, dystrophic changes occur in the cells of various organs, most often digestive and nervous system, skin; young children have growth retardation.

Treatment. Pyridoxine preparations 10-100 mg per day for 2-3 weeks orally, for chronic diseases of the gastrointestinal tract, parenterally; as well as the elimination of etiological factors.

10. Nicotinic acid deficiency

Deficiency of nicotinic acid (vitamins PP, B3, etc.) is due to insufficient intake of this vitamin from food or insufficient absorption in the intestines in various diseases of the stomach, small intestine, diseases accompanied by absorption deficiency syndrome, increased need for it (hard physical work, pregnancy and etc.).

The clinic is manifested by damage to the digestive, nervous system and skin. Decreased appetite, dryness and burning sensation in the mouth, nausea, vomiting, diarrhea alternating with constipation, general increasing weakness are characteristic. Tongue bright red, swollen, with painful ulcerations. Atrophic and erosive changes are also detected in other parts of the digestive tract. With damage to the nervous system, general irritability, symptoms of polyneuritis, and sometimes signs of damage to the spinal cord (usually posterolateral columns) appear. Skin lesions are manifested by erythema pellagra, the presence of pruritus, hyperpigmentation (mostly in open areas of the body and extremities), skin peeling, and the appearance of follicular papules. The function of the endocrine system is disturbed, hypoproteinemia develops. In severe cases, secretion is suppressed by the symptoms of pellagra, which is most common in Asia and Africa. Nicotinic acid and its amide are an effective antipellagric agent involved in cellular respiration. With their deficiency in the body, significant metabolic disorders develop, the functions of many organs, degenerative and dystrophic changes in organs.

The diagnosis is established on the basis of anamnesis, clinical and laboratory data: biochemical studies are characterized by the concentration of 1\N-methylnicotinomide in daily urine below 4 mg, in hourly urine - below 0,3 mg, the content of nicotinic acid is below 0,2 mg. In the blood and urine, the content of other B vitamins is reduced.

Treatment in severe and moderate cases in a hospital with full fractional nutrition with a gradual increase in calorie content, nicotinic acid or nicotinamide is administered orally at 25-100 mg / day for 2-3 weeks when combined with other B vitamins. In endogenous forms, parenteral administration is prescribed nicotinic acid and its amide.

Prevention consists in a varied balanced diet with a sufficient content of foods rich in nicotinic acid (chicken, meat, legumes, liver, green vegetables, fish). In the endogenous form - timely diagnosis and treatment of intestinal pathology, prophylactic use of nicotinic acid and its amide; additional use of vitamin PP for persons with an increased need for it.

11. Deficiency of vitamins C, D, K

Vitamin C plays an important role in redox processes, in carbohydrate metabolism, in the synthesis of collagen and procollagen, and in the normalization of vascular permeability.

Clinical manifestations begin with general weakness, increased fragility of capillaries with the formation of petechiae, increased bleeding of the gums, hemorrhagic effusions in the joints and pleura, dystrophic changes in the mucous membranes, anemization, and a violation of the nervous system.

Treatment. Complete nutrition and the appointment of drugs containing vitamin C.

Prevention. A complete balanced diet, prophylactic intake of ascorbic acid, when there is a possibility of developing its insufficiency (on long hikes with a predominant use of canned and dried foods, women during pregnancy and lactation, etc.); proper cooking, preventing the loss of ascorbic acid during cooking.

Of great importance is the deficiency of vitamin D2 (er-gocalciferol) and vitamin D3 (cholecalciferol-la). A large amount of vitamin D is formed in the skin when it is exposed to light, ultraviolet rays, and only a small part comes from food. In the human body, vitamin D is involved in the regulation of calcium and phosphorus metabolism. With a deficiency of vitamin D in food products, as well as with insufficient exposure to sunlight on the skin, a clinical picture of deficiency of this vitamin develops in the form of rickets

When establishing a diagnosis in an adult, biochemical laboratory data will be of great importance (a decrease in the concentration of inorganic phosphorus in the blood below 30 mg / l; an increase in the activity of alkaline phosphatase).

Treatment. Preparations of vitamins D2 and D3 in combination with calcium preparations and ultraviolet irradiation. Then they are transferred to dispensary observation and the courses of treatment are repeated (in case of an overdose, intoxication may occur).

Vitamin K deficiency occurs in children and is rare in adults. It is caused by the cessation of the flow of bile into the intestine, which is necessary for the absorption of phylloquinones, with obstruction and compression of the biliary tract, as well as with chronic bowel diseases, which are accompanied by absorption deficiency syndrome. It is also observed with an overdose of dicoumarin. Vitamin K enters the body with food and is formed in small amounts by the intestinal microflora.

Clinical manifestations in the form of hemorrhagic syndrome, which is manifested by bleeding from the nose, gums, gastrointestinal tract, (intradermal and subcutaneous hemorrhages).

The diagnosis is based on clinical observation data (diseases leading to vitamin K deficiency), as well as laboratory biochemical studies: hypoprothrombinemia below 30-35%, proconvertin deficiency, as well as factors IX and X are typical.

Treatment is carried out in a hospital with vitamin K - vikasol.

12. Open arterial (Botallov) duct

Congenital heart defects with enrichment of the pulmonary circulation are similar to hemodynamic disorders, when more than normal amount of blood enters the pulmonary circulation. Features in these hemodynamic disorders: the development of hypervolemia and hypertension in the pulmonary circulation.

There are three phases in the development of pulmonary hypertension.

I - hypervolemic, when there is a discrepancy between the vascular bed and the volume of flowing blood, but there is no spasm of arterioles.

II - mixed; spasm of the pulmonary vessels, accompanied by an increase in pressure in the pulmonary artery and an increase in pulmonary resistance.

III - sclerotic; hypervolemia and spasm of the pulmonary vessels, which cause sclerotic changes in the pulmonary vessels.

An open arterial (Botallov) duct is a defect due to non-closure after the birth of a child of a vessel connecting the aorta to the pulmonary trunk in the fetus, which can be combined with other congenital defects, often with a ventricular septal defect. Hemodynamic disorders are characterized by loss of blood from the aorta to the pulmonary artery and lead to hypervolemia of the pulmonary circulation and overload of both ventricles of the heart. The discharge from the aorta occurs during systole and the onset of diastole.

Clinical manifestations with a small section of the duct may be absent for a long time; children are developing normally. With a large defect, changes appear earlier, the child lags behind in development, fatigue appears, a tendency to respiratory infections, shortness of breath on exertion, pulmonary hypertension and heart failure develop. Blood pressure rises due to an increase in systolic and a decrease in diastolic blood pressure. A continuous systole-diastolic murmur appears during auscultation in the II intercostal space to the left of the sternum, which decreases with a deep breath, and increases with holding the breath on exhalation. A mitral valve murmur may be heard above the apex of the heart. The weakening of the noise and the increased emphasis of the II tone on the pulmonary artery are symptoms indicating the approach of the terminal phase of the defect.

Diagnosis: X-ray examination reveals an increase in the left heart. The trunk of the pulmonary artery is dilated, and the arch of the pulmonary artery bulges. On the ECG, the electrical axis of the heart is normal or deviated to the left. There are signs of left ventricular hypertrophy. The defect is confirmed by aortography (a discharge of contrast through the duct is visible) and catheterization of the pulmonary trunk and heart (an increase in pressure and blood oxygen saturation in the pulmonary trunk is observed).

Surgical treatment - ligation of the open ductus arteriosus.

13. Ventricular septal defect

The ventricular septal defect is localized in the membrane or muscular part of the septum, but it also happens that the septum is absent. If the defect is located above the supragastric ridge, at the aortic root or directly in it, then this defect is accompanied by aortic valvular insufficiency. Hemodynamic disorders are determined by its size and the ratio of pressure in the systemic and pulmonary circulation. Small defects (0,5-1 cm) are manifested by discharge through the defect from left to right with small volumes of blood, which does not disturb hemodynamics. The larger the defect and the more blood discharged through it, the earlier hypervolemia and hypertension appear in the pulmonary circulation, sclerosis of the pulmonary vessels with the outcome in heart failure. Severe defect proceeds with high pulmonary hypertension (Eisenmenger complex), when blood is discharged through the defect from right to left, it will be accompanied by severe arterial hypoxemia. Violations appear at the age of 2-4 months, when pulmonary vascular resistance decreases. With a small defect in the muscular part of the septum - Tolochinov-Roger disease - the defect can be asymptomatic for many years, children develop mentally and physically according to age, and can manifest themselves as characteristic frequent pneumonias. In the first 10 years of life, spontaneous closure of a small defect may occur, if the defect remains for subsequent years, then pulmonary hypertension gradually develops, which leads to heart failure. During auscultation, the intensity of the systolic murmur depends on the volume of the rate of blood discharge through the defect. As pulmonary hypertension increases, the noise weakens (may disappear completely), while there is an increase and accent of the II tone over the pulmonary trunk; some patients develop diastolic murmur.

Clinical manifestations: complaints of difficulty in breastfeeding, shortness of breath, cough, intermittent cyanosis when crying, weakness, fatigue, pulmonary infections. Children lag behind in development, inactive, pale; as pulmonary hypertension increases, shortness of breath, cyanosis during exercise, and a heart hump are formed. The borders of the heart are expanded in the transverse size and upwards. The apex beat is diffuse, rising and shifted down. When the right ventricle is overloaded, there is an epigastric pulsation. On palpation in the III-IV intercostal space to the left of the sternum, systolic trembling is determined. Auscultation in the III-IV intercostal space on the left edge of the sternum is determined by intense systolic murmur and systolic trembling. Strengthening and accent of the II heart sound above the pulmonary trunk. During auscultation of the lungs in the posterior lower sections (often on the left), congestive fine bubbling rales are heard. Patients with large septal defects, as a rule, do not survive to 1 year, as severe heart failure or infective endocarditis develops. Clinical manifestations of Eisenmenger's symptom complex: the child has crimson then purple cyanosis of the cheeks, lips, nail phalanges with the development of "drum sticks". The systolic murmur decreases, and the emphasis of the II tone on the pulmonary artery increases, shortness of breath increases, pains appear in the region of the heart.

14. Atrial septal defect

Atrial septal defect is one of the most common heart defects. Hemodynamic disorders are characterized by the discharge of blood through the existing defect from the left to the right atrium, which leads to an overload with a volume that is greater, the greater the defect of the right ventricle and pulmonary circulation. Young children have right ventricular hypertrophy and increased resistance in the vessels of the pulmonary circulation. The defect is recognized after the 2nd year of life.

Clinical manifestations of defect with a small defect may be absent. More typical is limited exercise tolerance from a young age, followed by and during exercise with shortness of breath, a feeling of heaviness, or abnormal heart rhythms, and the child may be more prone to respiratory infections. As pulmonary hypertension increases, there is a complaint of shortness of breath with little exertion, cyanosis, which at first during physical exertion is periodic, then becomes persistent, clinical manifestations of right ventricular failure gradually increase. Patients with a large defect develop a heart hump. The borders of the heart are expanded in diameter and to the right. Expansion of the vascular bundle to the left due to the enlargement of the pulmonary artery. Auscultation determines the splitting and accent of the II tone over the pulmonary trunk, in some patients a systolic murmur appears in the II-III intercostal space to the left of the sternum, which increases with holding the breath on exhalation. Perhaps the development of atrial fibrillation, which is not characteristic of other congenital heart defects.

Diagnosis: an x-ray examination can see an increase in the right atrium, the diameter of the shadow is increased due to the right sections and the pulmonary artery. On the ECG, the electrical axis of the heart is located vertically or shifted to the right. Signs of hypertrophy of the right atrium and right ventricle, there is an incomplete blockade of the right leg of the bundle of His. Sometimes there is a violation of the rhythm (flicker, atrial flutter, paroxysmal tachycardia).

Echocardiography confirms an atrial septal defect by the presence of an echo signal interruption in the part of the atrial septum distant from the atrioventricular valves.

The differential diagnosis is carried out most often with primary pulmonary hypertension (the pulmonary pattern with it is depleted) and with mitral stenosis, in contrast to which, with an atrial septal defect, there is no significant dilatation of the left atrium; in addition, mitral stenosis is reliably excluded by echocardiography. The diagnosis is finally confirmed by atrial catheterization, as well as angiocardiography with the introduction of contrast into the left atrium.

Surgical treatment: suturing or plastic defect. Patients without surgery live an average of about 40 years.

15. Pulmonary stenosis and tetralogy of Fallot

Pulmonary artery stenosis may be isolated or combined with other anomalies, in particular septal defects. Often isolated stenosis of the pulmonary artery is valvular, sometimes there is a subcapped isolated stenosis or stenosis due to hypoplasia of the valve ring. There may be dysplasia of the pulmonic valve leaflets, which are thickened, rigid, and may have calcium deposits. Hemodynamic disorders are manifested by a decrease in blood flow to the pulmonary circulation, determined by high resistance to blood flow into the stenosis zone, which leads to overload, hypertrophy, dystrophy and decompensation of the right ventricle of the heart.

Clinical manifestations depend on the degree of stenosis. An early symptom is shortness of breath on exertion, fatigue, dizziness. Subsequently, there are pains behind the sternum, palpitations, increased shortness of breath. With severe stenosis, right ventricular heart failure occurs early with peripheral cyanosis. Diffuse cyanosis indicates interatrial communication. Cyanosis appears with the development of chronic heart failure. An objective examination can reveal signs of right ventricular hypertrophy (cardiac impulse, often a heart hump, palpable ventricular pulsation in the epigastric region), expansion of the boundaries of the heart in a transverse size. During auscultation in the II intercostal space, a rough systolic murmur and systolic trembling are heard in the II intercostal space to the left of the sternum, you can listen to the splitting of the II tone with its weakening over the pulmonary artery, the I tone at the apex is enhanced.

