Description PKU sphere is a powdered, low-phenylalanine, glycomacropeptide (GMP) isolate casein protein substitute with essential and non-essential amino acids, carbohydrates, fats, vitamins, minerals and docosahexaenoic acid (DHA). With sugars and sweeteners. PKU sphere is indicated for the dietary treatment of phenylketonuria (PKU) in children from 6 years of age and in adults. Suitable for ages 4 and up.
Presence of allergens: fish (tuna oil), soy (soy lecithin) and milk (milk protein).
Nutritional characteristics
| Average values |
per 100 g |
per 35 g |
| Energy |
1.432 kJ
338 kcal |
508 kJ
120 kcal |
Fats
of which saturates
of which DHA |
4,7 g
1 g
314 mg |
1,6 g
0,35 g
110 mg |
Carbohydrates
of which sugars |
18 g
6,3 g |
6,3 g
2,2 g |
| Proteins |
56 g |
20 g |
| L-fenilalanina |
104 mg |
36 mg |
| Sale |
2 g |
0,71 g |
| Vitamin A (RE) |
740 mcg |
259 mcg |
| Vitamin E (alpha-TE) |
15 mg |
5,3 mg |
| Vitamin d |
14 mcg |
5 mcg |
| Vitamin k |
67 mcg |
23 mcg |
| Vitamin C |
74 mg |
26 mg |
| Thiamine |
1,7 mg |
0,60 mg |
| Riboflavin |
1,7 mg |
0,60 mg |
| Vitamin B6 |
1,7 mg |
0,60 mg |
| Niacin |
9.1 mg (28 NE) |
3.2 mg (9.8 NE) |
| Folic acid |
290 mcg |
102 mcg |
| Vitamin B12 |
4,6 mcg |
1,6 mcg |
| Pantothenic acid |
5,7 mg |
2,0 mg |
| Biotin |
36 mg |
13 mg |
| Sodium |
810 mg
35 mmol |
283 mg
12 mmol |
| Chlorine |
20 mg
0,56 mmol |
7 mg
0,2 mmol |
| Potassium |
670 mg
17 mmol |
234 mg
5,9 mmol |
| Calcium |
1.140 mg
29 mmol |
399 mg
10 mmol |
| Phosphorus |
1.180 mg
38 mmol |
413 mg
13 mmol |
| Magnesium |
330 mg
13 mmol |
115 mg
4,6 mmol |
| Iron |
21 mg |
7,4 mg |
| Zinc |
21 mg |
7,4 mg |
| Copper |
1,7 mg |
0,6 mg |
| Iodine |
240 mcg |
84 mcg |
| Selenium |
86 mcg |
30 mcg |
| Manganese |
1,1 mg |
0,4 mg |
| Chromium |
34 mcg |
12 mcg |
| Molybdenum |
57 mcg |
20 mcg |
| Hill |
570 mg |
200 mg |
| L-alanine |
2,36 g |
0,83 g |
| L-arginine |
2,73 g |
0,96 g |
| L-aspartic acid |
3,73 g |
1,31 g |
| L-cystine |
0,69 g |
0,24 g |
| L-glutamine |
7,72 g |
2,70 g |
| Glycine |
2,02 g |
0,71 g |
| L-histidine |
2,01 g |
0,70 g |
| L-isoleucina |
4,06 g |
1,42 g |
| L-leucine |
8,62 g |
3,02 g |
| L-lisina |
2,72 g |
0,95 g |
| L-metionina |
0,80 g |
0,28 g |
| L-fenilalanina |
0,10 g |
0,036 g |
| L-prolina |
4,58 g |
1,60 g |
| L-serine |
2,88 g |
1,01 g |
| L-Threonine |
6,55 g |
2,29 g |
| L-tryptophan |
1,15 g |
0,40 g |
| L-tyrosine |
6,43 g |
2,25 g |
| L-valine |
3,27 g |
1,14 g |
How to use 1) Add 120 ml of cold water to a glass.
