Lack of association between porphyria cutanea tarda and alpha 1-antitrypsin deficiency.

Abstract:

OBJECTIVE:To determine whether alpha 1-antitrypsin deficiency is involved in the pathogenesis of chronic liver disease in patients with porphyria cutanea tarda and in their recently described high prevalence of hepatitis C virus infection. DESIGN:Consecutive patients diagnosed as having porphyria cutanea tarda and chronic liver disease. SETTING:A northern Italian hospital. METHODS:alpha 1-antitrypsin phenotypes were characterized by isoelectric focusing and the results confirmed by DNA analysis in 63 Italian patients with porphyria cutanea tarda. RESULTS:alpha 1-antitrypsin phenotypes different from the normal one were found in 13% of the patients. This prevalence did not differ from that in control subjects (9%). Clinical characteristics of patients with porphyria cutanea tarda with normal or altered alpha 1-antitrypsin phenotype, including age of presentation of the disease, prevalence of hepatitis C virus infection, liver histology, prevalence of iron overload and hepatocellular carcinoma occurrence, did not differ significantly. CONCLUSION:alpha 1-antitrypsin does not seem to play a role in the pathogenesis of chronic liver disease and hepatitis C virus infection in patients with porphyria cutanea tarda. Patients in whom the two defects coexist do not have a more severe disease.

authors

Fargion S,Sergi C,Bissoli F,Fracanzani AL,Suigo E,Carazzone A,Roberto C,Cappellini MD,Fiorelli G

doi

10.1097/00042737-199604000-00018

subject

Has Abstract

pub_date

1996-04-01 00:00:00

pages

387-91

issue

4

eissn

0954-691X

issn

1473-5687

journal_volume

8

pub_type

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