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Hospital pediatrics. Systemic vasculitis in children. Clinic, diagnosis, treatment (lecture notes) Directory / Lecture notes, cheat sheets Table of contents (expand) LECTURE No. 7. Systemic vasculitis in children. Clinic, diagnosis, treatment Systemic vasculitis is a heterogeneous group of diseases with primary inflammatory and necrotic changes in the vascular wall. Etiology of systemic vasculitis: viruses (cytomegaloviruses, hepatitis virus), bacteria (streptococci, staphylococci, salmonella), parasitic diseases (roundworm, filariotosis). Classification of vasculitis. 1. Primary vasculitis with the formation of granulomas (giant cell arteritis, Takayasu arteritis, Wegener's granulomatosis, Churg-Strauss syndrome) and without the formation of granulomas (polyarteritis nodosa, Kawasaki disease, microscopic polyangitis). 2. Secondary vasculitis in rheumatic diseases (aortitis in rheumatoid arthritis, rheumatoid vasculitis, vasculitis in rheumatic diseases), in infections (aortitis in syphilis, hepatitis B, HIV infection). 1. Microscopic polyangiitis Microscopic polyangiitis (microscopic polyarteritis) is a necrotizing vasculitis with a small number or absence of immune deposits, predominantly affecting small vessels. Clinic. The clinical picture is dominated by symptoms of glomerulonephritis and pulmonary capillaritis. Signs: early development of renal failure due to the presence of extracapillary glomerulonephritis (urinalysis shows hematuria, moderate proteinuria). No arterial hypertension. Pulmonary syndrome: damage to the upper respiratory tract - necrotic damage to the nasal mucosa (necrotizing rhinitis), not leading to destructive processes that deform the nose; in the lower respiratory tract - pronounced pulmonary hemorrhagic processes (hemorrhagic capillaritis, pulmonary hemorrhage). 2. Churg-Strauss syndrome Churg-Strauss syndrome is a granulomatous inflammation involving the respiratory tract associated with asthma and eosinophilia, and a necrotizing vasculitis affecting small and medium-sized vessels. Clinic. Bronchial asthma for several years. Increased levels of eosinophils in the peripheral blood and their migration into tissues (chronic eosinophilic infiltration of the lungs). Severe attacks of asthma + signs of systemic vasculitis (fever, myalgia, arthralgia, weight loss). Damage to the central nervous system (peripheral neuropathy, hemorrhagic stroke). Kidney damage. Damage to the gastrointestinal tract (ischemia and perforation of the wall of the stomach or intestines). Skin lesions (painful purpura mainly on the lower extremities). Diagnosis criteria. Asthma, eosinophilia > 10%, changes in the paranasal sinuses, sinusitis, mono- or polyneuropathy, variable (volatile) pulmonary infiltrates, extravascular tissue eosinophilia. Laboratory criteria: hypereosinophilia, increased immunoglobulin E, C-reactive protein, accelerated ESR. Treatment. Glucocorticoids, cytostatics, plasmapheresis. 3. Wegener's granulomatosis Wegener's granulomatosis is a granulomatous inflammation of the respiratory tract, necrotizing vasculitis of small and medium-sized vessels, usually combined with necrotizing glomerulonephritis. There are 3 forms: localized, transitional, generalized. Laboratory criteria: accelerated ESR, leukocytosis, hypergammaglobulinemia. Criteria for diagnosis. The criteria for diagnosis are as follows. 1. Ulcerative-necrotic lesion of the upper respiratory tract. 2. Progressive areas of necrosis of ENT organs with cartilage destruction. 3. Involvement of the eye orbit. 4. A large number of eosinophils, granulomas in the biopsy specimen of the nasal mucosa, septum, and palate. Treatment. Glucocorticoids, cytostatics, NSAIDs, antiaggregants, anticoagulants. 4. Behçet's disease Behçet's disease is a vasculitis that affects small and medium-sized arteries and is manifested by the clinical triad of recurrent aphthous stomatitis, ulcerative necrotic changes in the genital organs and eye damage. Diagnostic criteria. Recurrent oral ulcer (at least 3 times during the year): minor aphthae, major aphthae, herpetiform rash. Also 2 of the following signs: recurrent genital ulcers, eye lesions (anterior posterior uveitis). Skin lesions (erythema nodosum, pseudofolliculitis, papulopustular inflammation, acneiform nodules). Formation of pustules at injection sites. 