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单卵双胎女孩的良性复发性胆汁淤积症(作者译)

[Benign recurrent cholestasis in monozygotic twin girls (author's transl)].

作者信息

Leiber D U, Lagenstein I, Grüttner R

出版信息

Monatsschr Kinderheilkd (1902). 1979 Jan;127(1):48-51.

PMID:569764
Abstract

The cases of Monozygotic twin girls suffering from benign recurrent familiar intrahepatic cholestasis are reported. So far both children had six icteric episodes due to this disease. These episodes occurred during times of severe emotional stress therefore, it may be assumed that a latent enzyme deficiency in the metabolism of bile acids, which is determined genetically, could be influenced by psychosomatic mechanisms. During the acute phases phenobarbital and cholestyramine succesfully reduced serum bilirubin levels.

摘要

报告了一对患良性复发性家族性肝内胆汁淤积症的单卵双胞胎女孩的病例。到目前为止,两个孩子都因这种疾病出现了六次黄疸发作。这些发作发生在严重情绪应激期间,因此,可以推测由基因决定的胆汁酸代谢中潜在的酶缺乏可能受到心身机制的影响。在急性期,苯巴比妥和消胆胺成功降低了血清胆红素水平。

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