MSUD HCU Tyrosinaemia MMA/PA IVA (for PKU cooler see pages 11-13)

Transcription

1 (for PKU see pages 11-13) + Description A food for special medical purposes. Cooler is a ready-to-drink protein substitute containing essential and non-essential amino acids (but excluding the offending amino acids(s)), carbohydrate, vitamins, minerals, trace elements and the Omega-3 long chain polyunsaturated fatty acids (LCPs); Docosahexaenoic acid (DHA) and Eicosapentaenoic acid (EPA). Contains sugar and sweeteners. Product Indication Free From Flavour options Maple Syrup Urine Disease Leucine, Isoleucine and Valine and Orange Homocystinuria Methionine and Orange Tyrosinaemia Phenylalanine and Tyrosine and Orange Methylmalonic Acidaemia / Propionic Acidaemia Methionine, Threonine and Valine. Low Isoleucine. Isovaleric Acidaemia and other disorders requiring Leucine-free protein substitutes Leucine

2 MSUD HCU Tyrosinaemia MMA/PA IVA Age Range Suitable from 3 years of age. 1. The daily protein requirement is given as. 2. Additional protein is provided from exchanges of natural protein, to provide restricted amino acids in controlled amounts, which is essential for growth and development. 3. Foods high in carbohydrate and fat, but low in protein, should be provided to supply the remainder of the energy. Dosage and Administration To be determined by the clinician or dietitian, and is dependent on the age, body weight and medical condition of the patient. = 15g PE = 10g PE = 20g PE Preparation Guidelines 1. Shake well 2. Open Water or mitted drinks should be taken after the. 3. Drink

3 Important NOTICE Must only be given to patients under strict medical suvision. is for the dietary management of maple syrup urine disease. is for the dietary management of homocystinuria. is for the dietary management of tyrosinaemia. is for the dietary management of Methylmalonic Acidaemia and Propionic Acidaemia. Nutritional Information * Figures in brackets represent MMA/PA and IVA s Nutritional Information is for the dietary management of Isovaleric acidaemia and other disorders requiring leucine free protein substitute. Suitable from 3 years of age. 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. 100ml Cooler10 Cooler15 Cooler20 Energy kj (331)* (430)* kcal (79)* (103)* Protein equivalent g Carbohydrate g of which sugars g Fat g (1.3)* (1.7)* of which saturates g (0.25)* (0.33)* of which DHA mg of which EPA mg Fibre g

4 100ml Cooler10 Cooler15 Cooler20 Vitamins Vitamin A µg RE IU Vitamin D μg IU Vitamin E mg α TE IU Vitamin C mg Vitamin K μg Thiamin mg Riboflavin mg Niacin mg Niacin equivalent MSUD mg HCU mg TYR mg MMA/PA mg IVA mg Vitamin B6 mg Folic acid μg Vitamin B12 μg Biotin μg Pantothenic acid mg Choline mg Myo-Inositol mg Minerals Sodium mg mmol Potassium mg mmol Chloride mg mmol Calcium mg mmol Phosphorus mg mmol Magnesium mg mmol Trace Elements Iron mg Cop µg Zinc mg Manganese mg Iodine μg Molybdenum μg Selenium μg Chromium μg

5 Amino Acids 100ml MSUD L-Alanine g L-Arginine g L-Aspartic acid g L-Cystine g L-Glutamine g Glycine g L-Histidine g L-Isoleucine g L-Leucine g L-Lysine g L-Methionine g L-Phenylalanine g L-Proline g L-Serine g L-Threonine g L-Tryptophan g L-Tyrosine g L-Valine g L-Carnitine mg Taurine mg Osmolality MSUD, HCU and TYR s = 1900 mosm/kg MMA/PA = 1650 mosm/kg IVA = 1740 mosm/kg Allergen Declaration Contains oil derived from fish sources, milk and soya lecithin. Storage Store in a cool, dry place and refrigerate before use if desired. Once open drink immediately or refrigerate and use within 24 hours. HCU TYR MMA/PA Pack size/volume 30 x pouches = 2.61L. 30 x pouches = 3.9L. 30 x pouches = 5.22L. Shelf life 9 months. IVA MSUD HCU TYR MMA/PA IVA