Metabolic control during exercise with and without medium-chain triglycerides (MCT) in children with long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency

Molecular Genetics and Metabolism

Volumn 89, Issue 1-2, 2006, Pages 58-63

  Gillingham, Melanie B ^a,b   Scott, Bradley ^b   Elliott, Diane ^b   Harding, Cary O ^a,b  

^a OREGON HEALTH AND SCIENCE UNIVERSITY   (United States)

^b OREGON HEALTH AND SCIENCE UNIVERSITY   (United States)

Author keywords

Oxidation; Exercise; Fatty acid oxidation; Long chain 3 hydroxyacyl coA dehydrogenase; Long chain 3 hydroxyacyl coA dehydrogenase deficiency; Medium chain triglyceride; Rhabdomyolysis; Trifunctional protein; Trifunctional protein deficiency

Indexed keywords

3 HYDROXYBUTYRIC ACID; ACYLCARNITINE; CREATINE KINASE; LACTIC ACID; LONG CHAIN ACYL COENZYME A DEHYDROGENASE; MEDIUM CHAIN TRIACYLGLYCEROL; MITOCHONDRIAL PROTEIN;

ADOLESCENT; ARTICLE; CHILD; CLINICAL ARTICLE; CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; CONTROLLED STUDY; CREATINE KINASE BLOOD LEVEL; DIET SUPPLEMENTATION; DIETARY INTAKE; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ELECTROCARDIOGRAPHY MONITORING; ENERGY RESOURCE; ENZYME BLOOD LEVEL; ENZYME DEFICIENCY; EXERCISE TOLERANCE; FEMALE; HEART RATE; HUMAN; HYPOTHESIS; LACTATE BLOOD LEVEL; LEAN BODY WEIGHT; LONG CHAIN ACYL COENZYME A DEHYDROGENASE DEFICIENCY; LUNG GAS EXCHANGE; MALE; METABOLIC REGULATION; MITOCHONDRIA TRIFUNCTION PROTEIN DEFICIENCY; NUTRITIONAL SUPPORT; ORANGE JUICE; PREDICTION; PRIORITY JOURNAL; RANDOMIZED CONTROLLED TRIAL; RESPIRATORY QUOTIENT; RHABDOMYOLYSIS; RISK ASSESSMENT; STEADY STATE; TREADMILL EXERCISE;

3-HYDROXYACYL COA DEHYDROGENASES; 3-HYDROXYBUTYRIC ACID; ADOLESCENT; CARNITINE; CHILD; CREATINE KINASE; EXERCISE; FEMALE; HUMANS; LACTIC ACID; MALE; MULTIENZYME COMPLEXES; RHABDOMYOLYSIS; TRIGLYCERIDES;

EID: 33747011078     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2006.06.004     Document Type: Article

References (17)

1. Jeukendrup A.E., Saris W.H., and Wagenmakers A.J. Fat metabolism during exercise: a review-part II: regulation of metabolism and the effects of training. Int. J. Sports Med. 19 (1998) 293-302
2. Odle J. New insights into the utilization of medium-chain triglycerides by the neonate: observations from a piglet model. J. Nutr. 127 (1997) 1061-1067
3. Odle J., Benevenga N.J., and Crenshaw T.D. Utilization of medium-chain triglycerides by neonatal piglets: II. Effects of even- and odd-chain triglyceride consumption over the first 2 days of life on blood metabolites and urinary nitrogen excretion. J. Anim. Sci. 67 (1989) 3340-3351
4. Gillingham M.B., Weleber R.G., Neuringer M., Connor W.E., Mills M., van Calcar S., Ver Hoeve J., Wolff J., and Harding C.O. Effect of optimal dietary therapy upon visual function in children with long-chain 3-hydroxyacyl CoA dehydrogenase and trifunctional protein deficiency. Mol. Genet. Metab. 86 (2005) 124-133
5. Ibdah J.A., Bennett M.J., Rinaldo P., Zhao Y., Gibson B., Sims H.F., and Strauss A.W. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N. Engl. J. Med. 340 (1999) 1723-1731
6. Isaacs Jr. J.D., Sims H.F., Powell C.K., Bennett M.J., Hale D.E., Treem W.R., and Strauss A.W. Maternal acute fatty liver of pregnancy associated with fetal trifunctional protein deficiency: molecular characterization of a novel maternal mutant allele. Pediatr. Res. 40 (1996) 393-398
7. Spiekerkoetter U., Sun B., Khuchua Z., Bennett M.J., and Strauss A.W. Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations. Hum. Mutat. 21 (2003) 598-607
8. Treem W.R., Rinaldo P., Hale D.E., Stanley C.A., Millington D.S., Hyams J.S., Jackson S., and Turnbull D.M. Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. Hepat. 19 (1994) 339-345
9. Anderson S.D., and Godfrey S. Cardio-respiratory response to treadmill exercise in normal children. Clin. Sci. 40 (1971) 433-442
10. Gilliam T.B., Sady S., Thorland W.G., and Weltman A.L. Comparison of peak performance measures in children ages 6 to 8, 9 to 10, and 11 to 13 years. Res Q. 48 (1977) 695-702
11. Godfrey S., Davies C.T., Wozniak E., and Barnes C.A. Cardio-respiratory response to exercise in normal children. Clin. Sci. 40 (1971) 419-431
12. Lagerstedt S.A., Hinrichs D.R., Batt S.M., Magera M.J., Rinaldo P., and McConnell J.P. Quantitative determination of plasma c8-c26 total fatty acids for the biochemical diagnosis of nutritional and metabolic disorders. Mol. Genet. Metab. 73 (2001) 38-45
13. Eriksson B.O., and Koch G. Effect of physical training on hemodynamic response during submaximal and maximal exercise in 11-13-year old boys. Acta Physiol. Scand. 87 (1973) 27-39
14. Odle J., Benevenga N.J., and Crenshaw T.D. Utilization of medium-chain triglycerides by neonatal piglets: chain length of even- and odd-carbon fatty acids and apparent digestion/absorption and hepatic metabolism. J. Nutr. 121 (1991) 605-614
15. Gillingham M.B., Connor W.E., Matern D., Rinaldo P., Burlingame T., Meeuws K., and Harding C.O. Optimal dietary therapy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Mol. Genet. Metab. 79 (2003) 114-123
16. Duran M., Wanders R.J., de Jager J.P., Dorland L., Bruinvis L., Ketting D., Ijlst L., and van Sprang F.J. 3-Hydroxydicarboxylic aciduria due to long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency associated with sudden neonatal death: protective effect of medium-chain triglyceride treatment. Eur. J. Pediatr. 150 (1991) 190-195
17. Shen J.J., Matern D., Millington D.S., Hillman S., Feezor M.D., Bennett M.J., Qumsiyeh M., Kahler S.G., Chen Y.T., and Van Hove J.L. Acylcarnitines in fibroblasts of patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and other fatty acid oxidation disorders. J. Inherit. Metab. Dis. 23 (2000) 27-44