BNF for Children 2011-2012

BNFC 2011–2012 1.9.1 Drugs affecting biliary composition and flow 69Ursodeoxycholic acid may also relieve the severe itchingassociated with cholestasis.In sclerosing cholangitis, ursodeoxycholic acid is usedto lower liver enzyme and serum-bilirubin concentrations.Ursodeoxycholic acid is also used in the treatment ofintrahepatic cholestasis in pregnancy.Smith-Lemli-Opitz syndrome Chenodeoxycholicand ursodeoxycholic acid have been used with cholesterolin children with Smith-Lemli-Opitz syndrome.Chenodeoxycholic acid is also used in combinationwith cholic acid to treat bile acid synthesis defects butcholic acid is difficult to obtain. Chenodeoxycholic acidand cholesterol are available from ‘special-order’ manufacturersor specialist importing companies, see p. 809.URSODEOXYCHOLIC ACIDCautions interactions: Appendix 1 (bile acids)Contra-indications radio-opaque stones; non-functioninggall bladder (in patients with radiolucentgallstones)Hepatic impairment avoid in chronic liver disease(but used in primary biliary cirrhosis)Pregnancy no evidence of harm but manufactureradvises avoidBreast-feeding not known to be harmful but manufactureradvises avoidSide-effects rarely, diarrhoeaLicensed use not licensed for use in children forindications shown belowIndication and doseCholestasis. By mouthNeonate 5 mg/kg 3 times daily, adjust dose andfrequency according to response, max. 10 mg/kg 3times dailyChild 1 month–2 years 5 mg/kg 3 times daily,adjust dose and frequency according to response,max. 10 mg/kg 3 times dailyImprovement of hepatic metabolism of essentialfatty acids and bile flow, in children withcystic fibrosis. By mouthChild 1 month–18 years 10–15 mg/kg twicedaily; total daily dose may alternatively be given in3 divided dosesCholestasis associated with total parenteralnutrition. By mouthNeonate 10 mg/kg 3 times dailyChild 1 month–18 years 10 mg/kg 3 times dailySclerosing cholangitis. By mouthChild 1 month–18 years 5–10 mg/kg 2–3 timesdaily, adjusted according to response, max. 15 mg/kg 3 times dailyUrsodeoxycholic Acid (Non-proprietary) ATablets, ursodeoxycholic acid 150 mg, net price 60-tab pack = £20.48. Label: 21Capsules, ursodeoxycholic acid 250 mg, net price 60-cap pack = £38.86. Label: 21Destolit c (Norgine) ATablets, scored, ursodeoxycholic acid 150 mg, netprice 60-tab pack = £17.67. Label: 21Urdox c (Wockhardt) ATablets, f/c, ursodeoxycholic acid 300 mg, net price60-tab pack = £26.50. Label: 21Ursofalk c (Dr Falk) ACapsules, ursodeoxycholic acid 250 mg, net price 60-cap pack = £30.17, 100-cap pack = £31.88. Label: 21Suspension, sugar-free, ursodeoxycholic acid250 mg/5 mL, net price 250 mL = £26.98. Label: 21Ursogal c (Galen) ATablets, scored, ursodeoxycholic acid 150 mg, netprice 60-tab pack = £17.05. Label: 21Capsules, ursodeoxycholic acid 250 mg, net price 60-cap pack = £30.50. Label: 21Other preparations for bile synthesisdefectsCHENODEOXYCHOLIC ACIDCautions see under Ursodeoxycholic Acid; interactions:Appendix 1 (bile acids)Contra-indications see under Ursodeoxycholic AcidPregnancy avoid—fetotoxicity reported in animalstudiesSide-effects see under Ursodeoxycholic AcidLicensed use not licensedIndication and doseCerebrotendinous xanthomatosis. By mouthNeonate 5 mg/kg 3 times dailyChild 1 month–18 years 5 mg/kg 3 times dailyDefective synthesis of bile acid. By mouthNeonate initially 5 mg/kg 3 times daily, reduced to2.5 mg/kg 3 times dailyChild 1 month–18 years initially 5 mg/kg 3 timesdaily, reduced to 2.5 mg/kg 3 times dailySmith-Lemli-Opitz syndrome see notes above. By mouthNeonate 7 mg/kg once daily or in divided dosesChild 1 month–18 years 7 mg/kg once daily or individed dosesAdministration for administration by mouth, add thecontents of a 250-mg capsule to 25 mL of sodiumbicarbonate solution 8.4% (1 mmol/mL) to produce asuspension containing chenodeoxycholic acid 10 mg/mL; use immediately after preparation, discard anyremaining suspension1 Gastro-intestinal system