| Chenodeoxycholic acid therapy in erythrohepatic protoporphyria. | |
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MedLine Citation:
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PMID: 3559148 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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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:
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Type: Journal Article |
Journal Detail:
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Title: Journal of hepatology Volume: 3 ISSN: 0168-8278 ISO Abbreviation: J. Hepatol. Publication Date: 1986 |
Date Detail:
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Created Date: 1987-05-11 Completed Date: 1987-05-11 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 8503886 Medline TA: J Hepatol Country: NETHERLANDS |
Other Details:
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Languages: eng Pagination: 407-12 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Chenodeoxycholic Acid
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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:
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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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