Illness, surgery or injury - UCD

Acute management consists of a range of actions, from evaluating clinical and biochemical signs to dietary management, IV management up to extracorporeal detoxification, aiming for managing dehydration, correct acidosis and hyperglycemia.

Only dietary measurements will be discussed.

Emergency treatment includes promotion of anabolism using a special high-calorie diet, transient stop of natural protein intake or of the precursor amino acids.
Rehydration, due to cellular dehydration during severe ketoacidosis

Dietary emergency management:

To prevent acute decompensation, home enteral feeding might be indicated, aiming to provide adequate energy in a state of increased demand and prevent endogenous protein catabolism

Example of an emerengy regimen (Baumgartner) during illness and without gastrointestinal symptoms, home emergency feeding management is appropriate.
There should be provision of adequate energy to meet increased metabolic demands and prevent endogenous protein catabolism. Regular review should be conducted by the metabolic specialist team

Emergency regime for protein-free feeding in infants and children²
Age	Glucose polymer concentration % CHO	Kcal/100 ml	Suggested daily fluid volume ml/kg	Feeding frequency
Up to 6 m	10	40	150 ml/kg	2 to 3 hourly oral/bolus day and night or continuous tube feeds using enteral feeding pump
7-12m	10-15	48	120 ml/kg
1y	15	60	1200 ml
2-9y	20	80	*
>10 y	25	100	*

* For children >10 kg emergency regimen fluid requirements can be calculated as:
11-20 kg: 100 ml/kg for the first 10 kg, plus 50 ml/kg for the next 10 kg
20 kg:100 ml/kg for the first 10 kg, plus 50 ml/kg for the next 10 kg, plus 25 ml/kg thereafter up to a maximum of 2500 ml/day

References:

Baumgartner et al. Orphanet Journal of Rare Diseased, 2014;9:130.
Häberle et al. Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet Journal of Rare Diseases 2012;7:32