부산대학교 도서관

The medical literature has emphasized repeatedly the association of a1-antitrypsin deficiency with panacinar emphysema and cirrhosis of the fiver. Previous reports have linked bronchiectasis with a1-antitrypsin deficiency. The present case confirms this association and adds the presence of an hepatic abnormality characteristic of a1-antitrypsin deficiency to complete the syndrome. The patient's phenotype was found to be “Duarte”. It has been suggested that patients with diffuse bronchiectasis should be screened for a1-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 a1-antitrypsin.