Emphysema, Cirrhosis, and Hepatoma with Alpha-1 Antitrypsin Deficiency

Editorials1 December 1974Emphysema, Cirrhosis, and Hepatoma with Alpha-1 Antitrypsin DeficiencyJACK LIEBERMAN, M.D.JACK LIEBERMAN, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-81-6-850 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe role of alpha-1 antitrypsin deficiency in predisposing to pulmonary emphysema has been universally accepted, and even the likelihood that an intermediate deficiency may play such a role is receiving growing support. Twenty-four molecular variants of antitrypsin have been discovered in man, but antitrypsin deficiency is usually associated with the Z variant. This variant has the lowest blood levels of antitrypsin and is the only variant whose serum concentration does not rise after an appropriate stimulus. The Z variant is also characterized by the presence of unusual intrahepatic globules immunologically related to antitrypsin, and this finding has focused much of...References1. MATSUBARAYOSHIDALIEBERMAN SAJ: Immunologically cross-reacting material of alpha1-antitrypsin isolated from normal and variant human liver. Proc Natl Acad Sci USA. In press Google Scholar2. SABESINKOFF SR: Pathogenesis of experimental viral hepatitis. N Engl J Med 290:944-950, 996-1002, 1974 CrossrefGoogle Scholar3. PORTERMOWATCOOK CAP: Alpha1-antitrypsin deficiency and neonatal hepatitis. Br Med J 2:435-439, 1972 CrossrefGoogle Scholar4. BERGERIKSSON NS: Liver disease in adults with alpha1-antitrypsin deficiency. N Engl J Med 287:1264-1267, 1972 CrossrefMedlineGoogle Scholar5. NORKINCAMPAGNA-PINTO SD: Cytoplasmic hyaline inclusions in hepatoma. Arch Pathol 86:25-32, 1968 MedlineGoogle Scholar6. GORDONDIXONROGERS HJJ: Alpha 1-antitrypsin (A1AT) accumulation in livers of emphysematous patients with A1AT deficiency. Hum Pathol 3:361-370, 1972 CrossrefMedlineGoogle Scholar7. DEKKERKRAUSE AJ: Hyaline globules in human neoplasms. Arch Pathol 95:178-181, 1973 MedlineGoogle Scholar8. CLARKCLIFFTONNEWTON DEB: Antiproteolytic activity of human serum with particular reference to its changes in the presence, and considerations of its use for detection, of malignant neoplasms. Proc Soc Exp Biol Med 69:276-279, 1948 CrossrefMedlineGoogle Scholar9. GOETZWEINSTEINROBERTS ICE: Effects of protease inhibitors on growth of hamster tumor cells in culture. Cancer Res 32:2469-2474, 1972 MedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: JACK LIEBERMAN, M.D.Affiliations: Department of Respiratory Diseases City of Hope National Medical Center Duarte, California PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byPediatric Metabolic DiseasesBiochemical Tests in Diseases of the Intestinal Tract: Their Contributions to Diagnosis, Management, and Understanding the Pathophysiology of Specific Disease StatesMalignant liver disease in alpha 1 -antitrypsin deficiencyPrevalence of hepatocellular carcinoma in alpha-1-antitrypsin deficiencySerum α 1 -antitrypsin and duodenal ulcerCirrhosis and hepatocellular carcinoma in a patient with heterozygous (MZ) alpha-1 -antitrypsin deficiencyDemonstration of alpha1-antitrypsin in paraffin sections of hepatoma and cirrhosisAlpha 1-antitrypsin deficiency and liver disease.Hepatopathie im Kindes- und Erwachsenenalter bei Alpha-1-Antitrypsin-MangelThe concentration of alpha-1-antitrypsin in cerebrospinal fluid and serum in a series of 40 intracranial tumorsAcute Phase Reactant Proteins in CancerAlpha 1 -Antitrypsin DeficiencyObstructive airway disease associated with heterozygous alpha-1 antitrypsin deficiencyLiver, Lung and Malignant Disease in Heterozygous (Pi MZ) α1-antitrypsin Deficiency 1 December 1974Volume 81, Issue 6Page: 850-852KeywordsAttentionBloodCirrhosisEmphysemaHepatocellular carcinoma ePublished: 1 December 2008 Issue Published: 1 December 1974 PDF downloadLoading ...