Surgical treatment with valvoeloplasty, which in tetralogy of Fallot can be combined with the closure of the interatrial communication. Valvulotomy is less effective than valvuloplasty.

Tetralogy of Fallot is a complex congenital heart disease that is characterized by a combination of pulmonary stenosis with a large ventricular septal defect and aortic dextroposition, as well as severe right ventricular hypertrophy. Hemodynamic disturbances can be determined by pulmonary stenosis and ventricular septal defect. An insufficient amount of blood in the pulmonary circulation is characteristic, and venous blood is discharged into the pulmonary circulation through a ventricular septal defect into the aorta, which is the cause of diffuse cyanosis.

The clinical picture of the defect is formed in children of early childhood. In children with a severe form of Fallot's tetrad, the appearance of diffuse cyanosis can be detected in the first months after birth: at first it appears when crying, screaming, but after a while it becomes persistent. More often, cyanosis is detected at the time when the child begins to walk, but there are cases of late detection, already at the age of 6-10 years. For a severe course, attacks of a sharp increase in shortness of breath and cyanosis are characteristic, which can lead to coma and death from disorders of cerebral circulation.

Treatment of such patients can be palliative - this is the imposition of aortopulmonary anastomoses. Radical correction of the defect consists in eliminating stenosis and closing the defect of the interventricular septum.

16. Stenosis and coarctation of the aorta

Aortic stenosis, depending on its location, is divided into supravalvular, valvular and subvalvular. Hemodynamics: a small amount of blood enters the aorta. Violations are formed in connection with the obstruction of blood flow from the left ventricle to the aorta, which creates a pressure gradient between them. The level of overload of the left ventricle depends on the degree of development of stenosis, which leads to its hypotrophy, and decompensation develops already in the later stages of the defect. Supravalvular stenosis is usually accompanied by a change in the intima of the aorta, which can spread to the brachycephalic arteries and the orifices of the coronary arteries, which impairs their blood flow.

The clinical picture is determined by the degree of stenosis. On examination, the skin is pale, the child is restless. Complaints of shortness of breath, pain in the heart. The borders of the heart are not changed. During auscultation, a rough systolic murmur on the right and systolic trembling are heard in the II intercostal space. With subvalvular stenosis, a systolic murmur is heard in the III-IV intercostal space to the left of the sternum, the I tone is increased.

On the ECG - signs of hypertrophy of the left ventricle, left atrium, rhythm disturbance. The electrical axis of the heart is located normally or deviated to the left. Echocardiography reveals deformed aortic valve leaflets protruding into the aortic lumen.

Coarctation of the aorta is a narrowing of the aorta at the border of its arch and the descending section, usually below the origin of the left subclavian artery.

There are two main types of defect:

1) children's type with an open ductus arteriosus.

Distinguish with children's type:

a) productive coarctation (higher than the discharge of the defect);

b) postductal coarctation (below the discharge of the defect);

2) adult type, or isolated coarctation of the aorta.

Clinical manifestations: from an early age, pulmonary heart failure develops, upon examination - pallor of the skin. Complaints of shortness of breath, chilliness of the feet, fatigue when walking, running, palpitations in the form of strong beats, sometimes headaches and nosebleeds. On palpation, an increased apex beat of the heart is detected, it is also possible to determine the pulsation of the intercostal arteries (collateral blood supply to the tissues is carried out through them), occasionally - systolic trembling in the II-III intercostal spaces. The borders of the heart are expanded to the left. During auscultation, a systolic murmur is heard at the base of the heart, the II tone on the aorta is increased.

The main symptom of the defect is lower blood pressure in the legs than in the arms. If arterial pressure and arterial pulse are also lowered on the left arm, then coarctation proximal to the origin of the left subclavian artery can be suspected.

On the ECG - signs of left ventricular hypertrophy, but with a defect in children, the electrical axis of the heart is usually deviated to the right.

Treatment consists of excision of the narrowed portion of the aorta, replacing it with a prosthesis or an end-to-end anastomosis, or a shunt operation. The optimal age for surgery with a favorable course of the defect is 8-14 years.

17. Infective endocarditis

Infective endocarditis (IE) is a disease characterized by an inflammatory lesion of valvular structures in the area of ​​congenital malformation in the parietal endocardium of the endothelium and great vessels. The urgency of the problem of infective endocarditis is due to the high incidence; an increase in the proportion of primary forms; an increase in the proportion of pathogens resistant to antibiotic therapy; a change in the clinical picture and an increase in the number of atypical variants of the course; serious prognosis and high mortality, ranging from 15 to 45%. In the absence of the effect of antibiotic therapy within 2 weeks, it is regarded as "uncontrolled sepsis".

According to modern concepts, sepsis is the result of an uncontrolled systemic inflammatory response caused by any bacterial, viral, fungal or protozoal disease, as well as an infectious complication.

Systemic inflammatory response syndrome is one of the common symptoms in sepsis.

Criteria for diagnosis in children: rectal body temperature above 38 °C (oral above 37,8 °C), axillary - below 35,2 °C. Tachycardia: an increase in heart rate above the upper limit of the age norm. Tachypneous: an increase in respiratory rate above the upper limit of the age norm, the number of leukocytes in the peripheral blood is more than 1210 or less than 410, or at least 10% of immature forms (total of metamyelocytes, myelocytes and stab leukocytes). The presence of SIRS criteria indicates the systemic nature of the body's response to any pathological process, but is not yet the basis for establishing a diagnosis of sepsis.

In order to confirm that a child has SIRS, at least two of the above criteria must be established.

For SIRS that developed against the background of an infection, there are three combination options.

1. SIRS + bacteremia = sepsis: a child with no obvious foci of infection developed signs of SIRS, and a positive blood culture was detected at the same time. A diagnosis of sepsis is made, which determines the indications for antibiotic therapy.

2. SIRS + local focus of infection = sepsis: in a child with a purulent-inflammatory focus of any localization, the disease is severe, SIRS joins, which means a threat of generalization - sepsis is diagnosed and treatment is intensified.

3. SIRS + clinic of infection = sepsis: a child with no obvious foci of infection developed SIRS, blood culture is negative, but there are obvious clinical symptoms of the infectious process. This is the most difficult situation to diagnose. It is set according to vital indications, since delay in prescribing antibiotic therapy can contribute to the development of septic shock with a fatal outcome, when objective evidence of sepsis will be obtained only at autopsy.

18. Diagnosis and treatment of infective endocarditis

The causes of infective endocarditis are many types of pathogens, but gram-positive cocci (streptococci, staphylococci), gram-negative bacteria, chlamydia, and viruses predominate.

The pathogenesis of infective endocarditis: a decrease in the immunobiological properties of the macroorganism, the presence of foci of infection is of particular importance. Morphological examination of the endocardium can reveal foci of ulceration with the formation of blood clots, with valvular damage, deformation of the valves develops. In acute septic endocarditis, foci of purulent fusion appear in the myocardium.

Major criteria for the diagnosis of infective endocarditis:

1) sowing from two separate blood cultures of typical pathogens (green streptococcus, enterococcus, Staphylococcus aureus);

2) echocardiographic signs of IE - vegetation on the heart valves or subvalvular structures, valvular blood regurgitation, abscess in the area of ​​the prosthetic valve.

Small Criteria:

1) previous valve damage or drug addiction (with the introduction of a drug into a vein);

2) fever over 38 °C;

3) vascular symptoms: pulmonary infarctions, arterial embolisms, mycotic aneurysms, intracranial hemorrhages, Lukin's symptom;

4) immunological symptoms in the form of manifestations of glomerulonephritis, Osler's nodules, Roth's spots, rheumatoid factor;

5) a positive result of the study on hemoculture, which does not meet the requirements of major criteria;

6) echocardiographic features that do not meet the major criteria.

Infective endocarditis is considered proven if two major criteria or one major and three minor criteria or five minor criteria are present.

Clinical and morphological classification:

1) primary IE - on intact valves;

2) secondary IE - on damaged valves, congenital heart defects, after heart surgery.

Laboratory diagnostics: in the general blood test - leukocytosis, an increase in ESR, in a biochemical blood test - an increase in C-reactive protein, globulins in the proteinogram. The causative agent of the disease is sown in the blood. With kidney damage by the type of glomerulonephritis in the urine, hematuria. Echocardiography can detect thrombotic vegetations on the valves.

The treatment is carried out for a long time with the use of antibiotics, the course is from 2 to 6 months (3rd generation cephalosporins, protected penicillins, aminoglycosides - netrimycin, etc.).

Anti-inflammatory therapy, glucocorticoids are prescribed. With thromboembolic complications, anticoagulants, heparin are prescribed. Mandatory sanitation of foci of infection of chronic tonsillitis, caries, sinusitis, etc. Indications for surgical treatment: intractable circulatory failure, persistent embolism, progressive valve destruction.

19. Classification and clinic of myocarditis

Myocarditis is an inflammation of the heart muscle, caused by infectious and non-infectious direct effects or indirectly through immune mechanisms, occurring acutely, subacutely or chronically.

Etiology of myocarditis: bacterial pathogens (diphtheria, typhoid fever, salmonellosis, tuberculosis). Fungi (aspergillosis, coccidioidomycosis, actinomycosis). There are two types of cards:

1) resulting from the impact on the body of protein drugs, physical and chemical objects (serum, medicinal, post-vaccination);

2) in diffuse connective tissue diseases (DBST): hereditary factors (genetically determined defect in antiviral immunity).

Classification of myocarditis.

1. Congenital, or antenatal, carditis:

1) early carditis: fibroelastosis - the predominance of elastic tissue or elastofibrosis - the predominance of fibrous tissue;

2) late carditis (late fetopathy) - this morphological feature is absent.

2. Acquired carditis of early age, up to 2 years.

3. Acquired carditis - from 2 to 5 years. Primary subacute myocarditis is characterized by a gradual onset, a delineated acute phase, turning into a long chronic process from 5 to 18 years.

4. Myocarditis in older children.

Classification according to the course: 1) acute course;

2) subacute course of carditis - from 5 to 18 months;

3) chronic course - more than 18 months. Options:

1) stagnant;

2) hypertrophic;

3) restrictive.

Classification according to the severity of carditis: mild, moderate, severe.

Outcomes and complications: cardiosclerosis, myocardial hypertrophy, rhythm and conduction disturbances, pulmonary hypertension, valvular lesions, thromboembolic syndrome.

The clinical picture, regardless of their cause, is the same: an increase in the size of the heart, an increase in the sonority of tones, especially the first tone, the appearance of apical systolic murmur, tachycardia, less often bradycardia, rhythm rigidity. In infectious parenchymal endocarditis, a pronounced cardiac syndrome, cardiomegaly, deafness of heart tones, and a rigid rhythm are characteristic. Total circulatory failure develops. With vascular lesions of the myocardium, pain syndrome, a violation of excitation processes are characteristic. When the myocardium is affected, vascular lesions are detected. Feature: undulating character, torpidity of the course, exacerbations with the addition of intercurrent infections.

20. Diagnosis and treatment of myocarditis

X-ray examination - expansion of the boundaries of the heart in all directions. On the ECG - rhythm disturbance, blockade.

Signs of great significance (4 points): an increase in the overall size of the heart or its cavities in the absence of effusion in the pericardium according to X-ray or ultrasound; decrease in myocardial contractility (according to echocardiography, rheography) or the presence of heart failure.

Signs of medium significance (2 points): signs of the absence of influence of the autonomic nervous system on the activity of the heart (rhythm rigidity, absence of respiratory arrhythmia), confirmed by cardiointervalography; detection of cardiac antigen and anticardiac antibodies in the blood; increase in the blood of cardiospecific fractions of isoenzymes LDH, malate hydrogenase; a complex of ECG signs of hypertrophy of the heart, ECG signs of ischemia.

Minor signs (1 point): pain in the region of the heart, weakening of the sonority of the first tone; tachy or bradycardia; apical systolic murmur; gallop rhythm; sinoauricular blockade; violation of antroventricular or intraventricular conduction; ectopic rhythm; extrasystole; shift of the interval S - T, change in the T wave.

The diagnosis of myocarditis is reliable if the total score is 5 or more; obligatory presence of at least one of the signs of great significance. Probable diagnosis if the score is 3-4; obligatory presence of a sign of average significance.

The New York Heart Association has identified an association with past infection, which was proven by clinical and laboratory data: identification of the pathogen, complement fixation reaction, results of the neutralization reaction, hemagglutination reaction, accelerated ESR, the appearance of C-reactive ESR.

Small signs: an increase in the size of the heart, tachycardia (sometimes bradycardia), a weakening of the I tone, a gallop rhythm.

Big signs: pathological changes on the ECG (impaired repolarization, rhythm and conduction disturbances), increased blood concentrations of cardio-selective enzymes and proteins (CPK, MF, LDH, troponin T). An increase in the size of the heart according to x-ray or echocardiography. Congestive circulatory failure. Cardiogenic shock.

The diagnosis of myocarditis is qualified when a previous infection is combined with one major and two minor criteria.

