Adult Liver Disease in SZ Phenotype Alpha-1-Antitrypsin Deficiency
Authors: Philip E. Palmer, Gherardo J. Gherardi, Joseph M. Baldwin, Hubert J. Wolfe
Journal: Annals of Internal Medicine (1978)

Brief Reports1 January 1978Adult Liver Disease in SZ Phenotype Alpha-1-Antitrypsin DeficiencyPHILIP E. PALMER, M.D., GHERARDO J. GHERARDI, M.D., JOSEPH M. BALDWIN, M.D., HUBERT J. WOLFE, M.D.PHILIP E. PALMER, M.D.Search for more papers by this author, GHERARDO J. GHERARDI, M.D.Search for more papers by this author, JOSEPH M. BALDWIN, M.D.Search for more papers by this author, HUBERT J. WOLFE, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-88-1-59 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptLiver disease associated with alpha-1-antitrypsin deficiency, classically described in protease inhibitor (Pi)-type ZZ phenotype children, characteristically shows variable fibrosis or cirrhosis and distinctive periodic acid-Schiff-positive (diastase-resistant) globules, containing alpha-1-antitrypsin, within periportal hepatocytes. In adults, liver involvement may occur without lung disease, and severely affected heterozygotes (non-ZZ individuals) have been reported. However, the significance of alpha-1-antitrypsin liver disease in adults remains undefined (1, 2).Case 1, a 73-year-old man, presented with biochemical evidence of liver disease in 1971 during hospitalization for unrelated medical problems. Total bilirubin was 1.0 mg/dl (normal < 1.0), alkaline phosphatase 145 IU (normal, 9 to 35), serum...References1. TRIGERMILLWARD-SADLERCZAYKOWSKITROWELLWRIGHT DGAJR: Alpha-1-antitrypsin deficiency and liver disease in adults. Q J Med 45:B51-B72, 1976 MedlineGoogle Scholar2. FISHERTAYLORSHERLOCK RLS: α-1-antitrypsin deficiency in liver disease: the extent of the problem. Gastroenterology 71:646-651, 1976 CrossrefMedlineGoogle Scholar3. PALMERDELELLISWOLFE PRH: Immunohistochemistry of liver in alpha-1-antitrypsin deficiency. A comparative study. Am J Clin Pathol 62:350-354, 1974 CrossrefMedlineGoogle Scholar4. MORINMARTINFELDMANNRUEFFBENHAMOUROPARTZ TJGBJP: Heterozygous alpha1-antitrypsin deficiency and cirrhosis in adults, a fortuitous association. Lancet 1:250-251, 1975 CrossrefMedlineGoogle Scholar5. SVEGER T: Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants. N Engl J Med 294:1316-1321, 1976 CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: PHILIP E. PALMER, M.D.; GHERARDO J. GHERARDI, M.D.; JOSEPH M. BALDWIN, M.D.; HUBERT J. WOLFE, M.D. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byMalignant liver disease in alpha 1 -antitrypsin deficiency?SZ like? ?1-antitrypsin phenotypes in PI ZZ children with liver diseaseIn vitro amplification of the α1-antitrypsin gene: Application to prenatal diagnosisNatural history of alpha-1-protease inhibitor deficiencyNatural history of alpha-1-protease inhibitor deficiencyVitamin-K-Resorptions- und Verwertungsstörungen, Hämostasedefekte bei LebererkrankungenMinimal Liver Disease in Young Persons with Homozygous and Heterozygous Alpha-1-Antitrypsin DeficiencyCase 25-1983Heterozygous MZ Alpha1-Antitrypsin Deficiency in Adults with Chronic Active Hepatitis and Cryptogenic CirrhosisExpression of protein markers in malignant hepatoma. Evidence for genetic and epigenetic mechanismsAlpha1-antitrypsin deficiency with cirrhosis associated with the protease inhibitor phenotype SZ 1 January 1978Volume 88, Issue 1Page: 59-60KeywordsCirrhosisHepatocytesHospitalizationsLiverLiver diseasesLiver fibrosisLungsPhenotypesPhosphatasesProtease inhibitors ePublished: 1 December 2008 Issue Published: 1 January 1978 PDF downloadLoading ...