Managing the diet - UCD

Tabel 4: Nutricia Metabolic formula characteristics Example
Product	Anamix
Form	Powder
Availability	400g/can
USE: Scoop or measurements
Recommended age	0-12 months or as a supplementary feed up to 3 years
Added vit/min
Protein equivalent
Carbohydrate
Fat
DHA
Fiber
Energy/kcal

Diet calculations of a newly diagnosed infant^1,2
Diagnosis	UCD
Dietary management in newborn UCD infants is depending on clinical presentation at initiation and centres preference for disease management	The main aim at metabolic stability and normal growth to reduce nitrogen load in the urea cycle The amount of natural whole protein tolerated depends on the severity of the disorder and medication to prevent hyperammonaemia. It is determined individually by monitoring parameters such as growth velocity, disorder nature and severity and frequency of intercurrent illnesses. Clinical practise differs for decisions on the level of natural protein and/or amino acid supplementation.
Protein recommendation	Age adequate and based on bodyweight
Energy provision	Age adequate and based on bodyweight
Volume	Age adequate and based on bodyweight 6-8 feeds in 24 hours/on demand
Essential amino acid infant formula	Standard solution: 15 grams (or 3 scoops) essential amino acid infant formula (UCD Anamix Infant) 3 scoops + 90 mls water provides 100 ml essential amino acid formula
Breast feeding	Mostly 6-8 feeds are advised daily. Consider naso gastric tube feeding at an early stage of inadequate intake to prevent catabolism Techniques for breastfeeding Fixed frequency of breast feeding and essential amino acid infant formula alternating Measured expressed breast milk
Standard infant formula	Preparation of the standard infant formula as indicated. Mostly 6-7 feeds are advised daily, the total daily amount is divided by the amount of feeds. Consider naso gastric tube feeding at an early stage of inadequate intake to prevent catabolism Techniques for bottle feeding Mixing the standard infant formula with the essential amino acid infant formula requires the infant need to finish the full amount and does not get used to the taste of the amino acids, which can be a problem once low protein foods are introduced and the standard infant formula amount is lowered. But it assures a balanced provision of essential amino acids
Frequency of monitoring	Daily, reducing to 2-3 times a week Weighing: daily
Increasing essential amino acid intake	Increase amount of standard formula Or increase frequency of feeding or volume of breast milk
Transition to solid products/weaning	In line with recommendations on food introduction for healthy population Start the introduction on low protein foods gradually in line with local recommendations, like vegetables and fruits Provide the low protein foods after the breast milk or formula feeds Manage energy intake, low protein foods contain often lower energy density than breast or bottle feeds Once 8 – 12 tablespoons are consumed at one time, this can be given instead of the equivalent of the standard infant formula or breast feed. Gradually introduce more texture and lumpier foods, starting at 6-8 months Finger foods can be introduced from 7 months onwards. From 9 months the low protein pasta, low protein bread and potato (products) can be introduced.

References

Dixon M, MacDonald A, White F, Stafford J. Disorders of Amino Acid Metabolism, Organic Acidaemias and Urea Cycle Disorders. In: Clinical Paediatric Dietetics. 4^th ed: Shaw V. West Sussex: John Wiley & Sons Limited, 2014:381-525: 19914636
Häberle et al. Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet Journal of Rare Diseases 2012;7:32