1. Medical Metabolic Formulas Every Pediatrician should know
Dr. Nawal Makhseed
MBBS, FAAP, FRCPC, DABMGG, FCCMG
Pediatric Metabolic Consultant
Jahra, Adan, Farwaniya, and Maternity Hospitals

2. Management goals Giving Hope in Treatment Plan results
Organizing patients support groups
Support diet compliance
Raise diet awareness and discussing the varieties !!! A fact ignored by central medical stores
Enhancing disease knowledge again and again and again and again and again and …………..

3. Dietary management of IEM
Restricted dietary management is the core of treating certain IEM
Highly effective treatment in protecting the patient, and leading a normal life
BUT ……
In certain metabolic disorders EMERGENCY management is as important as long term management in protecting the child
The circle goes on and on …… BECAUSE strict Long term management prevents catabolic crisis
What are those diseases ?

4. Teamwork!!!!! Patient Patient Caregiver Metabolic doctor
Doctor on call
Caregiver
Dietitian
Local Doctor
Laborotory
Industry
Food experts
Food experts
Doctor on call
Caregiver
Industry
Patient
Laborotory
Dietitian
Local Doctor

5. Dietary management  deliver patient benefits
Better quality of life
Children catch up in growth
Patients gain strength and energy
Faster recovery during illness
Lower cost of care

6. Medical formulas/food: FSMPs
Formulas/Foods for Special Medical Purposes or medical foods intended for patients who have been medically diagnosed and must be used under medical supervision.
A fact ………………………………………………………..????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????????
Ignored by ???????????????????????????????

7. b-Energy containing formulas 2-Avoidance diet: Fat/ lactose/Fructose
How?
Condition specific nutritional treatment
Non- condition specific nutritional support
Low protein products
1- Avoidance diet: protein disorders a-Concentrated protein containing formulas
b-Energy containing formulas
2-Avoidance diet: Fat/ lactose/Fructose
FAOD/ Galactosemia/ Fructose intolerance
Providing missing elements: Carbohydrate disorders
GSD
Gluconeogenesis dis
Vitamins/ minerals
Individual amino acids / mixed AA
Essential fatty acids
Oli: Medium chain TG
Flavor packs
Engineered foods
Many in the market

8. Principles are clear Avoid offending compounds
Provide deficient product
Provide adequate energy to prevent catabolism
Make sure all nutritional elements are met to ensure healthy and normal growth

9. Dietary management: Amino acids disorders
Natural protein restriction
Supplementation of the AA whose breakdown is blocked due to the metabolic defect, its recommended to divide the prescribed amount throughout the day
Supplementation of the other nutrients
Supplement adequate energy

10. Natural protein Breast feeding either directly or as EBM
Every 100 ml EBM:
contains 1 gram protein
72 calories
Baby formulas
Mainly use Nan1
Every 100 grams powder contains 9.6 grams protein
Each scoop measure 4.3 grams:
0.3 grams protein
22.3 Kcal

11. Amino acids supplementation
Supplementation of the amino acids whose breakdown is blocked……. ?
Some are essential amino acids that the body needs to meet growth requirement and other molecules production
There are several companies that does those special amino acid mixtures
Nutricia
Vitaflo
Others

12. Nutricia Amino acid mixtures
Used to be names according to the absence of the offending amino acid
XP = NO pheynalanine
XP Analog = from Zero to 1 year (per 100 grams powder: 13.1 grams protein, 457 Kcal)
XP Maximaid = from 1-8 years ( per 100 grams powder: 25 grams protein, 339 Kcal)
XP Maximum = from 8 years onward (per 100 grams powder: 39 grams, 309 Kcal)
What is the difference between each type is
Protein content
Caloric intake

13. Nutricia PKU range – Infant/young child
Per 36 gm sachet
457
135 10

14. Nutricia PKU range – older child/adult
297 39

15. MMA/PPA formulas XMTVI Analog XMTVI Maxamaid (1 – 8 years)
XMTVI Maxamum (From 8 years)
Methionine-, threonine-, valine-free and
isoleucine –low powdered drink mix, containing amino acids, carbohydrate, vitamins, minerals and trace elements
MMA PA Anamix Infant
isoleucine –low powdered infant formula
Supplemented with DHA & ARA and prebiotic fibres
XMTVI Asadon
isoleucine –low powdered amino acid mix

