Cholic acid oral
- Drugs List
- Therapeutic Indications
- Dosage
- Administration
- Contraindications
- Precautions and Warnings
- Pregnancy and Lactation
- Side Effects
- Monograph
Presentation
Oral formulations of cholic acid.
Drugs List
Therapeutic Indications
Uses
Inborn errors of bile acid synthesis
Dosage
The dose must be adjusted for each patient in a specialised unit according to blood and/or urine chromatographic bile acid profiles.
The daily dose may be divided if it consists of more than one capsule in order to mimic the continuous production of cholic acid in the body, and to reduce the number of capsules that need to be taken per administration.
The lowest dose of cholic acid that effectively reduces the bile acid metabolites to as close to zero as possible should be chosen.
Adults
5mg/kg to 15mg/kg daily with food at the same time each day, in the morning and/or evening.
It is recommended that the minimum dose is 50mg and that the dose is adjusted in 50mg steps, up to a maximum of 500mg daily in adults.
Children
Children aged over 1 month
5mg/kg to 15mg/kg daily with food at the same time each day, in the morning and/or evening.
It is recommended that the minimum dose is 50mg and that the dose is adjusted in 50mg steps.
Patients with Hepatic Impairment
The dose of cholic acid should be adjusted individually in patients with inborn errors of bile acid metabolism and hepatic impairment.
No data regarding cholic acid treatment are available in patients with inborn errors of bile acid metabolism with hepatic impairment unrelated to their primary disease, and as such no recommendations on dosage adjustment can be made. All patients with hepatic impairment being treated with cholic acid should be monitored closely.
Additional Dosage Information
Familial hypertriglyceridaemia
The cholic acid dose in patients with newly diagnosed or a family history of familial hypertriglyceridaemia may need to be elevated due to the poor absorption of cholic acid in the intestines. Doses will have to be established and adjusted as necessary. The manufacturer suggests that an elevated dose, notably higher than the 500mg daily limit for adult patients, may be required and safe.
Administration
For infants and children who cannot swallow capsules, the capsules may be opened and the content added to infant formula or juice.
Contraindications
Neonates under 1 month
Galactosaemia
Precautions and Warnings
Familial hypertriglyceridaemia
Glucose-galactose malabsorption syndrome
Hepatic impairment
Lactose intolerance
Pregnancy
Treatment to be initiated and supervised by a specialist
Contains lactose
Monitor hepatic function
Monitor prothrombin times
Monitor serum and/or urine bile acid levels prior & during dose adjustment
Discontinue if hepatic function deteriorates
Discontinue treatment if prothrombin time does not improve within 3 months
Familial hypertriglyceridaemia: Higher doses than 500mg daily may be needed
Advise to avoid antacids &/or bile acid sequestrants within 5 hours of dose
During the initiation of therapy and dose adjustment, serum and/or urine bile acid levels should be monitored intensively using suitable analytical techniques. It is recommended that monitoring takes place at least every 3 months during the first year of treatment, 6 monthly during the subsequent three years and annually thereafter.
Liver parameters should be monitored, preferentially more frequently than serum and/or urine bile acid levels. Concurrent elevation of serum gamma glutamyltransferase (GGT), alanine aminotransferase (ALT) and/or serum bile acids above normal levels may indicate overdose. Transient elevations of transaminases at the initiation of cholic acid treatment have been observed and do not indicate the need for a dose reduction if GGT is not elevated and if serum bile acid levels are falling or in the normal range.
Treatment with cholic acid in patients with pre-existing hepatic impairment should be given under close monitoring.
Treatment with cholic acid should be stopped if abnormal hepatocellular function, as measured by prothrombin time, does not improve within 3 months of the initiation of cholic acid treatment for all patients. Treatment should be stopped earlier if there are clear indicators of severe hepatic failure.
In case of persistent lack of therapeutic response to cholic acid monotherapy, other treatment options should be considered.
Pregnancy and Lactation
Pregnancy
Use cholic acid with caution during pregnancy.
The manufacturer advises that it is extremely important that pregnant women continue their therapy during pregnancy but pregnant women and their unborn children should be closely monitored.
There is a limited amount of data (less than 20 pregnancy outcomes) from the use of cholic acid in pregnant women. The exposed pregnancies, however, showed no adverse reactions to cholic acid and resulted in normal, healthy children.
Animal studies do not indicate direct or indirect harmful effects with respect to reproductive toxicity.
Lactation
Cholic acid is considered safe to use during breastfeeding.
The manufacturer advises that cholic acid may be used during breastfeeding.
Cholic acid and its metabolites are excreted in human milk, but at therapeutic doses, no effects on the breastfed newborn/infant are anticipated.
Side Effects
Diarrhoea
Gallstones
Increase in serum transaminases
Pruritus
Overdosage
It is strongly recommended that the UK National Poisons Information Service be consulted on cases of suspected or actual overdose where there is doubt over the degree of risk or about appropriate management.
The following number will direct the caller to the relevant local centre (0844) 892 0111
Information may be obtained if you have access to ToxBase the primary clinical toxicology database of the National Poisons Information Service. This is available via password on the internet ( www.toxbase.org ) or if this is unavailable at the backup site ( www.toxbasebackup.org ).
Further Information
Last Full Review Date: August 2019.
Reference Sources
Summary of Product Characteristics: Orphacol 50 milligram capsules. Laboratoires CTRS. Revised July 2020.
Summary of Product Characteristics: Orphacol 250 milligram capsules. Laboratoires CTRS. Revised July 2020.
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