Alpha1-antitrypsin deficiency with diffuse bronchiectasis and cirrhosis of the liver

Chest. 1977 Apr;71(4):535-8. doi: 10.1378/chest.71.4.535.


The medical literature has emphasized repeatedly the association of alpha1-antitrypsin deficiency with panacinar emphysema and cirrhosis of the liver. Previous reports have linked bronchiectasis with alpha1-antitrypsin deficiency. The present case confirms this association and adds the presence of an hepatic abnormality characteristics of alpha1-antitrypsin deficiency to complete the syndrome. The patient's phenotype was found to be MDuarteZ. It has been suggested that patients with diffuse bronchiectasis should be screened for alpha1-antitrypsin deficiency. We would add that a complete assessment of hepatic function, including liver biopsy, should be carried out in those individuals with reduced levels of alpha1-antitrypsin.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Bronchiectasis / complications*
  • Bronchiectasis / diagnostic imaging
  • Bronchiectasis / enzymology
  • Child
  • Humans
  • Infant
  • Liver Cirrhosis / complications*
  • Liver Cirrhosis / enzymology
  • Liver Cirrhosis / pathology
  • Liver Function Tests
  • Middle Aged
  • Phenotype
  • Radiography
  • Respiratory Function Tests
  • alpha 1-Antitrypsin Deficiency*