Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency
(2005) In Nutrition & Metabolism 2.- Abstract
Background: Multiple Acyl-CoA-Dehydrogenase deficiency (MADD) is an inherited metabolic disorder characterized by impaired oxidation of fatty acids and some amino acids. Methods: We 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.... (More)
Background: Multiple Acyl-CoA-Dehydrogenase deficiency (MADD) is an inherited metabolic disorder characterized by impaired oxidation of fatty acids and some amino acids. Methods: We 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. Results: The 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. Conclusion: We 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.
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- author
- Takken, T. ; Custers, J. W.H. ; Visser, G. ; Dorland, L. ; Helders, P. J.M. and De Koning, T. J. LU
- publishing date
- 2005-05-20
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Nutrition & Metabolism
- volume
- 2
- article number
- 12
- publisher
- BioMed Central (BMC)
- external identifiers
-
- scopus:23044510127
- ISSN
- 1743-7075
- DOI
- 10.1186/1743-7075-2-12
- language
- English
- LU publication?
- no
- id
- cacd8ffb-ddbc-4638-b428-0519c3a7a255
- date added to LUP
- 2020-03-03 19:05:15
- date last changed
- 2022-04-19 11:08:47
@article{cacd8ffb-ddbc-4638-b428-0519c3a7a255,
abstract = {{<p>Background: Multiple Acyl-CoA-Dehydrogenase deficiency (MADD) is an inherited metabolic disorder characterized by impaired oxidation of fatty acids and some amino acids. Methods: We 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. Results: The 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. Conclusion: We 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.</p>}},
author = {{Takken, T. and Custers, J. W.H. and Visser, G. and Dorland, L. and Helders, P. J.M. and De Koning, T. J.}},
issn = {{1743-7075}},
language = {{eng}},
month = {{05}},
publisher = {{BioMed Central (BMC)}},
series = {{Nutrition & Metabolism}},
title = {{Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency}},
url = {{http://dx.doi.org/10.1186/1743-7075-2-12}},
doi = {{10.1186/1743-7075-2-12}},
volume = {{2}},
year = {{2005}},
}