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P O Box 137 Glenvista 2058 | 11 Rooigras Avenue Bassonia www.nutr-e-volution.co.za

LE

AD IN GT HE WA Y IN

peter@nutr-e-volution.co.za

KET OGE NIC A ND

METAB OLIC MANA GEMENT


Aminomed® macronutritional information

AMINOMED®

Nutritional component Energy (kJ) (kCal)

per 100g 2046 489

per 100ml** 307 73

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).

Protein equivalent * Amino acids

g g

13.5 16.2

2.03 2.4

Carbohydrates Of which - Lactose Maltodextrin Starch

g g g g

52.6 0 52.6 0

7.9 0 7.9 0

Indication

Fat Of which - Saturated Monounsaturated Polyunsaturated - Linoleic acid (ω-6) - α-linolenic acid ω-3) Arachidonic acid (ω-3) Docosahexaenoic acid (ω-3)

g g g g g g mg mg

24.9 10.2 11.2 3.5 2.85 0.37 86 55

3.7 1.5 1.7 0.5 0.43 0.056 12 8

Dietary fibre

g

0

0

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

Dosage and application

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 toddlers 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.

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

Nutritional component Minerals Sodium Potassium Chloride Calcium Phosphorus Magnesium Iron Trace elements Zinc Copper Iodine Chromium Flouride Manganese Molybdenum Selenium Vitamins

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

Key Features

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)

Vitamin A Vitamin D Vitamin E Vitamin K Vitamin C Vitamin B1 Vitamin B2 Niacin Vitamin B6 Folic acid Pantothenic acid Vitamin B12 Biotin L-Carnitine Choline Inositol Taurine

per 100g

per 100ml**

mg mg mg mg mg mg mg

121 510 330 495 266 63 5.8

18 77 50 74 40 9.5 0.87

mg mg µg µg µg µg µg µg

5.25 0.347 84 10 170 400 25 14.4

0.79 0.052 12.6 1.5 25.5 60 3.8 2.2

mg µg mg µg mg mg mg mg mg µg mg µg µg mg mg mg mg

0.8 10 9 22 80 0.42 0.9 5.35 0.5 63 3.6 1.16 12 26 69 46 36

0.12 1.5 1.35 3.3 12 0.063 0.14 0.8 0.075 9.5 0.54 0.17 1.8 3.9 10.4 6.9 5.4

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

Osmolarity

mOsmol/kg = 320 -330

mOsmol/l

= 290 -300


Aminomed® macronutritional information

AMINOMED®

Nutritional component Energy (kJ) (kCal)

per 100g 2046 489

per 100ml** 307 73

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).

Protein equivalent * Amino acids

g g

13.5 16.2

2.03 2.4

Carbohydrates Of which - Lactose Maltodextrin Starch

g g g g

52.6 0 52.6 0

7.9 0 7.9 0

Indication

Fat Of which - Saturated Monounsaturated Polyunsaturated - Linoleic acid (ω-6) - α-linolenic acid ω-3) Arachidonic acid (ω-3) Docosahexaenoic acid (ω-3)

g g g g g g mg mg

24.9 10.2 11.2 3.5 2.85 0.37 86 55

3.7 1.5 1.7 0.5 0.43 0.056 12 8

Dietary fibre

g

0

0

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

Dosage and application

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 toddlers 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.

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

Nutritional component Minerals Sodium Potassium Chloride Calcium Phosphorus Magnesium Iron Trace elements Zinc Copper Iodine Chromium Flouride Manganese Molybdenum Selenium Vitamins

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

Key Features

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)

Vitamin A Vitamin D Vitamin E Vitamin K Vitamin C Vitamin B1 Vitamin B2 Niacin Vitamin B6 Folic acid Pantothenic acid Vitamin B12 Biotin L-Carnitine Choline Inositol Taurine

per 100g

per 100ml**

mg mg mg mg mg mg mg

121 510 330 495 266 63 5.8

18 77 50 74 40 9.5 0.87

mg mg µg µg µg µg µg µg

5.25 0.347 84 10 170 400 25 14.4

0.79 0.052 12.6 1.5 25.5 60 3.8 2.2

mg µg mg µg mg mg mg mg mg µg mg µg µg mg mg mg mg

0.8 10 9 22 80 0.42 0.9 5.35 0.5 63 3.6 1.16 12 26 69 46 36

0.12 1.5 1.35 3.3 12 0.063 0.14 0.8 0.075 9.5 0.54 0.17 1.8 3.9 10.4 6.9 5.4

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

Osmolarity

mOsmol/kg = 320 -330

mOsmol/l

= 290 -300


CaloJOULE®

CaloJOULE® macronutritional information Nutritional information Energy

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.

