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Hepatic copper in primary biliary cirrhosis: biliary excretion and response to penicillamine treatment

Excessive hepatic copper accumulation occurs in long-lasting cholestatic liver disorders especially in primary biliary cirrhosis. As in Wilson's disease, penicillamine has recently been introduced for the treatment of primary biliary cirrhosis. In Wilson's disease there is decreased biliary excretio... Full description

Journal Title: Gut 1981, Vol.22 (11), p.901-906
Main Author: Salaspuro, M P
Other Authors: Pikkarainen, P , Sipponen, P , Vuori, E , Miettinen, T A
Format: Electronic Article Electronic Article
Language: English
Subjects:
Quelle: Alma/SFX Local Collection
Publisher: England: BMJ Publishing Group Ltd and British Society of Gastroenterology
ID: ISSN: 0017-5749
Link: https://www.ncbi.nlm.nih.gov/pubmed/7308845
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recordid: cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_1419468
title: Hepatic copper in primary biliary cirrhosis: biliary excretion and response to penicillamine treatment
format: Article
creator:
  • Salaspuro, M P
  • Pikkarainen, P
  • Sipponen, P
  • Vuori, E
  • Miettinen, T A
subjects:
  • Abridged Index Medicus
  • Adult
  • Aged
  • Bile - metabolism
  • Bile Acids and Salts - metabolism
  • Copper - metabolism
  • Female
  • Humans
  • Liver - metabolism
  • Liver Cirrhosis, Biliary - drug therapy
  • Liver Cirrhosis, Biliary - metabolism
  • Male
  • Middle Aged
  • Penicillamine - therapeutic use
  • Research Article
ispartof: Gut, 1981, Vol.22 (11), p.901-906
description: Excessive hepatic copper accumulation occurs in long-lasting cholestatic liver disorders especially in primary biliary cirrhosis. As in Wilson's disease, penicillamine has recently been introduced for the treatment of primary biliary cirrhosis. In Wilson's disease there is decreased biliary excretion of copper. The present study shows that as compared with controls the biliary excretion of copper is not decreased in primary biliary cirrhosis; instead it may be increased in some patients. However, when compared with high hepatic copper concentration biliary copper excretion was low. In contrast with copper, biliary secretion of bile acids was decreased in eight of the 17 patients. Treatment with oral penicillamine (600 mg/day) for one year resulted in a significant decrease of hepatic copper concentration, but had no consistent effect on the biliary excretion of copper or on the amount of histologically stainable orcein-positive copper-binding protein. The results suggest that excessive hepatic copper accumulation in primary biliary cirrhosis may not be primarily caused by a decreased biliary excretion, or that a new equilibrium is achieved in advanced primary biliary cirrhosis. D-penicillamine appears to improve significantly the biliary excretion of bile acids.
language: eng
source: Alma/SFX Local Collection
identifier: ISSN: 0017-5749
fulltext: fulltext
issn:
  • 0017-5749
  • 1468-3288
url: Link


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descriptionExcessive hepatic copper accumulation occurs in long-lasting cholestatic liver disorders especially in primary biliary cirrhosis. As in Wilson's disease, penicillamine has recently been introduced for the treatment of primary biliary cirrhosis. In Wilson's disease there is decreased biliary excretion of copper. The present study shows that as compared with controls the biliary excretion of copper is not decreased in primary biliary cirrhosis; instead it may be increased in some patients. However, when compared with high hepatic copper concentration biliary copper excretion was low. In contrast with copper, biliary secretion of bile acids was decreased in eight of the 17 patients. Treatment with oral penicillamine (600 mg/day) for one year resulted in a significant decrease of hepatic copper concentration, but had no consistent effect on the biliary excretion of copper or on the amount of histologically stainable orcein-positive copper-binding protein. The results suggest that excessive hepatic copper accumulation in primary biliary cirrhosis may not be primarily caused by a decreased biliary excretion, or that a new equilibrium is achieved in advanced primary biliary cirrhosis. D-penicillamine appears to improve significantly the biliary excretion of bile acids.
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subjectAbridged Index Medicus ; Adult ; Aged ; Bile - metabolism ; Bile Acids and Salts - metabolism ; Copper - metabolism ; Female ; Humans ; Liver - metabolism ; Liver Cirrhosis, Biliary - drug therapy ; Liver Cirrhosis, Biliary - metabolism ; Male ; Middle Aged ; Penicillamine - therapeutic use ; Research Article
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abstractExcessive hepatic copper accumulation occurs in long-lasting cholestatic liver disorders especially in primary biliary cirrhosis. As in Wilson's disease, penicillamine has recently been introduced for the treatment of primary biliary cirrhosis. In Wilson's disease there is decreased biliary excretion of copper. The present study shows that as compared with controls the biliary excretion of copper is not decreased in primary biliary cirrhosis; instead it may be increased in some patients. However, when compared with high hepatic copper concentration biliary copper excretion was low. In contrast with copper, biliary secretion of bile acids was decreased in eight of the 17 patients. Treatment with oral penicillamine (600 mg/day) for one year resulted in a significant decrease of hepatic copper concentration, but had no consistent effect on the biliary excretion of copper or on the amount of histologically stainable orcein-positive copper-binding protein. The results suggest that excessive hepatic copper accumulation in primary biliary cirrhosis may not be primarily caused by a decreased biliary excretion, or that a new equilibrium is achieved in advanced primary biliary cirrhosis. D-penicillamine appears to improve significantly the biliary excretion of bile acids.
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