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.
Lack of association between porphyria cutanea tarda andalpha 1-antitrypsin deficiency / S.R. Fargion, C. Sergi, F. Bissoli, A.L. Fracanzani, E. Suigo, A. Carazzone, R. Ceriani, M.D. Cappellini, G. Fiorelli. - In: EUROPEAN JOURNAL OF GASTROENTEROLOGY & HEPATOLOGY. - ISSN 0954-691X. - 8:4(1996 Apr), pp. 387-391.
|Titolo:||Lack of association between porphyria cutanea tarda andalpha 1-antitrypsin deficiency|
FARGION, SILVIA ROSSANA (Primo)
CAPPELLINI, MARIA DOMENICA (Penultimo)
|Parole Chiave:||α1-antitrypsin deficiency; Chronic liver disease; Hepatitis C virus; Porphyria cutanea tarda|
|Settore Scientifico Disciplinare:||Settore MED/09 - Medicina Interna|
|Data di pubblicazione:||apr-1996|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1097/00042737-199604000-00018|
|Appare nelle tipologie:||01 - Articolo su periodico|