Treatment. Etiotropic therapy, antibiotic therapy. Pathogenetic therapy of myocarditis: NSAIDs, duration of treatment is 4-6 weeks (indomethacin, brufen, voltaren), GCS, duration of treatment is 2-5 weeks, prednisolone. Immunosuppressants (aminoquinoline drugs) duration of treatment 4-8 months (delagil, plaquenil). If complications such as heart rhythm disturbance, development of heart failure, thromboembolism occur, symptomatic therapy is prescribed, including diuretics, antiarrhythmics, thrombolytics, anticoagulants, etc.

21. Acute bronchitis

Acute bronchitis is an acute diffuse inflammation of the tracheobronchial tree.

The disease is caused by viral (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.

The damaging agent enters the trachea and bronchi with the inhaled air by hematogenous and lymphogenous routes. 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 manifestations of bronchitis of infectious etiology begin with rhinitis, nasopharyngitis, moderate intoxication, fever, weakness, feeling of weakness, soreness behind the sternum, a dry cough appears, turning into a wet cough. With a moderate course of 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 subfebrile. 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 exhalation, dry wheezing can be heard during auscultation), 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.

Treatment. Bed rest, plenty of warm drink with raspberries, honey, lime blossom. Assign antiviral and antibacterial therapy, vitamin therapy. With a strong dry cough - antitussive drugs: codeine, libexin, etc. With a wet cough - mucolytic drugs: bromhexine, ambrobene and others.

22. 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 year 1 for 2 years in a row.

The disease is associated with prolonged irritation of the bronchi by various harmful factors (smoking, 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) rarely 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.

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 the secret, 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. 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. Bronchial obstruction develops due to hyperplasia of the epithelium of the mucous glands, edema and inflammatory compaction of the bronchial wall, bronchial obstruction with an excess of viscous bronchial secretions, and bronchospasm. Violation of the elastic structures of the alveolar walls. In response to alveolar hypoxia, a spasm of the pulmonary arterioles develops and an increase in total pulmonary and pulmonary arteriolar resistance develops; Pericapillary pulmonary hypertension develops. Chronic hypoxemia leads to an increase in blood viscosity, which is accompanied by metabolic acidosis, which further increases vasoconstruction in the pulmonary circulation.

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.

23. Diagnosis and treatment of chronic bronchitis

A slight leukocytosis with a stab 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: predominantly neutrophilic leukocytes appear in it, the level of acid mucopolysaccharides and DNA fibers increases, which increase the viscosity of sputum, the amount of lysozyme decreases, etc. With the help of bronchoscopy, endobronchial manifestations of the inflammatory process, stages of development of the inflammatory process are assessed: catarrhal, purulent, atrophic, hypertrophic, hemorrhagic and severity, but mainly to the level of subsegmental bronchi.

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. Antibiotic bacterial therapy is prescribed, 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 drugs, drink plenty of water to restore and improve bronchial patency. Phytotherapy using marshmallow root, coltsfoot leaves, plantain. Assign proteolytic enzymes (trypsin, chymotrypsin) that reduce the viscosity of sputum. 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, broncho-antispasmodics are added to the treatment: eufillin, anticholinergics (atropine in aerosols), adrenostimulants (ephedrine, salbutamol, berotek). In a hospital, intratracheal lavage with 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. Patients with chronic bronchitis, complicated by respiratory failure and chronic cor pulmonale, are shown to use veroshpiron (up to 150-200 mg / day).

24. Pneumonia

Pneumonia is an inflammation of the lungs, characterized by inflammation of the parenchymal, respiratory part of the lungs.

Classification. According to the morphological form: focal, focal confluent, segmented, lobar, interstitial.

Downstream: acute, protracted (in the absence of resolution of the pneumonic process within 6 to 8 weeks).

For the development of complications:

1) pulmonary (synpneumatic pleurisy, metapneumonic pleurisy, pulmonary destruction, lung abscess, pneumothorax, pyopneumothorax);

2) extrapulmonary (toxic shock, cardiovascular insufficiency, DIC, respiratory distress syndrome).

The etiological factor is various bacteria: pneumococci, staphylococci, streptococci, Klebsiella pneumonia, gram-negative flora and mycoplasmas (community-acquired form); staphylococcus, Pseudomonas aeruginosa (nosocomial form); chlamydia, cytomegaloviruses (with perinatal infection); various bacteria in immunocompromised patients.

Acute pneumonia usually begins with an acute period, often after hypothermia, the patient begins to experience a tremendous chill: body temperature rises to febrile figures of 39-40 ° C, less often to 38-41 ° C; pain when breathing on the side of the affected lung is aggravated by coughing, at the beginning dry, then wet with purulent viscous sputum. The patient's condition is serious. The skin of the face is hyperemic and cyanotic. Breathing is rapid, shallow, with flaring of the wings of the nose. Pneumococcal pneumonia and staphylococcal pneumonia proceed similarly. More often, staphylococcal pneumonia is more severe, accompanied by destruction of the lungs with the formation of thin-walled air cavities, abscesses in the lung tissue. This type of pneumonia is characterized by severe intoxication, manifested by fever, chills, flushing of the skin and mucous membranes, dizziness, headache, severe shortness of breath, hemoptysis, tachycardia, nausea, and vomiting. If the patient has a severe infectious-toxic shock, then vascular insufficiency develops, blood pressure is 90-80 and 60-50 mm Hg. Art., when viewed from the pallor of the skin, sticky sweat, cold extremities. With the progression of the intoxication syndrome, cerebral disorders are detected, heart failure increases, the heart rhythm is disturbed, a shock lung develops, hepatorenal syndrome, DIC, toxic enterocolitis. These pneumonias can be rapidly fatal.

25. Diagnosis and treatment of pneumonia

On the basis of clinical and laboratory data, shortening of percussion sound is taken into account, increased vesicular respiration with foci of bronchial respiration is noted, crepitus, fine and medium bubbling rales are heard, and focal shading is observed on radiographs (sometimes on tomograms). Examine sputum or throat swabs for bacteria, including Mycobacterium tuberculosis, viruses, Mycoplasma pneumoniae.

Treatment of pneumonia with a mild course of the disease and with a favorable course and favorable living conditions can be treated at home, but many patients need inpatient treatment. According to emergency indications, patients are hospitalized with a lobar lesion of the lung tissue and with a pronounced infectious-toxic syndrome. At the height of the disease, bed rest is prescribed, a mechanically and chemically sparing diet with limited salt and an increase in the amount of vitamins, especially A and C. With the disappearance or significant decrease in the phenomena of intoxication, it is recommended to expand the regimen, apply physiotherapy exercises, if there are no contraindications. Immediately after taking sputum, swabs and swabs from the bronchi for bacteriological examination, etiotropic antibiotic therapy is started, which is carried out under the control of clinical efficacy, and in the subsequent treatment, the results of the study of the seeded microflora and its sensitivity to antibiotics are taken into account.

In community-acquired pneumonia, semi-synthetic penicillins, protected penicillins, new generation macrolides are prescribed. In case of nosocomial pneumonia, "protected" penicillins, aminoglycosides, 2nd-3rd generation cephalosporins, fluoroquinolones and other antibiotics of the reserve group are prescribed. In pneumonia with intrauterine infection, new generation macrolides (spiromycin, roxithromycin, azithromycin). With pneumonia in patients with immunodeficiencies, cephalosporins of the 3rd-4th generation, fluoroquinolones are prescribed.

In severe viral-bacterial pneumonia, often developing as a result of the interaction of the influenza virus and staphylococcus, along with intravenously administered broad-spectrum antibacterial drugs, the administration of a specific donor anti-influenza g-globulin is prescribed.

Combinations of antibiotics for the treatment of complicated pneumonia: cephalosporins + semi-synthetic penicillins; semi-synthetic penicillins + aminoglycosides; cephalosporins + aminoglycosides. Detoxification agents (hemodez, etc.) are also used.

Treatment of respiratory failure, elimination of obstructive syndrome. Mucolytic therapy, bronchodilator therapy, physiotherapy, immunocorrective therapy, exercise therapy are prescribed. With severe tachycardia, a decrease in systolic pressure to 100 mm Hg. Art. and below, patients with pneumonia are shown strophanthin, sulfokamphokain. Increase the immunological reactivity of the patient (immunoglobulin, dibazol, methyluracil). Vitamin therapy is carried out.

26. Bronchial asthma

Bronchial asthma is a chronic disease that occurs with relapses, with a predominant lesion of the respiratory tract, which is based on chronic allergic inflammation of the bronchi, accompanied by their hyperreactivity and intermittent attacks of shortness of breath and suffocation as a result of widespread bronchial obstruction, which is caused by bronchospasm, mucus hypersecretion, and edema. bronchial walls.

There are two forms of bronchial asthma - immunological and non-immunological - and a number of clinical and pathogenetic variants: infectious-allergic, atopic, autoimmune, adrenergic imbalance, dyshormonal, neuropsychic, primary altered bronchial reactivity, cholinergic.

Etiology and risk factors for bronchial asthma in children: atopy, bronchial hyperreactivity, heredity. Causes (sensitizing): household allergens (house dust, house dust mites), epidermal allergens of animals, birds, and other insects, fungal allergens, pollen allergens, food allergens, drugs, viruses and vaccines, chemicals.

A common pathogenetic mechanism is an altered sensitivity and reactivity of the bronchi, determined by the reaction of bronchial patency in response to the influence of physical, chemical, and pharmacological factors.

The disease often begins with a paroxysmal cough, accompanied by shortness of breath with a discharge of a small amount of vitreous sputum (asthmatic bronchitis). With the advent of asthma attacks of mild severity, moderate severity, severe severity. An asthma attack can begin with precursors: this is a copious discharge of watery secretions from the nose, sneezing, paroxysmal coughing, etc. An asthma attack is characterized by a short inhalation and a prolonged exhalation. On examination, the chest is in the position of maximum inspiration, the patient takes a forced position: sitting on the bed, hanging his legs down, tilting his body slightly forward. In breathing, the muscles of the shoulder girdle, back, and also the abdominal wall take an active part. On percussion over the lung fields, a box sound is determined; on auscultation, a lot of dry rales are heard. The attack often ends with the separation of viscous sputum. Severe prolonged attacks can turn into an asthmatic state - this is one of the most formidable options for the course of the disease. The asthmatic condition is manifested by increasing resistance to bronchodilator therapy and unproductive cough.

The course of the disease is cyclical: an exacerbation phase with clinical symptoms and data from laboratory and instrumental studies is replaced by an improvement phase. Complications of bronchial asthma: emphysema, infectious bronchitis, cor pulmonale, pulmonary heart failure.

27. Treatment of bronchial asthma

Treatment for bronchial asthma should be selected individually, taking into account the variant of the course, the phase of the disease, the presence of complications, concomitant diseases, patient tolerance of drugs and the most rational use of them during the day.

In atopic bronchial asthma, first of all, elimination therapy is necessary, the most complete or permanent cessation of contact with the allergen. If the allergen is identified, then it is impossible to isolate the patient from it, since specific hyposensitization is shown in a specialized allergological institution in the remission phase. Patients with atonic asthma, if it is an uncomplicated form of the disease, are prescribed cromolyn sodium (Intal) in the treatment, spraying it with a special inhaler. If bronchial asthma is combined with other allergic manifestations, oral ketotifen is preferable. The effect of both drugs comes gradually. In the absence of effect, glucocorticoids are prescribed; in moderate cases, it is advisable to prescribe in the form of inhalations (becotide). In severe exacerbations, glucocorticoids are prescribed orally, starting with prednisolone, after achieving a clinical effect, the dose is gradually reduced. In case of allergies to food products, the use of unloading and dietary therapy is prescribed, which is carried out in a hospital.

Treatment with vaccines is carried out only in specialized hospitals. In case of violation in the immune system, appropriate immunocorrective therapy is prescribed. During the period of remission, sanation of foci of chronic infection is carried out. In case of violation of mucociliary clearance, it is necessary to prescribe liquefying therapy: heavy drinking, alkaline warm inhalations, herbal decoction, mucolytic agents.

With "aspirin" asthma, foods that contain acetylsalicylic acid are excluded from the diet. Non-steroidal anti-inflammatory drugs are strictly prohibited. If necessary, you can prescribe Intal, Zaditen, corticosteroid hormones. To stop asthma attacks, bronchodilator therapy is individually selected and prescribed (the optimal dose of bronchodilators is selected from a small dose to the most effective). A positive effect in most patients is exerted by selective /3-2-adrenergic stimulants (salbutamol, berotek, etc.), which are available in the form of pocket metered-dose inhalers.

During an attack, two breaths of the aerosol are recommended. In mild cases of the disease, these drugs can be used in the form of tablets. For more severe attacks, injections of aminophylline intravenously or in tablet form and suppositories are used.

Anticholinergics (atropine, belladonna, platifillin) are preferred in the infectious-allergic form of the disease, especially with obstruction of large bronchi. Sometimes these drugs can be added to other bronchodilators. Atrovent is an effective drug in this group. Different mechanisms of bronchial obstruction in each patient are caused by a combination of drugs. An effective remedy is berodual, which combines berotek and atrovent in the form of a metered dose inhaler.

28. Respiratory failure

Respiratory failure is a pathological condition of the body in which the normal maintenance of the gas composition of the blood is not ensured or it is achieved due to the tension of the compensatory mechanisms of external respiration.

There are five groups of factors leading to impaired external respiration.

