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Kawasaki disease


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Kawasaki disease

  1. 1. Dr. Sachin Soni DNB Pediatrics Indraprastha Apollo Hospital, New Delhi
  2. 2. What is it?  Medium vessel vasculitis presents as acute febrile illness of childhood, characterized by coronary arteries anomaly in 15-25% of affected individuals
  3. 3. Epidemiology  Its common pediatric disorder with the annual incidence range 60-150 per 100,000  Commonest pediatric vasculitis in children below 5 years of age(1-3)  Commonest vasculitic disorder amongst all ages
  4. 4. Etiology  Genetics  Autoantigen  Toxin mediated (STSS) super antigen mediated
  5. 5. Pathogenesis Acute or subacute stages: Inflammatory infiltration of vascular wall, initially polymorphonuclear cells thereafter by macrophages, lymphocytes (primarily CD8+ T cells), and plasma cells Eedema of endothelial and smooth muscle cells IgA plasma cells are prominent in the inflammatory infiltrate In most severely affected vessels, inflammation involves all three layers of the vascular wall Destruction of the internal elastic lamina Loss of structural integrity weakens the vessel wall Dilation (ectasia) saccular or fusiform aneurysm formation Thrombi in lumen and obstruct blood flow vascular wall can become progressively fibrotic, marked intimal proliferation Arterial stenosis or occlusion
  6. 6. EPIDEMIOLOGIC CASE DEFINITION (CLASSIC CLINICAL CRITERIA) Fever persisting for at least 5 days  Presence of at least 4 principle feature 1- Changes in extremities:A- Acute:- Erythema of palm, soles and edema of hand and feet B- Sub acute:- Periungul peeling of finger, toes in 2-3 polymorphus exanthema 2- B/L Bulbar conjunctival injection without exudates 3- Changes in lip and oral cavity:- Erythema, lip cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosa 4- Cervical lymphadenopathy (>1.5 cm diameter), usually unilateral 
  7. 7. Other Clinical Findings Cardiovascular findings Congestive heart failure, myocarditis, pericarditis, valvular regurgitation, Coronary artery abnormalities, Aneurysms of medium-sized noncoronary arteries Raynaud phenomenon, Peripheral gangrene Musculoskeletal system Arthritis, arthralgias Gastrointestinal tract Diarrhea, vomiting, abdominal pain, Hepatic dysfunction, Hydrops of gallbladder Central nervous system Extreme irritability, Sensoryneural hearing loss, Aseptic meningitis, Genitourinary system Urethritis/meatitis
  8. 8. Clinical Menifestation
  9. 9. Percentages of cases
  10. 10. Laboratory finndings Complete Hemogramme Anemia Leukocytosis with neutrophilia and immature forms Thrombocytosis after one week Elevated erythrocyte sedimentation rate (ESR) Acute phase reactant Elevated CRP LFTs Hypoalbuminemia, Elevated serum transaminases, Elevated serum gamma glutamyl transpeptidase, Abnormal plasma lipids CSF CSF Pleocytosis others Hyponatremia, Leukocytosis in synovial fluid, sterile pyuria
  11. 11. Strawberry tongue
  12. 12. Congestion of bulbar conjunctiva
  13. 13. Indurative edema of the hands
  14. 14. Desquamation of the fingers
  15. 15. Coronary Artery Aneurysm Coronary angiogram demonstrating giant aneurysm of the left anterior descending coronary artery (LAD) with obstruction and giant aneurysm of the right coronary artery (RCA) with an area of severe narrowing in 6 yr old boy
  16. 16. Two-dimensional echocardiography     Most useful test to monitor development of coronary artery abnormalities Brightness of the arterial walls and lack of normal tapering of the vessels Coronary artery dimensions, adjusted for body surface area (BSA), are significantly increased in the first 5 wk after presentation BSA-adjusted coronary artery dimensions on baseline echocardiography in the first 10 days of illness to be good predictors of CAD
  17. 17. Aneurysm defined by Japanese Ministry of Health classified as: Small (<5 mm internal diameter)  Medium (5-8 mm internal diameter)  Giant (>8 mm internal diameter)   Echocardiography performed at diagnosis and after 2-3 wk of illness If the results are normal, repeat study should be performed 6-8 wk after onset of illness
  18. 18. Diagnosis Classic KD:- Diagnostic criteria Fever for at least 4 days and at least four of five of above principal characteristics of the illness Atypical or incomplete KD:   Patients have persistent fever but fewer than four of the five characteristics In these patients, laboratory and echocardiographic data can assist in the diagnosis Incomplete cases are most frequent in infants, who, unfortunately, also have the highest likelihood of development of coronary artery abnormalities
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  20. 20. Differential Diagnosis VIRAL INFECTIONS BACTERIAL INFECTIONS Adenovirus,Enterovirus,Measles,EBV Scarlet fever, Rocky Mountain spotted fever, Leptospirosis, Bacterial cervical lymphadenitis Systemic-onset juvenile idiopathic arthritis RHEUMATOLOGIC DISEASE OTHER Toxic shock syndrome, Staphylococcal scalded skin syndrome, Drug hypersensitivity reactions, StevensJohnson syndrome
  21. 21. TREATMENT Acute phase: Intravenous immunoglobulin 2 g/kg over 10-12 hr AND  Aspirin 80-100 mg/kg/day divided every 6 hr orally until patient is afebrile for at least 48 hr
  22. 22. CONVALESCENT STAGE  Aspirin 3-5 mg/kg once daily orally until 6-8 wk after illness onset
  23. 23. LONG-TERM THERAPY FOR PATIENTS WITH CORONARY ABNORMALITIES  Aspirin 3-5 mg/kg once daily orally  Clopidogrel 1 mg/kg/day (max 75 mg/day)  Most experts add warfarin or low-molecular-weight heparin for those patients at particularly high risk of thrombosis
  24. 24. ACUTE CORONARY THROMBOSIS  Fibrinolytic therapy with tissue plasminogen activator Or  Other thrombolytic agent under supervision of a pediatric cardiologist
  25. 25. Complication     Coronary artery aneurysm Acute thrombosis Coronary artery stenosis Reyes syndrome
  26. 26. • Defined by persistent or recrudescent fever 36 hr after completion of the initial IVIG infusion • Another dose of IVIG at 2 g/kg is administered to patients with IVIG resistance. • Intravenous methylprednisolone • If a second dose of IVIG or corticosteroids are ineffective • Cyclophosphamide and plasmapheresis. • Tumor necrosis factor inhibitor infliximab
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