Single center blind testing of a US multi-center validated diagnostic algorithm for Kawasaki disease in Asia

Abstract

ABSTRACTKawasaki disease (KD) is the leading cause of acquired heart disease in children. A key objective of research in KD is to reduce the risk of long-term cardiovascular sequelae by expediting timely diagnosis. The major challenge in KD diagnosis is that it shares clinical signs with other childhood febrile illnesses. Our previously single-center developed computer-based two-step algorithm was further tested by a five-center validation in US. This first blinded multi-center trial validated our approach, with sufficient sensitivity and positive predictive value, to identify most patients with KD diagnosed at centers across the US.We sought to determine if our algorithmic approach applied to an Asian cohort. This study involved 418 KD and 259 febrile controls (FC) from the Chang Gung Memorial Hospital in Taiwan. Our diagnostic algorithm retained sensitivity (379 of 418; 90.7%), specificity (223 of 259; 86.1%), PPV (379 of 409; 92.7%), and NPV (223 of 247; 90.3%) comparable to previous US 2016 single center and US 2020 fiver center results. Only 4.7% (15 of 418) of KD and 2.3% (6 of 259) of FC patients were identified as indeterminate. The algorithm identified 18 of 50 (36%) KD patients who presented 2 or 3 principal criteria. Of 418 KD patients, 157 were infants younger than one year and 89.2% (140 of 157) were classified correctly. Of the 44 patients with KD who had coronary artery abnormalities, our diagnostic algorithm correctly identified 43 (97.7%) including all patients with dilated coronary artery but one who found to resolve in 8 weeks.We assessed the performance of our KD diagnostic algorithm with a single center Asian cohort. This work demonstrates the applicability of our algorithmic approach and diagnostic portability, providing evidence to support the launch of an adequately powered, multicenter study for future Asian application in the emergency department setting. If deployed in Asia, our tool promises a cost-effective diagnostic approach to allow the timely management of Asian KD patients even in the absence of KD experts, to potentially enhance the outcome for KD patients and reduce the risk of coronary artery aneurysms.