Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms

Abstract

Prompt diagnosis and the administration of intravenous immunoglobulin(IVIG) has reduced the incidence of coronary artery abnormalities (CAA)in Kawasaki Disease (KD). The resistance to treatment and development ofthe coronary sequelae remain the most important problems in KD. We aimedto determine the predicting factors of nonresponse to initial IVIG therapyand to analyze the cases who had giant coronary aneurysms. A total of 120KD cases, including 61 children fulfilling the criteria for KD and 59 withincomplete KD were enrolled into this study. Demographic, laboratory, clinical,echocardiographic characteristics, and treatment regimens were reviewed,retrospectively. The median age of the patients was 33.5 months (range: 3-168months). Coronary artery aneurysms were detected in 35 patients (29%) atthe time of diagnosis. Twenty-eight patients had coronary aneurysms smallor medium in size, one had a large, and seven had giant coronary aneurysms.CAA persisted in 8 cases in the follow-up, all of which were large or giantaneurysms. A ten month-old girl with a giant coronary aneurysm wasreferred to coronary bypass surgery in the subacute phase of follow-up, dueto myocardial ischemia. Eighteen patients were unresponsive to the initialIVIG therapy (%15), of whom 10 were diagnosed as cKD and 8 were iKD.Patients who did not respond to initial IVIG therapy, had higher white bloodcell (WBC) count, higher C-reactive protein (CRP) and lower albumin levelsthan those who did (P