Dichloroacetate (DCA) appears to have some beneficial effect in patients with mitochondrial electron transport chain defects. This medication acts by stimulating the activity of the pyruvate dehydrogenase complex enzyme. The stimulation of this enzyme results in increased metabolism of pyruvate to acetyl CoA and away from lactate production. As such, it has been postulated that this medication would benefit patients with conditions such as mitochondrial cytopathies that result in elevated lactate levels. Although the clinical use of this medication has been limited, certain side effects have been identified. These have included an alteration of blood glucose metabolism such that hypoglycemia may result. Alterations in nerve conduction velocity have also been reported. The hypoglycemia effects of this drug appear to be more prominent in the initial phases of treatment and for this reason patients are monitored closely in the hospital setting with frequent blood glucose evaluations. it is also recommended that nerve conduction velocities be documented prior to the onset of this treatment and at regular intervals thereafter. The effect on nerve conduction is reportedly dose-related and a reduction in nerve conduction velocity would necessitate a decrease and possibly a cessation of this therapy. The patient will be followed at regular intervals with neurological examinations and nerve conduction studies.

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
3M01RR002172-17S2
Application #
6220607
Study Section
Project Start
1998-12-01
Project End
1999-11-30
Budget Start
1998-10-01
Budget End
1999-09-30
Support Year
17
Fiscal Year
1999
Total Cost
Indirect Cost
Name
Children's Hospital Boston
Department
Type
DUNS #
076593722
City
Boston
State
MA
Country
United States
Zip Code
02115
Cassidy, Adam R; Bernstein, Jane Holmes; Bellinger, David C et al. (2018) Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease. Clin Neuropsychol :1-19
Bean Jaworski, Jessica L; White, Matthew T; DeMaso, David R et al. (2018) Visuospatial processing in adolescents with critical congenital heart disease: Organization, integration, and implications for academic achievement. Child Neuropsychol 24:451-468
Hron, Bridget M; Ebbeling, Cara B; Feldman, Henry A et al. (2017) Hepatic, adipocyte, enteric and pancreatic hormones: response to dietary macronutrient composition and relationship with metabolism. Nutr Metab (Lond) 14:44
Rollins, Caitlin K; Asaro, Lisa A; Akhondi-Asl, Alireza et al. (2017) White Matter Volume Predicts Language Development in Congenital Heart Disease. J Pediatr 181:42-48.e2
Sakai Bizmark, Rie; Chang, Ruey-Kang R; Tsugawa, Yusuke et al. (2017) Impact of AHA's 2007 guideline change on incidence of infective endocarditis in infants and children. Am Heart J 189:110-119
Selamet Tierney, Elif Seda; Hollenbeck-Pringle, Danielle; Lee, Caroline K et al. (2017) Reproducibility of Left Ventricular Dimension Versus Area Versus Volume Measurements in Pediatric Patients With Dilated Cardiomyopathy. Circ Cardiovasc Imaging 10:
Cousminer, Diana L; Widén, Elisabeth; Palmert, Mark R (2016) The genetics of pubertal timing in the general population: recent advances and evidence for sex-specificity. Curr Opin Endocrinol Diabetes Obes 23:57-65
Keerthy, Divya; Youk, Ada; Srinath, Arvind I et al. (2016) Effect of Psychotherapy on Health Care Utilization in Children With Inflammatory Bowel Disease and Depression. J Pediatr Gastroenterol Nutr 63:658-664
Cassidy, Adam R; White, Matthew T; DeMaso, David R et al. (2016) Processing speed, executive function, and academic achievement in children with dextro-transposition of the great arteries: Testing a longitudinal developmental cascade model. Neuropsychology 30:874-885
Faden, Maheer; Holm, Mari; Allred, Elizabeth et al. (2016) Antenatal glucocorticoids and neonatal inflammation-associated proteins. Cytokine 88:199-208

Showing the most recent 10 out of 463 publications