LEADING THE WAY IN KETOGENIC AND METABOLIC MANAGEMENT

0861 999 666P O Box 137 Glenvista 2058 | 11 Rooigras Avenue Bassonia

www.nutr-e-volution.co.za peter@nutr-e-volution.co.za

086 536 7181

LEADING THE WAY IN KETOGENIC AND METABOLIC MANAGEMENT

AMINOMED®Aminomed® is an amino acid based infant formula suitable for individuals with cow’s milk protein allergy and multiple food intolerances. The protein component of the Aminomed® is derived from amino acids, the main source of carbohydrates is maltodextrin and the fat blend contains a healthy blend of omega 3 and 6 fatty acids, including docosahexaenoic acid (DHA) and arachidonic acid (AA).

Aminomed® is used in the dietary treatment of severe food allergies/intolerance, protracted diarrhoea, maldigestion and malabsorbtion syndrome.

Aminomed® is suitable as a starting point for an elimination diet and the basis for a search diet.

Aminomed® may be used in patient with inflammatory bowel diseases and short bowel syndrome

Elemental amino acids increase absorption and eliminate the risk of protein based allergies, and the concomitant immune response

Carbohydrates derived from highly digestible maltodextrin, with a low dextrose equivalence. Aminomed® is lactose and sucrose free

High in long chain polyunsaturated fatty acids with an excellent omega 3 to omega 6 ratio. Linoleic acid: α-linolenic acid (7.7:1)

Contains very long chain polyunsaturated fatty acids DHA and AA

Meets 100% of RDA and DRI’s

Contains nucleotides to enhance immunity (24.43mg/100g)

Indication Dosage and application

Aminomed® macronutritional information

Key Features

Aminomed® can be administered in the same manner as a normal infant formula (15% dilution) and is suitable as a sole source of nutrition, or for supplementary feeding in tod-dlers and small children.

The amount of Aminomed® used per day is specific to the individuals needs and will vary between individuals. The diet should be monitored and adjusted regularly by a qualified medical professional.

Nutritional component per 100g per 100ml** Energy (kJ) 2046 307

(kCal) 489 73 Protein equivalent * g 13.5 2.03 Amino acids g 16.2 2.4 Carbohydrates g 52.6 7.9 Of which - Lactose g 0 0

Maltodextrin g 52.6 7.9 Starch g 0 0

Fat g 24.9 3.7 Of which - Saturated g 10.2 1.5

Monounsaturated g 11.2 1.7 Polyunsaturated g 3.5 0.5

- Linoleic acid (ω-6) g 2.85 0.43 - α-linolenic acid ω-3) g 0.37 0.056 Arachidonic acid (ω-3) mg 86 12 Docosahexaenoic acid (ω-3) mg 55 8 Dietary fibre g 0 0

*Conversion calculated as follows:1g Protein = 1.2g Amino acids = 17kJ = 4kcal

**Values obtained using the standard dilution of 15.0g in 90ml water (100ml)Energy distribution:Protein 11%Carbohydrates 44%Fat 45%

Nutritional component per 100g per 100ml**Minerals

Sodium mg 121 18Potassium mg 510 77Chloride mg 330 50Calcium mg 495 74Phosphorus mg 266 40Magnesium mg 63 9.5Iron mg 5.8 0.87

Trace elementsZinc mg 5.25 0.79Copper mg 0.347 0.052Iodine µg 84 12.6Chromium µg 10 1.5Flouride µg 170 25.5Manganese µg 400 60Molybdenum µg 25 3.8Selenium µg 14.4 2.2

VitaminsVitamin A mg 0.8 0.12Vitamin D µg 10 1.5Vitamin E mg 9 1.35Vitamin K µg 22 3.3Vitamin C mg 80 12Vitamin B1 mg 0.42 0.063Vitamin B2 mg 0.9 0.14Niacin mg 5.35 0.8Vitamin B6 mg 0.5 0.075Folic acid µg 63 9.5Pantothenic acid mg 3.6 0.54Vitamin B12 µg 1.16 0.17Biotin µg 12 1.8L-Carnitine mg 26 3.9Choline mg 69 10.4Inositol mg 46 6.9Taurine mg 36 5.4

**Values obtained using standard dilution of 15.0g in 90ml water (100ml)

Osmolality Osmolarity

mOsmol/kg = 320 -330 mOsmol/l = 290 -300

LEADING THE WAY IN KETOGENIC AND METABOLIC MANAGEMENT

AMINOMED®Aminomed® is an amino acid based infant formula suitable for individuals with cow’s milk protein allergy and multiple food intolerances. The protein component of the Aminomed® is derived from amino acids, the main source of carbohydrates is maltodextrin and the fat blend contains a healthy blend of omega 3 and 6 fatty acids, including docosahexaenoic acid (DHA) and arachidonic acid (AA).

Aminomed® is used in the dietary treatment of severe food allergies/intolerance, protracted diarrhoea, maldigestion and malabsorbtion syndrome.

Aminomed® is suitable as a starting point for an elimination diet and the basis for a search diet.

