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Cholic acid

Orphacol · ATC code A05AA03

Also known as: Kolik asit

Key facts
Active substance
Cholic acid
ATC code
A05AA03
Authorisation
centrally authorised across the EU (EMA)
Dosage forms
Orphacol 50 mg hard capsules / Orphacol 250 mg hard capsules
Marketing authorisation holder
Theravia
Information as of
2026-10-28

Key facts

Cholic acid is a primary bile acid that replaces missing endogenous bile acids. It is used for inborn errors of primary bile acid synthesis from 1 month of age.

What it is used for

  • Treatment of inborn errors in primary bile acid synthesis due to 3β-Hydroxy-Δ5-C27-steroid oxidoreductase deficiency or Δ4-3-Oxosteroid-5β-reductase deficiency in infants, children and adolescents aged 1 month to 18 years and adults.

Dosing

  • Orphacol (hard capsules): daily dose 5 to 15 mg/kg in all age groups; minimum dose 50 mg, adjusted in 50 mg steps; in adults not more than 500 mg per day.
  • The dose is adjusted individually in a specialised unit based on blood and/or urine bile acid profiles; the lowest effective dose is chosen.
  • The daily dose may be divided; take with food at about the same time each day. For children who cannot swallow capsules, the content may be added to infant formula or juice.
  • In familial hypertriglyceridaemia, a higher dose, notably above the 500 mg daily limit for adults, may be required.
  • Monitoring: every 3 months in the first year, every 6 months in the subsequent three years, then annually.

Contraindications

  • Hypersensitivity to the active substance or to any of the excipients.
  • Concomitant use of phenobarbital or primidone.

Important warnings

  • Severe hepatotoxicity, including fatal cases, has been reported; close monitoring in pre-existing hepatic impairment.
  • Treatment should be stopped if hepatocellular function (prothrombin time) does not improve within 3 months, or earlier with clear signs of severe hepatic failure.
  • Concurrent rises in GGT, ALT and/or serum bile acids may indicate overdose; monitor liver parameters regularly.
  • Contains lactose.

Side effects

  • Frequency not known: diarrhoea, increased transaminases, pruritus and gallstones.
  • Diarrhoea, pruritus and increased transaminases were associated with overdose and disappeared after dose reduction; gallstones after long-term treatment in very few patients.

Interactions

  • Phenobarbital and primidone antagonise cholic acid (contraindicated).
  • Ciclosporin alters pharmacokinetics and effect; avoid co-administration, otherwise monitor bile acid levels closely.
  • Bile acid sequestrants (cholestyramine, colestipol, colesevelam) and certain antacids (e.g. aluminium hydroxide): separate from the cholic acid dose by 5 hours; ursodeoxycholic acid also affects the effect.

Pregnancy and breastfeeding

  • No contraception needed. Limited data showed no adverse reactions in pregnancy; therapy should be continued with close monitoring.
  • Can be used during breast-feeding.

Summary of the official product information, written with AI on 2026-10-29. Every number was checked automatically against the original text. Not reviewed by a physician – the full official text is authoritative.

Full official text →

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For patients

Package leaflet, verbatim

This product has no separate US patient information.

Authorisation and brand names by country

Medicines with exactly this active-substance composition, as listed in the national registers – alphabetical and without ranking. Whether a product is currently available is not shown here.

EU – centrally authorised (all member states)

Source: European Medicines Agency (EMA), product information

Brand names in 3 more European countries
Iceland
Orphacol
Liechtenstein
Orphacol
Norway
Orphacol

EMA – Article 57 product data (EU/EEA), 2026-10-29

Medelix receives no money from pharmaceutical companies. Brand names are listed alphabetically and without ranking.

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Sources

  1. European Medicines Agency. Orphacol – EPAR, Product Information. 2026-10-28. https://www.ema.europa.eu/en/documents/product-information/orphacol-epar-product-information_en.pdf

Editorial: Medelix editorial team · last updated 2026-10-28 · How these pages are made · Report an error

Source: European Medicines Agency (EMA), product information. Not advertising, not a treatment recommendation. For questions about your own treatment, ask your doctor or pharmacist.