Abstract:

Objective    To investigate the clinical characteristics and treatment of hypereosinophilic syndrome（HES） in children. Methods    The clinical manifestations， laboratory examinations，gastroscopy and imaging features，pathological results and therapy experience in 13 HES children admitted in Beijing Children’s Hospital，Capital Medical University from January 2009 to February 2016 and related literatures were analyzed retrospectively. Results    Of the 13 patients evaluated， 7 were male（54%） and 6 were female（46%）. The median age at diagnosis was 9.6 years old（ranging 3 to 14 years old）. The median course of disease was 23 months（ranging 1 to 72 months）. The peripheral eosionophil counts ranged （4.5—29.2）×109/L（mean 13.5×109/L）. Gastrointestinal tract was the most commonly involved organ， and was reported in 92%（12/13） of patients. It was followed in frequency by urinary system（84%），pulmonary（53%），cardiac（23%），and skin（8%） and liver（8%）. A total of 2 sites were involved in 6 patients，3 sites were involved in 5 patients and 4 sites were involved in 2 patients. Treatment of oral prednisone therapy was given and follow-up of 10 patients had no clinical symptoms（2 patients had stopped prednisone）. But eosionophil counts still increased to varying degrees. Three patients were lost to follow-up. Conclusion    HES in children is more common in school age and adolescent children. Gastrointestinal tract，urinary system and pulmonary involvement are more common. Glucocorticoid treatment is effective，which requires to be maintained in small dose in the long term.

Key words: hypereosinophilic syndrome, children, diagnosis, treatment

摘要：

目的    探讨儿童高嗜酸性粒细胞综合征（HES）的临床特点以及治疗。方法　回顾性分析2009年1月至2016年2月首都医科大学附属北京儿童医院收治的13例儿童HES临床表现、实验室检查、胃镜和影像、病理、治疗经过，结合相关文献对儿童HES临床特点以及诊治进行探讨。结果　13例HES中男7例（54%），女6例（46%）；年龄3～14岁；病程1～72个月；外周血嗜酸性粒细胞计数（4.5～29.2）×109/L。受累器官：胃肠12例（92%）， 肾脏膀胱11例（84%）， 肺部7例（53%），可疑心脏受累3例（23%），皮肤１例（8%），肝脏1例（8%）；2个部位受累共6例，3个部位受累5例，4个部位受累2例。治疗上均给予泼尼松口服治疗，随访10例均无临床不适症状（2例已停激素），但嗜酸性粒细胞仍有不同程度升高，3例失访。结论　儿童HES多见于学龄以及青春期儿童，以胃肠道、泌尿系统、肺部受累多见，糖皮质激素治疗有效，需要小剂量长期维持。

关键词: 高嗜酸性粒细胞综合征, 儿童, 诊断, 治疗