1. Damage to the bronchi and respiratory structures of the lungs:

1) damage to the bronchial tree: an increase in the tone of the smooth muscles of the bronchi (bronchospasm), edematous and inflammatory changes in the bronchial tree, a violation of the supporting structures of the small bronchi, a decrease in the tone of the large bronchi (hypotonic hypokinesia);

2) damage to respiratory structures (infiltration of lung tissue, destruction of lung tissue, dystrophy of lung tissue, pneumosclerosis);

3) a decrease in the functioning lung parenchyma (underdevelopment of the lung, compression and atelectasis of the lung, the absence of a part of the lung tissue after surgery).

2. Damage to the musculoskeletal skeleton of the chest and pleura (limited mobility of the ribs and diaphragm, pleural adhesions).

3. Damage to the respiratory muscles (central and peripheral paralysis of the respiratory muscles, degenerative-dystrophic changes in the respiratory muscles).

4. Violation of blood circulation in the pulmonary circulation (reduction of the vascular bed of the lungs, spasm of the pulmonary arterioles, blood stasis in the pulmonary circulation).

5. Violation of the regulation of breathing (oppression of the respiratory center, respiratory neurosis, violation of local regulatory relationships).

Classification. Form: ventilation, alveolorespiratory.

Type of ventilation failure:

1) obstructive;

2) restrictive;

3) combined. Severity:

1) DN I degree;

2) DN II degree;

3) DN III degree.

Obstructive ventilation insufficiency is associated with difficulty in advancing the flow of gas through the airways of the lungs due to a decrease in the lumen of the bronchial tree.

Restrictive ventilation failure is a consequence of processes that limit the extensibility of the lungs and reduce lung volumes: pneumosclerosis, adhesions, lung resections, etc.

Combined ventilation failure occurs when there is a combination of restrictive and obstructive disorders.

Alveorespiratory insufficiency is a violation of pulmonary gas exchange due to a decrease in the diffusion capacity of the lungs, uneven distribution of ventilation and ventilation-perfusion relations of the lungs.

29. Diagnosis and treatment of respiratory failure

Respiratory insufficiency of the I degree: shortness of breath at rest is absent; intermittent cyanosis, pallor of the face, anxiety, irritability, blood pressure normal or moderately elevated. Indicators of external respiration: minute volume of respiration (MOD) increased, vital capacity (VC) reduced, respiratory reserve (RD) reduced, respiratory volume (OD) slightly reduced, respiratory equivalent (DE) increased, oxygen utilization factor (KIO2) reduced. Blood gas composition, acid-base state (CBS): blood gas composition at rest is not changed, or blood oxygen saturation is moderately reduced to 90% P / D (normal 80-100 mm Hg. Art.).

Respiratory insufficiency II degree: shortness of breath at rest is constant, with the participation of auxiliary muscles, the ratio of P / D 2-1,5: 1, tachycardia; cyanosis. Generalized pallor of the skin, sweating, pallor of the nail beds. BP is elevated. Indicators of external respiration: MOD is increased, VC is reduced by more than 25-30%, OD and RD are reduced to 50%, DE is significantly increased, which indicates a pronounced decrease in oxygen utilization in the lungs. Blood gas composition, CBS: oxygen saturation of the blood is 70-85%, i.e., it decreases to 60 mm Hg. Art.; normocapnia or hypercapnia above 45 mm, respiratory or metabolic acidosis: pH 7,34-7,25 (normal 7,35-7,45), base deficiency (BE) increased.

Respiratory insufficiency III degree: severe shortness of breath, respiratory rate more than 150% of the norm, aperiodic breathing, periodically bradypnoe, non-synchronization of breathing, paradoxical breathing; reduction or complete absence of respiratory sounds on inspiration. The P/D ratio varies; cyanosis is generalized, generalized pallor is possible, marbling of the skin, sticky sweat, blood pressure is reduced; consciousness and reaction to pain are suppressed, decreased tone of skeletal muscles, convulsions, coma. Indicators of external respiration: MOD is reduced, VC and OD are reduced by more than 50%, RD is 0. Blood gas composition, CBS: blood oxygen saturation is below 70% (40 mm Hg); decompensated mixed acidosis: pH less than 7,2, BE more than 6-8, hypercapnia more than 70 mm Hg. Art., the level of bicarbonates and buffer bases is reduced. The directions of treatment are as follows:

1) creating a microclimate;

2) maintaining free airway patency;

3) oxygen therapy;

4) spontaneous breathing under true positive pressure;

5) normalization of pulmonary blood flow (eufillin, petamine, benzohexonium);

6) CBS correction;

7) to improve the utilization of oxygen by tissues, a glucose-vitamin-energy complex (glucose 10-20%, ascorbic acid, cocarboxylase, riboflavin, cytochrome C, calcium pantothenate, unitiol);

8) treatment of the underlying disease and concomitant pathological conditions.

30. Acute pyelonephritis

Pyelonephritis is a nonspecific infectious disease that affects the kidney parenchyma, mainly with damage to the interstitial tissue.

Causes: infection, impaired urodynamics, impaired immunity. Most often, pyelonephritis is caused by Escherichia coli, Proteus, Enterococcus, Streptococcus, Staphylococcus aureus.

The infection enters the kidney, pelvis, then into its calyces by the hematogenous or lymphogenous route from the lower urinary tract along the wall of the ureter, along its lumen in the presence of retrograde refluxes. Of particular importance in the formation of pyelonephritis is urinary stasis, impaired venous and lymphatic outflow from the kidney.

The disease begins acutely with an increase in body temperature up to 40 ° C, chills, sweating, pallor of the skin and mucous membranes, pain in the lumbar region; sharp pain in the costovertebral angle; weakness, thirst, dysuria or pollakiuria, painful urination. Joining headache, nausea, vomiting, which indicates a rapidly growing intoxication. Symptom Pasternatsky, as a rule, is positive. With bilateral acute pyelonephritis, renal failure may develop.

Acute pyelonephritis can be complicated by paranephritis, necrosis of the renal papillae.

Clinical and laboratory data: neutrophilic leukocytosis, anemia, aneosinophilia, increased ESR are noted in the blood test; in the analysis of urine - leukocyturia, pyuria with moderate proteinuria and hematuria; in the sample according to Zimnitsky - a decrease in the density of urine during the day; in the sample according to Nechiporenko - leukocytosis; in a biochemical study of blood - an increase in the content of sialic acids, creatinine, urea, the appearance of C-reactive protein. On a survey radiograph, one can detect an increase in one of the kidneys in volume, when performing excretory urography - a sharp limitation of the mobility of the affected kidney during breathing, the absence or later appearance of a shadow of the urinary tract on the side of the lesion.

Tasks in treatment: elimination and reduction of the microbial-inflammatory process in the renal tissue and urinary tract; normalization of metabolic disorders and the functional state of the kidneys; stimulation of regenerative processes; reduction of sclerotic processes in the interstitial tissue.

In the acute period, table No. 7a is shown, consumption of up to 2 liters of fluid per day.

Antibacterial therapy is carried out with nalidixic acid (nevigramon, blacks), nitroxoline (5-NOC), nitrofuran derivatives (furadonin). The use of these drugs should be alternated. It is impossible to use nalidixic acid and nitrofuran drugs at the same time, as this weakens the antibacterial effect.

Effectively combined treatment with antibiotics and sulfonamides. Antibiotics are selected depending on the sensitivity of the microflora to them. Prescribe drugs of the penicillin group ampicillin, drugs of the aminoglycoside series, long-acting sulfonamides.

31. Chronic pyelonephritis

Chronic pyelonephritis is a consequence of untreated acute pyelonephritis and can proceed without acute events from the onset of the disease.

Clinical manifestations. Unilateral chronic pyelonephritis is manifested by dull constant pain in the lumbar region on the side of the affected kidney. Dysuric disorders are absent in most patients.

Diagnosis is based on anamnesis, clinical and laboratory data, neutrophilic leukocytosis is noted in the blood test. When analyzing urine in the sediment, the predominance of leukocytes over other formed elements of urine is revealed. The relative density of urine remains normal. One of the symptoms of the disease is bacteriuria, if the number of bacteria in 1 ml exceeds 100, it is necessary to determine the sensitivity to antibiotics and chemotherapy drugs. The most common symptom of chronic pyelonephritis, especially bilateral process, is arterial hypertension. The functional state of the kidneys is determined using chromocystoscopy, excretory urography, clearance methods. In chronic pyelonephritis, the concentration ability of the kidneys is disturbed early, and the nitrogen excretion function has been acting for many years. With infusion urography, the concentration ability of the kidneys, delayed release of a radiopaque substance, deformation of the cups, local spasms, and deformation of the pelvis are determined. Then the spastic phase is replaced by atony, the pelvis and calyx expand.

In differential diagnosis with chronic glomerulonephritis, the nature of the urinary syndrome with a predominance of leukocyturia over hematuria, the presence of active leukocytes, significant bacteriuria in pyelonephritis, and excretory urography data are of great importance. Nephrotic syndrome indicates the presence of glomerulonephritis. With arterial hypertension, a differential diagnosis is made between pyelonephritis and glomerulonephritis.

Treatment of chronic pyelonephritis should be carried out for a long time. Treatment should begin with the appointment of nitrofurans (furadonin, furadantin, etc.), 5-NOC, nalidixic acid (blacks, nevigramon), sulfonamides (urosulfan, atazol, etc.), alternating them alternately. At the same time, it is recommended to treat with cranberry juice or extract. With the ineffectiveness of drugs during an exacerbation of the disease, broad-spectrum antibiotics are used. Before prescribing antibiotics, each time it is necessary to determine the sensitivity of the microflora to it.

32. Acute glomerulonephritis

Glomerulonephritis is an immunoallergic disease characterized by a predominant lesion of the glomerular vessels, which occurs as an acute or chronic process with relapses and remissions.

Variants of glomerulonephritis:

1) nephritic - manifested by hematuria, proteinuria, hypertension, oliguria, cylinduria, leukocyturia, hypovolemia, hypocomplementemia, encephalopathy;

2) nephrotic - high proteinuria, edema, hypoproteinemia, possible arterial hypertension, erythrocyturia, azotemia;

3) mixed - severe nephrotic syndrome, significant hematuria, hypertension;

4) hematuric - hematuria predominates in the urinary syndrome;

5) isolated - urinary syndrome is manifested by extrarenal symptoms, which are slightly expressed.

Acute glomerulonephritis is a cyclic infectious-allergic kidney disease that develops more often 1-3 weeks after an infectious disease (usually streptococcal etiology). Acute glomerulonephritis can occur at any age but is more common before age 40.

The disease develops after angina, tonsillitis, upper respiratory tract infections, scarlet fever, etc. 12/? - group A hemolytic streptococcus, pneumococcus, respiratory viruses, parasitic invasion, hypothermia, injuries.

Clinical manifestations of glomerulonephritis in children are as follows.

1. Extrarenal:

1) neurovegetative syndrome: malaise anorexia, lethargy, nausea, vomiting, poor appetite, headache;

2) cardiovascular syndrome - hypertension, muffled heart sounds, murmurs and accents of heart sounds, enlargement of the liver;

3) edematous syndrome is manifested by pastosity, limited or generalized edema.

2. Renal manifestations:

1) urinary syndrome - oliguria, proteinuria, hematuria, cylinduria, transient, lymphocytic-mononuclear leukocyturia;

2) pain syndrome is manifested by pain in the lumbar region or undifferentiated pain in the abdomen;

3) renal failure syndrome - azotemia, manifested by three main symptoms: edematous, hypertonic and urinary.

Hospitalization in a hospital, bed rest is prescribed, a diet with a restriction of salt in food leads to increased excretion of water and the elimination of edematous and hypertensive syndrome. Antibacterial therapy is prescribed if there is a connection between glomerulonephritis and an existing infection.

33. Chronic glomerulonephritis

Chronic glomerulonephritis is a long-term immunological bilateral kidney disease, in which changes in the urine persist without significant dynamics for more than a year, or edema and hypertension are observed for more than 3-5 months. Chronic glomerulonephritis may be the outcome of acute glomerulonephritis or primary chronic, without a previous acute attack.

During chronic glomerulonephritis, two stages are distinguished.

Stage I of renal compensation, i.e. sufficient nitrogen excretion function of the kidneys (and this stage is accompanied by a pronounced urinary syndrome, proceeds latently and is manifested by slight albuminuria or hematuria).

Stage II of renal decompensation, manifested by insufficiency of the nitrogen excretion function of the kidneys (urinary symptoms are less significant; high arterial hypertension is observed, edema is mostly moderate; hypoisostenuria and polyuria are expressed at this stage, the outcome of which is azotemic uremia).

Forms of chronic glomerulonephritis:

1) the nephrotic form is the most common form of primary nephrotic syndrome. The clinical manifestations of the disease are determined by the nephrotic syndrome, and further progression of glomerulonephritis occurs with nitrogen excretion of the kidneys and arterial hypertension;

2) hypertonic form. Among the symptoms, arterial hypertension predominates over the urinary syndrome, which is mild. Sometimes chronic glomerulonephritis can manifest itself as a hypertensive type after the first violent attack of glomerulonephritis. But it also develops when a latent form of acute glomurulonephritis occurs. Arterial pressure rises to 180-200 mm Hg. Art. and fluctuates throughout the day under the influence of various factors. Hypertrophy of the left ventricle of the heart develops, with auscultation, an accent of the II tone over the aorta is heard. Hypertension does not become malignant. Revealed changes in the fundus in the form of neuroretinitis;

3) mixed form. This form is simultaneously manifested by nephrotic and hypertensive syndrome;

4) latent form. This often developing form is manifested by urinary syndrome without arterial hypertension and without edema. This form has a long course (10-20 years or more), later leads to the development of uremia.

