Department of Pediatrics, Kasturba Medical College, A-14, A Block, KMC Staff Quarters, Light House Hill Road, Mangalore, 575003, India.
Indian J Pediatr. 2013 Feb;80(2):124-7. doi: 10.1007/s12098-012-0821-x. Epub 2012 Jun 28.
To highlight the varied clinical manifestations and management of idiopathic hypereosinophilic syndrome, a rare disorder in children.
Retrospective review of case records of 3 patients who were diagnosed to have idiopathic hypereosinophilic syndrome (IHES) in a tertiary referral centre between 1997 and 2010 was performed. These 3 children presented with different symptoms and the first case had cardiac involvement. All had very high absolute eosinophil count (cells/mm(3)) 43,206, 9,082, 2,925, respectively. Diagnosis was confirmed by bone marrow biopsy in all three cases and supported by liver biopsy in the first two cases and inguinal lymphnode biopsy in the last case. All 3 children responded to treatment with steroids, with only second patient requiring hydroxyurea to control disease.
Case 1 and 2 are on follow up for 13 y and 10 y respectively and both are asymptomatic. Case 3 expired due to sepsis 1 mo after diagnosis.
Treatment with steroids with or without hydroxyurea gave good response in all 3 cases. Hence, they still remain the gold standard for the treatment of IHES in children.
强调儿童罕见疾病特发性嗜酸性粒细胞增多综合征的各种临床表现和治疗方法。
对 1997 年至 2010 年间在一家三级转诊中心诊断为特发性嗜酸性粒细胞增多综合征（IHES）的 3 例患者的病历进行回顾性分析。这 3 名儿童表现出不同的症状，第一个病例有心脏受累。所有患者的绝对嗜酸性粒细胞计数（细胞/mm(3)）均非常高，分别为 43206、9082、2925。所有 3 例均通过骨髓活检确诊，前 2 例通过肝活检和最后 1 例通过腹股沟淋巴结活检得到支持。所有 3 例患儿均对类固醇治疗有反应，仅第 2 例患儿需要羟基脲来控制疾病。
第 1 例和第 2 例患者分别随访 13 年和 10 年，均无症状。第 3 例患儿在诊断后 1 个月因脓毒症死亡。
类固醇加或不加羟基脲治疗在所有 3 例患儿中均有良好反应。因此，它们仍然是儿童 IHES 治疗的金标准。
