Calabri G B, Falcini F, Favilli S, Pollini I, Capuzzo L, Simonini G, De Simone L
U.O. di cardiologia Pediatrica, Ospedale A Meyer Via L. Giordano no. 13 Firenze.
Pediatr Med Chir. 2002 Sep-Oct;24(5):352-7; discussion 397-9.
Kawasaki disease is an acute generalized self-limiting vasculitis of small and medium size arteries of unknown ethiology, mostly affecting children younger then 5 years of age. Coronary arteries may be involved with aneurysms lesions, thrombotic occlusion, acute coronary syndrome and sudden death. The mortality rate has been significantly reduced by combined therapy of intravenous immunoglobuline (IVIG) and acetil salicylic acid (ASA) from 3% to 0.2%. From February 1986 to November 2001, 121 children, 76 male and 45 females with Kawasaki disease, have been observed at the Paediatric Department, University of Florence. Coronary changes (i.e. coronary ectasia and coronary aneurysms) have been observed in 22.3%. In only one 3-months old child, giant aneurysms of coronary arteries and myocardial necrosis have been detected. In 26 of 27 children at 2D echo a remodelling of the coronary changes within 12 months from the onset of the illness was observed. The mean follow-up has been five years (range 6-138 months) and in no patient further cardiac manifestations have been observed; however, as little is known about the progression of vascular damage, a more protracted follow-up is needed.
川崎病是一种病因不明的急性全身性自限性中小动脉血管炎，主要影响5岁以下儿童。冠状动脉可能会出现动脉瘤病变、血栓闭塞、急性冠状动脉综合征和猝死。静脉注射免疫球蛋白（IVIG）和乙酰水杨酸（ASA）联合治疗已使死亡率从3%显著降低至0.2%。1986年2月至2001年11月，佛罗伦萨大学儿科对121例川崎病患儿进行了观察，其中男76例，女45例。观察到冠状动脉改变（即冠状动脉扩张和冠状动脉瘤）的发生率为22.3%。仅在一名3个月大的儿童中检测到冠状动脉巨大动脉瘤和心肌坏死。在27例患儿中的26例，二维超声心动图显示在疾病发作后12个月内冠状动脉改变有重塑。平均随访时间为5年（范围6 - 138个月），未观察到任何患者有进一步的心脏表现；然而，由于对血管损伤的进展了解甚少，需要进行更长时间的随访。