The hematuric form should also be distinguished, since in some cases chronic glomerulonephritis is manifested by hematuria, without severe proteinuria with general symptoms of hypertension and edema.

It is necessary to eliminate foci of chronic infection (removal of the tonsils, sanitation of the oral cavity, etc.). Prolonged dietary restrictions (salt and protein).

34. Hereditary renal diabetes

Hereditary phosphate diabetes (hypophosphatomic, vitamin D-resistant rickets):

Diagnostic criteria. Viral type and progressive nature of bone deformities of the lower extremities, short stature, strong physique; normal intelligence, type of inheritance, low serum phosphorus (0,45-0,6 mmol/l), hyperphosphaturia (urine phosphate clearance 0,3-0,9 ml/s), normal blood calcium (2,25 -2,6 mmol / l), increased activity of blood alkaline phosphatase (more than 2100 nmol / l), increased levels of parathyroid hormone in the blood, decreased absorption of calcium and phosphorus in the intestine.

Treatment. Vitamin D - 10-000 IU per day, after 20-000 weeks, increase by 4-6 IU. Until an increase in phosphorus in the blood and a decrease in the activity of alkaline phosphatase (determine every 10-000 days), the maximum daily dose is 15 IU per day. Calcium gluconate 000-10 g / day. Phosphates up to 14 g / day, phytin 300 g / day, calcium glycerophosphate 000-1,5 g / day. Ksidifon. citrate blend.

Renal diabetes insipidus is the absence of a response of epithelial cells of the distal tubules of the kidneys to antidiuretic hormone (ADH, vasopressin). The type of inheritance is recessive, sex-linked (mostly boys get sick, girls get sick less often and more easily), a codominant type of inheritance is possible.

Diagnostic criteria. Polyuria (in newborns up to 2 liters per day, in older ones 5-10 liters). Polydipsia (no thirst for up to a year), hypoisostenuria below 1005, dehydration (fever, convulsions, vomiting, constipation). Hypotrophy, decreased skin turgor, constipation, growth retardation, delayed psychomotor development. Sample with ADH (3-8 units) or pituitrin - 0,1-0,5 ml (1 units in 5 ml).

Treatment. Rehydration (2,5% glucose + 2% NaCl), hypothiazide (increases the reabsorption of Na in the distal tubules and water in the proximal). Prostaglandin inhibitors (indomitacin, ibuprofen).

Renal salt diabetes (pseudohypoaldosteronism) is a violation of the reabsorption of sodium ions due to the low sensitivity of the receptors of the distal tubular epithelium to aldosterone.

It is inherited in an autosomal recessive manner.

Diagnostic criteria. Polyuria, polydipsia, anorexia, adynamia, acidosis, hyperkalemia. Delayed growth and mental development, ossification of the bones of the skeleton. In the blood, hyponatremia is below 130 mmol / l, hyperkalemia, hypovolemia. Increased content of aldosterone in the urine (60-80 mcg per day), increased excretion of sodium in the urine. In the urine, the level of ketosteroids and oxycorticoids is normal, and during the load, ACTH increases, which excludes adrenal hypoplasia.

Treatment. Correction of hyponatremia according to the formula: Na (in mol) = (140 - p) h 1,5 body weight (in kg), where p is the sodium content in blood serum (in mmol / l).

35. Hereditary nephritis

Hereditary nephritis is a genetically determined non-immune glomerulopathy, manifested by hematuria and (or) proteinuria, tending to progress, up to the development of CRF, often combined with hearing and vision pathology.

The type of inheritance is dominant, linked to the X chromosome.

Clinically, there are two options:

1) hereditary nephritis with hearing loss (Alport syndrome);

2) hereditary nephritis without hearing loss. Phenotypically, the polymorphism of clinical symptoms is due to the different expressivity of the mutant gene, which is located on the X chromosome. The presence of sick children with hereditary nephritis in clinically healthy parents indicates different penetrance of the mutant gene or codominance.

Morphology of hereditary nephritis: thinning of the basement membrane, degeneration, splitting or thickening, dystrophy, fragmentation. Proliferative changes in the glomeruli - hematuria. Violation of the structure of basement membranes and podocytes, the development of proteinuria. Lymphohistiocytic infiltration of the interstitium - abacterial leukocyturia. Focal glomerulosclerosis-hypertension.

Clinical picture and diagnosis.

Genealogical history (presence of diseases of hearing, vision, diseases of the cardiovascular system in relatives). Frequent SARS, infections (deficiency of Ig A). Urinary syndrome (hematuria, less often proteinuria, leukocyturia). Neuritis of the auditory nerve (hearing loss from moderate to complete deafness) is parallel to the severity of nephropathy. Changes in the organ of vision (spherophakia, lenticonus, cataracts, myopia). Changes in the nervous system (tremor, myasthenia gravis, decreased memory, intelligence). Violation of the functional state of the kidneys (hyperaminaciduria, decreased reabsorption of bicarbonates, concentration function). Metabolic acidosis, dyselectrolytemia. Stages of the course of the disease:

1) stage of compensation: arterial hypotension, absence or minimal changes in urine tests;

2) stage of subcompensation: symptoms of intoxication, sometimes hypertension, hematuria, moderate changes in protein and lipid metabolism, partial decrease in renal function;

3) stage of decompensation: vascular hypertension, metabolic shifts, increased hematuria and proteinuria, severe renal dysfunction.

Treatment. Prevention of progression of impaired renal function. There is no pathogenic therapy. Restriction of motor activity depending on the stage up to the appointment of bed rest. Complete and balanced nutrition. Sanitation of foci of infection.

To reduce hematuria - rutin, calcium gluconate, chokeberry, nettle, yarrow, etc. Steroids and cytostatics are not indicated. Delagil stabilizes lysosomal membranes, vitamin B6 10-14 days. Anabolic hormones (retabolil). Immunostimulants (levamisole).

Treatment of chronic renal failure, hemodialysis, kidney transplant.

36. Primary and secondary hyperoxaluria

Primary hyperoxaluria (oxalosis) is a hereditary pathology of glyoxylic acid metabolism, characterized by recurrent oxalate, calcium nephrolithiasis, gradually leading to CRF.

The disease is inherited in an autosomal recessive manner, a dominant type of inheritance is possible.

Clinical manifestations, diagnosis. At an early age, there are recurrent pains in the joints and their swelling, abdominal pain, hematuria, leukocyturia, proteinuria, renal colic; daily excretion of oxalates in the urine increases to 250 mg per day per 1,73 m2 of body surface; radiologically determined nephro- and urolithiasis; increase in renal clearance of oxalates up to 50 ml / min. Urine sediment with oxalaturia saturated color.

Differential diagnosis is carried out with glomerulonephritis, pyelonephritis, interstitial nephritis.

Treatment. The directions of treatment are as follows:

1) a decrease in the synthesis and excretion of oxalates and the prevention of the formation of oxalate-calcium salts (magnesium oxide);

2) vitamin B6 (in large doses);

3) orthophosphate;

4) inorganic pyrophosphates (hydroxyethylidene diphosphonic acid).

Secondary hyperoxaluria (oxalate nephropathy)

Etiology. An increase in the synthesis of oxalates, in addition to a hereditary metabolic pathology, may be associated with excessive formation from precursors, ethylene glycol poisoning, gout, inflammatory bowel diseases, bowel operations, and chronic pancreatitis.

Clinical manifestations. Frequent recurrent abdominal pain, possible enuresis; decrease in the volume of urine during the day, precipitation of salts; signs of skin allergies; changes in the urine: proteinuria, slight erythrocyturia, leukocyturia; increase in phospholipase activity of blood serum and urine; increased excretion of oxalates (up to 100 mg per day); phospholipiduria.

Differential diagnosis is carried out with chronic glomerulonephritis, pyelonephritis, interstitial nephritis.

Treatment. Increased fluid intake helps to reduce crystal formation and stone formation. Diet with the exclusion of foods containing a large amount of oxalates (cocoa, chocolate, beets, sorrel, parsley, extractives). Membrane stabilization (antioxidants, retinol, potassium, magnesium salts, pyridoxine).

37. Chronic gastroduodenitis

Chronic gastroduodenitis is a chronic inflammation of the mucous membrane of the antrum of the stomach and duodenum, which is accompanied by a violation of the physiological regeneration of the epithelium, the secretory and motor functions of the stomach.

Etiology and classification

Etiological factors:

1) endogenous factors (hereditary predisposition, high type of acid formation, impaired mucus formation, chronic diseases accompanied by hypoxia, local vascular disorders, intoxication, chronic diseases of the liver and biliary tract);

2) endogenous factors (malnutrition, poor-quality roughage, eating dry food, in a hurry, long breaks in eating; past food poisoning, prolonged and frequent medication, psycho-emotional stress, neurogenic stress, colonization of the mucous membrane of the antrum of the stomach and duodenum with bacteria ).

Classification.

1. By the period of the disease: exacerbations, subremissions, remissions.

2. According to the mechanism of development, there are:

1) chronic gastritis type A, based on an autoimmune mechanism of development with the production of antibodies to the parietal cells of the mucous membrane and the internal factor;

2) chronic type B gastritis develops as a result of various factors (long-term medication, nutritional disorders, persistence of Helicobacter pylori in the mucosa);

3) chronic gastritis type C has a reflux mechanism of development or medication as a result of taking NSAIDs.

Clinical manifestations. Pain in the abdomen is localized in the epigastric and pyloroduodenal zone, often occurs on an empty stomach and decreases after eating. Sometimes there is an early pain that appears after eating after 20-30 minutes, hungry pains are less often noted 1,5-2 hours after eating. The rhythm of pain in older children: hunger - pain - eating - relief - hunger. Reducing pain contributes to the intake of a small amount of food, and increases the pain of overeating, eating spicy, acidic foods, physical activity. Dyspeptic syndrome is caused by a violation of the motor and secretory functions of the stomach and duodenum, manifested by nausea, vomiting, belching, heartburn, stool disorders in the form of constipation or unstable stools with polyfecal matter. Asthenovegetative syndrome is manifested by weakness, fatigue, neurosis-like states. Palpation of the abdomen reveals moderate diffuse pain in the epigastric and pyloroduodenal region.

38. Diagnosis and treatment of gastroduodenitis

Based on the anamnesis of clinical and laboratory data in the blood test - a decrease in erythrocytes, hemoglobin, moderate leukocytosis; during endoscopic examination, superficial gastroduodenitis is isolated, where hyperemia and mucosal edema are revealed. With hypertrophic gastroduodenitis, the mucosa is edematous, hyperemic, has a "granular" appearance, punctate hemorrhages. With erosive gastritis - against the background of hyperemia, multiple, less often single, erosions with a flat bottom. With atrophic (subatrophic) gastroduodenitis, the mucosa is pale, the folds are thinned, smoothed, the vascular pattern is enhanced. In all forms, there may be signs of duodenogastric reflux (pylorus gaping, an admixture of bile in the contents of the stomach).

They carry out tests for the determination of Helicobacter Pilori - this is enzyme immunoassay, the determination of antibodies in the blood, urine, saliva; smear microscopy - prints of the gastric mucosa. X-ray examination according to indications, if there are changes in the folds, a large amount of contents on an empty stomach, spasms of the pylorus, duodenum, a change in the shape of the stomach.

Treatment. It is necessary to adhere to the medical and protective regimen, night sleep for at least 8 hours, the head of the bed should be higher than the foot. Sharp physical exertion, hard physical labor are contraindicated, it is necessary to treat carious teeth, diseases of the nasopharynx, giardiasis in a timely manner.

Diet therapy: food should be complete and varied, contain a sufficient amount of vegetables, fruits, dairy products. Food is taken 5-6 times a day, the last meal no later than 19.00. Dry food is not allowed. Do not take a horizontal position within 2-3 hours after eating. Highly carbonated drinks, chewing gum are contraindicated for use, especially on an empty stomach. Antacid therapy is carried out, almagel, maalox, phosphalugel are prescribed. Antisecretory therapy: H-2-histamine blockers: ranitidine 150 mg in the morning and evening, M-anticholinergics: gastrocepin 35 mg 2 times a day before meals.

Prescribe drugs that improve the protective properties of the mucosa - these are protective basic drugs (venter, denol, before meals and at night the tablet is chewed and washed down with water); synthetic prostaglandins (cytotec); non-specific mucosal protectors (actovegin, folic acid, vitamins A, E, B).

Helicobacter therapy is carried out, bismuth preparations (denol, bismofalk), antibacterial drugs (amoxacillin), antimicrobial drugs (metronidazole) are used.

In case of violation of the motor-evacuation function, motilium is used, adsorbents (smecta, enterosgel, wheat bran) are prescribed to correct the pathological reflux of duodenal contents into the stomach.

Physiotherapy is prescribed: UHF, laser therapy, inductothermy.

39. Peptic ulcer

Peptic ulcer of the stomach or duodenum is a chronic disease, the main symptom of which is the formation of ulcers in the digestive tract during the period of exacerbation.

The main etiological factor is Helicobacter pylori infection. An important role in the formation of pathology is played by psychosocial factors, toxic-allergic factors, hereditary-constitutional factors.

Classification:

1) by localization: stomach, duodenal bulb, mixed localization;

2) by phase: exacerbation, incomplete clinical remission, clinical remission;

3) in form: complicated, uncomplicated (bleeding, perforation, penetration, pyloric stenosis);

4) downstream: newly diagnosed, often recurrent (less than 3 years), rarely recurrent (more than 3 years);

5) according to the nature of the acid-forming function: with a preserved function, with an increased function, with a reduced function.

