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[洛美大学医院中心葡萄糖-6-磷酸脱氢酶缺乏症儿童的溶血性尿毒症]

[Post-hemolytic renal failure in children with glucose-6-phosphate dehydrogenase deficiency at the University Hospital Center in Lome].

作者信息

Balaka B, Agbèrè D, Bonkoungou P, Gnamey D, Kessie K, Assimadi K

机构信息

Service de Pédiatrie, Centre hospitalier universitaire de Lomé, Togo.

出版信息

Med Trop (Mars). 2003;63(2):151-4.

Abstract

The purpose of the study was to identify predisposing factors for acute hemolysis and post-hemolytic renal failure in children with glucose-6-phosphate dehydrogenase deficiency (G6PD). Any child presenting hemoglobinuria during the study period was prospectively evaluated. Evaluation included detection of the presence of hemolytic agents, laboratory tests to measure hemolysis, G6PD activity, infection and renal failure, and assessment of outcome and management of hemolysis and renal failure. G6PD deficiency was documented in 32.1% of the 230 children admitted with hemoglobinuria. Anuric renal failure occurred during the hemolysis episode in 35.1% of patients with G6PD deficiency (21 boys and 5 girls between 30 months to 13 years old). Acute hemolysis associated with infection occurred before any treatment in 53.8% of cases and after beginning treatment in 46.1%. In 84.6% of cases, occurrence of acute hemolysis involved association of drugs considered as nonhemolytic either with themselves or with other drugs. Anuric renal failure occurred after beginning treatment in all cases and was most severe in patients with of multiple-germ infection (30.7%) and drug association (84.6%). Renal failure was reversible in 80.7% and fatal in 19.2%. Multiple-germ infection and drug association appeared as the main predisposing factors for post hemolytic anuric renal failure in patients with G6PD deficiency. The high frequency of these factors in tropical areas suggests implication of local endemic infections.

摘要

本研究的目的是确定葡萄糖-6-磷酸脱氢酶缺乏症(G6PD)患儿急性溶血和溶血后肾衰竭的诱发因素。对研究期间出现血红蛋白尿的任何儿童进行前瞻性评估。评估包括检测溶血剂的存在、测量溶血、G6PD活性、感染和肾衰竭的实验室检查,以及对溶血和肾衰竭的结局及处理进行评估。在230例因血红蛋白尿入院的儿童中,32.1%被记录为G6PD缺乏。35.1%的G6PD缺乏患者(21名男孩和5名女孩,年龄在30个月至13岁之间)在溶血发作期间出现无尿性肾衰竭。53.

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