Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia

Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. Aim: To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. Material and methods: Retrospective review of medical records of...

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Autores principales: Valera M,José Miguel, Smok S,Gladys, Poniachik T,Jaime, Oksenberg R,Danny, Silva P,Guillermo, Ferrario B,Mario, Buckel G,Erwin, Brahm B,Javier
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2006
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872006000400010
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spelling oai:scielo:S0034-988720060004000102014-01-23Cirrosis biliar primaria: experiencia de trece años en dos centros de referenciaValera M,José MiguelSmok S,GladysPoniachik T,JaimeOksenberg R,DannySilva P,GuillermoFerrario B,MarioBuckel G,ErwinBrahm B,Javier Liver Cirrhosis, Bilary Liver Cirrhosis Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. Aim: To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. Material and methods: Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver transplantation, were recorded. Result: Seventy eight percent of patients were symptomatic at presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in 56%. No clinical or laboratory differences were observed between symptomatic patients or those with positive antimitochondrial antibodies and the rest of the study group. Sjögren syndrome was present in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61% had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred. Conclusions: An early diagnosis and treatment of a progressive disease such as PBC will reduce the incidence of complications and the use of costly treatmentsinfo:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.134 n.4 20062006-04-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872006000400010es10.4067/S0034-98872006000400010
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Liver Cirrhosis, Bilary
Liver Cirrhosis
spellingShingle Liver Cirrhosis, Bilary
Liver Cirrhosis
Valera M,José Miguel
Smok S,Gladys
Poniachik T,Jaime
Oksenberg R,Danny
Silva P,Guillermo
Ferrario B,Mario
Buckel G,Erwin
Brahm B,Javier
Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia
description Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. Aim: To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. Material and methods: Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver transplantation, were recorded. Result: Seventy eight percent of patients were symptomatic at presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in 56%. No clinical or laboratory differences were observed between symptomatic patients or those with positive antimitochondrial antibodies and the rest of the study group. Sjögren syndrome was present in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61% had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred. Conclusions: An early diagnosis and treatment of a progressive disease such as PBC will reduce the incidence of complications and the use of costly treatments
author Valera M,José Miguel
Smok S,Gladys
Poniachik T,Jaime
Oksenberg R,Danny
Silva P,Guillermo
Ferrario B,Mario
Buckel G,Erwin
Brahm B,Javier
author_facet Valera M,José Miguel
Smok S,Gladys
Poniachik T,Jaime
Oksenberg R,Danny
Silva P,Guillermo
Ferrario B,Mario
Buckel G,Erwin
Brahm B,Javier
author_sort Valera M,José Miguel
title Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia
title_short Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia
title_full Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia
title_fullStr Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia
title_full_unstemmed Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia
title_sort cirrosis biliar primaria: experiencia de trece años en dos centros de referencia
publisher Sociedad Médica de Santiago
publishDate 2006
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872006000400010
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