Clinical and endoscopic stage: "fresh" ulcer, the beginning of the epithelialization of the ulcer, healing of the ulcerative defect of the mucous membrane with preserved duodenitis, clinical and endoscopic remission.

Clinical manifestations. Pain of a persistent and persistent nature, localized in the epigastric or pyloroduodenal zone. The rhythm of pain in older children: hunger - pain - eating - relief - hunger. Characterized by the appearance of night pain, pain in the early morning hours.

The course of peptic ulcer can be latent, and for a long time children do not complain of abdominal pain, occasionally there is nausea, vomiting, belching, a feeling of rapid satiety, and heaviness in the abdomen. Asthenovegetative syndrome is manifested by sleep disturbance, emotional lability, irritability, arterial hypotension, impaired appetite.

Treatment. Bed rest, diet #1a, #1b, then #1.

Treatment is aimed at:

1) suppression of the aggressive properties of gastric juice. Apply selective blockers M-1-cholinergic receptors: gastrocepin, pyroncepin; H-2-histamine receptor blockers: ranitidine, famotidine; antacids: almagel, phosphalugel, gastrogel;

2) increase in the protective layer of the mucous membrane. Cytoprotectors are prescribed: bismuth preparations, cytotec, sucrafalk;

3) neurohumoral regulation: psychotropic drugs, dopamine receptor blockers.

Also apply:

1) antibacterial and antiprotozoal drugs;

2) physiotherapy: EHF, magnetic and laser therapy, hyperbaric oxygenation.

40. Biliary dyskinesia

Biliary dyskinesia is a functional disorder of the motility of the gallbladder and biliary tract, manifested by pain in the right hypochondrium, leading to a violation of the outflow of bile into the duodenum.

Dyskinesias are divided into primary and secondary. Primary dyskinesias cause a change in neurohumoral mechanisms, develop with intoxication against the background of allergic diseases, endocrine-hormonal disorders, and neurosis. Secondary dyskinesias occur reflexively in diseases of the abdominal organs by the type of viscero-visceral reflexes, joining chronic cholecystocholangitis, cholelithiasis. The occurrence of biliary dysmotility and biliary hypertension leads to a change in the normal blood flow in the gallbladder and bile ducts, which causes hypoxia with a subsequent change in the permeability of cell membranes and biochemical processes in the cells of the gallbladder mucosa and liver.

Clinical manifestations. It is manifested by pain in the abdomen, mainly in the right hypochondrium. Aching or cramping pains are accompanied by dyspeptic disorders (nausea, vomiting, bitterness in the mouth, intolerance to fatty foods, unstable stools), a characteristic feature is the connection of pain with neuropsychic and physical overload.

The pain syndrome in hypotonic dyskinesia is characterized by constant, periodically increasing pain and a feeling of fullness in the right hypochondrium. Pain syndrome in hypertensive dyskinesia is characterized by paroxysmal pains (cramping, stabbing, cutting) associated with emotional and physical overstrain with irradiation to the right shoulder, epigastric and near the umbilical region.

On examination, attention is drawn to astheno-getative disorders, pain on palpation in the right hypochondrium, positive symptoms of Kerr, Ortner, Murphy, Mussy.

Diagnostics. Based on the anamnesis, clinical and laboratory data, fractional duodenal sounding can reveal hypertonicity of the sphincters of Oddi, Lutkens, there is an increase in the duration of the 2nd and 3rd phases of FDD from 10 to 30 minutes, with hypotension, a decrease to 1-3 minutes; hyperkenesia of the gallbladder is characterized by rapid emptying, occurring immediately or in the first 3-5 minutes, the volume of portion B is not changed, with hypokinesia, gallbladder reflux is normal or slowed down, the amount of bile in portion B is greater than normal.

Treatment. Cholagogue therapy for hypomotor dyskinesia: drugs that stimulate bile formation (cholagol, cholenzym) are used; preparations containing bile acids (allohol, lyobil); drugs that cause an increase in the tone of the biliary tract (sorbitol, xylitol, magnesium sulfate); herbal preparations: dandelion, rosehip, mint, corn).

Cholagogue therapy for hypermotor dyskinesia: drugs are used that cause relaxation of the tone of the biliary tract (eufillin); preparations of plant origin (St. John's wort, chamomile, stinging nettle). A good cholekinetic effect is given by tubages.

41. Chronic cholecystitis

Chronic cholecystitis is an inflammatory process in the wall of the gallbladder of bacterial, viral origin.

Clinical manifestations. The onset of the disease is often erased with periodic exacerbations, the causes of which are errors in nutrition, physical activity, psycho-emotional disorders, intercurrent diseases. During the period of exacerbation, symptoms of intoxication increase, dyspeptic disorders intensify.

Complaints of pain in the right hypochondrium of a paroxysmal or dull nature, aggravated after eating fatty foods, when running, walking. The duration is from several minutes to 1-2 hours. On palpation of the abdomen, there is pain in the right hypochondrium.

Diagnosis. Based on the anamnesis, clinical and laboratory data, in the blood test in the acute course - leukocytosis, neutrophilia, increased ESR, in the chronic course - the inflammatory reaction is moderately and constantly expressed; in a biochemical study of blood during exacerbation - an increase in the content of sialic acids, fibrin, transaminases, alkaline phosphatase. On echographic examination, a decrease or increase in the gallbladder, a thickening of the wall of more than 1 mm, a violation of the contraction of the gallbladder. In the study of bile, a decrease in specific gravity (normally, the specific gravity in portion A is 1006-1007, portion B is 1024-1032, portion C is 1007-1010), a shift in pH to the acid side, (normally 6,2-7,5 ), with microscopy of the sediment - mucus, leukocytes, cylindrical epithelium, an increase in cholesterol crystals, calcium bilirubinate, the presence of cystiamblia or opisthorchiasis eggs.

Differential diagnosis is carried out with duodenitis, gastritis, pancreatitis, peptic ulcer, helminthic invasion, appendicitis.

Treatment. Diet therapy No. 5, nutrition mechanically, chemically, thermally sparing. Dairy products should be included in the morning and evening. Exclude from the diet dishes containing extractives, essential oils, peppers, onions, garlic, smoked meats, pastry, chocolate, coffee.

Antibacterial therapy (semi-synthetic penicillins, macrolides, cephalosporins), antiparasitic drugs when helminths and protozoa are detected. Choleretics and cholekinetics - depending on the type of dyskinetic disorders. Vitamin therapy. Preparations for improving liver function (karsil, Essentiale, legalon). Reflexology, physiotherapy, exercise therapy, depending on dyskinetic.

42. Acute pancreatitis

Acute pancreatitis is an acute inflammatory lesion of the pancreas, characterized by autolysis (due to the activation of its own enzymes) and dystrophy of the gland.

Etiology:

1) damage to the parenchyma of the pancreas;

2) obstructive disorders in congenital anomalies of the pancreas;

3) dysmetabolic causes;

4) acute circulatory disorders;

5) toxic and drug-induced lesions;

6) alimentary overload, especially in combination with alcohol;

7) pancreatic cancer.

Clinical manifestations. Pain syndrome: intense pain in the epigastrium with irradiation to the left or throughout the abdomen, or around the navel, diffuse, radiating, often girdle; lack of appetite, feeling of heaviness and fullness in the upper abdomen, flatulence, belching.

Dyspeptic syndrome: nausea, vomiting that does not bring relief, constipation or maldigestion syndrome; toxicosis syndrome: fever, headache, weakness, malaise, acute vascular insufficiency, toxic manifestations from other organs (brain, heart, kidneys, liver, DIC).

An objective examination: the skin is pale, the tongue is lined, the abdomen is swollen, tenderness on palpation, symptoms of "acute abdomen"; in severe destructive processes, a local discoloration of the skin is possible due to the toxic effect of proteolytic enzymes.

The following symptoms are positive:

1) Hodsted's symptom (cyanosis of certain areas of the anterior surface of the abdomen);

2) a symptom of Gray-Turner (bluish or green pigmentation on the lateral parts of the abdomen);

3) Grunwald's symptom (secondary chymoses or petechiae around the navel, in the buttocks);

4) Cullen's symptom (yellowish-cyanotic color in the navel);

5) Mondor's symptom (purple spots on the skin of the face and trunk); palpation of the abdomen reveals: pain in the region of the transverse abdominal muscle in the projection of the pancreas;

6) Kach's symptom - soreness at the edge of the rectus abdominis muscle on the left, 2 cm above the navel;

7) Chauffard's area - pain in the choledochopan-creatic triangle on the right;

8) Mayo-Robson point - on the border of the outer and middle third of the line connecting the navel with the middle of the left costal arch;

9) Desjardins point - located on the line connecting the navel to the top of the right armpit at a distance of 4-6 cm from the navel;

10) symptom Mayo-Robson - pain in the costal-vertebral zone on the left;

11) a symptom of Voskresensky - the absence of pulsation of the abdominal aorta with pressure in the epigastrium;

12) a symptom of "turn according to Tulzhilin".

43. Diagnosis and treatment of acute pancreatitis

Survey plan.

1. General analysis of blood, urine.

2. Determination of amylase in urine.

3. Biochemical blood test (amylase, lipase, trypsin, trypsin inhibitor, phospholipase, total protein and its fractions).

4. Amylase curve with a double load of glucose.

5. Prozerin test.

6. Coprogram.

7. Examination of feces for common fats, worm eggs, Giardia cysts, dysbacteriosis.

8. X-ray examination (endoscopic retrograde cholangiopancreatography, angiography, computed tomography, oral cholangiopancreatoscopy).

9. Ultrasound examination.

10. Radionuclide research (scanning).

11. Biopsy of the pancreas.

12. Laparoscopy.

13. Splenography.

Principles of treatment:

Elimination of pain, inhibition of the functional activity of the pancreas, reduction of enzymatic toxemia.

1. Diet therapy: hunger is recommended in the first 3 days, fractional drinking of mineral water is allowed, nutrition should be chemically and mechanically sparing, reduction in the diet of animal fats and proteins, refined sugar and other products that have a stimulating effect on pancreatic, gastric and cholesecretion).

2. Detoxification infusion therapy for 3-5 days (isotonic solutions of glucose and sodium chloride, albumin, plasma, hemodez, rheopolyglucin, cardiac drugs, glucocorticoids).

3. Antispasmodic (papaverine, noshpa, etc.), analgesic (baralgin, analgin) and anticholinergic painkillers (platifillin, metacin).

4. Antihistamines (suprastin, tavegil).

5. Pharmacological blockade of the vagus nerve to suppress pancreatic and gastric secretion (atropine, platifillin, ganglioblockers, trasylol, contrical, gordox).

6. To suppress the functional activity of the pancreas, antisecretory therapy is prescribed: H-histamine blockers (famotidine, sandostatin).

7. To reduce enzymatic toxemia, proteolysis inhibitors (kontrykal, gordox, trasilyl) are prescribed.

8. Assign pancreatic enzymes that do not contain bile (pancreatin, mesimforte, creon).

9. Broad-spectrum antibiotics for the prevention of purulent complications and the development of a secondary infection.

10. Prescribe prokinetics for nausea, vomiting, a feeling of fullness (cerucal, motilium).

Recommend phytotherapy, mineral waters of low salinity.

44. Chronic pancreatitis

Chronic pancreatitis is a progressive inflammatory disease characterized by progressive sclerosis (replacement of gland tissue by connective tissue) and progressive focal, segmental, or diffuse destruction of exocrine tissue.

Classification.

1. By origin: primary and secondary.

2. By shape:

1) recurrent;

2) with constant pain syndrome;

3) latent.

3. According to the period of illness:

1) exacerbation;

2) remission.

4. Clinical course: relapsing.

5. By severity: light, moderate, heavy.

6. Posyndromic characteristics: pancreatohepatic, cerebral, renal syndrome, etc.

7. Stages of the disease: initial; extended manifestations, final.

8. Status of pancreatic function:

1) the state of external secretion (without manifestations of external secretory insufficiency, with phenomena of exogenous insufficiency);

2) the state of internal secretion (without violation of internal secretion, with violations of internal secretion (hyper- and hypofunction of the insular apparatus)).

9. Complications from the pancreas. Clinical manifestations of recurrent chronic pancreatitis: pain syndrome, dyspeptic syndrome, toxicosis syndrome are provoked after an error in the diet. The pain is accompanied by bitterness in the mouth, nausea. An objective examination: blue under the eyes, grayish skin tone, sometimes subicteric, hypovitaminosis phenomena - dryness, peeling of the skin, cracks in the corners of the mouth; the degree of nutrition is reduced; swollen belly; positive symptoms of Mayo-Robson, Kach, Desjardins, Chauffard, Grotto (atrophy of fatty tissue of the anterior abdominal wall in the left hypochondrium).

After overeating, signs of exocrine insufficiency appear: increased and thinning of stools, polyfecal matter, in the capprogram - steatorrhea with neutral fat.

With exocrine insufficiency - polyfecal matter, stools are greasy, shiny, poorly washed off, and body weight deficit is increasing.

The manifestations of the disease depend on the severity of the inflammatory process and the severity of atrophy (sclerosis) of the pancreatic parenchyma, toxic damage to other organs (heart, blood vessels, liver, kidneys, nervous system).