Aminomed® may be used in patient with inflammatory bowel diseases and short bowel syndrome

Elemental amino acids increase absorption and eliminate the risk of protein based allergies, and the concomitant immune response

Carbohydrates derived from highly digestible maltodextrin, with a low dextrose equivalence. Aminomed® is lactose and sucrose free

High in long chain polyunsaturated fatty acids with an excellent omega 3 to omega 6 ratio. Linoleic acid: α-linolenic acid (7.7:1)

Contains very long chain polyunsaturated fatty acids DHA and AA

Meets 100% of RDA and DRI’s

Contains nucleotides to enhance immunity (24.43mg/100g)

Indication Dosage and application

Aminomed® macronutritional information

Key Features

Aminomed® can be administered in the same manner as a normal infant formula (15% dilution) and is suitable as a sole source of nutrition, or for supplementary feeding in tod-dlers and small children.

The amount of Aminomed® used per day is specific to the individuals needs and will vary between individuals. The diet should be monitored and adjusted regularly by a qualified medical professional.

Nutritional component per 100g per 100ml** Energy (kJ) 2046 307

(kCal) 489 73 Protein equivalent * g 13.5 2.03 Amino acids g 16.2 2.4 Carbohydrates g 52.6 7.9 Of which - Lactose g 0 0

Maltodextrin g 52.6 7.9 Starch g 0 0

Fat g 24.9 3.7 Of which - Saturated g 10.2 1.5

Monounsaturated g 11.2 1.7 Polyunsaturated g 3.5 0.5

- Linoleic acid (ω-6) g 2.85 0.43 - α-linolenic acid ω-3) g 0.37 0.056 Arachidonic acid (ω-3) mg 86 12 Docosahexaenoic acid (ω-3) mg 55 8 Dietary fibre g 0 0

*Conversion calculated as follows:1g Protein = 1.2g Amino acids = 17kJ = 4kcal

**Values obtained using the standard dilution of 15.0g in 90ml water (100ml)Energy distribution:Protein 11%Carbohydrates 44%Fat 45%

Nutritional component per 100g per 100ml**Minerals

Sodium mg 121 18Potassium mg 510 77Chloride mg 330 50Calcium mg 495 74Phosphorus mg 266 40Magnesium mg 63 9.5Iron mg 5.8 0.87

Trace elementsZinc mg 5.25 0.79Copper mg 0.347 0.052Iodine µg 84 12.6Chromium µg 10 1.5Flouride µg 170 25.5Manganese µg 400 60Molybdenum µg 25 3.8Selenium µg 14.4 2.2

VitaminsVitamin A mg 0.8 0.12Vitamin D µg 10 1.5Vitamin E mg 9 1.35Vitamin K µg 22 3.3Vitamin C mg 80 12Vitamin B1 mg 0.42 0.063Vitamin B2 mg 0.9 0.14Niacin mg 5.35 0.8Vitamin B6 mg 0.5 0.075Folic acid µg 63 9.5Pantothenic acid mg 3.6 0.54Vitamin B12 µg 1.16 0.17Biotin µg 12 1.8L-Carnitine mg 26 3.9Choline mg 69 10.4Inositol mg 46 6.9Taurine mg 36 5.4

**Values obtained using standard dilution of 15.0g in 90ml water (100ml)

Osmolality Osmolarity

mOsmol/kg = 320 -330 mOsmol/l = 290 -300

Nutritional information Per 100g Per 100kcal Energy (kJ) 2104 418 (kCal) 503 100 Protein g 0 0 Carbohydrates g 76.9 15.3

Of which sugars

g 7.7 1.5 Fat g 21 4.2

MCT g 9.4 1.9 Saturated g 9.3 1.8 Monounsaturated g 1.9 0.4 Polyunsaturated g 0.3 0.1 Linoleic acid g 0.31 0.1 α-Linolenic acid g 0.0372 0.0

Ratio LA/ALA 8.3 :1

CaloJOULE®CaloJOULE® is a protein free energy supplement, composed exclusively of carbohydrates and fatty acids. CaloJOULE® is free of electrolytes and is suitable for electrolyte and protein restricted diets.

CaloJOULE® is free of protein and electrolytes, making it suitable for use in diets that require protein and electrolyte restriction and energy supplementation. CaloJOULE® may be used in renal diets that require electrolyte and protein restriction

CaloJOULE® is suitable for use as an energy supplement for individuals with failure to thrive

CaloJOULE® may be used as a carbohydrate and fatty acid module in modular feeding

CaloJOULE® may be used in the ketogenic diet as a module to reduce the effective ratio between the macronutrients

Indication

CaloJOULE® macronutritional information

High energy density 500kCal per 100g

Contains MCT and LCT

Protein free

Electrolyte free

Carbohydrates derived from highly digestible maltodextrin with a low dextrose equivalence (Low GI)

Lactose free

Sucrose free

Key Features

Energy Distribution

Dosage and applicationCaloJOULE® may be mixed directly into solid foods to increase the energy content or mixed with water or predissolved formula to increase the caloric density of the liquid.

CaloJOULE® may be used in tube-feeding application if adequate care is taken to ensure that the powder is fully dissolved.

