Cholic acid

How it works

Replacing the missing bile acid restores negative feedback on bile acid synthesis, reducing the production of hepatotoxic intermediate metabolites while supporting bile flow and fat-soluble vitamin absorption.

Prescribing in practice

• Liver function should be monitored throughout treatment, as worsening liver biochemistry may indicate an inappropriate dose or progression requiring review.
• It is indicated for rare inborn errors of bile acid synthesis and should be managed by clinicians experienced in these metabolic disorders.
• Bile-acid sequestrants and some antacids reduce its absorption, so administration should be appropriately separated.

Monitoring

Monitor liver function and serum and urinary bile acid metabolites to guide dosing and confirm therapeutic response.

Counselling the patient

• This replaces a bile acid your body cannot make properly and is taken long term.
• Keep your appointments for the blood and urine tests that guide your dose.
• Leave a gap between this and any cholesterol-lowering resin or antacid.

Evidence & guidelines

Cholic acid replacement is the recognised treatment for inborn errors of primary bile acid synthesis and is supported by long-term observational outcome data.