BNF for Children (BNFC) 2018-2019

lINTERACTIONS→Appendix 1 : chenodeoxycholic acid

lSIDE-EFFECTSConstipation

lPREGNANCYManufacturer advises avoid—fetotoxicity
reported inanimalstudies.
lHEPATIC IMPAIRMENTManufacturer advises monitor—no
information available.

lRENAL IMPAIRMENTManufacturer advises monitor—no
information available.

lMONITORING REQUIREMENTSManufacturer advises to
monitor serum cholestanol levels and/or urine bile
alcohols every 3 months during the initiation of therapy
and dose adjustment, and then at least annually; liver
function should also be monitored during initiation of
therapy and then at least annually; additional or more
frequent investigations may need to be undertaken to
monitor therapy during periods of fast growth or
concomitant disease.

lDIRECTIONS FOR ADMINISTRATIONFor administrationby
mouthin patients who are unable to swallow capsules
and/or need to take a dose below 250 mg, manufacturer
advises to add capsule contents to sodium bicarbonate
solution 8. 4 %—for further information, consult product
literature.

lMEDICINAL FORMS
There can be variation in the licensing of different medicines
containing the same drug.
Capsule
▶Chenodeoxycholic acid (non-proprietary)A
Chenodeoxycholic acid 250 mgChenodeoxycholic acid 250 mg
capsules| 100 capsuleP£ 14 , 000. 00

Cholic acid 21-Mar-2016

lDRUG ACTIONCholic acid is the predominant primary bile
acid in humans, which can be used to provide a source of
bile acid in patients with inborn deficiencies in bile acid
synthesis.

lINDICATIONS AND DOSE

Inborn errors of primary bile acid synthesis (initiated by a

specialist)

▶BY MOUTH

▶Child (body-weight up to 10 kg): 50 mg daily, then

increased in steps of 50 mg daily in divided doses;

usual dose 5 – 15 mg/kg daily in divided doses, dose to

be given with food at the same time each day

▶Child (body-weight 10 kg and above):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

lINTERACTIONS→Appendix 1 : cholic acid

lSIDE-EFFECTSCholelithiasis (long term use).diarrhoea.
pruritus
SIDE-EFFECTS, FURTHER INFORMATIONPatients presenting
with pruritus and/or persistent diarrhoea should be
investigated for potential overdose by a serum and/or
urine bile acid assay.

lPREGNANCYLimited data available—not known to be
harmful, manufacturer advises continue treatment.
MonitoringManufacturer advises monitor patient
parameters more frequently in pregnancy.

lBREAST FEEDINGPresent in milk but not known to be
harmful.

lHEPATIC IMPAIRMENT
MonitoringManufacturer advises monitor closely.

lMONITORING REQUIREMENTSManufacturer advises
monitor serum and/or urine bile-acid concentrations every
3 months for thefirst year, then every 6 months for three

years, then annually; monitor liver function tests at the

same or greater frequency.

lDIRECTIONS FOR ADMINISTRATIONManufacturer advises

capsules may be opened and the content added to infant

formula, juice, fruit compote, or yoghurt for

administration.

lPATIENT AND CARER ADVICECounselling advised on

administration.

lMEDICINAL FORMS

There can be variation in the licensing of different medicines

containing the same drug.

Capsule

CAUTIONARY AND ADVISORY LABELS 25

▶Orphacol(Laboratoires CTRS)A

Cholic acid 50 mgOrphacol 50 mg capsules| 30 capsuleP

£ 1 , 860. 00 | 60 capsuleP£ 3 , 720. 00

Cholic acid 250 mgOrphacol 250 mg capsules| 30 capsuleP

£ 6 , 630. 00

Ursodeoxycholic acid

lINDICATIONS AND DOSE

Cholestasis

▶BY MOUTH

▶Neonate: 5 mg/kg 3 times a day (max. per dose 10 mg/kg

3 times a day), adjusted according to response.

▶Child 1–23 months: 5 mg/kg 3 times a day (max. per dose

10 mg/kg 3 times a day), adjusted according to

response

Improvement of hepatic metabolism of essential fatty

acids and bile flow, in children with cystic fibrosis

▶BY MOUTH

▶Child: 10 – 15 mg/kg twice daily, total daily dose may

alternatively be given in 3 divided doses

Cholestasis associated with total parenteral nutrition

▶BY MOUTH

▶Neonate: 10 mg/kg 3 times a day.

▶Child: 10 mg/kg 3 times a day

Sclerosing cholangitis

▶BY MOUTH

▶Child: 5 – 10 mg/kg 2 – 3 times a day (max. per dose

15 mg/kg 3 times a day), adjusted according to

response

lUNLICENSED USENot licensed for use in children for the

treatment of cholestasis, sclerosing cholangitis,

cholestasis associated with total parenteral nutrition or

the improvement of hepatic metabolism of essential fatty

acids and bileflow in cysticfibrosis.

lCONTRA-INDICATIONSNon-functioning gall bladder.

radio-opaque stones

lINTERACTIONS→Appendix 1 : ursodeoxycholic acid

lSIDE-EFFECTS

▶Common or very commonDiarrhoea.pale faeces

▶Rare or very rareCholelithiasis calcification.skin

reactions

▶Frequency not knownNausea.vomiting

lPREGNANCYNo evidence of harm but manufacturer

advises avoid.

lBREAST FEEDINGNot known to be harmful but

manufacturer advises avoid.

lHEPATIC IMPAIRMENTAvoid in chronic liver disease (but

used in primary biliary cirrhosis).

lPATIENT AND CARER ADVICEPatients should be given

dietary advice (including avoidance of excessive

cholesterol and calories).

BNFC 2018 – 2019 Biliary disorders 65

Gastro-intestinal system

1