Progressive familial intrahepatic cholestasis and inborn errors of bile acid synthesis

Clinics and Research in Hepatology and Gastroenterology

Volumn 36, Issue 3, 2012, Pages 271-274

  Jankowska, Irena ^a   Socha, Piotr ^a  

^a CHILDREN'S MEMORIAL HEALTH INSTITUTE   (Poland)

Author keywords

[No Author keywords available]

Indexed keywords

BILE ACID; CHOLIC ACID; CYANOCOBALAMIN; FOLIC ACID; GAMMA GLUTAMYLTRANSFERASE; URSODEOXYCHOLIC ACID;

ARTICLE; BILE ACID BLOOD LEVEL; BILE ACID SYNTHESIS; CHOLECYSTECTOMY; GAMMA GLUTAMYL TRANSFERASE BLOOD LEVEL; HUMAN; INBORN ERROR OF BILE ACID SYNTHESIS; INTRAHEPATIC CHOLESTASIS; LIVER BIOPSY; LIVER CELL CARCINOMA; LIVER FAILURE; LIVER TRANSPLANTATION; PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS;

AVITAMINOSIS; BILE ACIDS AND SALTS; CHENODEOXYCHOLIC ACID; CHILD; CHOLESTASIS, INTRAHEPATIC; CHOLIC ACID; DIGESTIVE SYSTEM SURGICAL PROCEDURES; GAMMA-GLUTAMYLTRANSFERASE; GASTROINTESTINAL AGENTS; GROWTH DISORDERS; HEPATOMEGALY; HUMANS; JAUNDICE; METABOLISM, INBORN ERRORS; PRURITUS; SPLENOMEGALY; TRANSAMINASES;

EID: 84861737843     PISSN: 22107401     EISSN: 2210741X     Source Type: Journal    
DOI: 10.1016/j.clinre.2012.03.020     Document Type: Article

References (5)

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3. Whitington P.F., Whitngton G.L. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 1988, 95:130-136. [The first description of partial external biliary diversion].
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5. Gonzales E., Gerhardt M.F., Fabre M., Setchell K.D., Davit-Spraul A., Vincent I., et al. Oral cholic acid for hereditary defects of primary bile acid synthesis: a safe and effective long-term therapy. Gastroenterology 2009, 137:1310-1320. [A large series with long term follow-up].