Protein NilCarbohydrates 63% Fat 37% (MCT 16.6% LCT 20.1%)

Nutritional information Per 100g Per 100kcal Energy (kJ) 2104 418 (kCal) 503 100 Protein g 0 0 Carbohydrates g 76.9 15.3

Of which sugars

g 7.7 1.5 Fat g 21 4.2

MCT g 9.4 1.9 Saturated g 9.3 1.8 Monounsaturated g 1.9 0.4 Polyunsaturated g 0.3 0.1 Linoleic acid g 0.31 0.1 α-Linolenic acid g 0.0372 0.0

Ratio LA/ALA 8.3 :1

CaloJOULE®CaloJOULE® is a protein free energy supplement, composed exclusively of carbohydrates and fatty acids. CaloJOULE® is free of electrolytes and is suitable for electrolyte and protein restricted diets.

CaloJOULE® is free of protein and electrolytes, making it suitable for use in diets that require protein and electrolyte restriction and energy supplementation. CaloJOULE® may be used in renal diets that require electrolyte and protein restriction

CaloJOULE® is suitable for use as an energy supplement for individuals with failure to thrive

CaloJOULE® may be used as a carbohydrate and fatty acid module in modular feeding

CaloJOULE® may be used in the ketogenic diet as a module to reduce the effective ratio between the macronutrients

Indication

CaloJOULE® macronutritional information

High energy density 500kCal per 100g

Contains MCT and LCT

Protein free

Electrolyte free

Carbohydrates derived from highly digestible maltodextrin with a low dextrose equivalence (Low GI)

Lactose free

Sucrose free

Key Features

Energy Distribution

Dosage and applicationCaloJOULE® may be mixed directly into solid foods to increase the energy content or mixed with water or predissolved formula to increase the caloric density of the liquid.

CaloJOULE® may be used in tube-feeding application if adequate care is taken to ensure that the powder is fully dissolved.

Protein NilCarbohydrates 63% Fat 37% (MCT 16.6% LCT 20.1%)

CarniVOLVE powder complies with the standards outlined by the Potchefstroom laboratory of inborn errors of metabolism (PLIEM) for the treatment of primary and secondary L-carnitine deficiencies that result from inborn errors of metabolism or any other factors that affect l-carnitine homeostasis, such as medications (Valproate, Doxyrubicin).

CarniVOLVE® powder is also indicated in diets high in fat, such as the ketogenic diet. Fat metabolism is dependent on L-carnitine as a conjugate for lipid transport to the inner mitochondria for β-oxidation.

CarniVOLVE® is a mild antioxidant and has been recommended as a conjunctive therapy in liver disease and renal failure.

Indication Dosage and applicationL-carnitine supplementation varies from patient to patient and should be monitored by a professional. In general the guideline dosages are stipulated below: 1. Inborn error of metabolism (Not MCADH) 100mg/kg/day 2. Secondary L-carnitine deficiency between 300-2000mg/day

Excessive L-carnitine intake can result in diarrhoea and fishy odor, this can be treated by reducing the dosage and will subside within a few hours. It is best to split the daily dosage into smaller doses across the day as it is quickly cleared from the body. A liquid L-carnitine is also available with a concentration of 200mg/ml.

CaloMALT® CarniVOLVE Powder (L-Carnitine)

CaloMALT® is a high energy carbohydrate supplement, with a low dextrose equivalence, which should be taken orally. CaloMALT® has a characteristic neutral flavour. CaloMALT® contains no protein or fat.

CarniVOLVE powder is a pure L-carnitine powder used in the supplementation of L-carnitine as recommended for inherited and secondary L-carnitine deficiencies, such as inborn errors of metabolism.

CaloMALT is a nutritional supplement where additional energy is required, such as Oncology Pre and Post surgery Anorexia Fat mal-absorption Poor appetite Bowel preparation HIV energy supplementation

Indication

CaloMalt® nutritional information

Easy absorptionFree of fructose, sucrose, galactose, gluten, fibre and lactoseNeutral flavour which allows it to be added to either sweet or savoury foods or beveragesSuitable for cookingNo added minerals or electrolytes

Pure l-carnitine free of impurities500mg scoop 50g unit

Key Features

Key Features

Contra-indications:CaloMALT® is not suitable as a sole source of nutrition. Precautionary note: Only suitable as a supplement to a normal diet.

Nutritional information per 100g powder Energy kcal 398 kJ 1667 Protein Nil Carbohydrates g 99.7 Glucose g 3.7 Maltodextrin g 96.0 Fats g Nil Dietary fibre g Nil

CarniVOLVE powder complies with the standards outlined by the Potchefstroom laboratory of inborn errors of metabolism (PLIEM) for the treatment of primary and secondary L-carnitine deficiencies that result from inborn errors of metabolism or any other factors that affect l-carnitine homeostasis, such as medications (Valproate, Doxyrubicin).

CarniVOLVE® powder is also indicated in diets high in fat, such as the ketogenic diet. Fat metabolism is dependent on L-carnitine as a conjugate for lipid transport to the inner mitochondria for β-oxidation.

CarniVOLVE® is a mild antioxidant and has been recommended as a conjunctive therapy in liver disease and renal failure.

Indication Dosage and applicationL-carnitine supplementation varies from patient to patient and should be monitored by a professional. In general the guideline dosages are stipulated below: 1. Inborn error of metabolism (Not MCADH) 100mg/kg/day 2. Secondary L-carnitine deficiency between 300-2000mg/day

Excessive L-carnitine intake can result in diarrhoea and fishy odor, this can be treated by reducing the dosage and will subside within a few hours. It is best to split the daily dosage into smaller doses across the day as it is quickly cleared from the body. A liquid L-carnitine is also available with a concentration of 200mg/ml.

