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[慢性肝性卟啉病(迟发性皮肤卟啉病)患者红细胞中δ-氨基乙酰丙酸(ALAD)的活性]

[Activity of delta-aminolaevulinic acid (ALAD) in erythrocytes of patients with chronic hepatic porphyria (porphyria cutanea tarda)].

作者信息

Stankiewicz-Wróblewska J

机构信息

Kliniki Gastroenterologii Akademii Medycznej, Gdańsku.

出版信息

Pol Arch Med Wewn. 1993 Mar;89(3):212-6.

PMID:8100994
Abstract

Erythrocyte delta-aminolaevulinic acid dehydratase (ALAD) activity was estimated in chronic hepatic porphyria, correlated with some tests of liver function and compared with enzyme activity in chronic liver diseases. 24 patients with chronic hepatic porphyria, 22--with chronic liver diseases and 30--controls were examined. European standardised method for the determination of ALAD activity blood was used. The significantly lowest activity was found in porphyric patients even after reactivation with zinc and dithiothreitol. There was no significant difference between ALAD activity in chronic liver diseases and controls. These results and lack of correlation between enzyme activity and liver function tests suggest that ALAD activity is decreased in chronic hepatic porphyria and is independent of liver damage.

摘要

在慢性肝卟啉病患者中测定了红细胞δ-氨基-γ-酮戊酸脱水酶(ALAD)活性,并将其与一些肝功能测试结果进行关联,同时与慢性肝病患者的酶活性进行比较。对24例慢性肝卟啉病患者、22例慢性肝病患者和30例对照者进行了检查。采用欧洲标准化的血液ALAD活性测定方法。即使在经锌和二硫苏糖醇重新激活后,卟啉病患者的ALAD活性仍显著最低。慢性肝病患者与对照者的ALAD活性无显著差异。这些结果以及酶活性与肝功能测试之间缺乏相关性表明,慢性肝卟啉病患者的ALAD活性降低,且与肝损伤无关。

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