Seth G. S Medical College and K.E.M. Hospital, Mumbai, Maharashtra, India.
Pediatric Hepatology, Nanavati Hospital, Mumbai, Maharashtra, India.
Ann Hepatol. 2018 Mar 1;17(2):332-334. doi: 10.5604/01.3001.0010.8665.
Kawasaki's disease (KD) is a systemic vasculitis often seen with viral and bacterial infections. Cholangitis is a known complication in biliary atresia patients post Kasai Portoenterostomy (KP). However KD, in a biliary atresia patient post KP has not been previously reported. A 1 years old girl who had previously undergone a KP for BA, presented with cholangitis which was presumed to be caused by a previous enterobacter infection that she had 2 months ago. However, on treating the cholangitis, the patient developed fever again after ten days which persisted even after changing the antibiotics. By this time she also displayed three of five characteristic features of KD in form of fever, strawberry tongue and cervical adenopathy. Investigations showed high ESR, high CRP, thrombocythemia and dilated coronary vessels on echocardiography. Treatment with intravenous immunoglobulin (IVIG) and steroids caused the symptoms to subside.
川崎病（KD）是一种常与病毒和细菌感染相关的全身性血管炎。胆管炎是卡塞氏 portaenterostomy（KP）后胆道闭锁患者的已知并发症。然而，KD 在 KP 后的胆道闭锁患者中尚未有报道。一名 1 岁女孩曾因 BA 接受 KP，出现胆管炎，推测是由她 2 个月前的肠杆菌感染引起的。然而，在治疗胆管炎时，患者在 10 天后再次发热，即使在更换抗生素后仍持续发热。此时，她还表现出川崎病的五个特征中的三个，即发热、草莓舌和颈部淋巴结肿大。检查显示血沉、C 反应蛋白高、血小板增多和超声心动图显示冠状动脉扩张。静脉注射免疫球蛋白（IVIG）和类固醇治疗使症状缓解。
