Abstract

The purpose of this study is to determine whether oral docosahexaenoic acid (DHA) supplementation is beneficial to patients with Long-Chain-3-Hydroxyacyl -coA Dehydrogenase Deficiency (LCHADD), an inborn error in fatty acid metabolism. The following hypothesis is to be tested: Supplementation of DHA, as an adjunct to treatment with a low fat, medium-chain triglyceride (MCT) supplemented diet, will retard or prevent the early development of retinal degeneration in patients with LCHADD. Patients will be supplemented with DHA for one year. DHA status will be measured by plasma and red blood cell fatty acid analysis. Deficient DHA levels will be normalized and maintained throughout the supplementation period. Electroretinograms (ERG) and visual evoked potentials (VEP) will be measured at baseline, after six months of supplementation and after twelve months supplementation. Efficacy will be measured by normalization of plasma levels of DHA and improvement in ERG and/or VEP results.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
5M01RR003186-14
Application #
6121707
Study Section
Project Start
1998-12-01
Project End
1999-11-30
Budget Start
1998-10-01
Budget End
1999-09-30
Support Year
14
Fiscal Year
1999
Total Cost
Indirect Cost

  Institution

Name
University of Wisconsin Madison
Department
Type
DUNS #
161202122
City
Madison
State
WI
Country
United States
Zip Code
53715

  Publications

Burgess-Hull, Albert J; Roberts, Linda J; Piper, Megan E et al. (2018) The social networks of smokers attempting to quit: An empirically derived and validated classification. Psychol Addict Behav 32:64-75
Kelly, