Malignant liver disease in alpha 1-antitrypsin deficiency.

  title={Malignant liver disease in alpha 1-antitrypsin deficiency.},
  author={J. Rainer Poley},
  journal={Acta paediatrica (Oslo, Norway : 1992). Supplement},
Ever since the first report by Sharp et al. (1) that alpha1-antitrypsin deficiency (AATD) PiZ is associated with liver disease in infants and children, hepatologists were captured by this association. Whereas the major manifestation of alpha1-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… CONTINUE READING