Suggested guidelines for the diagnosis and management of urea cycle disorders

Table 6 Emergency regimen for protein-free feeding in infants and children

Age	Glucose polymer concentration (% carbohydrates)	Energy/100ml		Suggested daily intake	Feeding frequency
		kcal	kJ
up to 6 m	10	40	167	150ml/kg	2 to 3 hourly oral/bolus
7-12 m	10-15	40-60	167-250	120ml/kg	day and night
1 y	15	60	250	1200ml	or continuous tube
2-9 y	20	80	334	Estimate as indicated^a	feeds using enteral
>10 y	25	100	418	Estimate as indicated^a	feeding pump

Adapted from Clinical Pediatric Dietetics[106]. Grade of recommendation, C-D.
^a For children >10 kg emergency regimen fluid requirements can be calculated as:
11–20 kg: 100ml/kg for the first 10 kg, plus 50ml/kg for the next 10 kg.
>20 kg: 100ml/kg for the first 10 kg, plus 50ml/kg for the next 10 kg, plus 25ml/kg thereafter up to a maximum of 2500ml/day.

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