Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency
<p>Abstract</p> <p>Background</p> <p>Multiple Acyl-CoA-Dehydrogenase deficiency (MADD) is an inherited metabolic disorder characterized by impaired oxidation of fatty acids and some amino acids.</p> <p>Methods</p> <p>We were interested whether ch...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2005-05-01
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| Series: | Nutrition & Metabolism |
| Online Access: | http://www.nutritionandmetabolism.com/content/2/1/12 |
| _version_ | 1811297696014663680 |
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| author | Helders PJM Dorland L Visser G Custers J WH Takken T de Koning TJ |
| author_facet | Helders PJM Dorland L Visser G Custers J WH Takken T de Koning TJ |
| author_sort | Helders PJM |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Background</p> <p>Multiple Acyl-CoA-Dehydrogenase deficiency (MADD) is an inherited metabolic disorder characterized by impaired oxidation of fatty acids and some amino acids.</p> <p>Methods</p> <p>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.</p> <p>Results</p> <p>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.</p> <p>Conclusion</p> <p>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> |
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| format | Article |
| id | doaj.art-9199779f94c8452da6b3f655b9dbf17c |
| institution | Directory Open Access Journal |
| issn | 1743-7075 |
| language | English |
| last_indexed | 2024-04-13T06:08:01Z |
| publishDate | 2005-05-01 |
| publisher | BMC |
| record_format | Article |
| series | Nutrition & Metabolism |
| spelling | doaj.art-9199779f94c8452da6b3f655b9dbf17c2022-12-22T02:59:10ZengBMCNutrition & Metabolism1743-70752005-05-01211210.1186/1743-7075-2-12Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiencyHelders PJMDorland LVisser GCusters J WHTakken Tde Koning TJ<p>Abstract</p> <p>Background</p> <p>Multiple Acyl-CoA-Dehydrogenase deficiency (MADD) is an inherited metabolic disorder characterized by impaired oxidation of fatty acids and some amino acids.</p> <p>Methods</p> <p>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.</p> <p>Results</p> <p>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.</p> <p>Conclusion</p> <p>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>http://www.nutritionandmetabolism.com/content/2/1/12 |
| spellingShingle | Helders PJM Dorland L Visser G Custers J WH Takken T de Koning TJ Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency Nutrition & Metabolism |
| title | Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency |
| title_full | Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency |
| title_fullStr | Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency |
| title_full_unstemmed | Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency |
| title_short | Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency |
| title_sort | prolonged exercise testing in two children with a mild multiple acyl coa dehydrogenase deficiency |
| url | http://www.nutritionandmetabolism.com/content/2/1/12 |
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