Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency

Nutrition & Metabolism

Table 2B Results prolonged exercise test subject 2.

	T = 0	T = 30	T = 60	T = 75	T = 90	T = 120
HR (b·min^-1)	80	120	120	120	120
VO₂ (ml·min^-1)	260	450	620	620	600
RER (VCO₂·VO₂^-1)	0.9	0.9	0.95	0.95	0.9
Glucose (mmol·l^-1)	5.2	4.6	4.3	4.4		4.5
Lactate (mmol·l^-1)	2.0	0.9	0.9	1.0		1.2
CK (u·l^-1)	58		80	78		78
3-keto-B (mmol·l^-1)	<0.05	<0.05	0.07			0.14
3-OH-B (mmol·l^-1)	<0.02	<0.02	0.04			0.24
FFA (umol·l^-1)	0.15	0.31	0.62	0.91		1.34
Urine
Ethylmalonic acid in mmol·mol^-1 creatinine	T = -60–0: 29					T = 120–300: 59

Abbreviations: HR: heart rate, RER: Respiratory Exchange Ratio, VO₂: oxygen uptake, CK: creatine kinase, 3-keto-B: 3-ketobutyric acid, 3-OH-B: 3-hydroxybutyric acid, FFA: Free Fatty Acids.

ISSN: 1743-7075