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Malignant liver disease in alpha 1 ‐antitrypsin deficiency
Author(s) -
Poley JR
Publication year - 1994
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1994.tb13204.x
Subject(s) - alpha 1 antitrypsin deficiency , medicine , hepatocellular carcinoma , chronic liver disease , liver disease , disease , alpha (finance) , gastroenterology , lung , pathology , cirrhosis , surgery , construct validity , patient satisfaction
Ever since the first report by Sharp et al. (1) that alpha 1 ‐antitrypsin deficiency (AATD) PiZ is associated with liver disease in infants and children, hepatologists were captured by this association. Whereas the major manifestation of alpha 1 ‐antitrypsin (AAT) PiZ deficiency in infants and children involves the liver, many adults affected with this phenotype develop chronic obstructive lung disease. However, in addition to severe panacinar emphysema, adults with AATD also face the risk of the development of chronic liver disease and hepatocellular carcinoma (HCC).