Treatment of choice of kawasaki disease?
Ans. is 'a' i.e., Intravenous immunoglobulin Kawasaki disease formerly called mucocutaneous lymph node syndrome is the most common vasculitides of childhood.It is typically a self-limited condition with fever and manifestation of acute inflammation lasting for an average of 12 days without therapy.However, cardiovascular complications particularly coronary artery (CA) aneurysms which lead to occlusion and ischemia may develop and lead to significant morbidity and mortality.The frequency of coronary artery aneurysm development and associated mortality and morbidity have been dramatically decreased as a result of intravenous immune globulin (IVIG)QThis therapy is most effective for preventing coronary artery abnormalities, but the benefit in children who have already developed coronary artery aneurysms are more equivocal. Thus expeditious diagnosis and timely treatment are critical to achieve the optimal clinical outcome.All children diagnosed with Kawasaki disease are treated at the time of diagnosis.The recommended initial therapy includes: -Intravenous immunoglobulin administered as single infection andAspirinIntravenous immunoglobulinSince the first report of intravenous immune globulin (IVIG) therapy, randomized controlled studies and meta analyses have confirmed that IVIG plus aspirin compared to aspirin alone reduces the risk of coronary artery aneurysm.Although aspirin does not appear to affect aneurysm formation all trials of IVIG treatment have included aspirin as well since it was treatment of choice at the time IVIG was introduced.The mechanism of beneficial effect of IVIG remains unknown.IVIG appears to have a generalized anti-inflammatory effect with reduction of fever and acute markers of inflammation.Possible mechanisms for these actions are cytokine level and production, neutralizing bacterial super antigens.Treatment of Kawasaki diseaseAcute stageIntravenous immunoglobulin with aspirin.Convalescent stageAspirin 3-5 mg/kg once daily orally until 6-8 week 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
Intravenous immunoglobulin
Steroids
Dapsone
Methotrexate
Vasculitis