BNF for Children 2011-2012

782 A2.7 Nutritional supplements for metabolic diseases BNFC 2011–2012Appendix 2: Borderline substancesHCU cooler c (Vitaflo)Liquid, protein (essential and non-essential amino acidsexcept methionine) 15 g, carbohydrate 7 g, fat 500 mg,energy 386 kJ (92 kcal)/130 mL, with vitamins, minerals andtrace elements. Orange or red flavour, net price 30 130-mLpouch = £277.20A methionine-free protein substitute for use as a nutritionalsupplement in children over 3 years with homocystinuriaHCU Express c (Vitaflo)Powder, protein (essential and non-essential amino acidsexcept methionine) 15 g, carbohydrate 3.8 g, fat 30 mg,energy 315 kJ (75.3 kcal)/25 g with vitamins, minerals andtrace elements. Unflavoured, (flavouring: see Flavour Pac c ,p. 777), net price 30 25- g sachets = £277.88A methionine-free protein substitute for use as a nutritionalsupplement in children over 8 years with homocystinuriaHCU gel c (Vitaflo)Powder, protein (essential and non-essential amino acidsexcept methionine) 8.4 g, carbohydrate 8.6 g, fat 30 mg,energy 286 kJ (68 kcal)/20 g with vitamins, minerals andtrace elements. Unflavoured, (flavouring: see Flavour Pac c ,p. 777), net price 30 20- g sachets = £151.93A methionine-free protein substitute for use as a nutritionalsupplement for the dietary management of children 1–10 yearswith homocystinuriaHCU LV c (SHS)Powder, protein (essential and non-essential amino acidsexcept methionine) 20 g, carbohydrate 2.5 g, fat 190 mg,energy 390 kJ (92 kcal)/27.8-g sachet, with vitamins,minerals, and trace elements. Unflavoured (flavouring: seeModjul c Flavour System, p. 777), or tropical flavour (formulationvaries slightly), net price 30 27.8-g sachets =£434.40Nutritional supplement for the dietary management of hypermethioninaemiaor vitamin B 6 non-responsive homocystinuria inchildren over 8 yearsXMET Homidon (SHS)Powder, protein equivalent (essential and non-essentialamino acids, except methionine) 77 g, carbohydrate 4.5 g, fatnil, energy 1386 kJ (326 kcal)/100 g. Unflavoured,(flavouring: see Modjul c Flavour System, p. 777), net price500 g = £156.42Nutritional supplement for the dietary management of hypermethioninaemiaor homocystinuria in children1 XMET Maxamaid (SHS)Powder, protein equivalent (essential and non-essentialamino acids except methionine) 25 g, carbohydrate 51 g, fatless than 500 mg, energy 1311 kJ (309 kcal)/100 g, withvitamins, minerals, and trace elements. Unflavoured(flavouring: see Modjul c Flavour System, p. 777), net price500 g = £82.57Nutritional supplement for the dietary management of hypermethioninaemiaor homocystinuria2 XMET Maxamum c (SHS)Powder, protein equivalent (essential and non-essentialamino acids except methionine) 39 g, carbohydrate 34 g, fatless than 500 mg, energy 1260 kJ (297 kcal)/100 g, withvitamins, minerals, and trace elements. Unflavoured,(flavouring: see Modjul c Flavour System, p. 777), net price500 g = £132.36Nutritional supplement for the dietary management of hypermethioninaemior homocystinuria1. Maxamaid products are generally intended for use inchildren 1–8 years2. Maxamum products are generally intended for use inchildren over 8 yearsHyperlysinaemiaHYPER LYS Anamix c Infant (SHS)Powder, protein equivalent (essential and non-essentialamino acids except lysine) 13.1 g, carbohydrate 49.5 g, fat23 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, 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-g measuring scoop provided)Nutritional supplement for the dietary management of provenhyperlysinaemia in children from birth to 3 years1 XLYS Maxamaid (SHS)Powder, protein equivalent (essential and non-essentialamino acids except lysine) 25 g, carbohydrate 51 g, fat lessthan 500 mg, energy 1311 kJ (309 kcal)/100 g with vitamins,minerals, and trace elements. Unflavoured, (flavouring: seeModjul c Flavour System, p. 777), net price 500 g = £82.57Nutritional supplement for the dietary management of hyperlysinaemiaIsovaleric acidaemiaIVA Anamix c Infant (SHS)Powder, protein equivalent (essential and non-essentialamino acids except leucine) 13.1 g, carbohydrate 49.5 g, fat23 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, 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-g measuring scoop provided)Nutritional supplement for the dietary management of provenisovaleric acidaemia or other proven disorders of leucine metabolismin children from birth to 3 yearsXLEU Faladon (SHS)Powder, protein equivalent (essential and non-essentialamino acids except leucine) 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 200 g =£62.55Nutritional supplement for the dietary management of isovalericacidaemia in children1 XLEU Maxamaid (SHS)Powder, protein equivalent (essential and non-essentialamino acids except leucine) 25 g, carbohydrate 51 g, fat lessthan 500 mg, energy 1311 kJ (309 kcal)/100 g with vitamins,minerals, and trace elements. Unflavoured, (flavouring: seeModjul c Flavour System, p. 777), net price 500 g = £82.57Nutritional supplement for the dietary management of isovalericacidaemiaMaple syrup urine diseaseIsoleucine Amino Acid Supplement (Vitaflo)Powder, isoleucine 50 mg, carbohydrate 4 g, fat nil, energy64 kJ (15 kcal)/4 g, net price 30 4-g sachets = £44.48Nutritional supplement for use in the dietary management ofmaple syrup urine disease and other inborn errors of amino acidmetabolism in children over 1 year and adultsMSUD Aid III c (SHS)Powder, protein equivalent (essential and non-essentialamino acids except isoleucine, leucine, and valine) 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 dietary management of maplesyrup urine disease and related conditions in children and adultswhere it is necessary to limit the intake of branched chain aminoacids