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

Nutrition & Metabolism

Table 2A Results prolonged exercise test subject 1

	T = 0	T = 30	T = 60	T = 75	T = 90	T = 120
HR (b·min^-1)	70	140	145	145	150
VO₂ (ml·min^-1)	300	700	700	720	720
RER (VCO₂·VO₂^-1)	0.8	0.95	0.95	0.9	0.9
Glucose (mmol·l^-1)	7.1	5.6	4.9	4.8	5.5	4.8
Lactate (mmol·l^-1)	1.1	1.2	1.0	1.3	1.1	1.0
CK (u·l^-1)	103		98	105	119	117
3-keto-B (mmol·l^-1)	0.07	<0.05	<0.05	0.05	0.1	0.15
3-OH-B (mmol·l^-1)	0.09	<0.02	<0.02	0.05	0.12	0.26
FFA (umol·l^-1)	0.76	0.28	0.49	0.88	1.03	0.93
Urine
Ethylmalonic acid in Mmol·mol^-1 creatine	T = -60–0: 16					T = 120–300: 74

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