CaloMALT® CarniVOLVE Powder (L-Carnitine)

CaloMALT® is a high energy carbohydrate supplement, with a low dextrose equivalence, which should be taken orally. CaloMALT® has a characteristic neutral flavour. CaloMALT® contains no protein or fat.

CarniVOLVE powder is a pure L-carnitine powder used in the supplementation of L-carnitine as recommended for inherited and secondary L-carnitine deficiencies, such as inborn errors of metabolism.

CaloMALT is a nutritional supplement where additional energy is required, such as Oncology Pre and Post surgery Anorexia Fat mal-absorption Poor appetite Bowel preparation HIV energy supplementation

Indication

CaloMalt® nutritional information

Easy absorptionFree of fructose, sucrose, galactose, gluten, fibre and lactoseNeutral flavour which allows it to be added to either sweet or savoury foods or beveragesSuitable for cookingNo added minerals or electrolytes

Pure l-carnitine free of impurities500mg scoop 50g unit

Key Features

Key Features

Contra-indications:CaloMALT® is not suitable as a sole source of nutrition. Precautionary note: Only suitable as a supplement to a normal diet.

Nutritional information per 100g powder Energy kcal 398 kJ 1667 Protein Nil Carbohydrates g 99.7 Glucose g 3.7 Maltodextrin g 96.0 Fats g Nil Dietary fibre g Nil

Nutritional information per 100g powder Energy kcal 398 kJ 1667 Protein Nil Carbohydrates g 99.7 Glucose g 3.7 Maltodextrin g 96.0 Fats g Nil Dietary fibre g Nil

Whey UP® Nutritional information

Whey UP is a whey based protein module suitable for protein supplementation. Whey protein is more digestible than conventional milk protein based protein supplements, with a digestibility of 93% as opposed to 85%. The amino acid profile is also of a high quality, scoring the highest protein score of 1.0. Whey proteins are high in essential amino acids, especially branched chain amino acids and large neutral amino acids. Large neutral amino acids are known to stabilise serotonin production and enhance brain function.

Whey UP is suitable for protein supplementation in persons with casein and/or gluten intolerances.

Whey UP may be used as a modular component of the ketogenic diet. The low gastric clearance time allows for regular feeding with shortened intervals.

Excellent protein digestibilityHigh PDCAA score of 1.0High in essential amino acidsGood solubility Milky flavourDioxin and antibiotic free

Indication

Dosage and application

Key Features

The amount of whey protein to be used is specific to the individual and should be determined by a qualified healthcare professional.

MCT OilMedium Chain Triglyceride (MCT) oil is a fractionated coconut oil that has all long chain triglycerides removed. MCTs bypass normal fatty acid absorptive pathways. This makes MCT’s suitable for persons that have difficulty absorbing and metabolising long chain triglycerides.

MCT oils are suitable as a fat based energy source in person with chylothorax and LCT fat malabsorption.

MCT oil may also be used as an emergency ketone boost in the ketogenic diet. This is due to the shortened metabolism caused by the shortened absorbtive pathway.

Indication

High in C8 and C10 MCTsHigh ketone yield per gram Drop resistant 500ml PET bottle2 year shelf life

Key Features

Dosage and applicationMCT oils are used as a fatty acid energy source in diets that are restricted in long chain triglycerides. The dosage is calculated specifically for each individual and use should be monitored by a qualified medical practitioner. Smaller dosages are recommended to prevent cramping or the onset of diarrhoea.

Whey UP®

Nutritional Information Per 100g Per 100 kcal Energy (kcal) 397 100 (KJ) 1657 418 Protein 80 20 Fats (g) 6.5 1.6 Carbohydrates 4.5 1.1 Lactose 4.5 1.1 Calcium 578 146 Magnesium 64 16 Iron 1 0.3 Sodium 724 182 Potassium 973 245 Chloride 117 29

Nutritional information per 100g powder Energy kcal 398 kJ 1667 Protein Nil Carbohydrates g 99.7 Glucose g 3.7 Maltodextrin g 96.0 Fats g Nil Dietary fibre g Nil

Whey UP® Nutritional information

Whey UP is a whey based protein module suitable for protein supplementation. Whey protein is more digestible than conventional milk protein based protein supplements, with a digestibility of 93% as opposed to 85%. The amino acid profile is also of a high quality, scoring the highest protein score of 1.0. Whey proteins are high in essential amino acids, especially branched chain amino acids and large neutral amino acids. Large neutral amino acids are known to stabilise serotonin production and enhance brain function.

Whey UP is suitable for protein supplementation in persons with casein and/or gluten intolerances.

Whey UP may be used as a modular component of the ketogenic diet. The low gastric clearance time allows for regular feeding with shortened intervals.

Excellent protein digestibilityHigh PDCAA score of 1.0High in essential amino acidsGood solubility Milky flavourDioxin and antibiotic free

Indication

Dosage and application

Key Features

The amount of whey protein to be used is specific to the individual and should be determined by a qualified healthcare professional.

