Which one of the following is the treatment of choice for Kawasaki's diseases?
Ans. is 'c' i.e., intravenous immunoglobulin Kawasaki diseaseIt is an acute febrile vasculitis of childhood.The illness occurs predominantly in young children, 80% of the patients are younger than 5 years.PathogenesisKawasaki disease causes a severe vasculitis of all blood vessels but predominantly affecting the medium sized arteries with predilection for the coronary arteries.Inflammation (vasculitis) involves all three layers of the vessel wall.The vessel loses its structural integrity and weakens resulting in dilatation and aneurysm formation.Clinical manifestations and diagnosisThe diagnosis of Kawasaki disease is based on demonstration of characteristic clinical signs.Diagnostic criteria for Kawasaki disease*Fever lasting for at least 5 days.Presence of at least four of the following five signs.Bilateral bulbar conjunctival injection, generally non purulent.Changes in the mucosa of the Oropharynx, including injected pharynx, injected and/dry fissured lips, strawberry tongue.changes of the peripheral extremities, such as edema and or erythema of the hands or feet in the acute phase; or periungual desquamation in the acute phase.Rash, primarily truncal; polymorphous but nonvascular.Cervical adenopathy, >1.5 cm, usually unilateral lymphadenopathy, illness not explained by other known disease process.Cardiac involvement is the most important manifestation of Kawasaki disease. These includeMyocarditis - seen in about 50% of the patients, manifested by tachycardia and decreased ventricular function.PericarditisCoronary artery aneurysm - Complications are rupture, thrombosis, stenosis, myocardial infarction.Laboratory findingsNo specific diagnostic test exists for Kawasaki disease, but certain laboratory findings are characteristic:Elevated E.S.R.Elevation of acute phase reactants i.e. (C reactive protein)Normocytic anemiaPlatelet count is normal in the first week and rapidly increases during 2nd and 3ld week.Treatment of Kawasaki diseaseAcute stageIntravenous immunoglobulin with aspirinConvalescent stageAspirin 3-5 mg/kg once daily orally until 6-8 week after illness onset.Long term therapy for those with coronary abnormalitiesAspirin 3-5 mg/kg once daily orally + dipyridamole 4-6 mg/kg/24 hr, divided in two-three doses orally.Acute coronary thrombosisPrompt fibrinolytic therapy with tissue plasminogen activator, streptokinase or urokinase under supervision of cardiologist.
Cyclosporine
Dapsone
Intravenous immunoglobulin
Methotrexate
Vasculitis