Kao Chun-Chieh, Ou Liang-Shiou, Lin Shy-Jae, Huang Jing-Long
Department of Pediatrics, Chang Gung Children's Hospital and Chang Gung University, Taoyuan, Taiwan.
Asian Pac J Allergy Immunol. 2002 Jun;20(2):121-6.
We described a 15-year-old girl who presented with persistent fever, bilateral flank pain, and worsening dyspnea. The peripheral blood cell count showed remarkable eosinophilia at the time of admission. Severe pleural effusion with eosinophilic Infiltrations as well as pericardial effusion were noted thereafter. Bone marrow examination disclosed markedly increased eosinophils. Bilateral ectasia of the renal pelvis was found in an ultrasonographic study of the kidneys. Spiking fever and progressive shortness of breath persisted despite treatment with empiric antibiotics for infection. Based on the clinical course and histological findings, a tentative diagnosis of idiopathic HES was made. After treatment with oral prednisolone daily (1 mg/kg/day) for one week, there was a rapid improvement in her clinical condition. She was discharged a few days later and the steroids were withdrawn gradually when she was asymptomatic. The absolute eosinophil count (AEC) was monitored during follow-up. At 3 weeks, the AEC had fallen from 8,060/mm3 to 4,792/mm3 and it further fell to 1,591/mm3 at 5 months, and to 855/mm3 at 8 months during follow-up. There is no evidence of any other organ involvement until now. The clinical manifestations, diagnosis and management of idiopathic HES in children are also reviewed.
我们描述了一名15岁女孩，她出现持续发热、双侧胁腹疼痛和进行性加重的呼吸困难。入院时外周血细胞计数显示明显的嗜酸性粒细胞增多。此后发现严重的胸腔积液伴嗜酸性粒细胞浸润以及心包积液。骨髓检查显示嗜酸性粒细胞明显增多。肾脏超声检查发现双侧肾盂扩张。尽管使用经验性抗生素治疗感染，但高热和进行性呼吸急促仍持续存在。根据临床病程和组织学检查结果，初步诊断为特发性高嗜酸性粒细胞综合征（HES）。口服泼尼松龙每日（1 mg/kg/天）治疗一周后，她的临床状况迅速改善。几天后她出院，无症状时逐渐停用类固醇。随访期间监测绝对嗜酸性粒细胞计数（AEC）。随访3周时，AEC从8060/mm³降至4792/mm³，5个月时进一步降至1591/mm³，8个月时降至855/mm³。迄今为止，没有任何其他器官受累的证据。本文还对儿童特发性HES的临床表现、诊断和治疗进行了综述。
