MSUD HCU Tyrosinaemia MMA/PA GA el (for PKU el see paes 7-10) Description A food for special medical purposes. Unflavoured powdered protein substitutes containin essential and non-essential amino acids (but excludin the offendin amino acid(s)), carbohydrate, suar, vitamins, minerals and trace elements. Product Indication Free From Ae Rane Maple Syrup Urine Disease Leucine, Isoleucine and Valine Homocystinuria Methionine Tyrosinaemia Phenylalanine and Tyrosine 6 months to 10 years Methylmalonic Acidaemia / Propionic Acidaemia Glutaric Aciduria type 1 Methionine, Threonine and Valine. Low Isoleucine. Lysine. Low Tryptophan. Dosae and Administration To be determined by the clinician or dietitian and is dependent on the ae, body weiht and medical condition of the patient. = 10 PE 1. The daily protein requirement is iven as el. 2. Additional protein is provided from exchanes of natural protein, to provide restricted amino acids in controlled amounts, which is essential for rowth and development. 3. Foods hih in carbohydrate and fat, but low in protein, should be provided to supply the remainder of the enery. 24
MSUD HCU Tyrosinaemia MMA/PA GA el Preparation Guidelines Gel can be either taken as a el or as a low volume drink. For el: For low volume drink: 1. Empty the contents of the sachet, plus ½ - 1 FlavourPac sachet if desired, into the beaker. 2. Add approximately 30ml of cold water. Secure the lid and shake well for 10 seconds. 1. Empty the contents of the sachet, plus ½ - 1 FlavourPac sachet if desired, into the beaker. 2. Add approximately 80ml (half way up the beaker) of cold water. Secure the lid and shake well for 10 seconds. 100 ml 80 60 40 20 100 ml 80 60 40 20 3. Leave to stand for 2 minutes, allowin a smooth el to form. Consume with a spoon. 3. Drink immediately. Separate FlavourPac sachets available. Suitable from 1 year of ae. Available in blackcurrant, lemon, orane, raspberry and tropical flavours. Ideally the product should be freshly prepared and used immediately. Any unused portions should be refrierated and used within 24 hours. Reshake before use. Water or permitted drinks should be taken after the el.
MSUD HCU Tyrosinaemia MMA/PA GA el Important NOTICE Must only be iven to patients under strict medical supervision. is for the dietary manaement of maple syrup urine disease. is for the dietary manaement of homocystinuria. is for the dietary manaement of tyrosinaemia. is for the dietary manaement of methylmalonic acidaemia/ propionic acidaemia. is for the dietary manaement of lutaric aciduria type 1. Suitable from 6 months to 10 years of ae. Diet must be supplemented with natural protein, water and other nutrients in prescribed quantities to supply additional nutritional requirements. Not for use as a sole source of nutrition. For enteral use only. Nutritional Information per per 24 100 sachet Enery kj 1413 339 kcal 338 81 Protein equivalent 41.7 10 Carbohydrate 42.9 10.3 of which suars 27.1 6.5 Fat 0.05 0.02 of which saturates 0 0 Fibre 0 0 Vitamins Vitamin A µ RE 600 144 Vitamin D μ 14.6 3.5 Vitamin E m α TE 9.0 2.2 Vitamin C m 63 15 Vitamin K μ 41 9.8 Thiamin m 1.0 0.24 Riboflavin m 1.2 0.29 Niacin m 14 3.4 Niacin equivalent: MSUD el m 38.2 9.2 HCU el m 31.8 7.7 TYR el m 32.5 7.9 MMA/PA el m 32.5 7.9 GA el m 18.5 4.4 Vitamin B6 m 1.1 0.26 Folic acid μ 208 50 Vitamin B12 μ 2.0 0.48 Biotin μ 25 6.0 per per 24 100 sachet Pantothenic acid m 5.0 1.2 Choline m 279 67 Myo-Inositol m 0 0 Minerals Sodium m 379 91 mmol 16.3 3.9 Potassium m 938 225 mmol 23.5 5.6 Chloride m 583 140 mmol 16.3 3.9 Calcium m 1083 260 mmol 27.1 6.5 Phosphorus m 825 198 mmol 26.4 6.3 Manesium m 167 40 mmol 6.8 1.6 Trace Elements Iron m 14 3.4 Copper µ 800 190 Zinc m 11 2.6 Mananese m 1.7 0.41 Iodine μ 138 33.1 Molybdenum μ 50 12 Selenium μ 35 8.4 Chromium μ 71 17
MSUD HCU Tyrosinaemia MMA/PA GA el 2.05 3.41 4.56 1.37 4.02 3.00 2.14 3.21 5.57 3.59 2.48 3.13 2.40 2.44 1.07 2. 3.53 3.12 3.83 5. 1.43 3.79 3.12 2.09 3.48 5.57 3.97 0.89 3.21 2.45 2.67 1.11 3.79 4.15 4.30 7. 1.77 5.06 1.95 0.15 6.58 4.58 2.02 2.93 1.11 Amino Acids per 100 L-Alanine L-Arinine L-Aspartic acid L-Cystine L-Glutamine Glycine L-Histidine L-Isoleucine L-Leucine L-Lysine L-Methionine L-Phenylalanine L-Proline L-Serine L-Threonine L-Tryptophan L-Tyrosine L-Valine L-Carnitine m Taurine m 5.11 4.85 6.13 1.45 5.51 4.06 1.74 3.65 1.05 2.73 4.29 2.90 2.61 1.45 2.90 4.07 3. 2.64 1.53 3.01 2.54 1.78 3.66 6.36 1.12 3.05 3.56 2.80 3.05 0.27 3.56 4.07 MSUD el TYR el HCU el MMA/PA el GA el
MSUD HCU Tyrosinaemia MMA/PA GA el Osmolality 24 + 30ml = 3000mOsm/k 24 + 80ml = 1540mOsm/k Storae Store in a cool, dry place. Ideally sachets should be used immediately after openin. Any unused powder in an open sachet should be kept in an airtiht container or sealed with a clipper and used within 24 hours. Pack size/weiht 30 x 24 sachets = 720 Shelf life 18 months. MSUD HCU TYR MMA/PA GA