eISSN: 1897-4317
ISSN: 1895-5770
Gastroenterology Review/Przegląd Gastroenterologiczny
Current issue Archive Manuscripts accepted About the journal Editorial board Abstracting and indexing Subscription Contact Instructions for authors Ethical standards and procedures
SCImago Journal & Country Rank
3/2008
vol. 3
 
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abstract:

Liver cirrhosis due to α1-antitrypsin deficiency in two infants

Agnieszka Bakuła
,
Piotr Socha
,
Joanna Pawłowska
,
Jerzy Socha
,
Piotr Kaliciński

Przegląd Gastroenterologiczny 2008; 3 (3): 136–138 Przegląd Gastroenterologiczny 2008; 3 (3): 136–138
Online publish date: 2008/06/09
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α1-antitrypsin deficiency is a genetic disorder associated with liver disease mainly during infancy or childhood and with emphysema in adults. It is the most common metabolic disease as an indication for liver transplantation in children. Liver injury is observed only in 10-15% of children with homozygous form of α1-antitrypsin deficiency. The natural course of the disease is variable and pathogenesis of liver changes is not clear. Liver transplantation was performed at the age of 1-17 years in our patients. The rapid progress of liver injury in two infants, seldom observed at this age, is reported in this paper.
keywords:

α1-antitrypsin deficiency, liver cirrhosis, children

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