16. MSUD– infants (Nutricia)
MSUD Anamix Infant
Isoleucine-, leucine-, and valine- free powdered infant formula
Supplemented with DHA & ARA and prebiotic fibres
MSUD Anamix Junior (1-10 years)
Unflavoured isoleucine-, leucine- and valine-free powder drink mix containing amino acids, carbohydrate, fat, vitamins, minerals and trace elements
Convenient pre-weighed sachet presentation
10 gm Protein/ 135 Kcal per 36g sachet
Contains essential fatty acids (LA and alpha-LNA)
MSUD Anamix Junior LQ (From 1 year)
Orange flavour isoleucine-, leucine and valine-free drink containing amino acids, carbohydrate, fat, vitamins minerals and trace elements
Contains DHA
Ready to drink
10g Protein per 125ml bottle

17. MSUD– older children and adults (Nutricia)
MSUD Lophlex LQ (from 4 years)
Ready to drink, isoleucine-, leucine- and valine-free protein substitute with vitamins, minerals and trace elements
20 g Protein/ 125 Kcal per 125ml pack
Contains DHA
Juicy berries flavour

18. IVA (Nutricia) IVA Anamix Infant
Leucine-free and powdered infant formula
Supplemented with DHA & ARA and prebiotic fibres
5 grams scoop
XLEU Analog /Maxamaid/ Maximum
Leucine-free powdered amino acids formula
Containing carbohydrate, vitamins, minerals
and trace elements
XLEU Faladon
Leucine-free powdered formula

19. Glutaric Aciduria type 1 (Nutricia)
GA1 Anamix Infant
Lysine free, low tryptophan powdered infant formula
Supplemented with DHA & ARA and prebiotic fibres
5 grams scoop
XLYS Low TRY Maxamaid (1 – 8 years)
XLYS Low TRY Maxamum (From 8 years)
Lysine free, low tryptophan powdered drink mix,
containing amino acids, carbohydrate, vitamins,
minerals and trace elements
XLYS Low TRY Glutaridon
Lysine free, low tryptophan powdered amino acid mix

20. Homocystinuria infants and young children (Nutricia)
HCU Anamix Infant
Methionine free powdered infant formula
Supplemented with DHA & ARA and prebiotic fibres
5 grams scoop
HCU Anamix Junior LQ (from 1 year)
Orange flavour methionine-free drink containing amino acids, carbohydrate, fat, vitamins minerals and trace elements
Ready to drink
Contains DHA
10g Protein/ 125 Kcal per 125ml bottle

21. Homocystinuria: older children & adults (Nutricia)
XMET Maxamaid (1 – 8 years) XMET Maxamum (From 8 years) Methionine free powdered drink mix, containing amino acids, carbohydrate, vitamins, minerals and trace elements XMET Homidon Methionine free powdered amino acid mix

22. Homocystinuria: older children & adults (Nutricia)
HCU Lophlex LQ (from 4 years)
Ready to drink, methionine-free protein substitute with vitamins, minerals and trace elements
20g Protein/ 125 Kcal per 125ml pack
Contains DHA
Juicy berries flavour
HCU LV (HCU Lophlex Advance) (from 8 years)
Concentrated methionine free powdered protein
substitute with vitamins, minerals and trace elements
20g Protein/ 92 Kcal per 27.8 gm sachet

23. Tyrosinaemia - infants (Nutricia)
TYR Anamix Infant
Tyrosine and phenylalanine -free powdered infant formula
Supplemented with DHA & ARA and prebiotic fibres
5 grams scoop
Phe/Tyrosine and Methionine-free TYR Anamix Infant also available for the dietary management of tyrosinaemia when plasma methionine levels are raised.

