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Cholic acid

Bile Acid · Treatment for Inborn Errors of Primary Bile Acid Synthesis

Bile AcidTreatment for Inborn Errors of Primary Bile Acid Synthesis
CDSCO approvedSchedule H
EXCRETION
not curated
INTERACTIONS
none in our sources
PREGNANCY
g Ursodeoxycholic acid is effective for the treatment of
FDA category + note

Mechanism

Inborn errors of primary bile acid synthesis are characterized by insufficient production of primary bile acids due to enzyme deficiencies. Cholic acid acts by replacing some of these missing primary bile acids, thereby alleviating the symptoms of the disease.

Indications

Inborn errors in primary bile acid synthesis due to an inborn deficiency of two specific liver enzymes

Dosing

Adult
l INDICATIONS AND DOSE Inborn errors of primary bile acid synthesis (initiated by a specialist) ▶ BY MOUTH Description of condition ▶ Adult: Usual dose 5–15 mg/kg daily; increased in steps of 50 mg daily in divided doses if required, dose to be given with food at the same time each day; Usual maximum 500 mg/24 hours Primary biliary cholangitis (or primary biliary cirrhosis) is a chronic cholestati…

Pregnancy & lactation

Pregnancy

g Ursodeoxycholic acid is effective for the treatment of

Related guidelines

Other Bile Acid drugs

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Sources: BNF·Verified: 2026-05-13 · House clinical team