5. Polyarteritis nodosa Polyarteritis nodosa is a necrotizing inflammation of medium and small caliber arteries without the formation of granulomas. Clinic. clinical options. 1. Thromboangiitis (juvenile) - damage to the vessels of the skin. 2. Visceral (classic): 1) damage to the vessels of the lungs; 2) damage to the vessels of the gastrointestinal tract; 3) damage to the vessels of the kidneys; 4) damage to the vessels of the heart. Diagnostic criteria. Loss of body weight by more than 4 kg, livedo reticularis on the skin, myalgia, weakness, decreased blood pressure (especially diastolic), arteriographic changes in blood vessels (aneurysm, occlusion). Biopsy - necrotizing vasculitis. Treatment. Glucocorticoids, cytostatics, symptomatic therapy (angioprotectors, antihypertensives, anticoagulants). Hemorrhagic vasculitis is a systemic inflammatory disease, Ig A, deposits, causing damage mainly to capillaries, arterioles and venules of the skin, joints, abdominal cavity and kidneys, which is based on multiple thrombovasculitis. It is more common in children 4-11 years old and is a classic immune complex disease. Clinical syndromes. Skin syndrome - maculopapular rashes on the lower extremities with transition to the buttocks. stomach. Articular syndrome - polyarthralgia, arthritis of large joints without deformation. Abdominal syndrome - sharp cramping pain in the abdomen, worsening after eating; nausea, vomiting. Renal syndrome - micro-, macrohematuria, severe capillary-toxic glomerulonephritis. Damage to the central nervous system - headaches, strokes, subdural hematomas, ubdural hematomas. Diagnostic criteria. Palpable purpura, age of onset of the disease - younger than 20 years, attacks of abdominal pain, hematuria, in the biopsy specimen there is granulocytic infiltration in the walls of arterioles and venules. Treatment. Exclusion of provoking factors, sanitation of foci of infection, treatment of the underlying disease, anticoagulants and antiplatelet agents, anti-inflammatory therapy, local treatment. Takayasu arteritis is a disease from the SV group, characterized by granulomatous inflammation of the aorta and its branches with the development of panarteritis; more often manifests itself in women 10-20 years old, chronic inflammation with destructive changes in the walls of large arteries, the aorta and its branches, which is accompanied by their stenosis and ischemia of the blood supplying organs. Localization of the process (IV anatomical type). 1. Aortic arch (I type). 2. Region of the descending abdominal aorta (type II). 3. Mixed forms (type III). 4. Involvement of the pulmonary artery and its branches (type IV). Clinical syndromes. Clinical syndromes in the acute phase of inflammation: general inflammatory, cardiovascular, peripheral blood flow insufficiency syndrome, cerebrovascular, pulmonary, arterial hypertension syndrome. Diagnosis criteria. The onset of the disease is before 40 years of age, a decrease in the pulse in the brachial artery, the difference in blood pressure in the arms is more than 10 mm Hg. Art., murmur heard above the subclavian artery and in the aorta, intermittent claudication, changes in the arteriogram. Treatment. Conservative (glucocorticoids, NSAIDs, aminoquinoline drugs, cytostatics, treatment of concomitant pathologies - tuberculosis, anticoagulants and antiplatelet agents, α-blockers). Surgical (excision of the occlusal part). Excision of the occlusive part of the vessel. Author: Pavlova N.V. << Back: Cardiac arrhythmias in children. Clinic, diagnosis, treatment (Dysfunction of automaticity function. Disorders of conduction function) >> Forward: Renal failure. Clinic, diagnosis, treatment (Acute renal failure. Chronic renal failure (CRF)) We recommend interesting articles Section Lecture notes, cheat sheets: ▪ General biology. Lecture notes ▪ Teria of the organization. Crib ▪ Enterprise economy. 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