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 of the coronary sequelae remain the most important problems in KD. We aimed to determine the predicting factors of nonresponse to initial IVIG therapy and to analyze the cases who had giant coronary aneurysms. A total of 120 KD cases, including 61 children fulfilling the criteria for KD and 59 with incomplete 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: 3-168 months). Coronary artery aneurysms were detected in 35 patients (29%) at the time of diagnosis. Twenty-eight patients had coronary aneurysms small or 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 giant aneurysms. A ten month-old girl with a giant coronary aneurysm was referred to coronary bypass surgery in the subacute phase of follow-up, due to myocardial ischemia. Eighteen patients were unresponsive to the initial IVIG 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 blood cell (WBC) count, higher C-reactive protein (CRP) and lower albumin levels than those who did (P<0.05). In univarite analysis; CRP, WBC and albumin were found to be significant predictors of nonresponse to initial IVIG therapy, while a stepwise multiple linear regression analysis showed that WBC count and albumin levels were significantly correlated with nonresponse to initial treatment with IVIG. Our study showed that WBC count and albumin levels might be used as predictors of nonresponse to the IVIG therapy in Turkish children with KD.

Is there an association between intravenous immunoglobulin resistance and coronary artery lesion in Kawasaki disease?—Current evidence based on a meta-analysis

“…However, 73 studies were excluded by reading the complete researches due to article type (n = 18), using the modified IVIG dosing as the initial treatment (n = 7), and lacking specific numbers of CAL cases in KD patients with IVIG resistance or response (n = 48). Ultimately, 53 articles [8,10,11,13, were included in the meta-analysis. The kappa test showed the kappa value of agreement during the systematic searches was 0.86.…”

“…Even though the precise molecular mechanism of CAL and IVIG in KD are still unclear, many clinical studies suggest IVIG resistance has deeply associated with the occurrence of CAL [7][8][9]. Thus, a great deal of literature have used this as a starting point to study the indicators for predicting IVIG resistance [10][11][12][13], with the expectation that additional therapeutic measures can be taken to reduce the incidence of CAL through early diagnosis of IVIG resistance.…”

Cardiovascular Compromise in Pediatrics in the Times of COVID-19: SARS-CoV-2 Infection and Kawasaki Disease

“…Other complications include myocarditis, pericardial effusion with pericarditis, mitral valvulitis, and coronary artery stenosis. Coronary aneurysms occur in 20-25% of all patients who do not receive treatment with intravenous immunoglobulin [26].…”