BNF for Children 2011-2012

468 9.4 Oral nutrition BNFC 2011–20129 Nutrition and bloodAdditrace c (Fresenius Kabi) ASolution, trace elements for addition to Vamin csolutions and glucose intravenous infusions, traces ofFe 3þ , Zn 2þ , Mn 2þ , Cu 2þ , Cr 3þ , Se 4þ , Mo 6þ ,F,I . Forchildren over 40 kg. Net price 10-mL amp = £2.31Cernevit c (Baxter) ASolution, dl-alpha tocopherol 11.2 units, ascorbicacid 125 mg, biotin 69 micrograms, colecalciferol220 units, cyanocobalamin 6 micrograms, folic acid414 micrograms, glycine 250 mg, nicotinamide 46 mg,pantothenic acid (as dexpanthenol) 17.25 mg, pyridoxinehydrochloride 5.5 mg, retinol (as palmitate)3500 units, riboflavin (as dihydrated sodium phosphate)4.14 mg, thiamine (as cocarboxylase tetrahydrate)3.51 mg. Dissolve in 5 mL water for injections.Net price per vial = £4.64Decan c (Baxter) ASolution, trace elements for addition to infusionsolutions, Fe 2þ , Zn 2þ , Cu 2þ , Mn 2þ ,F, Co 2þ I , Se 4þ ,Mo 6þ , Cr 3þ . For children over 40 kg. Net price 40-mLvial = £2.00Dipeptiven c (Fresenius Kabi) ASolution, N(2)-L-alanyl-L-glutamine 200 mg/mL(providing L-alanine 82 mg, L-glutamine 134.6 mg).For addition to infusion solutions containing aminoacids. Net price 50 mL = £16.40, 100 mL = £30.50DoseAmino acid supplement for hypercatabolic or hypermetabolicstates300–400 mg/kg daily; max. 400 mg/kg daily, dose not toexceed 20% of total amino acid intakeGlycophos c Sterile Concentrate (Fresenius Kabi) ASolution, sterile, phosphate 20 mmol, Na + 40 mmol/20 mL. For addition to Vamin c and Vaminolact csolutions, and glucose intravenous infusions. Netprice 20-mL vial = £4.60Peditrace c (Fresenius Kabi) ASolution, trace elements for addition toVaminolact c , Vamin c 14 Electrolyte-Free solutionsand glucose intravenous infusions, traces of Zn 2þ ,Cu 2þ , Mn 2þ , Se 4þ ,F,I . For use in neonates (whenkidney function established, usually second day oflife), infants, and children. Net price 10-mL vial =£4.18Cautions reduced biliary excretion especially in cholestatic liverdisease or in markedly reduced urinary excretion (careful biochemicalmonitoring required); total parenteral nutrition exceeding1 month (measure serum manganese concentration and checkliver function before commencing treatment and regularly duringtreatment)—discontinue if manganese concentration raised or ifcholestasis developsSolivito N c (Fresenius Kabi) ASolution, powder for reconstitution, biotin 60 micrograms,cyanocobalamin 5 micrograms, folic acid400 micrograms, glycine 300 mg, nicotinamide 40 mg,pyridoxine hydrochloride 4.9 mg, riboflavin sodiumphosphate 4.9 mg, sodium ascorbate 113 mg, sodiumpantothenate 16.5 mg, thiamine mononitrate 3.1 mg.Dissolve in water for injections or glucose intravenousinfusion for adding to glucose intravenous infusion orIntralipid c ; dissolve in Vitlipid N c or Intralipid c foradding to Intralipid c only. Net price per vial = £2.32Vitlipid N c (Fresenius Kabi) AEmulsion, adult, vitamin A 330 units, ergocalciferol20 units, dl-alpha tocopherol 1 unit, phytomenadione15 micrograms/mL. For addition to Intralipid c . Foradults and children over 11 years. Net price 10-mLamp = £2.32Emulsion, infant, vitamin A 230 units, ergocalciferol40 units, dl-alpha tocopherol 0.7 unit, phytomenadione20 micrograms/mL. For addition to Intralipid c .Net price 10-mL amp = £2.329.4 Oral nutrition9.4.1 Foods for special diets9.4.2 Enteral nutrition9.4.1 Foods for special dietsThese are preparations that have been modified toeliminate a particular constituent from a food or thatare nutrient mixtures formulated as food substitutes forchildren who either cannot tolerate or cannot metabolisecertain common constituents of food.Coeliac disease Intolerance to gluten in coeliacdisease is managed by completely eliminating glutenfrom the diet. A range of gluten-free products is availablefor prescription—see Appendix 2 (p. 777).Phenylketonuria Phenylketonuria (hyperphenylalaninaemia,PKU), which results from the inability to metabolisephenylalanine, is managed by restricting dietaryintake of phenylalanine to a small amount sufficient fortissue building and repair.Aspartame (used as a sweetener in some foods andmedicines) contributes to the phenylalanine intake andmay affect control of phenylketonuria. If alternatives areunavailable, children with phenylketonuria should notbe denied access to appropriate medication; the amountof aspartame consumed can be taken in to account inthe management of the condition. Where the presenceof aspartame in a preparation is specified in the productliterature, aspartame is listed as an excipient in therelevant product entry in BNF for Children; the childor carer should be informed of this.For further information on special dietary products usedin the management of metabolic diseases, see Appendix2.Some rare forms of phenylketonuria are caused by adeficiency of tetrahydrobiopterin. Treatment involvesoral supplementation of tetrahydrobiopterin; in somesevere cases, the addition of the neurotransmitter precursors,levodopa (section 4.9.1) and 5-hydroxytryptophan,is also necessary.Sapropterin, a synthetic form of tetrahydrobiopterin, islicensed as an adjunct to dietary restriction of phenylalaninein the management of patients with phenylketonuriaand tetrahydrobiopterin deficiency.