BNF for Children 2011-2012

BNFC 2011–2012 9.4.2 Enteral nutrition 469TETRAHYDROBIOPTERINRenal impairment use with caution—accumulationof metabolitesPregnancy crosses the placenta; use only if benefitoutweighs riskBreast-feeding present in milk, effects unknownSide-effects diarrhoea, urinary frequency, disturbedsleepLicensed use not licensedIndication and doseMonotherapy in tetrahydrobiopterin-sensitivephenylketonuria (specialist use only). By mouthChild 1 month–18 years 10 mg/kg twice daily(total daily dose may alternatively be given in 3divided doses), adjusted according to responseIn combination with neurotransmitter precursorsfor tetrahydrobiopterin-sensitive phenylketonuria(specialist use only). By mouthChild 1 month–2 years initially 250–750 micrograms/kg4 times daily (total daily dose mayalternatively be given in 3 divided doses), adjustedaccording to response; max. 7 mg/kg dailyChild 2–18 years initially 250–750 micrograms/kg 4 times daily (total daily dose may alternativelybe given in 3 divided doses), adjusted according toresponse; usual max. 10 mg/kg dailyTetrahydrobiopterin (Non-proprietary)Tablets, tetrahydrobiopterin 10 mg and 50 mgAvailable from ‘special-order’ manufacturers or specialistimporting companies, see p. 809SAPROPTERIN DIHYDROCHLORIDENote Sapropterin is a synthetic form of tetrahydrobiopterinCautions monitor blood-phenylalanine concentrationbefore and after first week of treatment—if unsatisfactoryresponse increase dose at weekly intervals tomax. dose and monitor blood-phenylalanine concentrationweekly; discontinue treatment if unsatisfactoryresponse after 1 month; monitor blood-phenylalanineand tyrosine concentrations 1–2 weeks after doseadjustment and during treatment; history of convulsionsHepatic impairment manufacturer advises caution—no information availableRenal impairment manufacturer advises caution—noinformation availablePregnancy manufacturer advises caution—consideronly if strict dietary management inadequateBreast-feeding manufacturer advises avoid—noinformation availableSide-effects diarrhoea, vomiting, abdominal pain;nasal congestion, cough, pharyngolaryngeal pain;headacheIndication and dosePhenylketonuria (specialist use only). By mouthChild 4–18 years initially 10 mg/kg once daily,preferably in the morning, adjusted according toresponse; usual dose 5–20 mg/kg dailyTetrahydrobiopterin deficiency (specialist useonly). By mouthNeonate initially 2–5 mg/kg once daily, preferablyin the morning, adjusted according to response;max. 20 mg/kg daily; total daily dose may alternativelybe given in 2–3 divided dosesChild 1 month–18 years initially 2–5 mg/kg oncedaily, preferably in the morning, adjusted accordingto response; max. 20 mg/kg daily; total dailydose may alternatively be given in 2–3 divideddosesKuvan c (Merck Serono) TADispersible tablets, sapropterin dihydrochloride100 mg, net price 30-tab pack = £597.22, 120-tab pack= £2388.88. Label: 13, 21, counselling, tablets shouldbe dissolved in water and taken within 20 minutes9.4.2 Enteral nutritionChildren have higher nutrient requirements per kgbody-weight, different metabolic rates, and physiologicalresponses compared to adults. They have lownutritional stores and are particularly vulnerable togrowth and nutritional problems during critical periodsof development. Major illness, operations, or traumaimpose increased metabolic demands and can rapidlyexhaust nutritional reserves.Every effort should be made to optimise oral food intakebefore beginning enteral tube feeding; this may includechange of posture, special seating, feeding equipment,oral desensitisation, food texture changes, thickening ofliquids, increasing energy density of food, treatment ofreflux or oesophagitis, as well as using age-specificnutritional supplements.Enteral tube feeding has a role in both short-termrehabilitation and long-term nutritional managementin paediatrics. It can be used as supportive therapy, inwhich the enteral feed supplies a proportion of therequired nutrients, or as primary therapy, in which theenteral feed delivers all the necessary nutrients. Mostchildren receiving tube feeds should also be encouragedto take oral food and drink. Tube feeding should beconsidered in the following situations:. unsafe swallowing and risk of aspiration;. inability to consume at least 60% of energy needsby mouth;. total feeding time of more than 4 hours per day;. weight loss or no weight gain for a period of 3months (less for younger children and infants);. weight for height (or length) less than 2nd percentilefor age and sex.Most feeds for enteral use (Appendix 2) contain proteinderived from cows’ milk or soya. Elemental feeds containingprotein hydrolysates or free amino acids can beused for children who have diminished ability to breakdown protein, for example in inflammatory bowel diseaseor pancreatic insufficiency.9 Nutrition and blood