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感染相关噬血细胞综合征:50例儿童病例报告

Infection associated hemophagocytic syndrome: a report of 50 children.

作者信息

Ningsanond V

机构信息

Hematology Division, Department of Pediatrics, Queen Sirikit National Institute of Child Health, Bangkok 10400, Thailand.

出版信息

J Med Assoc Thai. 2000 Oct;83(10):1141-9.

Abstract

Fifty children were diagnosed with infection associated hemophagocytic syndrome (IAHS) over the 10 year period from January 1988 through December 1997 at Queen Sirikit National Institute of Child Health, Thailand. Their ages ranged from 2 months to 14 years (mean 4.14 years). There was no difference in sex. Bacterial, mycobacterial, viral, fungal and protozoa were the associated infections in some of these patients. Supportive with specific therapy for the underlying disease was administered aggressively in all patients. Intravenous immuneglobulin (IVIG) was given in 8 patients. Thirty-five patients (70%) died, mostly as a result of coagulopathy with multiple organ failure and opportunistic infections. Two patients developed acute lymphoblastic leukemia 25 days and 3 months after recovering from IAHS.

摘要

1988年1月至1997年12月的10年间,泰国诗丽吉王后国家儿童健康研究所确诊了50例感染相关噬血细胞综合征(IAHS)患儿。他们的年龄从2个月至14岁不等(平均4.14岁)。性别上无差异。其中部分患者的相关感染包括细菌、分枝杆菌、病毒、真菌和原生动物。所有患者均积极接受针对基础疾病的支持性及特异性治疗。8例患者接受了静脉注射免疫球蛋白(IVIG)治疗。35例患者(70%)死亡,主要死于凝血病伴多器官功能衰竭和机会性感染。2例患者在从IAHS康复后25天和3个月时发生了急性淋巴细胞白血病。

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