2) Pour the contents of the PKU Sphere 20 sachet into the glass.
3) Stir well for 10 seconds and consume immediately.
Warnings PKU sphere is a food for special medical purposes and should be used under strict medical supervision.
Preservation Store in a cool, dry place. The contents of the pouch should be consumed immediately after opening.
Valid for packaging intact: 9 months.
Post-opening validity: 24 hours.
Format Pack of 30 sachets of 35 g.
Net weight: 1,050 g.
Available in 3 flavors: chocolate, red fruits and vanilla.
Cod. 10BO23C00A90, 10BO21C00A31, 12405861
Bibliography 1. van Calcar SC, MacLeod EL, Gleason ST, Etzel MR, Clayton MK, Wolff JA, et al. Improved nutritional management of phenylketonuria by using a diet containing glycomacropeptide compared with amino acids. The same clean nut. 2009; 89(4):1068-77.
2. Data on file. Daly A, Chahal S, MacDonald A. The use of glycomacropeptide as an alternative protein substitute in PKU children. 2014.
3. Solverson P, Murali SG, Litscher SJ, Blank RD, Ney DM. Low bone strength is a manifestation of phenylketonuria in mice and is attenuated by a glycomacropeptide diet. PLoS One. 2012; The 45-day-a-month-time world no.100
4. Sawin EA, De Wolfe TJ, Aktas B, Stroup BM, Murali SG, Steele JL, et al. Glycomacropeptide is a prebiotic that reduces Desulfovibrio bacteria, increases cecal short-chain fatty acids, and is anti-inflammatory in mice. Am J Physiology Gastrointest Liver Physiol. 2015; 309(7):590-601.
5. Improved nutritional management of phenylketonuria by using a diet containing glycomacropeptide compared with amino acidsvan Calcar SC1, MacLeod EL, Gleason ST, Etzel MR, Clayton MK, Wolff JA, Ney DM. The same clean nut. 2009 Apr; 89(4):1068-77.
6. Phenylketonuria: nutritional advances and challenges Marcello Giovannini, Elvira Verduci, Elisabetta Salvatici, Sabrina Paci, and Enrica Riva-Nutr Metab (Lond). 2012; 9: 7
7. Low bone strength is a manifestation of phenylketonuria in mice and is attenuated by a glycomacropeptide diet. Solverson P1, Murali SG, Litscher SJ, Blank RD, Ney DM.- PLoS One. 2012; The 45-day-a-month-time world no.100 doi: 10.1371/journal.pone.0045165. Epub 2012 Sep 18
8. Glycomacropeptide is a prebiotic that reduces Desulfovibrio bacteria, increases cecal short-chain fatty acids, and is antiinflammatory in mice. Sawin EA1, De Wolfe TJ2, Aktas B2, Stroup BM1, Fife SG1, Steele JL2, Ne DM3. Am J Physiol Gastrointest Liver Physiol. 2015 Oct 1; 309(7):G590-601.
9. Glycomacropeptide: long-term use and impact on blood phenylalanine, growth and nutritional status in children with PKU. A. Daly, S. Evans,S. Chahal, S. Santra, A. Pinto, R. Jackson,, C. Gingell, J. Rocha, F. J. Van Spronsen,and A. MacDonald. 6. WHO/FAO/UNU(2007). Protein and Amino Acid requirments in Human nutrition: Report of a Joint WHO/FAO/UNU Expert Consultation. World health organization technical report series., WHO/FAO/UNU: 1-265
10. Rober, M. et al. 2013. Micronutrient status in phenylketonuria. Mole Genet Metab. 110 Suppl: S6-17
11. Fekete, K. and T. Decsi, Long-chain polyunsaturated fatty acids in inborn errors of metabolism. Nutrients, 2010. 2(9): p. 965-74.
12. Vilaseca, A. et al. Long-chain polyunsaturated fatty acid concentration in patients with inborn errors of metabolism. Nutar Hosp, 2011. 26(1): p. 128-136.
