Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiencyReportar como inadecuado

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Nutrition and Metabolism

, 2:12

First Online: 20 May 2005Received: 23 February 2005Accepted: 20 May 2005


BackgroundMultiple Acyl-CoA-Dehydrogenase deficiency MADD is an inherited metabolic disorder characterized by impaired oxidation of fatty acids and some amino acids.

MethodsWe were interested whether children with MADD could tolerate a prolonged low-intensity exercise test and if this test could have any additional diagnostic value. Therefore, we performed a maximal exercise test and a low-intensity prolonged exercise test in 2 patients with MADD and in 5 control subjects. During a prolonged exercise test the subjects exercised on a cycle ergometer at a constant workload of 30% of their maximum for 90 minutes and heart rate, oxygen uptake, fuel utilization and changes in relevant blood and urinary parameters were monitored.

ResultsThe tests were tolerated well. During the prolonged exercise test the fatty acid oxidation FAO was quite low compared to 5 control subjects, while characteristic metabolites of MADD appeared in plasma and urine.

ConclusionWe suggest that the prolonged exercise test could be of diagnostic importance and might replace the fasting test as a diagnostic procedure in some cases, particularly in patients with anamnestic signs of intolerance for prolonged exercise.

Electronic supplementary materialThe online version of this article doi:10.1186-1743-7075-2-12 contains supplementary material, which is available to authorized users.

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Autor: T Takken - JWH Custers - G Visser - L Dorland - PJM Helders - TJ de Koning


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