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High Prevalence of Viral Infection in Adults with Homozygous and Heterozygous Alpha1-Antitrypsin Deficiency and Chronic Liver Disease

Theresa Propst, MD; Albert Propst, MD; Otto Dietze, MD; Gert Judmaier, MD; Herbert Braunsteiner, MD; and Wolfgang Vogel, MD
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Requests for Reprints: Theresa Propst, MD, Department of Internal Medicine, University Hospital Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.

Current Author Addresses: Drs. T. Propst, A. Propst, Judmaier, Braunsteiner, and Vogel: Department of Internal Medicine, Innsbruck University, Anichstrasse 35, 6020 Innsbruck, Austria.

Dr. Dietze: Department of Pathology, Innsbruck University, Müllerstrasse 44, 6020 Innsbruck, Austria.

© 1992 American College of PhysiciansAmerican College of Physicians

Ann Intern Med. 1992;117(8):641-645. doi:10.7326/0003-4819-117-8-641
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Objective: To determine the prevalence of chronic liver disease in adults with homozygous (Pi ZZ) and heterozygous (Pi Z) α1-antitrypsin deficiency and to assess the presence of other possible risk factors for the development of chronic active hepatitis and cirrhosis of the liver in these patients.

Design: Cross-sectional study.

Setting: A referral-based university hospital.

Patients: Consecutive patients (164) with the Pi ZZ and Pi Z phenotype with and without chronic liver disease.

Measurements: The presence of antibody to hepatitis C virus (anti-HCV) was determined using an assay incorporating synthetic peptide antigen from capsid protein (United Biomedical [UBI] assay) and a second-generation enzyme immunoassay (Abbott test); the presence of antibody to hepatitis B virus (anti-HBV) was determined using radioimmunoassays incorporating hepatitis B surface antigen (HBsAg) and hepatitis B core antigen (HBcAg); assays for antinuclear antibody and antimitochondrial antibody (M2 subtype) were also done, and alcohol abuse was assessed by history.

Results: Among patients with cirrhosis (32%), 62% were anti-HCV positive by the Abbott test (P = 0.006), and 41% were anti-HCV positive by the UBI assay (P = 0.007). Thirty-three percent of patients with cirrhosis had hepatitis B virus (HBV) infection (P = 0.01); 41% had a history of alcoholism; and 12% had features of autoimmune liver disease. Only five patients (9%) with cirrhosis had no other risk factor for chronic liver disease. Among patients with chronic active hepatitis (7%), 80% were anti-HCV positive by the Abbott test (P = 0.002), and 75% were anti-HCV positive by the UBI assay (P < 0.001). Thirty percent of patients with chronic active hepatitis had HBV infection (P = 0.023); 18% had autoimmune hepatitis; and 8% abused alcohol. Only two patients (17%) had no additional risk factor for the development of chronic active hepatitis. Among patients with steatosis of the liver (48%), 5% were anti-HCV positive by the Abbott test, and none were anti-HCV positive by the UBI assay; 18% had serologic evidence of past HBV infection, and 28% abused alcohol. Among patients without chronic liver disease (13%), no viral infection could be found; 9% were alcoholics.

Conclusions: Chronic liver disease in patients with α1-antitrypsin deficiency is associated with a high prevalence of viral infection; this infection, rather than α1-antitrypsin deficiency alone, may be the cause of the liver disease in such patients.





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