Treatment of choice of Kawasaki disease ?
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Correct Answer:
IV immunoglobulin
Description:
Kawasaki disease is a vasculitis that mostly involves the coronary vessels. It presents as an acute systemic disorder, usually affecting children under 5 years.Kawasaki's disease is an acute, febrile, multisystem disease of children. Some 80% of cases occur prior to the age of 5, with the peak incidence occurring at <=2 years. It is characterized by nonsuppurative cervical adenitis and changes in the skin and mucous membranes such as edema; congested conjunctivae; erythema of the oral cavity, lips, and palms; and desquamation of the skin of the fingeips. Although the disease is generally benign and self-limited, it is associated with coronary aery aneurysms in ~25% of cases, with an overall case fatality rate of 0.5-2.8%.Presentation is with fever, generalised rash, including palms and soles, inflamed oral mucosa and conjunctival congestion resembling a viral exanthema. The cause is unknown but it is thought to be an abnormal immune response to an infectious trigger These complications usually occur between the third and fouh weeks of illness during the convalescent stage. Vasculitis of the coronary aeries is seen in almost all the fatal cases that have been autopsied. There is typical intimal proliferation and infiltration of the vessel wall with mononuclear cells. Beadlike aneurysms and thromboses may be seenalong the aery. Other manifestations include pericarditis, myocarditis,myocardial ischemia and infarction, and cardiomegaly . It occurs mainly in Japan and other Asian countries, such as China and Korea, but other ethnic groups may also be affected. Treatment is with aspirin (5 mg/kg daily for 14 days) and IV Ig (400 mg/kg daily for 4 days) . Ref Davidsons 23e p1041 ,Harrisons 20e p2588
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