Per 100g (kJ) 2104 (kCal) 503

Per 100kcal 418 100

Protein

g

0

0

Indication

Carbohydrates

g

76.9

15.3

Of which sugars

g

7.7

1.5

Fat MCT Saturated Monounsaturated Polyunsaturated Linoleic acid α-Linolenic acid

g g g g g g g

21 9.4 9.3 1.9 0.3 0.31 0.0372

4.2 1.9 1.8 0.4 0.1 0.1 0.0

8.3

:1

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

Dosage and application

CaloJOULE® 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.

Key Features 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

Ratio LA/ALA

Energy Distribution Protein Carbohydrates Fat

Nil 63% 37% (MCT 16.6% LCT 20.1%)


CaloJOULE®

CaloJOULE® macronutritional information Nutritional information Energy

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.

Per 100g (kJ) 2104 (kCal) 503

Per 100kcal 418 100

Protein

g

0

0

Indication

Carbohydrates

g

76.9

15.3

Of which sugars

g

7.7

1.5

Fat MCT Saturated Monounsaturated Polyunsaturated Linoleic acid α-Linolenic acid

g g g g g g g

21 9.4 9.3 1.9 0.3 0.31 0.0372

4.2 1.9 1.8 0.4 0.1 0.1 0.0

8.3

:1

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

Dosage and application

CaloJOULE® 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.

Key Features 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

Ratio LA/ALA

Energy Distribution Protein Carbohydrates Fat

Nil 63% 37% (MCT 16.6% LCT 20.1%)


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.

Indication

Indication

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).

Key Features

L-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

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.

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 is a nutritional supplement where additional energy is required, such as Oncology Pre and Post surgery Contra-indications: Anorexia CaloMALT® is not suitable as a sole source of nutrition. Fat mal-absorption Precautionary note: Only suitable as a supplement to a normal diet. Poor appetite Bowel preparation HIV energy supplementation Easy absorption Free of fructose, sucrose, galactose, gluten, fibre and lactose Neutral flavour which allows it to be added to either sweet or savoury foods or beverages Suitable for cooking No added minerals or electrolytes

CaloMalt® nutritional information Nutritional information per 100g powder Energy Protein Carbohydrates Glucose Maltodextrin Fats Dietary fibre

kcal kJ g g g g g

398 1667 Nil 99.7 3.7 96.0 Nil Nil

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

Key Features Pure l-carnitine free of impurities 500mg scoop 50g unit

Dosage and application


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.

Indication

Indication

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).

Key Features

L-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

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.

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 is a nutritional supplement where additional energy is required, such as Oncology Pre and Post surgery Contra-indications: Anorexia CaloMALT® is not suitable as a sole source of nutrition. Fat mal-absorption Precautionary note: Only suitable as a supplement to a normal diet. Poor appetite Bowel preparation HIV energy supplementation Easy absorption Free of fructose, sucrose, galactose, gluten, fibre and lactose Neutral flavour which allows it to be added to either sweet or savoury foods or beverages Suitable for cooking No added minerals or electrolytes

CaloMalt® nutritional information Nutritional information per 100g powder Energy Protein Carbohydrates Glucose Maltodextrin Fats Dietary fibre

kcal kJ g g g g g

398 1667 Nil 99.7 3.7 96.0 Nil Nil

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

Key Features Pure l-carnitine free of impurities 500mg scoop 50g unit

Dosage and application


MCT Oil

Whey UP®

Medium 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.

Indication

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.

Dosage and application

MCT 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.

Key Features

High in C8 and C10 MCTs High ketone yield per gram Drop resistant 500ml PET bottle 2 year shelf life Nutritional information per 100g powder Energy Protein Carbohydrates Glucose Maltodextrin Fats Dietary fibre

kcal kJ g g g g g

398 1667 Nil 99.7 3.7 96.0 Nil Nil

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.

Indication

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.

Dosage and application

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

Key Features Excellent protein digestibility High PDCAA score of 1.0 High in essential amino acids Good solubility Milky flavour Dioxin and antibiotic free

Whey UP® Nutritional information Nutritional Information Energy (kcal) (KJ)

Per 100g 397 1657

Per 100 kcal 100 418

Protein

80

20

Fats (g)

6.5

1.6

Carbohydrates Lactose

4.5 4.5

1.1 1.1

Calcium Magnesium Iron Sodium Potassium Chloride

578 64 1 724 973 117

146 16 0.3 182 245 29


MCT Oil

Whey UP®

Medium 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.

Indication

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.

Dosage and application

MCT 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.

Key Features

High in C8 and C10 MCTs High ketone yield per gram Drop resistant 500ml PET bottle 2 year shelf life Nutritional information per 100g powder Energy Protein Carbohydrates Glucose Maltodextrin Fats Dietary fibre

kcal kJ g g g g g

398 1667 Nil 99.7 3.7 96.0 Nil Nil

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.

Indication

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.

Dosage and application

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

Key Features Excellent protein digestibility High PDCAA score of 1.0 High in essential amino acids Good solubility Milky flavour Dioxin and antibiotic free

Whey UP® Nutritional information Nutritional Information Energy (kcal) (KJ)

Per 100g 397 1657

Per 100 kcal 100 418

Protein

80

20

Fats (g)

6.5

1.6

Carbohydrates Lactose

4.5 4.5

1.1 1.1

Calcium Magnesium Iron Sodium Potassium Chloride

578 64 1 724 973 117

146 16 0.3 182 245 29


Precautions

KetoVOLVE®

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

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 fatty acids

Failure to gain weight Low grade acidosis Hypoglycaemia – if the diet is initiated with a fast Increased cholesterol

Indication

Possible formation of renal stones

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:

Nutritional information for KetoVOLVE Per 100g powder Energy kCal

West syndrome/Infantile spasm

KJ

Protein g Carbohydrates g Sugars g Fats

Lennox-Gastaut syndrome GLUT-1 Deficiency syndrome

LCT1 g MCT2 g

Trans Fat g

Doose Syndrome/ MAE – Myoclonic astatic epilepsy

Fibre g Vitamins Vitamin A ug RE Vitamin C mg Vitamin D ug Vitamin E3 mg Vitamin K ug Thiamin (B1) mg Riboflavin (B2) mg Niacin (B3) mg Pyridoxine (B6) mg Folic Acid ug M-Inositol mg Cobalomin (B12) ug Pantothenate (B5) mg D-Biotin ug Choline mg Minerals Calcium4 mg Chromium ug Copper ug Iodine ug Iron mg Magnesium mg Manganese mg Molybdenum ug Phosphorus mg Selenium ug Zinc mg Potassium mg Sodium mg Chloride mg

Dravet Syndrome Pyruvate Dehydrogenase deficiency Mitochondrial deficiencies

Contraindications

KetoVOLVE is contraindicated in individuals with certain metabolic deficiencies:

1. L-Carnitine deficiencies 2. Pyruvate carboxylase deficiencies 3. Porphyria 4. Inborn errors of fat metabolism 5. Diabetes mellitus

1

Long Chain Triglycerides

2

Medium Chain Triglycerides

3

Per 100kCal

700 2926

100 418

14.5 2.5 1.1

2.1 0.4 0.2

57.0 15.0 <0.5

8.1 2.1 <0.07

Nil

Nil

445 75 10 7 55 0.6 0.6 6.7 0.6 200 58 1.2 3 12 250

65.6 10.7 1.4 1.0 7.9 0.1 0.1 0.9 0.1 28.6 8.3 0.2 0.4 1.7 35.7

800 15 440 90 10 100 1 22 550 30 5 889 309 723

114.3 2.1 62.9 12.8 1.4 14.3 0.1 3.1 78.6 4.3 0.7 127 44.1 103.3

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 Fatty acid description C 6:0 Caproic acid C 8:0 Caprylic acid C 10:0 Capric acid C 12:0 Lauric acid C 14:0 Myristic acid C 16:0 Palmitic acid C 18:1 n-6 to n-9 acid C 18:1 Trans acid C 18:2 Linoleic acid C 18:2 Trans acid C 18:3 α-Linolenic acid C 20:0 C 22:1 Erucic acid

g per 100g Powder 0.300 12.120 7.840 27.230 9.120 5.642 7.980 0.000 1.425 0.000 0.144 0.000 0.200 72.00

% of total fat 0.4% 16.8% 10.9% 37.8% 12.7% 7.8% 11.1% 0.0% 2.0% 0.0% 0.2% 0.0% 0.3% 100%

MCT Oils Saturated Monounsaturated Polyunsaturated

Preparation and administration

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.

