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部分鸟氨酸转氨甲酰酶缺乏症导致急性肝衰竭时肝脏过氧化物酶体损伤。

Liver peroxisome damage during acute hepatic failure in partial ornithine transcarbamylase deficiency.

作者信息

Landrieu P, François B, Lyon G, Van Hoof F

出版信息

Pediatr Res. 1982 Dec;16(12):977-81. doi: 10.1203/00006450-198212000-00001.

DOI:10.1203/00006450-198212000-00001
PMID:7155674
Abstract

A boy with ornithine transcarbamylase (OTC) deficiency was relatively symptom free for 9 months and then developed an acute episode with liver failure and metabolic imbalance. Subsequently there was severe cerebral atrophy. Liver ornithine transcarbamylase activity was 3% of the normal mean. Of considerable interest was the finding of an accelerated breakdown of liver peroxisomes during the acute phase.

摘要

一名患有鸟氨酸转氨甲酰酶(OTC)缺乏症的男孩在9个月内相对无症状,随后出现了伴有肝功能衰竭和代谢失衡的急性发作。随后出现了严重的脑萎缩。肝脏鸟氨酸转氨甲酰酶活性为正常平均值的3%。在急性期发现肝脏过氧化物酶体加速分解,这一发现颇受关注。

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