儿童高免疫球蛋白E综合征四例并文献复习

回顾性分析总结2010年1月至2019年7月于郑州大学第一附属医院住院的4例儿童高IgE综合征(HIES)的临床资料,患儿均出生后6个月内反复发生皮肤和呼吸道感染。1例患儿并发严重的二尖瓣重度关闭不全伴心肌梗死，1例患儿并发肾脓肿，3例患者并发复发性肝脓肿，其中1例口服复方新诺明治疗后获得了缓解。对于反复出现不明原因感染的患儿应尽早行HIES相关基因筛查，明确诊断后可给予针对性抗感染治疗。

Hyperimmunoglobulin E syndrome in pediatric patients:4 cases report and literature review

The data of 4 children with hyperimmunoglobulin E syndrome(HIES) from Jan. 2010 to Jul. 2019, including clinical manifestations, laboratory test results, treatments and prognoses, was retrospectively analyzed. The age of the patients at onset of the disease was younger than 6 months. All the patients presented with multiple infections. The most common infections were skin and respiratory tract. One case had severe mitral incompetence with myocardial infarction. One case had renal abscess. Three cases had liver abscess and were alleviated after antibiotic treatment and abscess drainage. One case was treated with continuous trimethoprim-sulfamethoxazole and had a remission. HIES should be suspected when a child suffering from repeated infection of undetermined origin and screening for HIES-related genes should be performed as soon as possible. The targeted anti-infection therapy can be given as early as possible after diagnosed.