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Reye syndrome and reye-like syndrome.

机译:雷氏综合症和类雷氏综合症。

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摘要

Reye syndrome is an acute metabolic encephalopathy, largely affecting children and adolescents. In Reye-like syndrome, because of inborn errors of metabolism, hypoglycemia, hypoketonemia, elevated ammonia, and organic aciduria are often evident. It is well-known that fatty-acid oxidation defects can present as Reye-like syndrome. The most commonly diagnosed metabolic disorder in association with Reye syndrome has been medium-chain acyl coenzyme A dehydrogenase deficiency. The present consensus seems to be that Reye syndrome is very rare, and that any child suspected of manifesting this disorder should undergo investigations for inborn errors of metabolism. We recently treated a child with Reye-like illness deficiency, and who had also ingested aspirin. We discuss the possible pathogenesis of the disorder in this child. The end results of mitochondrial dysfunction in Reye syndrome and Reye-like illness may be similar.
机译:瑞氏综合症是一种急性代谢性脑病,主要影响儿童和青少年。在类似Reye的综合症中,由于先天性代谢错误,通常会出现低血糖,低血钾,氨升高和有机酸尿。众所周知,脂肪酸氧化缺陷可表现为雷耶样综合症。与Reye综合征相关的最常被诊断的代谢紊乱是中链酰基辅酶A脱氢酶缺乏症。目前的共识似乎是瑞氏综合症非常罕见,任何怀疑表现出这种疾病的儿童都应接受先天性代谢异常的检查。我们最近对一名患有Reye样疾病缺乏症的儿童进行了治疗,并且该儿童还摄入了阿司匹林。我们讨论了这个孩子的疾病的可能发病机理。 Reye综合征和Reye样疾病的线粒体功能障碍的最终结果可能相似。

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