Vieni Giuseppe, Pusceddu Sara, Bruno Ivana, Ragni Luca, Marchetti Federico
U.O.C. Pediatria e Neonatologia, Ospedale S. Maria delle Croci, AUSL Romagna, Ravenna.
U.O. Cardiologia Pediatrica e dell'Età Evolutiva, Policlinico S. Orsola, Bologna.
G Ital Cardiol (Rome). 2018 Feb;19(2):111-114. doi: 10.1714/2868.28942.
Kawasaki disease is a systemic vasculitis that presents with stable vital signs. Although it is well known that Kawasaki disease can cause myocarditis, tachycardia and heart failure during the acute stage, Kawasaki disease shock syndrome (KDSS) has recently been described. It is characterized by hypotension, signs and symptoms of poor perfusion and a shock-like state. We report the case of a 7-year-old boy with KDSS who presented with persistent fever, conjunctival injection, laterocervical lymphadenitis, and echocardiography showing signs of myocardial dysfunction and shock. The patient's hemodynamic status markedly improved with immunoglobulin therapy. Early recognition of KDSS can be challenging; however, delay in diagnosis may increase the risk of coronary artery abnormalities and death.
川崎病是一种伴有生命体征稳定的全身性血管炎。虽然众所周知川崎病在急性期可导致心肌炎、心动过速和心力衰竭，但最近已描述了川崎病休克综合征（KDSS）。其特征为低血压、灌注不良的体征和症状以及休克样状态。我们报告了一例患有KDSS的7岁男孩病例，该患儿表现为持续发热、结膜充血、颈侧淋巴结炎，超声心动图显示有心肌功能障碍和休克的迹象。免疫球蛋白治疗后患者的血流动力学状态明显改善。早期识别KDSS可能具有挑战性；然而，诊断延迟可能会增加冠状动脉异常和死亡的风险。
