Chenodeoxycholic acid oral
- Drugs List
- Therapeutic Indications
- Dosage
- Administration
- Contraindications
- Precautions and Warnings
- Pregnancy and Lactation
- Side Effects
- Monograph
Presentation
Oral formulations of chenodeoxycholic acid.
Drugs List
Therapeutic Indications
Uses
Cerebrotendinous xanthomatosis
Unlicensed Uses
Inborn errors of bile acid synthesis
Smith-Lemli-Opitz syndrome
Dosage
The lowest dose of chenodeoxycholic acid that effectively reduces the serum cholestanol and/or the urine bile alcohols to within the normal range should be chosen.
Adults
Initial dose: 750mg daily in three divided doses. The dose may be increased in 250mg increments to a maximum of 1000mg daily.
Children
Treatment of cerebrotendinous xanthomatosis (CTX)
Children aged 1 month to 18 years
Initial dose: 5mg/kg daily in three divided doses. The dose may be increased to a maximum of 15mg/kg daily.
Smith-Lemli-Opitz syndrome (unlicensed)
7mg/kg daily.
Inborn error of bile acid synthesis (unlicensed)
Initial dose: 5mg/kg three times daily.
Maintenance dose: 2.5mg/kg three times daily.
Neonates
Smith-Lemli-Opitz syndrome (unlicensed)
7mg/kg daily.
Inborn error of bile acid synthesis (unlicensed)
Initial dose: 5mg/kg three times daily.
Maintenance dose: 2.5mg/kg three times daily.
Administration
For infants and children who are unable to swallow capsules, the capsules may be opened and the contents added to 50ml of sodium bicarbonate 8.4% (1mmol/ml) to form a suspension containing chenodeoxycholic acid 5mg/ml.
Contraindications
Neonates under 1 month
Non-functioning gallbladder
Pregnancy
Radio-opaque gallstones
Precautions and Warnings
Females of childbearing potential
Breastfeeding
Hepatic impairment
Renal impairment
Long term prophylaxis may be required to prevent recurrence
Treatment to be initiated and supervised by a specialist
Administer 1 hour before or 4 hours after any bile acid sequestrant
Perform liver function tests before commencing therapy and during therapy
Monitor hepatic function in patients with hepatic impairment
Monitor renal function in patients with renal impairment
Monitor serum and/or urine bile acid levels prior & during dose adjustment
Pregnancy confirmed: Advise patient to discontinue and consult doctor
Maintain treatment at the lowest effective dose
Avoid antacids within 2 hours of dose
Female: Barrier or non-hormonal contraception required during treatment
Female: Ensure adequate contraception during treatment
During the initial and dose adjustment period, serum cholestanol and/or urine bile alcohols should be monitored every 3 months and then annually.
Liver function should be monitored regularly as overdose of chenodeoxycholic acid may be indicated by elevation of liver enzymes.
Additional monitoring may be required during growth spurts, concomitant disease and pregnancy.
In case of persistent lack of therapeutic response to chenodeoxycholic acid monotherapy, other treatment options should be considered.
Oral contraceptives reduce the effectiveness of chenodeoxycholic acid in CTX and are not recommended.
Pregnancy and Lactation
Pregnancy
Chenodeoxycholic acid is contraindicated during pregnancy.
The manufacturer recommends chenodeoxycholic acid is not used in pregnancy and in women of child bearing potential not using contraception.
Animal studies have shown reproductive toxicity with chenodeoxycholic acid. At the time of writing there is limited human data available.
Lactation
Use chenodeoxycholic acid with caution during breastfeeding.
The manufacturer advises that as a precautionary measure, it is preferable to either discontinue breastfeeding or to discontinue chenodeoxycholic acid therapy taking into account the benefits for the child and mother.
It is unknown whether chenodeoxycholic acid is excreted in human milk.
Side Effects
Abnormal liver function tests
Constipation
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: Chenodeoxycholic acid Leadiant 250 mg hard capsules. Leadiant GmbH. Revised September 2017.
NICE Evidence Services Available at: www.nice.org.uk Last accessed: 9 August 2019
Medscape UK | Univadis prescription drug monographs & interactions are based on FDB Multilex Content
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