Table 1.

RQ, and ventilation of subjects following exercise

Sub	Age (yr)	Gender	Mutations		First testt	With MCT		No MCT
						Mean RQ	Steady-state VO2	Mean RQ	Steady-state VO2
1 [5]	7.4	F	c.1528G > C/c.274_278del	LCHAD def.	MCT	NA	NA	NA	NA
2 [5]	8.4	F	c.1528G > C/c.274-278del	LCHAD def.	No MCT	1.05	7.5	0.87	11.6
3*	8.7	F	c.901G> A β-subunit/?	TFP def.	MCT	0.97	18.2	0.86	15.2
4	10.3	M	c.1528G > C/c.1528G > C	LCHAD def.	No MCT	1.06	13.2	1.01	23.4
5	12.6	M	c.1528G > C/?	LCHAD def.	MCT	1.01	24.9	1.05	26.2
6 [7]	12.7	F	c.1528G > C/c.1528G > C	LCHAD def.	No MCT	0.99	14.2	1.07	13.5
7 [6]	13.6	M	c.1528G > C/c.1678C > T	TFP def.	MCT	0.93	14.4	1.01	15.2
8 [5]	14.1	F	c.1528G > C/c.479–482TAGC > AATA	LCHAD def.	No MCT	0.89	19.4	0.94	16.8
9* [8]	14.4	F	c.901G> A β-subunit/?	TFP def.	MCT	0.93	16.3	0.99	17.1
Mean						0.95	17.8	1.01	17.8

Data is given for exercise tests performed following 4 oz of orange juice (No MCT) and following 4 oz orange juice and 0.5 gm/kg lean mass MCT (with MCT). Mutations are given as the change in the cDNA (c.) of the α-subunit of the trifunctional protein unless and otherwise noted. References indicate previous published mutations and

^*indicates siblings. Mean RQ = mean RQ during last 5 min of exercise; steady-state VO₂ = median VO₂ (ml/min/kg) during last 5 min of exercise.