MCT OilMedium Chain Triglyceride (MCT) oil is a fractionated coconut oil that has all long chain triglycerides removed. MCTs bypass normal fatty acid absorptive pathways. This makes MCT’s suitable for persons that have difficulty absorbing and metabolising long chain triglycerides.

MCT oils are suitable as a fat based energy source in person with chylothorax and LCT fat malabsorption.

MCT oil may also be used as an emergency ketone boost in the ketogenic diet. This is due to the shortened metabolism caused by the shortened absorbtive pathway.

Indication

High in C8 and C10 MCTsHigh ketone yield per gram Drop resistant 500ml PET bottle2 year shelf life

Key Features

Dosage and applicationMCT oils are used as a fatty acid energy source in diets that are restricted in long chain triglycerides. The dosage is calculated specifically for each individual and use should be monitored by a qualified medical practitioner. Smaller dosages are recommended to prevent cramping or the onset of diarrhoea.

Whey UP®

Nutritional Information Per 100g Per 100 kcal Energy (kcal) 397 100 (KJ) 1657 418 Protein 80 20 Fats (g) 6.5 1.6 Carbohydrates 4.5 1.1 Lactose 4.5 1.1 Calcium 578 146 Magnesium 64 16 Iron 1 0.3 Sodium 724 182 Potassium 973 245 Chloride 117 29

Nutritional information for KetoVOLVE

Preparation and administration

KetoVOLVE®KetoVOLVE is a very high fat, adequate protein and low carbohydrate formula, with an off white colour and bland milky flavor. KetoVOLVE consists of palm fruit oil, fractionated coconut oil, whey protein, maltodextrin, vitamins and minerals. KetoVOLVE is a powdered ketogenic diet suitable for tube feeding and as an oral supplement. KetoVOLVE® has no added, gluten, sucrose, fructose, casein, artificial sweeteners and flavourants.

KetoVOLVE is a nutritionally complete powdered formula that is suitable for the administration or supplementation of the Ketogenic diet, which is used in the management of refractory epilepsy. KetoVOLVE is suitable for use as a sole source of nutrition in children between 1 and 8 years of age. It is particularly effective in the management of:

KetoVOLVE is a complete feed suitable for the administration of the Ketogenic diet. Therefore KetoVOLVE has the same precautions as the Ketogenic diet, namely:

KetoVOLVE contains a blend of fractionated coconut oil and palm fruit oil, which is blended to yield the fatty acid profile in the table to the right. KetoVOLVE’s fatty acid profile is free of trans fats, GM organisms and has a LA:α-LA = 9.8:1

The recommended intake of KetoVOLVE is dependent on age, bodyweight and medical condition of the patient. The recommended dilution is 1:4 eg: 20g KetoVOLVE powder plus 80ml of water to yield 100ml. This results in a caloric density of 1.40 kCal per ml.

KetoVOLVE is contraindicated in individuals with certain metabolic deficiencies:

Indication

Precautions

KetoVOLVE fatty acids

Contraindications

West syndrome/Infantile spasmLennox-Gastaut syndromeGLUT-1 Deficiency syndromeDoose Syndrome/ MAE – Myoclonic astatic epilepsyDravet SyndromePyruvate Dehydrogenase deficiencyMitochondrial deficiencies

ConstipationFailure to gain weight Low grade acidosisHypoglycaemia – if the diet is initiated with a fast Increased cholesterolPossible formation of renal stones

1 Long Chain Triglycerides 2 Medium Chain Triglycerides 3 Vitamin E levels comply with EU Directive 2006/141/EC requiring min 0.5mg/g PUFA 4 Calcium to Phosphorus ratio is 1.4:1 compliant with EU Directive 2006/141/ECPlease note values may vary from label specifications due to rounding differences

1. L-Carnitine deficiencies2. Pyruvate carboxylase deficiencies3. Porphyria4. Inborn errors of fat metabolism 5. Diabetes mellitus

Per 100g powder Per 100kCal

Energy kCal 700 100 KJ 2926 418 Protein g 14.5 2.1 Carbohydrates g 2.5 0.4 Sugars g 1.1 0.2 Fats LCT1 g 57.0 8.1 MCT2 g 15.0 2.1 Trans Fat g <0.5 <0.07 Fibre g Nil Nil Vitamins Vitamin A ug RE 445 65.6 Vitamin C mg 75 10.7 Vitamin D ug 10 1.4 Vitamin E3 mg 7 1.0 Vitamin K ug 55 7.9 Thiamin (B1) mg 0.6 0.1 Riboflavin (B2) mg 0.6 0.1 Niacin (B3) mg 6.7 0.9 Pyridoxine (B6) mg 0.6 0.1 Folic Acid ug 200 28.6 M-Inositol mg 58 8.3 Cobalomin (B12) ug 1.2 0.2 Pantothenate (B5) mg 3 0.4 D-Biotin ug 12 1.7 Choline mg 250 35.7 Minerals Calcium4 mg 800 114.3 Chromium ug 15 2.1 Copper ug 440 62.9 Iodine ug 90 12.8 Iron mg 10 1.4 Magnesium mg 100 14.3 Manganese mg 1 0.1 Molybdenum ug 22 3.1 Phosphorus mg 550 78.6 Selenium ug 30 4.3 Zinc mg 5 0.7 Potassium mg 889 127 Sodium mg 309 44.1 Chloride mg 723 103.3