Chronic pancreatitis with constant pain is sluggish. Complaints of moderate persistent pain, aggravated after an error in the diet in the projection of the pancreas. On palpation, pain is determined in the left hypochondrium and at the points of the pancreas.

45. Diagnosis and treatment of chronic pancreatitis

Laboratory data:

1) complete blood count (neutrophilic leukocytosis with a shift to the left, there may be lymphocytosis, eosinophilia, thrombocytopenia);

2) urinalysis (increased amylase, peptidase);

3) biochemical blood test (increase in amylase, lipase, trypsin and its inhibitor - elastase;

4) amylase and glycemic curve with a double load of glucose according to the Staub-Traugott test; with pancreatitis, amylase is increased on an empty stomach, increases even more after exercise and does not return to normal after 2,5 hours; blood sugar in pancreatitis - in the acute period there may be flat (hypoglycemic) curves, hyperinsular curves - more often in pain form, hypoinsular (diabetic) glycemic curves are usually found in painless pancreatitis;

5) coprogram (steatorrhea, creatorrhea, amylorrhea). Instrumental research:

1) ECHO-graphy: an increase in an organ or its departments due to edema, sclerosis;

2) duodenal-pancreatic sounding;

3) starch loading evaluates the amylase degrading ability; in case of enzyme deficiency, the sugar curve remains flat;

4) X-ray diagnostics;

5) duodenography under conditions of artificial hypotension reveals an expansion of the duodenal arch, an expansion of the relief of the medial wall, and sometimes a narrowing of its descending section.

Treatment is the following:

1) during the period of subremission, drugs are prescribed that improve metabolic processes (essentiale, lipostabil, lipamide). Normalization of intestinal microflora (bifidumbacterin, lactobacterin); 2) without exacerbation: diet (table number 5), antispasmodics (papaverine, no-shpa), vitamins, anabolic steroids (nerobol, retabolil); pancreatic enzymes.

Phytotherapy, biological products, enteroseptol or intestopan; physiotherapy and spa treatment. Indications for surgical treatment:

1) pancreatic cyst;

2) organic duodenospasm;

3) narrowing or obturation of the common bile duct;

4) cicatricial narrowing in the region of the papilla of Vater.

46. ​​Classification of diseases of the joints

1. Rheumatism (rheumatic fever).

2. Diffuse connective tissue diseases:

1) systemic lupus erythematosus;

2) systemic scleroderma;

3) diffuse fasciitis;

4) dermatomyositis;

5) Sjogren's disease;

6) rheumatic polyalgia;

7) recurrent polychondritis;

8) Tietze's disease.

3. Systemic vasculitis (angiitis, arteritis):

1) nodular periarteritis;

2) granulomatous arteritis;

3) hyperergic angiitis;

4) Behçet's syndrome.

4. Rheumatoid arthritis.

5. Juvenile arthritis.

6. Ankylosing spondylitis (Bekhterev's disease).

7. Arthritis associated with spondyloarthritis:

1) psoriatic arthritis;

2) Reiter's disease;

3) arthritis in chronic nonspecific bowel diseases.

8. Arthritis associated with infection:

1) infectious arthritis;

2) reactive arthritis.

9. Microcrystalline arthritis:

1) gout;

2) chondrocalcinosis;

3) osteoarthritis.

10. Other diseases of the joints:

1) palindromic rheumatism; 2) multiple reticulohistiocytosis. 11. Arthropathy in non-rheumatic diseases:

1) allergic diseases;

2) metabolic disorders;

3) congenital DMST;

4) endocrine diseases;

5) damage to the nervous system;

6) diseases of the blood system;

7) diseases of extra-articular soft tissues;

8) bone diseases and osteochondropathy;

9) bone diseases: osteoporosis, osteomalacia;

10) deforming osteitis (Paget's disease), osteolysis;

11) osteochondropathy;

12) Perthes disease.

47. Juvenile rheumatoid arthritis

Juvenile rheumatoid arthritis is a disease from the group of diffuse connective tissue diseases (DCT). It is based on immunopathological processes.

It is characterized in most patients by an acyclic protracted or chronic course with systemic damage to the connective tissue, mainly the musculoskeletal system. Girls get sick 1,5-2 times more often than boys. The peak of the disease occurs at 5-7 and 12-14 years.

Classification.

According to the kylinico-anatomical characteristics: rheumatoid arthritis, predominantly articular form (with or without eye damage): polyarthritis, oligoarthritis, monoarthritis.

Rheumatoid arthritis, articular-visceral form: with limited visceritis (damage to the heart, blood vessels, kidneys, lungs, central nervous system, skin, mucous eyes, amyloidosis of internal organs).

Still's syndrome: an allergoseptic syndrome of rheumatoid arthritis in combination with rheumatism and DBST.

According to clinical and immunological characteristics: seropositive rheumatoid arthritis (test for rheumatoid factor is positive), seronegative rheumatoid arthritis (test for rheumatoid factor is positive).

According to the course of the disease: rapidly progressive, slowly progressive, without noticeable progression.

According to the degree of activity: high, medium, low, remission.

According to the functional ability of the patient:

1) with the preservation of functional ability; י

2) with a violation of the state of the musculoskeletal system: the ability to self-service is preserved, the ability to self-service is partially lost, the ability to self-service is completely lost;

3) with a violation of the condition of the eyes or internal organs.

Clinical signs of juvenile rheumatoid arthritis are as follows.

1. Arthritis lasting more than 3 weeks (mandatory sign).

2. The defeat of three joints during the first 3 months.

3. Symmetrical damage to small joints.

4. Damage to the cervical spine.

5. Exudate in the joint cavity.

6. Morning stiffness.

7. Tenosynovitis or bursitis.

8. Rheumatoid eye damage.

9. Rheumatoid nodules.

10. X-ray changes: epiphyseal osteoporosis.

11. Narrowing of the joint space.

12. Signs of effusion in the joint.

13. Compaction of periarticular tissues.

14. Laboratory data: ESR more than 35 mm/hour.

15. Positive rheumatoid factors.

16. Positive synovial biopsy data.

The presence of 7-8 symptoms - classic RA, 4-5 symptoms - definite RA, 2-3 symptoms - probable RA.

48. Reactive arthritis

Reactive arthritis is an inflammatory disease of the joints, chronologically associated with a specific infection, in which the microorganism or its antigens are not detected in the joint cavity. Reactive arthritis has a wide worldwide distribution, accounting for half of all lesions of the musculoskeletal system in children.

Criteria for the diagnosis of reactive arthritis.

Anamnestic: 1-4 weeks before the present illness ARVI, enterocolitis, acute disease of the genitourinary tract. Exacerbation of the focus of chronic infection.

Clinical: arthritic symptom complex: asymmetric joint damage: swelling, pain, local temperature increase, skin hyperemia, dysfunction. Reactive arthritis is not characterized by morning stiffness and migration of lesions. Complete reversibility of the articular syndrome in the treatment of the underlying disease within 1-2 weeks.

Infectious and inflammatory symptoms: pallor of the skin and mucous membranes, swollen lymph nodes, subfebrile temperature, inflammatory reaction of the blood.

Residual manifestations of an acute disease preceding arthritis: dry unproductive cough, congestive hyperemia of the pharynx, granularity of the posterior pharyngeal wall, swollen cervical lymph nodes (after SARS); loss of appetite, nausea, unstable stool, dysbacteriosis (after enterocolitis); violation of urination, microproteinuria, leukocyturia, microhematuria, subfebrile temperature (after pyelonephritis).

Paraclinical: blood test, R-gram of the joints - the presence of effusion in the joint cavity, compaction of periarticular tissues. Joint puncture - aseptic inflammation. Basic principles of treatment of rheumatoid arthritis:

1) elimination of infectious foci that support the articular process;

2) regulation of immunological reactivity;

3) impact on the local process in the joint and periarticular tissues with the help of intra-articular injection of anti-inflammatory drugs; physical factors, exercise therapy, massage, orthopedic measures;

4) hormone therapy only for severe articular-visceral forms of rheumatoid arthritis, with the unsuccessful use of other types of treatment;

5) stimulating therapy (vitamins, non-specific adaptogens, anabolic steroid drugs) strictly according to indications;

6) replacement and symptomatic therapy (painkillers, muscle relaxants, diuretics, antihypertensives, cardiovascular drugs; enzymes);

7) rational nutrition and appropriate regimen (in the acute period - bed with immobilization of the joint up to a plaster splint);

8) radical methods of treatment: surgical in combination with orthopedic for severe joint deformities and for the ineffectiveness of a conservative method of treatment.

49. Hereditary metabolic diseases

Hereditary metabolic diseases are a monogenic pathology in which gene mutation entails certain pathochemical disorders.

The classification is as follows.

1. Hereditary diseases of amino acid metabolism: alkaptonuria, albinism, hypervalemia, histidinemia, homocystinuria, leucinosis, tyrosinosis, phenylketonuria.

2. Hereditary diseases of carbohydrate metabolism: galactosemia, glycogenosis, disaccharidase deficiency, lactic acidosis, fructose intolerance.

3. Hereditary diseases of lipid metabolism: plasma lipidosis (hereditary hyperlipidemia, hypercholesterolemia, mucolipidoses; deficiency of lecithincholesterol acetyl granferase); cellular lipidosis - agangliosidoses (Tay-Sachs disease); sphingolipidoses (Niemann-Pick disease), cerebrosidoses (Gaucher disease).

4. Hereditary diseases of purine and pyrimidine metabolism: gout, Lesh-Nihan syndrome, orotic aciduria.

5. Hereditary diseases of the metabolism of corticosteroid biosynthesis: adrenogenital syndrome, hypoaldosteronism.

6. Hereditary diseases of porphyrin and bilirubin metabolism: Crigler-Najjar, Gilbert syndromes.

7. Hereditary diseases of connective tissue metabolism: mucopolysaccharidoses, Marfan's disease, Ehlers-Danlos syndrome.

8. Hereditary diseases of metal metabolism: Wilson-Konovalov disease, Menkes disease (copper metabolism), hemochromatosis (iron metabolism), familial periodic paralysis (potassium metabolism).

9. Hereditary diseases of erythron metabolism: hemolytic anemia, Fanconi anemia.

10. Hereditary diseases of lymphocyte and leukocyte metabolism: immunodeficiency states with adenosine deaminase deficiency, septic granulomatosis.

11. Hereditary metabolic diseases of the transport systems of the kidneys (tubulopathies): renal tubular acidosis, de Toni-Debre-Fanconi disease, vitamin D-resistant rickets, etc.

12. Hereditary metabolic diseases of the gastrointestinal tract: malabsorption syndrome with disaccharidases deficiency, chloridorep, pathologies of intestinal transport of glucose, galactose.

Diagnostic criteria: mental retardation; athetosis, ataxia, convulsive syndrome, repeated coma; relapses of ketoacidosis; specific smell of urine; myopathies, skeletal anomalies, immunodeficiency; hair and skin changes, cataracts; hepatosplenomegaly, malabsorption syndrome; unexplained sibling deaths.

50. Diseases of amino acid metabolism. Phenylketonuria

Hereditary diseases in which the transport of amino acids through the intestinal mucosa and renal tubules is impaired or their catabolism is altered due to a deficiency of enzymes or coenzymes.

General diagnostic criteria for amino acid metabolism disorders require additional laboratory testing:

1) a combination of mental retardation with visual pathology (homocystinuria, sulfite oxidase deficiency);

2) mental retardation and convulsive syndrome (nonketotic hyperglycinemia, carnosinemia, phenylketonuria, arginine-succinic aciduria, citrullinemia, ornithine transcarbamylase deficiency, type I and II hyperlysinemia);

3) the presence of a changed color and smell of urine (phenyl-ketonuria, alkaptonuria, maple-scented urine disease);

4) damage to the liver and central nervous system (arginemia);

5) a combination of mental retardation with skin lesions (hereditary xanthinuria, phenylketonuria, histidinemia);

6) abnormal smell of urine (glutaric, type II and isovaleric acidemia - the smell of sweaty feet, phenyl-ketonuria - the smell of mice or musty, β3-methylcroto-nylglycinuria - the smell of cats, methionine malabsorption - the smell of cabbage, trimethylaminuria - the smell of rotting fish, tyrosinemia - rancid or fishy smell).

Phenylketonuria

Hereditary disease, which is based on a deficiency of the enzyme phenylalanine hydroxylase, which ensures the conversion of phenylalanine to tyrosine, as a result, phenylalanine and its derivatives accumulate in the body, which have a toxic effect.

Etiology: autosomal recessive inheritance.

Diagnostic criteria: at birth, children look completely normal, the manifestation of the disease appears at 2-6 months in the form of lethargy, lack of interest in the environment, regurgitation; muscle hypotension, seizures; retardation of static-motor and psycho-speech development. There is increased irritability, anxiety, subsequently - a deep degree of mental retardation; allergic dermatitis, hypopigmentation of the skin, hair, iris; mouse smell of urine.

Examination plan:

1) Felling's test: green coloration of urine, iron trichloride addition test;

2) determination of the level of phenylalanine in the blood (increased: more than 900-1200 µmol/l);

3) special methods for direct diagnosis of a mutant gene using synthetic oligonucleotides; probes;

4) biochemical test - loading with phenylalanine at a dose of 25 mg/kg;

5) consultations of an oculist, a neuropathologist;

6) molecular genetic methods for diagnosing a gene defect in PKU: blood is taken from newborns on the 4th-5th day of life in a full-term baby and on the 7th day of life in a premature baby.

