MSUD gel 24g sachets(Vitaflo International Ltd)
30 sachet (ACBS).NHS indicative price = £ 227. 50
MSUD Lophlex®LQ 20
▶Nutritional supplement for the dietary management of maple
syrup urine disease in children over 3 years.
LIQUID, protein equivalent (essential and non-essential amino
acids except isoleucine, leucine, and valine) 20 g, carbohydrate
8.8 g, fat less than 500 mg, energy 509 kJ (120kcal)/125 mL, with
vitamins, minerals, and trace elements.
MSUD Lophlex LQ 20 liquid(Nutricia Ltd)
125 ml (ACBS).NHS indicative price = £ 16. 82
MSUD Maxamum®
▶Nutritional supplement for the dietary management of maple
syrup urine disease.
POWDER, protein equivalent (essential and non-essential amino
acids except isoleucine, leucine, and valine) 39 g, carbohydrate
34 g, fat less than 500 mg, energy 1260 kJ (297 kcal)/100g, with
vitamins, minerals, and trace elements. Maxamum products are
generally intended for use in children over 8 years.
MSUD Maxamum powder orange(Nutricia Ltd)
500 gram (ACBS).NHS indicative price = £ 165. 09
MSUD Maxamum powder unflavoured(Nutricia Ltd)
500 gram (ACBS).NHS indicative price = £ 165. 09Methylmalonic or propionic acidaemia
MMA/PA Anamix®Infant
▶Nutritional supplement for the dietary management of proven
methylmalonic acidaemia or propionic acidaemia in children
from birth to 3 years.
POWDER, protein equivalent (essential and non-essential amino
acids except methionine, threonine, and valine, and low
isoleucine) 13.1 g, carbohydrate 49.5 g, fat 23 g,fibre 5.3 g,
energy 1915 kJ (457kcal)/100g, 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)/100mL.
MMA / PA Anamix Infant powder(Nutricia Ltd)
400 gram (ACBS).NHS indicative price = £ 40. 79
XMTVI Asadon®
▶Nutritional supplement for the dietary management of
methylmalonic acidaemia or propionic acidaemia in children
and adults.
POWDER, protein equivalent (essential and non-essential amino
acids except methionine, threonine, and valine, and low
isoleucine) 77 g, carbohydrate 4.5 g, fat nil, energy 1386 kJ
(326 kcal)/100g.
XMTVI Asadon powder(Nutricia Ltd)
200 gram (ACBS).NHS indicative price = £ 78. 04
MMA/PA Maxamum®
▶Nutritional supplement for the dietary management of
methylmalonic acidaemia or propionic acidaemia.
POWDER, protein equivalent (essential and non-essential amino
acids except methionine, threonine, and valine, and low
isoleucine) 39 g, carbohydrate 34 g, fat less than 500 mg, energy
1260 kJ (297kcal)/100g, with vitamins, minerals, and trace
elements. Maxamum products are generally intended for use in
children over 8 years.
MMA/PA Maxamum powder(Nutricia Ltd)
500 gram (ACBS).NHS indicative price = £ 165. 09Other inborn errors of metabolism
Cystine500®
▶Nutritional supplement for the dietary management of inborn
errors of amino acid metabolism in adults and children from
birth.
POWDER, cystine 500 mg, carbohydrate 3.3 g, fat nil, energy
63 kJ (15 kcal)/4 g
Cystine500 oral powder sachets(Vitaflo International Ltd)
30 sachet (ACBS).NHS indicative price = £ 57. 14DocOmega®
▶Nutritional supplement for the dietary management of inborn
errors of metabolism for adults and children from birth.
POWDER, protein (cows’milk, soya) 100 mg, carbohydrate 3.2 g,
fat 500 mg (of which docosahexaenoic acid 200 mg),fibre nil,
energy 74 kJ (18 kcal)/4 g, with minerals
DocOmega oral powder 4g sachets(Vitaflo International Ltd)
30 sachet (ACBS).NHS indicative price = £ 41. 35
EAA®Supplement
▶Nutritional supplement for the dietary management of
disorders of protein metabolism including urea cycle disorders
in children over 3 years.
POWDER, protein equivalent (essential amino acids) 5g,
carbohydrate 4 g, fat nil, energy 151 kJ (36 kcal)/12.5g, with
vitamins, minerals, and trace elements.
EAA Supplement oral powder 12.5g sachets(Vitaflo International Ltd)
50 sachet (ACBS).NHS indicative price = £ 215. 60
Isoleucine50®
▶Nutritional supplement for use in the dietary management of
inborn errors of amino acid metabolism in adults and children
from birth.
POWDER, isoleucine 50 mg, carbohydrate 3.8 g, fat nil, energy
63 kJ (15kcal)/4 g
Isoleucine50 oral powder 4g sachets(Vitaflo International Ltd)
30 sachet (ACBS).NHS indicative price = £ 57. 14
KeyOmega®
▶Nutritional supplement for the dietary management of inborn
errors of metabolism.
POWDER, protein (cows’milk, soya) 170 mg, carbohydrate 2.8 g,
fat 800 mg (of which arachidonic acid 200 mg, docosahexaenoic
acid 100 mg), energy 80 kJ (19kcal)/4 g.
KeyOmega oral powder 4g sachets(Vitaflo International Ltd)
30 sachet (ACBS).NHS indicative price = £ 42. 29
Leucine100®
▶Nutritional supplement for the dietary management of inborn
errors of amino acid metabolism in adults and children from
birth.
POWDER, leucine 100 mg, carbohydrate 3.7 g, fat nil, energy
63 kJ (15kcal)/4 g
Leucine100 oral powder sachets(Vitaflo International Ltd)
30 sachet (ACBS).NHS indicative price = £ 57. 14
Low protein drink
▶Nutritional supplement for the dietary management of inborn
errors of amino acid metabolism in adults and children over
1 year.
POWDER, protein (cows’milk) 4.5 g (phenylalanine 100 mg),
carbohydrate 59.5 g, fat 29.9g,fibre nil, energy 2194 kJ
(528 kcal)/100g, with vitamins, minerals, and trace elements.
Contains lactose.
TermedMilupa®lp-drinkby manufacturer.
Milupa LP drink(Nutricia Ltd)
400 gram (ACBS).NHS indicative price = £ 9. 68
Phenylalanine50®
▶Nutritional supplement for use in the dietary management of
inborn errors of metabolism in adults and children from birth.
POWDER, phenylalanine 50 mg, carbohydrate 3.8 g, fat nil,
energy 63 kJ (15 kcal)/4 g
Phenylalanine50 oral powder sachets(Vitaflo International Ltd)
30 sachet (ACBS).NHS indicative price = £ 55. 48
ProZero®
▶A protein-free nutritional supplement for the dietary
management of inborn errors of metabolism in children over
6 months and adults.
LIQUID, carbohydrate 8.1 g (of which sugars 3.5 g), fat 3.8 g,
energy 278 kJ (66 kcal)/100mL. Contains lactose.
ProZero liquid(Vitaflo International Ltd)
250 ml (ACBS).NHS indicative price = £ 1. 52 | 1000 ml (ACBS)
.NHS indicative price = £ 6. 091048 Nutritional supplements for metabolic diseases BNFC 2018 – 2019
Borderline substances|Appendix 2A2