Fatty acid description g per 100g Powder % of total fat C 6:0 Caproic acid 0.300 0.4% C 8:0 Caprylic acid 12.120 16.8% C 10:0 Capric acid 7.840 10.9% C 12:0 Lauric acid 27.230 37.8% C 14:0 Myristic acid 9.120 12.7% C 16:0 Palmitic acid 5.642 7.8% C 18:1 n-6 to n-9 acid 7.980 11.1% C 18:1 Trans acid 0.000 0.0% C 18:2 Linoleic acid 1.425 2.0% C 18:2 Trans acid 0.000 0.0% C 18:3 α-Linolenic acid 0.144 0.2% C 20:0 0.000 0.0% C 22:1 Erucic acid 0.200 0.3% 72.00 100%

MCT Oils

Saturated

Monounsaturated

Polyunsaturated

Nutritional information for KetoVOLVE

Preparation and administration

KetoVOLVE®KetoVOLVE is a very high fat, adequate protein and low carbohydrate formula, with an off white colour and bland milky flavor. KetoVOLVE consists of palm fruit oil, fractionated coconut oil, whey protein, maltodextrin, vitamins and minerals. KetoVOLVE is a powdered ketogenic diet suitable for tube feeding and as an oral supplement. KetoVOLVE® has no added, gluten, sucrose, fructose, casein, artificial sweeteners and flavourants.

KetoVOLVE is a nutritionally complete powdered formula that is suitable for the administration or supplementation of the Ketogenic diet, which is used in the management of refractory epilepsy. KetoVOLVE is suitable for use as a sole source of nutrition in children between 1 and 8 years of age. It is particularly effective in the management of:

KetoVOLVE is a complete feed suitable for the administration of the Ketogenic diet. Therefore KetoVOLVE has the same precautions as the Ketogenic diet, namely:

KetoVOLVE contains a blend of fractionated coconut oil and palm fruit oil, which is blended to yield the fatty acid profile in the table to the right. KetoVOLVE’s fatty acid profile is free of trans fats, GM organisms and has a LA:α-LA = 9.8:1

The recommended intake of KetoVOLVE is dependent on age, bodyweight and medical condition of the patient. The recommended dilution is 1:4 eg: 20g KetoVOLVE powder plus 80ml of water to yield 100ml. This results in a caloric density of 1.40 kCal per ml.

KetoVOLVE is contraindicated in individuals with certain metabolic deficiencies:

Indication

Precautions

KetoVOLVE fatty acids

Contraindications

West syndrome/Infantile spasmLennox-Gastaut syndromeGLUT-1 Deficiency syndromeDoose Syndrome/ MAE – Myoclonic astatic epilepsyDravet SyndromePyruvate Dehydrogenase deficiencyMitochondrial deficiencies

ConstipationFailure to gain weight Low grade acidosisHypoglycaemia – if the diet is initiated with a fast Increased cholesterolPossible formation of renal stones

1 Long Chain Triglycerides 2 Medium Chain Triglycerides 3 Vitamin E levels comply with EU Directive 2006/141/EC requiring min 0.5mg/g PUFA 4 Calcium to Phosphorus ratio is 1.4:1 compliant with EU Directive 2006/141/ECPlease note values may vary from label specifications due to rounding differences

1. L-Carnitine deficiencies2. Pyruvate carboxylase deficiencies3. Porphyria4. Inborn errors of fat metabolism 5. Diabetes mellitus

Per 100g powder Per 100kCal

Energy kCal 700 100 KJ 2926 418 Protein g 14.5 2.1 Carbohydrates g 2.5 0.4 Sugars g 1.1 0.2 Fats LCT1 g 57.0 8.1 MCT2 g 15.0 2.1 Trans Fat g <0.5 <0.07 Fibre g Nil Nil Vitamins Vitamin A ug RE 445 65.6 Vitamin C mg 75 10.7 Vitamin D ug 10 1.4 Vitamin E3 mg 7 1.0 Vitamin K ug 55 7.9 Thiamin (B1) mg 0.6 0.1 Riboflavin (B2) mg 0.6 0.1 Niacin (B3) mg 6.7 0.9 Pyridoxine (B6) mg 0.6 0.1 Folic Acid ug 200 28.6 M-Inositol mg 58 8.3 Cobalomin (B12) ug 1.2 0.2 Pantothenate (B5) mg 3 0.4 D-Biotin ug 12 1.7 Choline mg 250 35.7 Minerals Calcium4 mg 800 114.3 Chromium ug 15 2.1 Copper ug 440 62.9 Iodine ug 90 12.8 Iron mg 10 1.4 Magnesium mg 100 14.3 Manganese mg 1 0.1 Molybdenum ug 22 3.1 Phosphorus mg 550 78.6 Selenium ug 30 4.3 Zinc mg 5 0.7 Potassium mg 889 127 Sodium mg 309 44.1 Chloride mg 723 103.3

Fatty acid description g per 100g Powder % of total fat C 6:0 Caproic acid 0.300 0.4% C 8:0 Caprylic acid 12.120 16.8% C 10:0 Capric acid 7.840 10.9% C 12:0 Lauric acid 27.230 37.8% C 14:0 Myristic acid 9.120 12.7% C 16:0 Palmitic acid 5.642 7.8% C 18:1 n-6 to n-9 acid 7.980 11.1% C 18:1 Trans acid 0.000 0.0% C 18:2 Linoleic acid 1.425 2.0% C 18:2 Trans acid 0.000 0.0% C 18:3 α-Linolenic acid 0.144 0.2% C 20:0 0.000 0.0% C 22:1 Erucic acid 0.200 0.3% 72.00 100%

MCT Oils

Saturated

Monounsaturated

Polyunsaturated

Some separation is likely to occur when solubilised KetoVOLVE is left to stand. This is a normal characteristic of KetoVOLVE and can be remedied by stirring.