24. Tyrosinaemia - young children (Nutricia)
TYR Anamix Junior (1-10 years)
Unflavoured tyrosine- and phenylalanine-free powder drink mix containing amino acids, carbohydrate, fat, vitamins, minerals and trace elements
Convenient pre-weighed sachet presentation
8.4g PE per 29g sachet
Contains essential fatty acids (LA and alpha-LNA)
TYR Anamix Junior LQ (from 1 year)
Orange flavour tyrosine and phenylalanine free drink containing amino acids, carbohydrate, fat, vitamins minerals and trace elements
Contains DHA
Ready to drink
10g Protein/ 125 Kcal per 125ml bottle

25. Tyrosinaemia– older children and adults (Nutricia)
TYR Lophlex LQ (from 4 years)
Ready to drink, tyrosine- and phenylalanine-free protein substitute with vitamins, minerals and trace elements
20g Protein/120 Kcal per 125ml pack
Contains DHA
Juicy berries flavour

26. Urea Cycle Disorders infant Anamix infant
UCD Anamix infant
Nutritionally complete
All Essential Increased levels of BCAAs (branched chain amino acids)
Additional energy from fats and carbohydrates
Full micronutrient profile
Added LCPUFAs e.g. DHA
Fat source consists of a mixture of vegetable oils (olive, coconut and walnut oils)
Used in combination with standard infant
formula or breast milk
– never sole source of nutrition

27. Essential Amino Acid Mix
Essential Amino Acids mix is a powdered mix of essential amino acids.
For use as a supplementary protein source, where essential amino acids only are required, rather than the whole protein or peptides
Essential Amino Acid Mix may be used for those on low protein diets requiring essential amino acid supplementation and in the dietary management of certain urea cycle disorders and Arginase deficiency. 8

28. Urea cycle disorders > 6 Months of age
Dialamine is a pale orange powder containing carbohydrate, essential amino acids, flavouring and inherently low levels of minerals.
For the dietary management of urea cycle disorders, where an essential amino acid supplement is required. 8

29. OLD Monogen: 80% MCT and 20% LCT Nutritional content per 100 gm powder
Energy
420 Kcal
Protein
12.5 gm
Carbohydrate
68 gm
Fat in 100 gm powder
MCT (80%) (84%)
LCT (20%) (16%)
11 gm (12.9)
8.8 gm (10.84)
gm (2 gm)
Ratio n6:n3 fatty acids 4.6:1
% from energy
-linoleic acid
-α-linolenic acid
1.0 %
0.2 1 %

30. Additional energy supplementation
Carbohydrate modules : (UCD, organic acidemia, FAOD) (NOT in patients who needs ketogenic diet)
Maxijul Super Soluble (suitable from birth)
Polycal powder (suitable from birth)
Polycal Liquid (from 3 years up) (Not available)

31. Additional energy supplementation
Carbohydrate + fat modules: BUT Contraindicated in FAOD, avoided in Ketogenic diet
Duocal Super Soluble (suitable from birth)
Duocal Liquid (suitable from birth) (Not available)

32. Additional energy supplementation
Fat modules:
Medium chain triglycerides (MCT): : BUT Contraindicated in MCAD/MAAD
MCT oil (suitable from birth)
Liquigen (suitable from birth)
Long chain triglycerides (LCT): BUT Contraindicated in FAOD
Calogen Neutral (suitable from birth)
Calogen flavoured (suitable from 3 years up)

33. Additional energy supplementation Vitaflo
Neutral-tasting food fortifier and MCT supplement
Each 16 g packet: 10.1 gm MCT

34. Emergency/ Energy supply
SOS, color coded
10, 15, 20, 25 %
Used in all metabolic emergencies
Except in those on ketogenic diet

35. Vitalfo Cooler system

36. Vitaflo Cooler system

37. Cooler system

38. Express gel

39. Gel

40. UCCS with sustained release Lasting up to 8-10 hours GSD/ FAOD/Gluconeogenesis

41. Essential amino acids

42. Protein free milk
Used for any disorder that needs to be treated with a protein restricted diet

43. Dietary management Team work Many formulas
Dietary management has to be reviewed in each visit as compliance is the golden standard of managing these patients
Meet patient’s need according to his progress, Compliance, tolerability, adequate intake, growth, new circumstances ….etc
Always available to review things