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瑞氏综合征中的肝脏病变。

The hepatic lesion in Reye's syndrome.

作者信息

Bove K E, McAdams A J, Partin J C, Partin J S, Hug G, Schubert W K

出版信息

Gastroenterology. 1975 Sep;69(3):685-97.

PMID:1158086
Abstract

The hepatic lesion in Reye's syndrome (acute encephalopathy with fatty degeneration of viscera) was studied by light microscopy of sequential biopsy specimens obtained in 49 children. The hepatic lesion is a morphologically characteristic, rapidly evolving, and reversible toxic hepatitis. In specimens obtained with 48 hr of onset of neurological deterioration, the severity of the diffuse microvesicular steatosis is best appreciated in frozen sections stained for lipid content. Variation in severity of hepatocyte glycogen depletion in early biopsies correlates with other histological measures of severity, and with the occurrence of hypoglycemia, severity of the encephalopathy at the time of admission, and mortality rate. Histochemical studies suggest that the hepatic lesion is attributable to mitochondrial injury and other evidence that supports this hypothesis is briefly reviewed. The etiology of the syndrome and its relationship to the viral disease which usually precedes it are unknown.

摘要

通过对49名儿童连续获取的活检标本进行光学显微镜检查,对瑞氏综合征(急性脑病伴内脏脂肪变性)的肝脏病变进行了研究。肝脏病变是一种形态学特征明显、迅速发展且可逆的中毒性肝炎。在神经功能恶化发作48小时内获取的标本中,弥漫性微泡性脂肪变性的严重程度在经脂质含量染色的冰冻切片中最易观察到。早期活检中肝细胞糖原耗竭严重程度的差异与其他严重程度的组织学指标相关,也与低血糖的发生、入院时脑病的严重程度以及死亡率相关。组织化学研究表明肝脏病变归因于线粒体损伤,支持这一假说的其他证据也将简要回顾。该综合征的病因及其与通常在其之前发生的病毒性疾病的关系尚不清楚。

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