Lack of association between porphyria cutanea tarda and α1,-antitrypsin deficiency
OBJECTIVE:To determine whether α1-antitrypsin deficiency is involved in the patho-genesis 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 cutane...
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| Veröffentlicht in: | European journal of gastroenterology & hepatology 1996-04, Vol.8 (4), p.387-392 |
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| container_issue | 4 |
| container_start_page | 387 |
| container_title | European journal of gastroenterology & hepatology |
| container_volume | 8 |
| creator | Fargion, Silvia Sergi, Consolato Bissoli, Franco Ludovica, Anna Fracanzani Suigo, Elena Carazzone, Aurora Roberto, Ceriani Cappellini, Maria Domenica Fiorelli, Gemino |
| description | OBJECTIVE:To determine whether α1-antitrypsin deficiency is involved in the patho-genesis 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:α1-antitrypsin phenotypes were characterized by isoelectric focusing and the results confirmed by DNA analysis in 63 Italian patients with porphyria cutanea tarda.
RESULTS:α1-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 α1-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:α1-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. |
| doi_str_mv | 10.1097/00042737-199604000-00018 |
| format | Article |
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DESIGN:Consecutive patients diagnosed as having porphyria cutanea tarda and chronic liver disease.
SETTING:A northern Italian hospital.
METHODS:α1-antitrypsin phenotypes were characterized by isoelectric focusing and the results confirmed by DNA analysis in 63 Italian patients with porphyria cutanea tarda.
RESULTS:α1-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 α1-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:α1-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.</description><identifier>ISSN: 0954-691X</identifier><identifier>EISSN: 1473-5687</identifier><identifier>DOI: 10.1097/00042737-199604000-00018</identifier><language>eng</language><publisher>Hagerstown, MD: Lippincott-Raven Publishers</publisher><subject>Biological and medical sciences ; Errors of metabolism ; Medical sciences ; Metabolic diseases ; Proteins and glycoproteins</subject><ispartof>European journal of gastroenterology & hepatology, 1996-04, Vol.8 (4), p.387-392</ispartof><rights>Lippincott-Raven Publishers.</rights><rights>1996 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c2968-5cc4384a98803bd2de4504d5cee65ed84fa1473e3353aff96d12276ea568c84d3</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=3107073$$DView record in Pascal Francis$$Hfree_for_read</backlink></links><search><creatorcontrib>Fargion, Silvia</creatorcontrib><creatorcontrib>Sergi, Consolato</creatorcontrib><creatorcontrib>Bissoli, Franco</creatorcontrib><creatorcontrib>Ludovica, Anna</creatorcontrib><creatorcontrib>Fracanzani</creatorcontrib><creatorcontrib>Suigo, Elena</creatorcontrib><creatorcontrib>Carazzone, Aurora</creatorcontrib><creatorcontrib>Roberto, Ceriani</creatorcontrib><creatorcontrib>Cappellini, Maria Domenica</creatorcontrib><creatorcontrib>Fiorelli, Gemino</creatorcontrib><title>Lack of association between porphyria cutanea tarda and α1,-antitrypsin deficiency</title><title>European journal of gastroenterology & hepatology</title><description>OBJECTIVE:To determine whether α1-antitrypsin deficiency is involved in the patho-genesis 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:α1-antitrypsin phenotypes were characterized by isoelectric focusing and the results confirmed by DNA analysis in 63 Italian patients with porphyria cutanea tarda.
