BNF for Children 2011-2012

488 9.8 Metabolic disorders BNFC 2011–20129 Nutrition and blood2 micrograms, folic acid 100 micrograms, nicotinamide7.5 mg, pantothenic acid 2 mg, pyridoxine1 mg, riboflavin 1 mg, thiamine 1.5 mg, vitamin A1250 units, vitamin D 2 200 units, vitamin E 5 mg, vitaminK 1 25 micrograms), minerals and trace elements(chromium 50 micrograms, copper 1 mg, iodine75 micrograms, iron 5 mg, magnesium 1 mg, manganese1.25 mg, molybdenum 50 micrograms, selenium25 micrograms, zinc 5 mg), net price 30-cap pack =£3.52, 60-cap pack = £6.69DoseVitamin and mineral deficiency and as adjunct insynthetic dietsChild 5–12 years 2 junior capsules dailyKetovite c (Paines & Byrne)Tablets A, yellow, ascorbic acid 16.6 mg, riboflavin1 mg, thiamine hydrochloride 1 mg, pyridoxinehydrochloride 330 micrograms, nicotinamide 3.3 mg,calcium pantothenate 1.16 mg, alpha tocopherylacetate 5 mg, inositol 50 mg, biotin 170 micrograms,folic acid 250 micrograms, acetomenaphthone500 micrograms, net price 100-tab pack = £4.17DosePrevention of vitamin deficiency in disorders ofcarbohydrate or amino-acid metabolism and adjunctin restricted, specialised, or synthetic dietsChild 1 month—18 years 1 tablet 3 times daily; doseadjusted according to condition, diet, or age; use withKetovite c Liquid for complete vitamin supplementationAdministration may be crushed immediately before useLiquid, pink, sugar-free, vitamin A 2500 units, ergocalciferol400 units, choline chloride 150 mg, cyanocobalamin12.5 micrograms/5 mL, net price 150-mLpack = £2.70DosePrevention of vitamin deficiency in disorders ofcarbohydrate or amino-acid metabolism and adjunctin restricted, specialised, or synthetic dietsChild 1 month—18 years 5 mL daily; dose adjustedaccording to condition, diet, or age; use with Ketovite cTablets for complete vitamin supplementationAdministration may be mixed with milk, cereal, or fruit juice9.8.1 Drugs used in metabolicdisordersMetabolic disorders should be managed under the guidanceof a specialist. As many preparations are unlicensedand may be difficult to obtain, arrangements forcontinued prescribing and supply should be made inprimary care.General advice on the use of medicines in metabolicdisorders can be obtained from:Alder Hey Children’s HospitalMedicines Information CentreTel: (0151) 252 5381andGreat Ormond Street Hospital for ChildrenPharmacyTel: (020) 7405 9200Wilson’s diseasePenicillamine is used in Wilson’s disease (hepatolenticulardegeneration) to aid the elimination of copperions; it is also used for cystinuria. Children who arehypersensitive to penicillin may react rarely to penicillamine.Trientine is used for the treatment of Wilson’s diseaseonly, in patients intolerant of penicillamine; it is not analternative to penicillamine in other diseases such ascystinuria. Penicillamine-induced systemic lupuserythematosus may not resolve on transfer to trientine.Zinc prevents the absorption of copper in Wilson’sdisease. Symptomatic patients should be treated initiallywith a chelating agent because zinc has a slow onset ofaction. When transferring from chelating treatment tozinc maintenance therapy, chelating treatment shouldbe co-administered for 2–3 weeks until zinc produces itsmaximal effect.9.7 Bitters and tonicsClassification not included in BNF for Children.9.8 Metabolic disorders9.8.1 Drugs used in metabolic disorders9.8.2 Acute porphyriasThis section covers drugs used in metabolic disordersand not readily classified elsewhere.PENICILLAMINECautions concomitant nephrotoxic drugs (increasedrisk of toxicity); monitor urine for proteinuria; monitorblood and platelet count regularly (see below); neurologicalinvolvement in Wilson’s disease; interactions:Appendix 1 (penicillamine)Blood counts and urine tests Consider withdrawal if plateletcount falls below 120 000/mm 3 or white blood cellsbelow 2500/mm 3 or if 3 successive falls within referencerange (can restart at reduced dose when counts return towithin reference range but permanent withdrawal necessaryif recurrence of leucopenia or thrombocytopenia)Counselling Warn child and carer to tell doctor immediatelyif sore throat, fever, infection, non-specific illness, unexplainedbleeding and bruising, purpura, mouth ulcers, orrashes developContra-indications lupus erythematosusRenal impairment reduce dose and monitor renalfunction or avoid—consult product literaturePregnancy fetal abnormalities reported rarely; avoid ifpossibleBreast-feeding manufacturer advises avoid unlesspotential benefit outweighs risk—no informationavailable