Document Detail


Chenodeoxycholic acid therapy in erythrohepatic protoporphyria.
MedLine Citation:
PMID:  3559148     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The short-term effect of chenodeoxycholic acid administration on the excretion of protoporphyrin was investigated in 5 patients suffering from erythrohepatic protoporphyria. Faeces were collected for 7 days, 10 ml of bile was sampled daily and blood was drawn every 2 to 3 days. Chenodeoxycholic acid was given in a dosage of 15 mg/kg/day from the 8th day. Collection of faeces, bile and blood was then continued for 10 more days. Protoporphyrin concentration was measured by high-pressure liquid chromatography and fluorometry. Following the administration of chenodeoxycholic acid the concentration of protoporphyrin in faeces and bile decreased significantly. In addition, all patients showed a significant decrease in erythrocyte protoporphyrin concentration. These results indicate that chenodeoxycholic acid therapy causes a marked decrease in the excretion of protoporphyrin in patients with erythrohepatic protoporphyria. The subsequent decrease in erythrocyte protoporphyrin suggests that chenodeoxycholic acid inhibits the production of protoporphyrin in the liver.
Authors:
J Van Hattum; H Baart de la Faille; J W Van den Berg; A Edixhoven-Bosdijk; J H Wilson
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of hepatology     Volume:  3     ISSN:  0168-8278     ISO Abbreviation:  J. Hepatol.     Publication Date:  1986  
Date Detail:
Created Date:  1987-05-11     Completed Date:  1987-05-11     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8503886     Medline TA:  J Hepatol     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  407-12     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Chenodeoxycholic Acid / therapeutic use*
Erythrocytes / metabolism
Humans
Liver / metabolism
Liver Diseases / drug therapy*,  metabolism
Porphyrias / drug therapy*,  metabolism
Porphyrins / metabolism*
Protoporphyrins / metabolism*
Time Factors
Chemical
Reg. No./Substance:
0/Porphyrins; 0/Protoporphyrins; 474-25-9/Chenodeoxycholic Acid

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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