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Article

Variable degree of liver involvement in siblings with PiZZ alpha-1-antitrypsin deficiency-related liver disease.

Journal of pediatric gastroenterology and nutrition - 1 Jul 2006

Hinds R, Hadchouel A, Shanmugham N P, Al-Hussaini A, Chambers S, Cheeseman P, Mieli-Vergani G, Hadzić N

Abstract excerpt

PiZZ alpha-1-antitrypsin deficiency is the commonest genetic cause of chronic liver disease, but only 10-15% of PiZZ individuals develop liver disease in childhood. Studies have demonstrated varying patterns of disease progression within siblings with the PiZZ phenotype. We retrospectively analysed the case-notes of all patients diagnosed with PiZZ A1ATD between 1978-2002 and compared the pattern of liver disease...

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PMID: 16819392
DOI: 10.1097/01.mpg.0000226370.09085.39

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Source receipt: pubmed, retrieved 7 Sept 2026.

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