51. Histidinemia, arginemia, Hartnup's disease

Histidinemia

The basis of the disease is a violation of the metabolism of the amino acid histidine as a result of histidase deficiency.

Etiology: the type of inheritance is autosomal recessive.

The manifestation of the disease is different - from the first days to adulthood.

Diagnostic criteria vary widely, ranging from severe mental retardation to no symptoms.

Clinical signs: psychomotor and speech development is delayed; muscle hypotension, convulsive syndrome; blond hair and blue eyes.

Survey plan.

Determination of the level of histidine: in the blood and urine - increased.

Determination of the pathological reaction to the load with tidine (100 mg/kg of body weight).

Determination of the content of urokinic acid in urine or sweat.

Consultation of a neurologist.

The differential diagnosis is with phenylketonuria.

Treatment: diet - exclude foods rich in histidine (beef, chicken, eggs, milk, cottage cheese, cheese, peas, flour). Natural feeding of children of the first year of life is recommended.

Symptomatic therapy.

Arginemia

Hereditary disorders of arginine metabolism as a result of a decrease in the activity of the arginase enzyme, which catalyzes the breakdown of arginine into ornithine and urea.,

Etiology. The type of inheritance is autosomal recessive.

Diagnostic criteria: cerebral disorders in the form of profound mental retardation, spastic diplegia, convulsive syndrome; hepatomegaly.

Examination plan:

1) determination of the excretion of arginine-succinic acid in the urine;

2) consultation of a neurologist. Differential diagnosis is made with glyco

genoses, other types of amino acid metabolism disorders.

Treatment: diet (low protein with the addition of a mixture of essential amino acids). Symptomatic therapy. Hartnup disease

The disease is characterized by impaired intestinal and renal active transport of certain neutral α-amino acids (tryptophan, lysine, methionine, glycine).

Etiology: type of inheritance autosomal recessive.

Diagnostic criteria: pellagra-like photosensitive dermatitis; cerebellar ataxia, tremor, nystagmus, mental retardation, depression, phobias; generalized hyperaminoaciduria, absence of tryptophan in the urine, increased excretion of indole compounds.

Survey plan. Test for aminoaciduria (hyperaminoaciduria). Determination of indole and its derivatives in urine. Determination of amino acids (tryptophan, lysine, methionine, glycine) in blood serum. Consultation of a dermatologist, neuropathologist.

Treatment: A protein-restricted diet enriched with fruits. The introduction of nicotinamide, pyridoxine. Protecting the skin from exposure to sunlight.

52. Glycogenosis

A group of enzymopathies in which the processes of breakdown and synthesis of glycogen are disrupted, which leads to its accumulation in various organs (liver, kidneys, muscles)

Etiology. The type of inheritance is an autosomal recessive disease, depending on the nature of the enzyme defect, 12 types are distinguished.

Diagnostic criteria

Type I - hepatorenal glycogenosis (Girke's disease). Clinical criteria: hepatomegaly (dense liver with a smooth surface), large abdomen; growth retardation, "doll" appearance of the child (small stature, short limbs, enlarged abdomen, reduced muscle strength), xanthomas on the skin; in the first weeks of life: lethargy, weakness, sometimes vomiting, malnutrition; hypoglycemic conditions: pallor, sweating, convulsions, coma; increased appetite due to hypoglycemia; mental development does not suffer, sexual development is delayed. On palpation - an increase in the liver, dense, smooth, painless, the edge descends into the small pelvis.

Laboratory data: fasting hypoglycemia, diabetic nature of the glucose tolerance test, no increase in blood sugar in response to the administration of adrenaline and glucagon, hyperketonemia, acetonuria; an increase in the content of glycogen in the liver cells (in the study of a biopsy), an increase in the content of glycogen in the cells of peripheral blood. Hyperlactatemia and hyperuricemia are also characteristic.

Type II - glycogen cardiomegaly (Pompe disease).

It is manifested by the widespread deposition of glycogen in the liver, kidneys, heart muscle, in the nervous system, and skeletal muscles. Clinical manifestations after birth: anorexia, vomiting, muscle weakness, shortness of breath, cyanosis, enlargement of the heart, progressive right ventricular heart failure; frequent pneumonia against the background of atelectasis with severe respiratory failure; externally: round pasty face, enlarged tongue, muscular hypotension, delayed physical development. Neurological disorders associated with muscle weakness.

Laboratory data in the analysis of blood and urine are unchanged. Liver biopsy showed large amounts of glycogen.

Type III - limit dextrinosis (Forbes-Corey disease).

Incomplete breakdown of glycogen and the formation of a polysaccharide with shortened terminal branches of the molecule.

Clinic as in type I. The difference is an increase in blood sugar with a load of galactose.

Examination plan:

1) general analysis of blood, urine, urine for acetone;

2) determination of blood sugar on an empty stomach, after a load of carbohydrates;

3) test with adrenaline and glucagon;

4) examination of blood serum (proteinogram, cholesterol, lipids, activity of liver enzymes);

5) determination of glycogen in a liver biopsy, in peripheral blood cells;

6) ECG and FCG;

7) consultation of an oculist.

53. Diseases of lipid metabolism

Lipidoses are included in an extensive group of hereditary storage diseases, united by the phenomenon of accumulation in cells of various metabolic products.

Niemann-Pick disease (sphingolipidosis)

Hereditary enzymopathy, characterized by the accumulation of sphingomyelin in the brain, liver, RES.

Etiology: sphingomyelinase defect inherited in an autosomal recessive manner.

Diagnostic criteria

Clinical: early onset and malignant course of the disease, psychomotor retardation, spastic tetraparesis, deafness, blindness; progressive hepato- and splenomegaly; brown shade of the skin, on the fundus - a cherry-red spot.

Laboratory data: detection of Niemann-Pick cells in the punctate of the bone marrow and spleen; enzyme diagnostics (examination of skin fibroblasts or leukocytes after their treatment with ultrasound) - the establishment of a metabolic block.

Gaucher disease

Hereditary lipid metabolism disease associated with the accumulation of cerebrosides in the cells of the nervous and reticuloendothelial systems.

Etiology. The disease is caused by a defect in a lysosomal enzyme inherited in an autosomal recessive manner.

Diagnostic criteria. Acute form of Gaucher disease (children's type): from the first months of life, delay in physical and neuropsychic development, malnutrition, muscle hypertension, opisthotonus, convulsions, trismus; hepatosplenomegaly, respiratory failure.

Changes in the skeletal system, the appearance of pain during movement in the tubular bones, death in the 1st year of life.

Chronic form (juvenile type): can be at any age; splenomegaly, anemia, hemorrhagic syndrome; skeletal deformity; brown or ocher pigmentation on the front of the legs.

Laboratory studies: detection of Gaucher cells in bone marrow, spleen, lymph nodes.

Tay-Sachs disease

The basis is a violation of the metabolism of gangliosides with their increased deposition in the gray matter of the brain, liver, spleen.

Etiology. Hexosaminidase defect. The type of inheritance is autosomal recessive.

Diagnostic criteria: manifests in 3-4 months; the child is lethargic, inactive, loses interest in the environment; refusal to eat, vomiting, regurgitation; delayed psychomotor development, hepatosplenomegaly; progressive degradation of intelligence up to idiocy; various paralysis, including pseudobulbar with swallowing disorder; atrophy of the nipples of the optic nerves and a cherry-red spot in the macular region; decreased activity of hexosaminidase. Deafness and blindness develop. Children quickly lose weight and death occurs in 1-1,5 years.

54. Malformations of the bronchopulmonary system

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-Kun's syndrome);

6) Williams-Campbell syndrome;

7) congenital lobar emphysema.

2. Defects 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.

According to various authors, hereditary diseases of the respiratory system make up from 5 to 35% of the total number of patients with nonspecific lung diseases.

Chronic lung disease in children

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.

55. Classification of COPD in children

Congenital diseases:

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, immovable cilia syndrome, Kartagener's syndrome;

c) universal genetically determined exocrinopathy (abnormal viscosity of bronchial secretions).

cystic fibrosis;

2) local type of changes causing obstruction (malformations):

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

1. Stage I includes lung agenesis as a result of the absence of a primary bronchial kidney.

2. At stage II, 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 3rd-4th week of the embryonic period.

3. Stage III of the violation occurs on the 30-40th day of intrauterine development and is characterized by the presence of lung hypoplasia.

4. Stage IV (2-5th month of the prenatal period) is determined by a violation of the development of small bronchi and leads to the occurrence of polycystic lung disease.

56. Congenital and hereditary lung diseases

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.

Polycystic lung disease 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.

Congenital lobar emphysema is characterized by stretching of the parenchyma of the lobe (rarely of the segment) due to partial obstruction of the draining bronchus.

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.

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.

Idiopathic diffuse pulmonary fibrosis.

The disease is based on damage to small vessels of the lungs by deferred immune complexes. Morphologically, it is characterized by thickening of the walls of the alveoli due to mononuclear infiltration and fibrosis. In the walls of the alveoli, an increase in the network of reticular fibers, which are later replaced by collagen ones, was revealed.

Primary pulmonary hypertension (Aers syndrome) is characterized by hypertrophy of the right ventricular myocardium, 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.

Goodpasture's syndrome is a combination of pulmonary hemosiderosis and glomerulonephritis, characterized by immunological damage to the basement membranes of the lungs and kidneys.

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.

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.

Author: Pavlova N.V.

We recommend interesting articles Section Lecture notes, cheat sheets:

▪ Constitutional law of the Russian Federation. Lecture notes

▪ Pathological anatomy. Lecture notes

▪ Infectious diseases. Crib

See other articles Section Lecture notes, cheat sheets.

Read and write useful comments on this article.

<< Back

Latest news of science and technology, new electronics:

Artificial leather for touch emulation 15.04.2024

In a modern technology world where distance is becoming increasingly commonplace, maintaining connection and a sense of closeness is important. Recent developments in artificial skin by German scientists from Saarland University represent a new era in virtual interactions. German researchers from Saarland University have developed ultra-thin films that can transmit the sensation of touch over a distance. This cutting-edge technology provides new opportunities for virtual communication, especially for those who find themselves far from their loved ones. The ultra-thin films developed by the researchers, just 50 micrometers thick, can be integrated into textiles and worn like a second skin. These films act as sensors that recognize tactile signals from mom or dad, and as actuators that transmit these movements to the baby. Parents' touch to the fabric activates sensors that react to pressure and deform the ultra-thin film. This ... >>

Petgugu Global cat litter 15.04.2024

Taking care of pets can often be a challenge, especially when it comes to keeping your home clean. A new interesting solution from the Petgugu Global startup has been presented, which will make life easier for cat owners and help them keep their home perfectly clean and tidy. Startup Petgugu Global has unveiled a unique cat toilet that can automatically flush feces, keeping your home clean and fresh. This innovative device is equipped with various smart sensors that monitor your pet's toilet activity and activate to automatically clean after use. The device connects to the sewer system and ensures efficient waste removal without the need for intervention from the owner. Additionally, the toilet has a large flushable storage capacity, making it ideal for multi-cat households. The Petgugu cat litter bowl is designed for use with water-soluble litters and offers a range of additional ... >>

The attractiveness of caring men 14.04.2024

The stereotype that women prefer "bad boys" has long been widespread. However, recent research conducted by British scientists from Monash University offers a new perspective on this issue. They looked at how women responded to men's emotional responsibility and willingness to help others. The study's findings could change our understanding of what makes men attractive to women. A study conducted by scientists from Monash University leads to new findings about men's attractiveness to women. In the experiment, women were shown photographs of men with brief stories about their behavior in various situations, including their reaction to an encounter with a homeless person. Some of the men ignored the homeless man, while others helped him, such as buying him food. A study found that men who showed empathy and kindness were more attractive to women compared to men who showed empathy and kindness. ... >>

Random news from the Archive 3D Vision: second version 21.12.2011 NVIDIA delighted fans of 3D video by presenting its new developments in the field of stereoscopic imaging. Nvidia 3D Vision 2 is the name of a new software and hardware system for viewing stereo images on a PC screen. Its main differences from the first generation model are improved performance and a redesigned active shutter glasses. The size of the eyepieces has increased by about 20%, which significantly expands the field of view for users. Their closed design made it possible to almost completely get rid of side flares. NVIDIA engineers also worked on optimizing the algorithm for switching between the LCD shutters of the right and left eyepieces. In this way, the open time of both shutters has been reduced to a minimum, resulting in reduced flickering. Also noteworthy is the introduction of NVIDIA 3D LightBoost technology, which will now be mandatory for all NVIDIA 3D Vision 2 certified monitors. When the display is switched to stereoscopic mode, the brightness of the screen is greatly increased to compensate for the loss of brightness when light passes through the glasses.

News feed of science and technology, new electronics

Interesting materials of the Free Technical Library:

▪ section of the site Spy stuff. Article selection

▪ article by Hayao Miyazaki. Famous aphorisms

▪ article Do leeches suck bad blood? Detailed answer

▪ Article Carnation. Legends, cultivation, methods of application

▪ article Alcohol varnish for violins. Simple recipes and tips

▪ article Akan proverbs and sayings. Large selection

Leave your comment on this article:

All languages ​​of this page

Arabic	Hebrew	Polish
Bengali	Hindi	Portuguese
Chinese	Italian	Spanish
English	Japanese	Turkish
French	Korean	Ukrainian
German	Malay	Vietnamese

Home page | Library | Articles | Website map | Site Reviews

www.diagram.com.ua
2000-2024