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

Takken, T. ; Custers, J. W.H. ; Visser, G. ; Dorland, L. ; Helders, P. J.M. and De Koning, T. J. LU (2005) In Nutrition and 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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type
Contribution to journal
publication status
published
subject
in
Nutrition and Metabolism
volume
2
article number
12
publisher
Karger
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
2020-03-04 10:27:58
@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    = {Karger},
  series       = {Nutrition and 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},
}