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Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency
T Takken1, Jwh Custers, G Visser
1Department of Pediatric Physical Therapy & Exercise Physiology, University Hospital for Children and Youth 'Het Wilhelmina Kinderziekenhuis', University Medical Centre Utrecht, Utrecht, The Netherlands. t.takken@umcutrecht.nl.
Nutrition & Metabolism
|May 24, 2005
Summary
Children with Multiple Acyl-CoA-Dehydrogenase deficiency (MADD) can tolerate prolonged low-intensity exercise. This test reveals low fatty acid oxidation and MADD metabolites, suggesting its diagnostic value for metabolic disorders.
Area of Science:
- Biochemistry
- Exercise Physiology
- Metabolic Disorders
Background:
- Multiple Acyl-CoA-Dehydrogenase deficiency (MADD) is an inherited metabolic disorder.
- MADD impairs the oxidation of fatty acids and certain amino acids.
Purpose of the Study:
- To assess exercise tolerance in children with MADD.
- To determine the diagnostic value of a prolonged low-intensity exercise test for MADD.
Main Methods:
- Performed maximal and prolonged low-intensity exercise tests on 2 MADD patients and 5 controls.
- Monitored heart rate, oxygen uptake, fuel utilization, and biochemical parameters during a 90-minute exercise test at 30% maximum workload.
Main Results:
- MADD patients tolerated the prolonged exercise test well.
- Significantly lower fatty acid oxidation (FAO) was observed in MADD patients compared to controls.
- Characteristic MADD metabolites were detected in plasma and urine during the test.
Conclusions:
- Prolonged low-intensity exercise tests are well-tolerated by MADD patients.
- This exercise test can aid in diagnosing MADD by revealing impaired FAO and specific metabolites.
- The test may serve as an alternative to fasting tests for MADD diagnosis, especially in patients with exercise intolerance history.