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. [10.1097/00042737-199604000-00018]

Lack of association between porphyria cutanea tarda andalpha 1-antitrypsin deficiency

S.R. Fargion
Primo
;
A.L. Fracanzani;M.D. Cappellini
Penultimo
;
1996

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.
α1-antitrypsin deficiency; Chronic liver disease; Hepatitis C virus; Porphyria cutanea tarda
Settore MED/09 - Medicina Interna
apr-1996
Article (author)
File in questo prodotto:
Non ci sono file associati a questo prodotto.
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/209635
Citazioni
  • ???jsp.display-item.citation.pmc??? 2
  • Scopus 4
  • ???jsp.display-item.citation.isi??? 4
social impact