新生儿肝内胆汁淤积症与α1-抗胰蛋白酶缺乏症（作者译）
[Neonatal intrahepatic cholestasis and alpha-1-antitrypsin deficiency (author's transl)].
作者信息
Carnicer J, Martín J, Ballesta A, Amat L
出版信息
An Esp Pediatr. 1981 Mar;14(3):189-94.
PMID:6973942
Abstract
A case of alpha-1-antitrypsin deficiency in an 11 month-old Pi ZZ infant is reported. The patient exhibited intrahepatic cholestasis in the first months of life, with jaundice and hepatomegaly. Liver biopsy confirmed the diagnosis and showed the presence of hepatic fibrosis. Pi phenotypes, serum levels of alpha-1-antitrypsin, and symptoms in the family of the patient are reported. Clinical, biochemical and pathologic findings in liver disease in alpha-1-antitrypsin deficiency are discussed.
摘要
报告了一例11个月大的Pi ZZ型婴儿α-1抗胰蛋白酶缺乏症病例。该患者在生命的最初几个月出现肝内胆汁淤积，伴有黄疸和肝肿大。肝活检确诊了诊断，并显示存在肝纤维化。报告了患者家族中的Pi表型、α-1抗胰蛋白酶血清水平及症状。讨论了α-1抗胰蛋白酶缺乏症肝病的临床、生化和病理表现。
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