Potential Renal Solute Load (PRSL) 5.56 mOsm/100kJ 23.24 mOsm/100kcal

Mixing instructions1. Weigh out prescribed amount of powder and place into sealable container2. KetoVOLVE dissolves in hot tap water3. Make up to required volume4. Stir, shake or whisk vigorously until all the powder is dissolved 5. Remove carefully, chill in refrigerator6. Stir before use

Dilution TableKetoVOLVE (g) Water (ml) Final Volume (ml) Kcal/ml

20 80 100 1.4050 200 250 1.40

100 400 500 1.40200 800 1000 1.40

Some separation is likely to occur when solubilised KetoVOLVE is left to stand. This is a normal characteristic of KetoVOLVE and can be remedied by stirring.

Potential Renal Solute Load (PRSL) 5.56 mOsm/100kJ 23.24 mOsm/100kcal

Mixing instructions1. Weigh out prescribed amount of powder and place into sealable container2. KetoVOLVE dissolves in hot tap water3. Make up to required volume4. Stir, shake or whisk vigorously until all the powder is dissolved 5. Remove carefully, chill in refrigerator6. Stir before use

Dilution TableKetoVOLVE (g) Water (ml) Final Volume (ml) Kcal/ml

20 80 100 1.4050 200 250 1.40

100 400 500 1.40200 800 1000 1.40

L-Arginine, which ultimately acts as a substrate in the conversion of ammonia to urea, which is readily excreted through the urine. The L-Arginine is further metabolised to L-ornithine and urea, whereas the succinic acid is recycled into the Tricarboxylic acid cycle. L-Arginine is a known stimulant of growth hormone release and may contribute to ammonia fixation through direct stimulus of the urea cycle in tandem with stimulating the release of growth hormone, which will in turn slow the release ammonia through catabolism.

Secondary deammoniation is achieved through the supplementation of sodium benzoate and sodium phenylbutyrate, which conjugate glycine and glutamine respectively. Benzoic acid is a xenobiotic molecule that is quickly metabolised to benzoyl-coA. Benzoyl-coA is a substrate of the GLYAT enzyme that conjugates the benzoic acid portion of benzoyl-coA to glycine for excretion in the urine, as hippuric acid. During benzoic acid supplementation glycine is depleted resulting in a shift in the reaction direction of glycine synthase. This action by glycine synthase traps free ammonia and carbon dioxide, forming glycine, which is utilised as a substrate for GLYAT and the resulting hippuric acid is excreted through the urine. 1 molecule of ammonia is “trapped” within every hippuric acid molecule through this pathway. Phenylbutyrate is metabolised to phenylacetate, which is then conjugated to glutamine. 2 molecules are “trapped” and release through the excretion of phenylacetylglutamine. The use of two ammonia fixing substrates is justified through Michaelis-Menton kinetics, which

illustrates the saturable nature of enzyme reactions.

UCD Complete®Hyperammonaemia is a life threatening crisis caused by the accumulation of excessive ammonia. UCD complete is a supplement containing 2 ammonia scavengers and the amino acid L-arginine. These ingredients act to scavenge 3 ammonia molecules and enhance anabolic pathways reducing the flux of nitrogen to the urea cycle, respectively. The advantage of a dual scavenger system is that the rate of ammonia clearance is significantly increased when compared to competitive products.

Urea cycle defects are a group of autosomal recessive disorders, with the exception of Ornithine transcarbamylase deficiency, which is inherited in an X-linked manner. These diseases result from defects in the metabolism of waste nitrogen generated by the breakdown of nitrogen containing molecules, such as protein. In severe cases, infants appear normal at birth but decompensate metabolically within a few days. This decompensation results in cerebral edema, anorexia, hyper or hypoventilation, hypothermia, seizures, neurological posturing and coma. Milder conditions such as Arginase deficiency have later onset patterns and decompensation is normally triggered by intermittent illness or stress. Ammonia is a normal component of blood but is limited to levels between 10 -40µmol/L. These levels are strictly controlled and disturbances in ammonia homeostasis can be catastrophic. Minor elevations of levels to 100µmol and 200µmol are associated with disturbances in consciousness and neurological disturbances respectively. It was originally believed that the toxicity associated with hyperammonaemia was directly attributed to the ammonia, primarily through the depletion of ATP and the Tricarboxylic acid cycle intermediate α-ketoglutarate. However radio labelled studies showed that elevated ammonia did not affect the concentration of α-ketogluterate in the brain. It is now believed that the toxicological effects of elevated ammonia are a result of elevated L-glutamine, particularly in the brain This elevation in brain glutamine levels results in a complex pathophysiological and neurotoxic response, including disturbances in potassium ion homeostasis, Free radical generation, increase mitochondrial permeability and lactate production. Secondary effects of L-Glutamine elevation include astrocytes swelling and dysfunction. There is however evidence to suggest that ammonia does alter some aspects of cerebral energy metabolism, but this is not considered significant enough to be life threatening until end-stage disease.

