Titration of bile acid supplements in 3β-hydroxy-Δ5-C27-steroid dehydrogenase/isomerase deficiency

Journal of Pediatric Gastroenterology and Nutrition

Volumn 50, Issue 6, 2010, Pages 655-660

  Riello, L ^a   D'Antiga, L ^a   Guido, M ^a   Alaggio, R ^a   Giordano, G ^a   Zancan, L ^a  

^a UNIVERSITY OF PADOVA   (Italy)

Author keywords

Bile acids; Inborn errors of metabolism; Intrahepatic cholestasis

Indexed keywords

3BETA HYDROXY DELTA5 C27 STEROID DEHYDROGENASE ISOMERASE; 3BETA HYDROXY DELTA5 STEROID DEHYDROGENASE; 3BETA,7ALPHA DIHYDROXY 5 CHOLENOIC ACID; BILE ACID; CHENODEOXYCHOLIC ACID; ENZYME PRECURSOR; UNCLASSIFIED DRUG; URSODEOXYCHOLIC ACID;

BILIARY CIRRHOSIS; CHILD; CLINICAL ARTICLE; CLINICAL FEATURE; CONTROLLED STUDY; DISEASE CONTROL; DISEASE COURSE; DOSE RESPONSE; DRUG DOSE TITRATION; ENZYME DEFICIENCY; FEMALE; FOLLOW UP; HEPATITIS; HISTOPATHOLOGY; HUMAN; HUMAN TISSUE; LIQUID CHROMATOGRAPHY; LIVER BIOPSY; LIVER FUNCTION TEST; MALE; NEWBORN SCREENING; PRESCHOOL CHILD; PRIORITY JOURNAL; REVIEW; SCHOOL CHILD; TANDEM MASS SPECTROMETRY; TREATMENT DURATION; TREATMENT RESPONSE; URINE LEVEL;

3-HYDROXYSTEROID DEHYDROGENASES; BIOPSY; CHENODEOXYCHOLIC ACID; CHILD; CHILD, PRESCHOOL; CHOLAGOGUES AND CHOLERETICS; CHOLIC ACIDS; CHROMATOGRAPHY, LIQUID; DIETARY SUPPLEMENTS; DISEASE PROGRESSION; HUMANS; INFANT; INFANT, NEWBORN; ISOMERASES; LIVER DISEASES; STEROID METABOLISM, INBORN ERRORS; TANDEM MASS SPECTROMETRY; TREATMENT OUTCOME; URSODEOXYCHOLIC ACID;

EID: 77953146627     PISSN: 02772116     EISSN: None     Source Type: Journal    
DOI: 10.1097/MPG.0b013e3181b97bd2     Document Type: Review

References (22)

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