BNF for Children 2011-2012

BNFC 2011–2012 A2.7 Nutritional supplements for metabolic diseases 783MSUD Anamix c Infant (SHS)Powder, protein equivalent (essential and non-essentialamino acids except isoleucine, leucine, and valine) 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 provenmaple syrup urine disease in children from birth to 3 yearsMSUD Anamix c Junior (SHS)Powder, protein equivalent (essential and non-essentialamino acids except isoleucine, leucine, and valine) 8.4 g,carbohydrate 11 g, fat 3.9 g, energy 474 kJ (113 kcal)/29-gsachet, with vitamins, minerals, and trace elements. Unflavoured,(flavouring: see Modjul c Flavour System, p. 777),net price 30 29-g sachets = £166.73Nutritional supplement for the dietary management of maplesyrup urine disease in children 1–10 yearsMSUD Anamix c Junior LQ (SHS)Liquid, protein equivalent (essential and non-essential aminoacids except isoleucine, leucine, and valine) 10 g, carbohydrate8.8 g, fat 4.8 g, fibre 310 mg, energy 497 kJ (118 kcal)/125 mL, with vitamins, minerals, and trace elements. Lactose-free.Orange flavour, net price 125-mL carton = £7.58Nutritional supplement for the dietary management of maplesyrup urine disease in children 1–10 yearsMSUD express c (Vitaflo)Powder, protein equivalent (essential and non-essentialamino acids except leucine, isoleucine, and valine) 15 g,carbohydrate 3.8 g, fat less than 100 mg, energy 315 kJ(75 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 maplesyrup urine disease in children over 8 years and adultsMSUD cooler c (Vitaflo)Liquid, protein equivalent (essential and non-essential aminoacids except leucine, isoleucine, and valine) 15 g, carbohydrate7 g, fat 500 mg, energy 386 kJ (92 kcal)/130-mL pouch,with vitamins, minerals, and trace elements. Orange or redflavour, net price 30 130-mL = £277.20Nutritional supplement for the dietary management of maplesyrup urine disease in children over 3 years and adultsMSUD Gel c (Vitaflo)Powder, protein equivalent (essential and non-essentialamino acids except leucine, isoleucine, and valine) 8.4 g,carbohydrate 8.6 g, fat less than 100 mg, energy 286 kJ(68 kcal)/20 g, with vitamins, minerals, and trace elements.Unflavoured, (flavouring: see Flavour Pac c , p. 777), netprice 30 20-g sachets = £151.93Nutritional supplement for the dietary management of maplesyrup urine disease in children 1–10 years1 MSUD Maxamaid c (SHS)Powder, protein equivalent (essential and non-essentialamino acids except isoleucine, leucine, and valine) 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 maplesyrup urine disease2 MSUD Maxamum c (SHS)Powder, protein equivalent (essential and non-essentialamino acids except isoleucine, leucine, and valine) 39 g,carbohydrate 34 g, fat less than 500 mg, energy 1260 kJ(297 kcal)/100 g, with vitamins, minerals, and trace elements.Orange flavour or unflavoured, (flavouring: seeModjul c Flavour System, p. 777), net price 500 g = £132.36Nutritional supplement for the dietary management of maplesyrup urine diseaseValine Amino Acid Supplement (Vitaflo)Powder, valine 50 mg, carbohydrate 4 g, fat nil, energy 64 kJ(15 kcal)/4 g, net price 30 4-g sachets = £44.48Nutritional supplement for the dietary management of maplesyrup urine disease and other inborn errors of amino acidmetabolism in children over 1 yearMethylmalonic propionic acidaemiaMMA/PA Anamix c Infant (SHS)Powder, protein equivalent (essential and non-essentialamino acids except methionine, threonine, and valine, andlow isoleucine) 13.1 g, carbohydrate 49.5 g, fat 23 g, fibre5.3 g, energy 1915 kJ (457 kcal)/100 g, with vitamins,minerals, and trace elements; standard dilution (15%) providesprotein 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 provenmethylmalonic acidaemia or propionic acidaemia in children frombirth to 3 yearsXMTVI Asadon (SHS)Powder, protein equivalent (essential and non-essentialamino acids except methionine, threonine, and valine, andlow isoleucine) 77 g, carbohydrate 4.5 g, fat nil, energy1386 kJ (326 kcal)/100 g. Unflavoured, (flavouring: seeModjul c Flavour System, p. 777), net price 200 g = £62.55Nutritional supplement for the dietary management of methylmalonicacidaemia or propionic acidaemia in children and adults1 XMTVI Maxamaid (SHS)Powder, protein equivalent (essential and non-essentialamino acids except methionine, threonine, and valine, andlow isoleucine) 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 cFlavour System, p. 777), net price 500 g = £82.57Nutritional supplement for the dietary management of methylmalonicacidaemia or propionic acidaemia2 XMTVI Maxamum (SHS)Powder, protein equivalent (essential and non-essentialamino acids except methionine, threonine, and valine, andlow isoleucine) 39 g, carbohydrate 34 g, fat less than 500 mg,energy 1260 kJ (297 kcal)/100 g, with vitamins, minerals,and trace elements. Unflavoured, (flavouring: see Modjul cFlavour System, p. 777), net price 500 g = £132.36Nutritional supplement for the dietary management of methylmalonicacidaemia or propionic acidaemiaOther inborn errors of metabolismAppendix 2: Borderline substances1. Maxamaid products are generally intended for use inchildren 1–8 years2. Maxamum products are generally intended for use inchildren over 8 yearsCystine Amino Acid Supplement (Vitaflo)Powder, cystine 500 mg, carbohydrate 3.4 g, fat nil, energy63 kJ (15 kcal)/4 g, net price 30 4-g sachets = £44.48Nutritional supplement for the dietary management of inbornerrors of amino acid metabolism in children over 1 year