SWY To, CLS Yan, NC Fong, CW Leung

Abstract

Kawasaki disease shock syndrome (KDSS) is a newer entity being better recognised recently, which is defined as Kawasaki disease features plus systolic hypotension or clinical signs of poor perfusion. Patients with KDSS typically have more heightened levels of inflammatory markers and greater risks of coronary artery abnormalities, mitral regurgitation and persistent myocardial dysfunction. They also have greater likelihood of intravenous immunoglobulin (IVIG) resistance requiring additional anti-inflammatory treatment. We report two cases of KDSS presenting with shock, both with IVIG resistance and coronary arteries involvement. Clinicians should maintain high index of suspicion for KDSS to avoid delay in diagnosis and to facilitate timely treatment by IVIG.

川崎病休克綜合徵（KDSS）近來更為醫護人員所認識，定義為川崎病特徵加上收縮期低血壓／灌注不良的臨床表現。KDSS患者通常有更高水平的炎性標記物和更大風險的冠狀動脈異常，二尖瓣返流以及持續性的心肌功能障礙。他們也對靜脈免疫球蛋白（IVIG）有更大耐受的可能性，需要額外的抗炎治療。我們報導兩例表現出休克的KDSS，兩例皆有IVIG耐受和冠狀動脈介入。臨床醫生應當保持對KDSS的高度懷疑，從而避免診斷延誤，並給予及時的IVIG治療。