BNF for Children 2011-2012

BNFC 2011–2012 A2.7 Nutritional supplements for metabolic diseases 781Loprofin c (SHS)Low-protein. Mix, plain 500 g = £7.09; chocolate 500 g =£7.50; lemon 500 g = £7.50. Egg replacer 2 250 g = £13.03.Egg-white replacer 100 g = £8.38PK Foods c (Gluten Free Foods Ltd)Low-protein. Flour mix 750 g = £9.91. Egg replacer 350 g =£4.67ACBS IndicationsPastaLoprofin c (SHS)Low-protein. Pasta, animal shapes 500 g = £7.13; conchigle500 g = £7.15; spirals 500 g = £7.42; gnoccetti sardi 500 g =£7.15; lasagne 250 g = £3.61; macaroni elbows 250 g = £3.56;penne 500 g = £7.42; spaghetti 500 g = £7.42; tagliatelle 250 g= £3.56; vermicelli 250 g = £3.69. Rice, imitation 500 g =£7.20Promin c (Firstplay Dietary)Low-protein. Pasta, alphabet shapes 500 g = £6.48; lasagnesheets 200 g = £2.76; macaroni 500 g = £6.48; noodles, flat500 g = £6.48; shells 500 g = £6.48; spaghetti, short-cut 500 g= £6.48; spirals 500 g = £6.48. Rice, imitation 500 g = £6.48.Tricolour pasta, alphabet shapes 500 g = £6.48; shells 500 g= £6.48; spirals 500 g = £6.48Pizza basesJuvela c (Juvela)Low-protein. Pizza base 2 180 g = £7.55Ultra PKU c (Ultrapharm)Low-protein. Pizza base 5 80 g = £2.45Savoury meals and mixesLoprofin c (SHS)Low-protein. Snack pot, curry 47 g = £3.94Promin c (Firstplay Dietary)Low-protein. Burger mix 2 62 g = £5.77; lamb & mint 2 62 g = £5.77. Couscous 500 g = £6.48. Pasta elbows in cheeseand broccoli sauce 4 66 g = £7.54. Pasta meal 500 g =£6.48. Pasta shells in tomato, pepper, and herb sauce 4 72 g= £7.54. Pasta spirals in Moroccan sauce 4 72 g = £7.54.Sausage mix, apple & sage 4 30 g = £6.49; original 4 30 g= £6.49; tomato & basil 4 30 g = £6.49SpreadsTaranis c (Firstplay Dietary)Low-protein. Spread, hazelnut 230 g = £6.80A2.7 Nutritional supplementsfor metabolic diseasesGlutaric aciduria (type 1)GA1 Anamix c Infant (SHS)Powder, protein equivalent (essential and non-essentialamino acids except lysine, and low tryptophan) 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 provenglutaric aciduria (type 1) in children from birth to 3 yearsGA Gel c (Vitaflo)Gel, protein equivalent (essential and non-essential aminoacids except lysine, and low tryptophan) 8.4 g, carbohydrate8.6 g. fat trace, energy 286 kJ (68 kcal)/20 g, with vitamins,minerals, and trace elements. Unflavoured (flavouring: seeFlavourPac c , p. 777), net price 30 20-g sachets = £149.04Nutritional supplement for dietary management of type 1 glutaricaciduria in children 1–10 years1 XLYS, Low TRY, Maxamaid (SHS)Powder, protein equivalent (essential and non-essentialamino acids except lysine, and low tryptophan) 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 dietary management of type 1 glutaricaciduriaXLYS, TRY, Glutaridon (SHS)Powder, protein equivalent (essential and non-essentialamino acids except lysine and tryptophan) 79 g, carbohydrate4 g, energy 1411 kJ (332 kcal)/100 g, Lactose-free.Unflavoured, (flavouring: see Modjul c Flavour System,p. 777), net price 2 500 g = £312.84Nutritional supplement for type 1 glutaric aciduria in children andadults; requires additional source of vitamins, minerals and traceelementsGlucogen storage diseaseCorn flour and corn starchFor hypoglycaemia associated with glycogen-storage diseaseGlucose(Dextrose monohydrate)Net price 500 g = £1.18.For glycogen storage disease and sucrose/isomaltose intoleranceGlycosade c (Vitaflo)Powder, protein 200 mg, carbohydrate (maize starch) 47.6 g,fat 100 mg, fibre less than 600 mg, energy 803 kJ (192 kcal)/60 g, net price 30 60-g sachets = £92.03A nutritional supplement for use in the dietary management ofglycogen storage disease and other metabolic conditions where aconstant supply of glucose is essential. Not suitable for use inchildren under 2 yearsHomocystinuria orhypermethionimaemiaHCU Anamix c Infant (SHS)Powder, protein equivalent (essential and non-essentialamino acids except methionine) 13.1 g, carbohydrate 49.5 g,fat 23 g, fibre 5.3 g, energy 1915 kJ (457 kcal)/100 g, withvitamins, minerals, and trace elements; standard dilution(15%) provides protein equivalent 2 g, carbohydrate 7.4 g, fat3,5 g, fibre 800 mg, energy 287 kJ (69 kcal)/100 mL. Unflavoured,net price 400 g = £32.70 (5-g measuring scoopprovided)Nutritional supplement for the dietary management of provenvitamin B 6 non-responsive homocystinuria or hypermethioninaemiain children from birth to 3 years1. Maxamaid products are generally intended for use inchildren 1–8 yearsAppendix 2: Borderline substances