Orphanet Journal of Rare Diseases

Table 1 Energy delivery for each case per day of admission in intensive care, separated as IV (intravenous) and estimated food energy and total for case 1a, CACTD, 1b HMGCL2D and 1c CPT2D. Estimated energy requirement for neonates and infants is 100 Kcal / Kg /day and for adolescent case 1B approximately 50 Kcals / Kg / day

From: The use of sodium DL-3-Hydroxybutyrate in severe acute neuro-metabolic compromise in patients with inherited ketone body synthetic disorders

a
Day	4	5	6	7	8	9	10
IV Energy (Kcal)	45	110	95	84	38	16	100
Food energy (Kcal)		11	27	76	172	206	29
Total energy (Kcal)	45	121	122	160	210	222	128
Kcal/Kg	17	47	47	62	81	85	49
D,L-3-OHB (mg/kg/day)		300	600	600	600	600	600
b
Day	1	2	3	4	5	6	7
IV Energy (Kcal)	200	510	340	200	0	0	0
Food energy (Kcal)	0	250	1500	2090	1680	2500	2500
Total energy (Kcal)	200	760	1840	2290	1680	2500	2500
Kcal/Kg	4	15	37	46	34	50	50
D,L-3-OHB (mg/kg/day)	0	0	0	600	600	600	600
c
Day	1 (12 h after admission	2	3	4	5	6	7
IV Energy (Kcal)	84	76	0	100	0	0	0
Food energy (Kcal)	11	427	517	442	550	551	472
Total energy (Kcal)	95	503	517	542	550	551	472
Kcal/Kg	21	109	112	118	120	120	103
D,L-3-OHB (mg/kg/day)	330	978	815	978	978	978	815

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ISSN: 1750-1172

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