Document Detail

Chenodeoxycholic acid therapy in erythrohepatic protoporphyria.
MedLine Citation:
PMID:  3559148     Owner:  NLM     Status:  MEDLINE    
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.
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
Chenodeoxycholic Acid / therapeutic use*
Erythrocytes / metabolism
Liver / metabolism
Liver Diseases / drug therapy*,  metabolism
Porphyrias / drug therapy*,  metabolism
Porphyrins / metabolism*
Protoporphyrins / metabolism*
Time Factors
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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