BNF for Children 2011-2012

BNFC 2011–2012 A2.7 Nutritional supplements for metabolic diseases 785PKU Anamix c First Spoon (SHS)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine) 5 g, carbohydrate 4.8 g,fat 150 mg, fibre nil, energy 168 kJ (41 kcal)/12.5-g sachet,with vitamins, minerals, and trace elements, net price 30 12.5 g = £81.00Nutritional supplement for the dietary management of provenphenylketonuria in children from 6 months to 5 yearsPKU Anamix c Infant (SHS)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine) 13.1 g, carbohydrate49.5 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 equivalent 2 g, carbohydrate7.4 g, fat 3.5 g, fibre 800 mg, energy 287 kJ (69 kcal)/100 mL.Unflavoured, net price 400 g = £29.72 (5-g measuring scoopprovided)Nutritional supplement for the dietary management of provenphenylketonuria in children from birth to 3 yearsPKU Anamix c Junior (SHS)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine) 8.4 g, carbohydrate 9.9 g,fat 3.9 g, energy 455 kJ (108 kcal)/29-g sachet, with vitamins,minerals, and trace elements. Flavours: chocolate, pineapple-vanilla,or unflavoured (carbohydrate 11 g, energy 474 kJ(113 kcal)/29-g sachet), net price 30 29-g sachets =£106.20Nutritional supplement for the dietary management of phenylketonuriain children 1–10 yearsPKU Anamix Junior LQ c (SHS)Liquid, protein equivalent (essential and non-essential aminoacids except phenylalanine) 10 g, carbohydrate 8.8 g, fat4.8 g, fibre 310 mg, energy 497 kJ (118 kcal)/125 mL, withvitamins, minerals, and trace elements. Lactose-free. Flavours:Berry, orange, or unflavoured, net price 125-mLcarton = £4.72Nutritional supplement for the dietary supplement of phenyketonuriain children 1–10 yearsPKU cooler10 c (Vitaflo)Liquid, protein equivalent (essential and non-essential aminoacids except phenylalanine) 10 g, carbohydrate 5.1 g, energy258 kJ (62 kcal)/87-mL pouch, with vitamins, minerals, andtrace elements. Unflavoured (white) or flavoured (orange,purple, or red), net price 30 87-mL = £112.80Nutritional supplement for the dietary management of phenylketonuriain children over 3 yearsPKU cooler15 c (Vitaflo)Liquid, protein equivalent (essential and non-essential aminoacids except phenylalanine) 15 g, carbohydrate 7.8 g, energy386 kJ (92 kcal)/130-mL pouch, with vitamins, minerals, andtrace elements. Unflavoured (white) or flavoured (orange,purple, or red), net price 30 x 130 mL = £168.00Nutritional supplement for the dietary management of phenylketonuriain children over 3 yearsPKU cooler20 c (Vitaflo)Liquid, protein equivalent (essential and non-essential aminoacids except phenylalanine) 20 g, carbohydrate 10.2 g,energy 517 kJ (124 kcal)/174-mL pouch, with vitamins,minerals, and trace elements. Unflavoured (white) or flavoured(orange, purple, or red), net price 30 174 mL =£225.60Nutritional supplement for the dietary management of phenylketonuriain children over 3 yearsPKU express c (Vitaflo)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine) 15 g, carbohydrate 3.8 g,energy 315 kJ (76 kcal)/25 g, with vitamins, minerals, andtrace elements. Lemon, orange, tropical, or unflavoured, netprice 30 x 25-g sachets = £164.84Nutritional supplement for the dietary management of phenylketonuriain children over 8 yearsPKU gel c (Vitaflo)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine) 8.4 g, carbohydrate 8.6 g,fat less than 100 mg, energy 286 kJ (68 kcal)/20 g, withvitamins, minerals and trace elements. Orange, raspberry, orunflavoured (flavouring: see Flavour Pac c , p. 777), net price30 20-g sachets = £95.03For use as part of the low-protein dietary management ofphenylketonuria in children 1–10 yearsPKU Lophlex c LQ 10 (SHS)Liquid, protein equivalent (essential and non-essential aminoacids except phenylalanine) 10 g, carbohydrate 4.4 g, fibre170 mg, energy 245 kJ (58 kcal)/62.5 mL, with vitamins,minerals, and trace elements. Flavours: berry, citrus, orange,or tropical, net price 60 62.5 mL = £261.00Nutritional supplement for the dietary management of phenylketonuriain children over 4 years and adults including pregnantwomenPKU Lophlex c LQ 20 (SHS)Liquid, protein equivalent (essential and non-essential aminoacids except phenylalanine) 20 g, carbohydrate 8.8 g, fibre340 mg, energy 490 kJ (115 kcal)/125 mL, with vitamins,minerals, and trace elements. Flavours: berry, citrus, orange,or tropical, net price 3 125 mL = £26.04Nutritional supplement for the dietary management of phenylketonuriain children over 4 years and adults including pregnantwomenPKU Start c (Vitaflo)Liquid, protein equivalent (essential and non-essential aminoacids except phenylalanine) 2 g, carbohydrate 8.3 g, fat 2.9 g,energy 286 kJ (68 kcal)/100 mL with vitamins, minerals, andtrace elements. Contains lactose and fish oil. Net price 500-mL bottle = £5.58For the dietary management of phenylketonuria in children under12 monthsSno-Pro c (SHS)Liquid, protein (cows’ milk) 220 mg (phenylalanine 12.5 mg),carbohydrate 8 g, fat 3.8 g, energy 273 kJ (65 kcal)/100 mL.Contains lactose. Net price 200 mL = £1.05Nutritional supplement for the dietary management of phenylketonuria,chronic renal failure and other inborn errors of aminoacid metabolismL-Tyrosine (SHS)Powder, net price 100 g = £18.41Nutritional supplement for the dietary management of phenylketonuriain pregnant women with low plasma tyrosine concentrationsTyrosine Amino Acid Supplement (Vitaflo)Powder, tyrosine 1 g, carbohydrate 2.9 g, energy 62 kJ(15 kcal)/4-g sachet, net price 30 4-g sachets = £4.08Nutritional supplement for the dietary management of phenylketonuriaand other inborn errors of amino acid metabolism1 XP Maxamaid (SHS)Powder, protein equivalent (essential and non-essentialamino acids except phenylalanine) 25 g, carbohydrate 51 g,fat less than 500 mg, energy 1311 kJ (309 kcal)/100 g, withvitamins, minerals, and trace elements. Orange flavour, orunflavoured (flavouring; see Modjul c Flavour System,p. 777), net price 500 g = £48.85Nutritional supplement for the dietary management of phenylketonuriain children 1– 8 years1. Maxamaid products are generally intended for use inchildren 1–8 yearsAppendix 2: Borderline substances