title: Cholangiocarcinoma in two siblings with emphysema and alpha-1-antitrypsin deficiency.
abstract: Two siblings with homozygous ZZ alpha-1-antitrypsin deficiency were discovered to have primary liver tumours (both cholangiocarcinomas). This lends support to the view that alpha-1-antitrypsin deficiency plays a role in the development of some primary liver tumours.
authors.affiliation: Department of Pathology, Ninewells Hospital and Medical School, Dundee.
authors.forename: D M
authors.initials: DM
authors.lastname: Parham
authors.forename: J R
authors.initials: JR
authors.lastname: Paterson
authors.forename: A
authors.initials: A
authors.lastname: Gunn
authors.forename: W
authors.initials: W
authors.lastname: Guthrie
mesh_terms: D002759:Adenoma, Bile Duct / Q000150:complications / Q000201:enzymology / Q000235:genetics* / Q000473:pathology; D000328:Adult; D001650:Bile Duct Neoplasms / Q000150:complications / Q000201:enzymology / Q000235:genetics / Q000473:pathology; D001653:Bile Ducts, Intrahepatic; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000201:enzymology / Q000235:genetics* / Q000473:pathology; D008297:Male; D010375:Pedigree; D011656:Pulmonary Emphysema / Q000150:complications / Q000235:genetics* / Q000473:pathology; D000515:alpha 1-Antitrypsin / Q000235:genetics; D019896:alpha 1-Antitrypsin Deficiency
chemical_list: D000515:alpha 1-Antitrypsin