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Kawasaki disease


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Kawasaki disease

  1. 1. Kawasaki Disease: Dr.B.BALAGOBI
  2. 2. KAWASAKI DISEASE• Vasculitis of unknown etiology• Multisystem involvement and inflammation of small and medium sized arteries with aneurysm formation• More common among children of Asian decent• Usually children <5 years; peak 2-3 years
  3. 3. • KAWASAKI DISEASE In children < 3 months of age – Usually see atypical course leading to rapid and severe coronary artery damage (CAD) – ECHO mandatory if considered in this age group; diagnosis very difficult• Age is independent risk factor for CAD• CAD develops in 5% of timely treated patients• Prolonged fever is hallmark of the disease• Lymphadenopathy is least common finding (seen in 75% of cases compared with 90% for other signs)• Coronary lesions are usually not present until 10 days; therefore decision to treat made prior to knowledge of cardiac outcome• Other useful signs – Extreme irritability – Inflammation of BCG scar
  4. 4. Etiology• Infectious agent most likely – Age-restricted susceptible population – Seasonal variation – Well-defined epidemics – Acute self-limited illness similar to known infections• No causative agent identified – Bacterial, retroviral, superantigenic bacterial toxin – Immunologic response triggered by one of several microbial agents
  5. 5. KAWASAKI DISEASE – CLINICAL PRESENTATION• Acute phase (1-2 weeks) – Sudden onset of high fever followed by conjunctival erythema, mucosal changes, cervical adenopathy, swelling of hands and feet – Irritability – Abdominal pain, hydrops of gall bladder – Arthritis – Carditis – tachycardia, CHF, giant coronary artery aneurysms
  6. 6. KAWASAKI DISEASE – CLINICAL PRESENTATION• Subacute phase – Lasts up to 4th week – Resolution of fever and other symptoms – Desquamation of fingers and toes – Elevation of platelet count – Coronary artery aneurysms• Convalescent phase – Disappearance of clinical symptoms – 6-8 weeks after initial symptoms
  7. 7. Diagnostic Criteria• Fever for at least 5 days• At least 4 of the following 5 features: 1. Changes in the extremities  Edema, erythema, desquamation 2. Polymorphous exanthem, usually truncal 3. Conjunctival injection 4. Erythema&/or fissuring of lips and oral cavity 5. Cervical lymphadenopathy• Illness not explained by other known disease process
  8. 8. Trager, J. D. N Engl J Med 333(21): 1391. 1995.
  9. 9. Trager, J. D. N Engl J Med 333(21): 1391. 1995.
  14. 14. Circulation 103(2):335-336. 2001.
  15. 15. • Infectious Differential Diagnosis – Measles & Group A beta-hemolytic strep can closely resemble KD – Bacterial: severe staph infections w/toxin release – Viral: adenovirus, enterovirus, EBV, roseola – Spirocheteal: Lyme disease, Leptospirosis – Parasitic: Toxoplasmosis – Rickettsial: Rocky Mountain spotted fever, Typhus• Immunological/Allergic – JRA (systemic onset) – Atypical ARF – Hypersensitivity reactions – Stevens-Johnson syndrome
  16. 16. KAWASAKI DISEASE - COMPLICATIONS• Coronary artery thrombosis and coronary and peripheral artery Coronary artery thrombosis and coronary and peripheral artery aneurysm• Myocardial infarction• Myopericarditis• Congestive heart failure• Hydrops of gall bladder• Aseptic meningitis• Arthritis• Sterile pyuria (urethritis)• Thrombocytosis• Diarrhea• Pancreatitis• Peripheral gangrene
  17. 17. Kawasaki Disease: Labs • Late• Early – Thrombocytosis – Leukocytosis – Elevated CRP – Left shift – Mild anemia • EKG changes – Thrombocytosis – Arrhythmias – Elevated ESR – Abnormal Q waves – Elevated CRP – Prolonged PR and/or QT – Hypoalbuminemia intervals – Elevated transaminases – Low voltage – Sterile pyuria – ST-T–wave changes. • CXR–cardiomegaly
  18. 18. KAWASAKI DISEASE - TREATMENT• IV Immunoglobulin (mechanism unknown) – Single dose of 2 g/kg over 12 hours • Rapid defervescence and symptom resolution • Reduces incidence of coronary artery aneurysm• Aspirin 80-100 mg/kg/day divided q 6 hours for 48 hours then reduce by ½ – Continue for 6-8 weeks until cardiac ECHO shows no evidence of cardiac pathology
  19. 19. T/F features of kawasaki disease?A. Coronary artery aneurysm commonly occurs during acute phase.B. High dose Aspirin is used during acute phase.C. Cause polycythaemiaD. Desquamation of the skin is a feature.E. Common after 10 years of age
  20. 20. T/F regarding Kawasaki disease – Thrombocytosis is seen within 7days of the illness – Erythema and induration at the BCG immunization site may be seen – Early treatment with aspirin intravenous globulins will reduce the development of coronary arterial aneurysms – Desquamation may involve palms and soles – Pericarditis and pericardial effusion is a recognized complication