Liang Y.-C.Chang C.-H.MING-TAI LINKao F.-Y.Huang S.-K.MEI-HWAN WU2020-12-212020-12-2120200031-3998https://www.scopus.com/inward/record.uri?eid=2-s2.0-85075288445&doi=10.1038%2fs41390-019-0668-1&partnerID=40&md5=c37c1d37599e3d86047babcba4ef51cdhttps://scholars.lib.ntu.edu.tw/handle/123456789/529249Background: We aimed to investigate the clinical implications of unresponsiveness to single or repeated courses intravenous immunoglobulin (IVIG) and Kawasaki disease (KD) shock syndrome in patients with KD in an era of a single brand of IVIG. Methods: Data were collected from National Health Insurance database 2010–2013. Characteristics of the KD patients were analyzed, including age, gender, shock, and associated coronary aneurysms. Results: There were 3043 KD patients (male: 1872) identified. Among them, 46 (1.51%) had KDSS, 261 patients (8.5%) had IVIG unresponsiveness, and 225 patients (7.4%) developed coronary aneurysms. Moreover, 51 patients did not respond to the second course IVIG therapy, i.e., re-IVIG unresponsiveness. KDSS was associated with the occurrence of IVIG unresponsiveness (P < 10?4) and re-IVIG unresponsiveness (P = 0.02). In addition to male gender and KD shock syndrome, IVIG unresponsiveness (OR: 2.18, 95% CI: 1.48–3.22, P = 0.001) and re-IVIG unresponsiveness (OR: 2.87, 95% CI: 1.40–5.89, P = 0.004) were both independent risk factors for coronary aneurysms. Conclusions: In a nationwide KD cohort, both IVIG unresponsiveness and re-IVIG unresponsiveness increase the risk of coronary aneurysms. Such observation addresses the importance of refining the treatment for IVIG unresponsiveness, at least in those with KD shock syndrome. ? 2019, International Pediatric Research Foundation, Inc.[SDGs]SDG3immunoglobulin; steroid; immunoglobulin; Article; child; child hospitalization; cohort analysis; coronary artery aneurysm; data base; drug response; female; human; immunotherapy; lack of drug effect; major clinical study; male; mucocutaneous lymph node syndrome; preschool child; priority journal; recurrent disease; repeated drug dose; retrospective study; risk factor; sex difference; shock; single drug dose; steroid therapy; treatment response; age; complication; coronary artery aneurysm; epidemiology; factual database; infant; mucocutaneous lymph node syndrome; newborn; shock; Taiwan; Age Factors; Child; Child, Preschool; Coronary Aneurysm; Databases, Factual; Female; Humans; Immunoglobulins, Intravenous; Infant; Infant, Newborn; Male; Mucocutaneous Lymph Node Syndrome; Retrospective Studies; Risk Factors; Shock; Taiwanjournal article10.1038/s41390-019-0668-1317110682-s2.0-85075288445