BNF for Children 2011-2012

786 A2.7 Nutritional supplements for metabolic diseases BNFC 2011–2012Appendix 2: Borderline substances1 XP Maxamum c (SHS)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine) 39 g, carbohydrate 34 g,fat less than 500 mg, energy 1260 kJ (297 kcal)/100 g, withvitamins, minerals, and trace elements. Orange, or unflavoured(flavouring; see Modjul c Flavour System, p. 777),net price 30 50-g sachets = £226.50, 500 g = £75.54Nutritional supplement for the dietary management of phenylketonuriain children over 8 yearsTyrosinaemiaMethionine-free TYR Anamix c Infant (SHS)Powder, protein equivalent (essential and non-essentialamino acids except methionine, phenylalanine, and tyrosine)13.1 g, carbohydrate 49.5 g, fat 23 g, fibre 5.3 g, energy1915 kJ (457 kcal)/100 g, with vitamins, minerals, and traceelements; standard dilution (15%) provides protein equivalent2 g, carbohydrate 7.4 g, fat 3.5 g, fibre 800 mg, energy287 kJ (69 kcal)/100 mL. Unflavoured, net price 400 g =£32.70 (5-g measuring scoop provided)Nutritional supplement for the dietary management of proventyrosinaemia type 1 in children from birth to 3 yearsTYR Anamix c Infant (SHS)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine and tyrosine) 13.1 g,carbohydrate 49.5 g, fat 23 g, fibre 5.3 g, energy 1915 kJ(457 kcal)/100 g, with vitamins, minerals, and trace elements;standard dilution (15%) provides protein equivalent2 g, carbohydrate 7.4 g, fat 3.5 g, fibre 800 mg, energy 287 kJ(69 kcal)/100 mL. Unflavoured, net price 400 g = £32.70 (5-gmeasuring scoop provided)Nutritional supplement for the dietary management of proventyrosinaemia where plasma-methionine concentrations are normalin children from birth to 3 yearsTYR Anamix c Junior (SHS)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine and tyrosine) 8.4 g,carbohydrate 11 g, fat 3.9 g, energy 475 kJ (113 kcal)/29-gsachet, with vitamins, minerals, and trace elements. Unflavoured,net price 30 x 29-g sachets = £173.32Nutritional supplement for the dietary management of proventyrosinaemia in children 1–10 yearsTYR Anamix c Junior LQ (SHS)Liquid, protein equivalent (essential and non-essentialamino acids except phenylalanine and tyrosine) 10 g,carbohydrate 8.8 g, fat 4.8 g, fibre 310 mg, energy500 kJ (119 kcal)/125 mL, with vitamins, minerals andtrace elements. Orange flavour, net price 36 x 125-mLbottle = £272.79Nutritional supplement for the dietary management of tyrosinaemiatype 1 (when nitisinone (NTBC) is used, see section 9.8.1),type II, and type III, in children over 1 yearTYR cooler c (Vitaflo)Liquid, protein equivalent (essential and non-essential aminoacids except tyrosine and phenylalanine) 15 g, carbohydrate7 g, fat 500 mg, energy 386 kJ (92 kcal)/130 mL, with vitamins,minerals, and trace elements. Orange or red flavour,net price 30 130-mL pouch = £277.20Nutritional supplement for the dietary management of tyrosinaemiain children over 8 yearsTYR express c (Vitaflo)Powder, protein equivalent (essential and non-essentialamino acids except tyrosine and phenylalanine) 15 g,carbohydrate 3.8 g, fat less than 100 mg, energy 315 kJ(76 kcal)/25 g, with vitamins, minerals, and trace elements.Unflavoured (flavouring: see Flavour Pac c , p. 777), netprice 30 25-g sachets = £271.88Nutritional supplement for the dietary management of tyrosinaemiain children over 8 years1. Maxamum products are generally intended for use inchildren over 8 yearsTYR Gel c (Vitaflo)Gel, protein equivalent (essential and non-essential aminoacids except tyrosine and phenylalanine) 8.4 g, carbohydrate8.6 g, fat less than 100 mg, energy 286 kJ (68 kcal)/20 g, withvitamins, minerals and trace elements. Unflavoured(flavouring: see Flavour Pac c , p. 777), net price 30 20-gsachets = £151.93Nutritional supplement for the dietary management of tyrosinaemiain children 1–10 years2 XPHEN TYR Maxamaid (SHS)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine and tyrosine) 25 g,carbohydrate 51 g, fat less than 500 mg, energy 1311 kJ(309 kcal)/100 g, with vitamins, minerals, and trace elements.Unflavoured (flavouring: see Modjul c FlavourSystem, p. 777), net price 500 g = £82.57Nutritional supplement for the dietary management of tyrosinaemiain children 1–8 yearsXPHEN TYR Tyrosidon (SHS)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine and tyrosine) 77 g,carbohydrate 4.5 g, fat nil, energy 1386 kJ (326 kcal)/100 g.Unflavoured (flavouring: see Modjul c Flavour System,p. 777), net price 500 g = £156.42Nutritional supplement for the management of tyrosinaemia inchildren and adults where plasma-methionine concentrations arenormalXPTM Tyrosidon (SHS)Powder, protein equivalent (essential and non-essentialamino acids except methionine, phenylalanine, and tyrosine)77 g, carbohydrate 4.5 g, fat nil, energy 1386 kJ (326 kcal)/100 g. Unflavoured (flavouring: see Modjul c FlavourSystem, p. 777), net price 500 g = £156.42Nutritional supplement for the dietary management of tyrosinaemiatype I in children and adults where plasma-methionineconcentrations are above normalUrea cycle disorders (other thanarginase deficiency)L-Arginine (SHS)Powder, net price 100 g = £12.27For use as a supplement in urea cycle disorders other thanarginase deficiency, such as hyperammonaemia types I and II,citrullaemia, arginosuccinic aciduria, and deficiency of N-acetylglutamate synthetaseConditions for which ACBS productscan be prescribedNote This is a list of clinical conditions for which the ACBS hasapproved toilet preparations. For details of the preparations seeChapter 13.BirthmarksSee Disfiguring skin lesions, belowDermatitisAveeno c Bath Oil; Aveeno c Cream; Aveeno c Colloidal;Aveeno c lotion; E45 c Emollient Bath Oil; E45 cEmollient Wash Cream; E45 c LotionDermatitis herpetiformisSee also Gluten-sensitive enteropathies, p. 7772. Maxamaid products are generally intended for use inchildren 1–8 years