Please note values may vary from label specifications due to rounding differences 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/EC


Precautions

KetoVOLVE®

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

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 fatty acids

Failure to gain weight Low grade acidosis Hypoglycaemia – if the diet is initiated with a fast Increased cholesterol

Indication

Possible formation of renal stones

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:

Nutritional information for KetoVOLVE Per 100g powder Energy kCal

West syndrome/Infantile spasm

KJ

Protein g Carbohydrates g Sugars g Fats

Lennox-Gastaut syndrome GLUT-1 Deficiency syndrome

LCT1 g MCT2 g

Trans Fat g

Doose Syndrome/ MAE – Myoclonic astatic epilepsy

Fibre g Vitamins Vitamin A ug RE Vitamin C mg Vitamin D ug Vitamin E3 mg Vitamin K ug Thiamin (B1) mg Riboflavin (B2) mg Niacin (B3) mg Pyridoxine (B6) mg Folic Acid ug M-Inositol mg Cobalomin (B12) ug Pantothenate (B5) mg D-Biotin ug Choline mg Minerals Calcium4 mg Chromium ug Copper ug Iodine ug Iron mg Magnesium mg Manganese mg Molybdenum ug Phosphorus mg Selenium ug Zinc mg Potassium mg Sodium mg Chloride mg

Dravet Syndrome Pyruvate Dehydrogenase deficiency Mitochondrial deficiencies

Contraindications

KetoVOLVE is contraindicated in individuals with certain metabolic deficiencies:

1. L-Carnitine deficiencies 2. Pyruvate carboxylase deficiencies 3. Porphyria 4. Inborn errors of fat metabolism 5. Diabetes mellitus

1

Long Chain Triglycerides

2

Medium Chain Triglycerides

3

Per 100kCal

700 2926

100 418

14.5 2.5 1.1

2.1 0.4 0.2

57.0 15.0 <0.5

8.1 2.1 <0.07

Nil

Nil

445 75 10 7 55 0.6 0.6 6.7 0.6 200 58 1.2 3 12 250

65.6 10.7 1.4 1.0 7.9 0.1 0.1 0.9 0.1 28.6 8.3 0.2 0.4 1.7 35.7

800 15 440 90 10 100 1 22 550 30 5 889 309 723

114.3 2.1 62.9 12.8 1.4 14.3 0.1 3.1 78.6 4.3 0.7 127 44.1 103.3

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 Fatty acid description C 6:0 Caproic acid C 8:0 Caprylic acid C 10:0 Capric acid C 12:0 Lauric acid C 14:0 Myristic acid C 16:0 Palmitic acid C 18:1 n-6 to n-9 acid C 18:1 Trans acid C 18:2 Linoleic acid C 18:2 Trans acid C 18:3 α-Linolenic acid C 20:0 C 22:1 Erucic acid

g per 100g Powder 0.300 12.120 7.840 27.230 9.120 5.642 7.980 0.000 1.425 0.000 0.144 0.000 0.200 72.00

% of total fat 0.4% 16.8% 10.9% 37.8% 12.7% 7.8% 11.1% 0.0% 2.0% 0.0% 0.2% 0.0% 0.3% 100%

MCT Oils Saturated Monounsaturated Polyunsaturated

Preparation and administration

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.

Please note values may vary from label specifications due to rounding differences 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/EC


Mixing instructions 1. Weigh out prescribed amount of powder and place into sealable container 2. KetoVOLVE dissolves in hot tap water 3. Make up to required volume 4. Stir, shake or whisk vigorously until all the powder is dissolved 5. Remove carefully, chill in refrigerator 6. Stir before use KetoVOLVE (g) 20 50 100 200

Dilution Table Water (ml) Final Volume (ml) 80 100 200 250 400 500 800 1000

Kcal/ml 1.40 1.40 1.40 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 instructions 1. Weigh out prescribed amount of powder and place into sealable container 2. KetoVOLVE dissolves in hot tap water 3. Make up to required volume 4. Stir, shake or whisk vigorously until all the powder is dissolved 5. Remove carefully, chill in refrigerator 6. Stir before use KetoVOLVE (g) 20 50 100 200

Dilution Table Water (ml) Final Volume (ml) 80 100 200 250 400 500 800 1000

Kcal/ml 1.40 1.40 1.40 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


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.

Indication

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

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.

Indication

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

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.


Dosage and application

Due 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 application

Due 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.


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