At the Heart of Treating Kawasaki Disease: The Search for New Approaches to Prevent Coronary Artery Aneurysms.
At the Heart of Treating Kawasaki Disease: The Search for New Approaches to Prevent Coronary Artery Aneurysms.
复制标题
治疗川崎病的核心：寻找预防冠状动脉瘤的新方法。
DOI：
10.1002/art.42346
复制
发表时间：
2023
期刊：
影响因子：
--
通讯作者：
Lee,PuiY
中科院分区：
文献类型：
--
作者：
Du,Yan;Lee,PuiY
川崎是一种中等大小的血管炎，主要影响幼儿。KD患者表现为反复发热和多形性皮疹、粘膜炎、保留角膜缘的结膜炎、肢体变化（红斑和硬结）和单侧颈部淋巴结病的标志性症状。KD最可怕的并发症发生在冠状动脉中，该冠状动脉维持向心脏的含氧血液供应。如果不治疗，20-30%的患者会出现冠状动脉异常，从血管扩张到大动脉瘤（1）。在发达国家，KD是儿童获得性心脏病的主要病因，冠状动脉瘤（coronary artery aneurysms，CAA）是KD患者短期和长期死亡的主要原因。罕见的几周内猝死病例通常是由CAA血栓形成引起的，导致心肌梗死或巨大动脉瘤破裂。虽然在约50%的病例中冠状动脉异常完全消退，但持续性动脉瘤的血管仍有血栓形成和狭窄的风险，通常需要多次动脉介入治疗和显著的长期发病率（2）。
Kawasaki disease (KD) is a medium-sized vessel vasculitis primarily affecting young children. Patients with KD present with recurrent fever and the hallmark constellation of polymorphous skin rash, mucositis, limbal-sparing conjunctivitis, extremity changes (erythema and induration) and unilateral cervical lymphadenopathy. The most feared complication of KD occurs in the coronary arteries that maintain oxygenated blood supply to the heart. If untreated, 20–30% of patients develop coronary artery abnormalities ranging from vessel dilatation to large aneurysms (1). The preponderance of coronary involvement makes KD the leading cause of acquired heart disease among children in developed countries.Coronary artery aneurysms (CAA) are a major cause of short-term and long-term mortality associated with KD. Rare cases of sudden death within weeks of disease onset are typically caused by thrombosis of CAA, leading to myocardial infarction, or rupture of a giant aneurysm. While full resolution of coronary abnormalities occurs in~ 50% of cases, vessels with persistent aneurysms remain at risk for thrombosis and stenosis, often translating into a requirement for multiple arterial interventions and significant long-term morbidity (2).