Jin Ping, Luo Yong, Liu Xi, Xu Jinji, Liu Chunyi
Pediatric Intensive Care Unit, Baoan Maternal and Child Health Hospital, College of Medicine, Jinan University, Shenzhen, China.
Department of Cardiology, Baoan Maternal and Child Health Hospital, College of Medicine, Jinan University, Shenzhen, China.
Front Pediatr. 2019 Nov 1;7:423. doi: 10.3389/fped.2019.00423. eCollection 2019.
Macrophage activation syndrome (MAS) is a rare and severe complication of Kawasaki disease (KD). The clinical feature, early diagnosis and treatment options, and prognosis need to be further determined in patients with KD complicated with MAS. In this report, we retrospectively analyzed three KD patients complicated with MAS who were treated in pediatric intensive care units (PICU) and reviewed the relevant literatures. We find that being male, being age over 2 years old, incomplete KD, intravenous immunoglobulin (IVIG) non-responder, or persistent fever greater than 10 days are all highly associated with occurrence of MAS. Additional work-ups should be performed promptly in patient with above predisposing factors to rule out complication of MAS. Patients with KD complicated with MAS are at a higher risk of having coronary artery involvement or aneurysm formation, which can be reversed with timely treatment. Early identification and prompt treatment are key points for improving the prognosis of KD patients complicated with MAS.
巨噬细胞活化综合征（MAS）是川崎病（KD）一种罕见且严重的并发症。KD合并MAS患者的临床特征、早期诊断与治疗方案以及预后仍需进一步明确。在本报告中，我们回顾性分析了在儿科重症监护病房（PICU）接受治疗的3例KD合并MAS患者，并复习了相关文献。我们发现，男性、年龄大于2岁、不完全KD、静脉注射免疫球蛋白（IVIG）无反应者或持续发热超过10天均与MAS的发生高度相关。对于有上述易感因素的患者，应及时进行进一步检查以排除MAS并发症。KD合并MAS患者发生冠状动脉受累或动脉瘤形成的风险更高，及时治疗可使其逆转。早期识别和及时治疗是改善KD合并MAS患者预后的关键。
