L-alanine supplementation in Pompe disease (IOPD): a potential therapeutic implementation for patients on ERT? A case report

Table 2 REE parameters analysis obtained from indirect calorimetry (IC) compared to prediction with commonly used equations

		T0	T1	T2	Percentage variation (T0-T2)
IC-REE (kcal/day)		1265	1236	1219	-3.6
^£Harris Benedict-REE( Kcal/day)		1142	1153	1166	+ 2.1
^§Schofield-REE (Kcal/day)		1061	1083	1106	+ 4.2
^¤WHO-REE (Kcal/day)		1133	1158	1182	+ 4.3
IC REE/predictive equation (%)	Harris Benedict	11.1	10.7	10.5	-5.4
	Schofield	11.9	11.4	11.0	-7.6
	WHO	11.2	10.7	10.3	-8.0

T0: baseline pre-LAOS; T1: after 6 months of LAOS start at 15 g/day; T2: after 3 months of increasing LAOS dosage (18 g/day) for a total of 9 months supplementation. Notes: IC-REE: REE measured by indirect calorimetry; %IC-REE/predictive equation: ratio of IC-REE to predicted equations; £ Harris Benedict-REE: Harris JA, Benedict FG. A biometric study of basal metabolism in man. Washington: Carnegie Institute of Washington;1919. § Schofield-REE: Schofield WN. Predicting basal metabolic rate, new standards and review of previous work. Hum Nutr Clin Nutr 1985;39:5–41. ¤ WHO-REE: World Health Organization. Energy and protein requirements, Report of a joint FAO/WHO/UNU expert consultation. Geneva: World Health Organization;1985

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