RESULTS:α1-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 α1-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:α1-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.</description><subject>Biological and medical sciences</subject><subject>Errors of metabolism</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Proteins and glycoproteins</subject><issn>0954-691X</issn><issn>1473-5687</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1996</creationdate><recordtype>article</recordtype><recordid>eNp1kM9KAzEQh4MoWKvvkINHo8kmu0mOUvwHBQ8qeFumyYTGrrtLsqX0sXwRn8mtVW8ehmHg-w38PkKo4JeCW33FOVeFlpoJayuuxpONI8wBmQilJSsrow_JhNtSscqK12NykvPbSGgp9IQ8zcGtaBco5Ny5CEPsWrrAYYPY0r5L_XKbIlC3HqBFoAMkDxRaTz8_xAWDdohD2vY5ttRjiC5i67an5ChAk_HsZ0_Jy-3N8-yezR_vHmbXc-YKWxlWOqekUWCN4XLhC4-q5MqXDrEq0RsVYNcApSwlhGArL4pCVwhjJWeUl1Ni9n9d6nJOGOo-xXdI21rweien_pVT_8mpv-WM0fN9tIfsoAkJWhfzX14KrrmWI6b22KZrBkx51aw3mOolQjMs6__Uyy9ognUm</recordid><startdate>199604</startdate><enddate>199604</enddate><creator>Fargion, Silvia</creator><creator>Sergi, Consolato</creator><creator>Bissoli, Franco</creator><creator>Ludovica, Anna</creator><creator>Fracanzani</creator><creator>Suigo, Elena</creator><creator>Carazzone, Aurora</creator><creator>Roberto, Ceriani</creator><creator>Cappellini, Maria Domenica</creator><creator>Fiorelli, Gemino</creator><general>Lippincott-Raven Publishers</general><general>Lippincott Williams & Wilkins</general><scope>IQODW</scope><scope>AAYXX</scope><scope>CITATION</scope></search><sort><creationdate>199604</creationdate><title>Lack of association between porphyria cutanea tarda and α1,-antitrypsin deficiency</title><author>Fargion, Silvia ; Sergi, Consolato ; Bissoli, Franco ; Ludovica, Anna ; Fracanzani ; Suigo, Elena ; Carazzone, Aurora ; Roberto, Ceriani ; Cappellini, Maria Domenica ; Fiorelli, Gemino</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c2968-5cc4384a98803bd2de4504d5cee65ed84fa1473e3353aff96d12276ea568c84d3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1996</creationdate><topic>Biological and medical sciences</topic><topic>Errors of metabolism</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Proteins and glycoproteins</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Fargion, Silvia</creatorcontrib><creatorcontrib>Sergi, Consolato</creatorcontrib><creatorcontrib>Bissoli, Franco</creatorcontrib><creatorcontrib>Ludovica, Anna</creatorcontrib><creatorcontrib>Fracanzani</creatorcontrib><creatorcontrib>Suigo, Elena</creatorcontrib><creatorcontrib>Carazzone, Aurora</creatorcontrib><creatorcontrib>Roberto, Ceriani</creatorcontrib><creatorcontrib>Cappellini, Maria Domenica</creatorcontrib><creatorcontrib>Fiorelli, Gemino</creatorcontrib><collection>Pascal-Francis</collection><collection>CrossRef</collection><jtitle>European journal of gastroenterology & hepatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Fargion, Silvia</au><au>Sergi, Consolato</au><au>Bissoli, Franco</au><au>Ludovica, Anna</au><au>Fracanzani</au><au>Suigo, Elena</au><au>Carazzone, Aurora</au><au>Roberto, Ceriani</au><au>Cappellini, Maria Domenica</au><au>Fiorelli, Gemino</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Lack of association between porphyria cutanea tarda and α1,-antitrypsin deficiency</atitle><jtitle>European journal of gastroenterology & hepatology</jtitle><date>1996-04</date><risdate>1996</risdate><volume>8</volume><issue>4</issue><spage>387</spage><epage>392</epage><pages>387-392</pages><issn>0954-691X</issn><eissn>1473-5687</eissn><abstract>OBJECTIVE:To determine whether α1-antitrypsin deficiency is involved in the patho-genesis 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:α1-antitrypsin phenotypes were characterized by isoelectric focusing and the results confirmed by DNA analysis in 63 Italian patients with porphyria cutanea tarda.
RESULTS:α1-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 α1-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:α1-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.</abstract><cop>Hagerstown, MD</cop><pub>Lippincott-Raven Publishers</pub><doi>10.1097/00042737-199604000-00018</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record> |
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| issn | 0954-691X 1473-5687 |
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| source | Journals@Ovid Ovid Autoload |
| subjects | Biological and medical sciences Errors of metabolism Medical sciences Metabolic diseases Proteins and glycoproteins |
| title | Lack of association between porphyria cutanea tarda and α1,-antitrypsin deficiency |
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