UCD complete acts through two mechanisms that ameliorate the effects of ammonia toxicity. Primary ammonia removal is achieved through the supplementation of cytoplasmic intermediate

Indication

L-Arginine, which ultimately acts as a substrate in the conversion of ammonia to urea, which is readily excreted through the urine. The L-Arginine is further metabolised to L-ornithine and urea, whereas the succinic acid is recycled into the Tricarboxylic acid cycle. L-Arginine is a known stimulant of growth hormone release and may contribute to ammonia fixation through direct stimulus of the urea cycle in tandem with stimulating the release of growth hormone, which will in turn slow the release ammonia through catabolism.

Secondary deammoniation is achieved through the supplementation of sodium benzoate and sodium phenylbutyrate, which conjugate glycine and glutamine respectively. Benzoic acid is a xenobiotic molecule that is quickly metabolised to benzoyl-coA. Benzoyl-coA is a substrate of the GLYAT enzyme that conjugates the benzoic acid portion of benzoyl-coA to glycine for excretion in the urine, as hippuric acid. During benzoic acid supplementation glycine is depleted resulting in a shift in the reaction direction of glycine synthase. This action by glycine synthase traps free ammonia and carbon dioxide, forming glycine, which is utilised as a substrate for GLYAT and the resulting hippuric acid is excreted through the urine. 1 molecule of ammonia is “trapped” within every hippuric acid molecule through this pathway. Phenylbutyrate is metabolised to phenylacetate, which is then conjugated to glutamine. 2 molecules are “trapped” and release through the excretion of phenylacetylglutamine. The use of two ammonia fixing substrates is justified through Michaelis-Menton kinetics, which

illustrates the saturable nature of enzyme reactions.

UCD Complete®Hyperammonaemia is a life threatening crisis caused by the accumulation of excessive ammonia. UCD complete is a supplement containing 2 ammonia scavengers and the amino acid L-arginine. These ingredients act to scavenge 3 ammonia molecules and enhance anabolic pathways reducing the flux of nitrogen to the urea cycle, respectively. The advantage of a dual scavenger system is that the rate of ammonia clearance is significantly increased when compared to competitive products.

Urea cycle defects are a group of autosomal recessive disorders, with the exception of Ornithine transcarbamylase deficiency, which is inherited in an X-linked manner. These diseases result from defects in the metabolism of waste nitrogen generated by the breakdown of nitrogen containing molecules, such as protein. In severe cases, infants appear normal at birth but decompensate metabolically within a few days. This decompensation results in cerebral edema, anorexia, hyper or hypoventilation, hypothermia, seizures, neurological posturing and coma. Milder conditions such as Arginase deficiency have later onset patterns and decompensation is normally triggered by intermittent illness or stress. Ammonia is a normal component of blood but is limited to levels between 10 -40µmol/L. These levels are strictly controlled and disturbances in ammonia homeostasis can be catastrophic. Minor elevations of levels to 100µmol and 200µmol are associated with disturbances in consciousness and neurological disturbances respectively. It was originally believed that the toxicity associated with hyperammonaemia was directly attributed to the ammonia, primarily through the depletion of ATP and the Tricarboxylic acid cycle intermediate α-ketoglutarate. However radio labelled studies showed that elevated ammonia did not affect the concentration of α-ketogluterate in the brain. It is now believed that the toxicological effects of elevated ammonia are a result of elevated L-glutamine, particularly in the brain This elevation in brain glutamine levels results in a complex pathophysiological and neurotoxic response, including disturbances in potassium ion homeostasis, Free radical generation, increase mitochondrial permeability and lactate production. Secondary effects of L-Glutamine elevation include astrocytes swelling and dysfunction. There is however evidence to suggest that ammonia does alter some aspects of cerebral energy metabolism, but this is not considered significant enough to be life threatening until end-stage disease.

UCD complete acts through two mechanisms that ameliorate the effects of ammonia toxicity. Primary ammonia removal is achieved through the supplementation of cytoplasmic intermediate

Indication

Dosage and applicationDue the vast heterogeneity of human genetics, each individual will respond differently to UCD complete and should always use the product under medical supervision. The dosage in acute decompensation and hyperammonaemia is 1g/kg/day, once the ammonia has been stabilised this dosage should be reduced to a point where the plasma ammonia is within normal parameters.

During hyperammonaemia it is recommended that protein intake is restricted to prevent additional ammonia entering the system.

Dosage and applicationDue the vast heterogeneity of human genetics, each individual will respond differently to UCD complete and should always use the product under medical supervision. The dosage in acute decompensation and hyperammonaemia is 1g/kg/day, once the ammonia has been stabilised this dosage should be reduced to a point where the plasma ammonia is within normal parameters.

During hyperammonaemia it is recommended that protein intake is restricted to prevent additional ammonia entering the system.