Risk of hepatobiliary disease in adults with severe ??1-antitrypsin deficiency (PiZZ): is chronic viral hepatitis B or C an additional risk factor for cirrhosis and hepatocellular carcinoma?

作者：Abdul-Nasser Elzouki, Sten Eriksson · 发表于：European Journal of Gastroenterology & Hepatology · 年份：1996 · DOI：10.1097/00042737-199610000-00010 · 被引用次数：102 · 研究领域：Protease and Inhibitor Mechanisms、Bone health and treatments、Blood Coagulation and Thrombosis Mechanisms

OBJECTIVES: To assess homozygous alpha 1-antitrypsin deficiency (PiZZ) as a risk factor for cirrhosis, hepatocellular carcinoma (HCC) and gallstone disease, and to analyse the respective interrelation-ships and those suggested to exist between PiZZ, alpha 1-antitrypsin and chronic hepatitis B and C. DESIGN/METHODS: This study was based on 31 autopsied adults with severe alpha 1-antitrypsin deficiency diagnosed during the period 1963-94, in the city of Malmö, Sweden. For each autopsied PiZZ individual, four age- and sex-matched controls were selected from the same autopsy register. The autopsy rate during the study period was 57.2% of all deaths in the city and 85% of deaths at the hospital. Relative risks were estimated in terms of Mantel-Haenszel odds ratios (ORmh). RESULTS: In the PiZZ group, we found 13 cases of cirrhosis (ORmh = 8.3; 95% CI, 3.8-18.3; P < 0.0001), 5 cases of HCC (ORmh = 5.0; 95% CI, 1.6-15.8; P = 0.008), and 8 cases of gallstone disease (ORmh = 1.0; 95% CI, 0.4-2.3; P = 0.924), compared with 7, 4 and 29 cases, respectively, in the control group. Stratification of the data by age and sex showed the difference in relative risk of cirrhosis between the PiZZ and control groups to be significant in both sexes, but that of HCC to be significant only in the male subgroup. There was no correlation between PiZZ state and gallstone disease in either sex. All PiZZ patients with cirrhosis and HCC had had negative tests for anti-hepatitis B core antigen and/or hepatit...