Table 2.

Energy and body composition data. Resting energy expenditure (REE) as measured by indirect calorimetry (gold standard) and as predicted by the FAO/WHO/UNU in subjects with PKAN and controls is given as well as comparison between the two and normalized to fat free mass (FFM). Respiratory quotient given for PKAN subjects using indirect calorimetry. Body composition done by bioelectrical impedance for PKAN subjects but not available for controls.

		PKAN participants
		All	Ages 18 & up	Ages 18 & under	Females	Males
Gold-standard measurements of REE (mREE) and FFM
Indirect calorimetry:	REE (kcal/day)	1570 ± 310	1610 ± 266	1500 ± 393	1401 ± 209	1810 ± 248
	RQ	0.90 ± 0.04	0.89 ± 0.04	0.91 ± 0.04	0.89 ± 0.03	0.91 ± 0.05
Bioelectrical impedance:	FM (kg)	13.2 ± 8.90	15 ± 10	10 ± 6	13 ± 11	13 ± 4
	FFM (kg)	45.3 ± 15.2	50 ± 14	36 ± 14	38 ± 10	58 ± 14
REE normalized to FFM	REE (kcal/day)/FFM (kg)	36.9 ± 7.27	33.6 ± 4.62	43.4 ± 7.51	37.8 ± 5.22	35.4 ± 10.0
Prediction equation of REE (pREE)
FAO/WHO/UNU	REE (kcal/day)	1450 ± 300	1490 ± 296	1386 ± 323	1280 ± 193	1740 ± 205
Comparing mREE to pREE
Predicted as % of measured	(pREE/mREE) * 100	108.7 ± 10.6%	109.0 ± 10.6%	108.1 ± 11.5%	110 ± 9.7%	106.5 ± 12.5%
			Control participants
			All	Females		Males
Gold-standard measurements of REE (mREE) and FFM
Indirect calorimetry:	REE (kcal/day)		1466.1 ± 266.5	1328.8 ± 170.3		1680.6 ± 249.3
	RQ
Bioelectrical impedance:	FM (kg)
	FFM (kg)
REE normalized to FFM	REE (kcal/day)/FFM (kg)
Prediction equation of REE (pREE)
FAO/WHO/UNU	REE (kcal/day)		1463.3 ± 243.0	1317.5 ± 141		1690.0 ± 287.3
Comparing mREE to pREE
Predicted as % of measured	(pREE/mREE) * 100		100.5 ± 10.2%	101.1 ± 9.6%		99.6 ± 11.4%

Footnotes: mREE = measured resting energy expenditure; FM = fat mass; FFM = fat free mass; pREE = predicted resting energy expenditure.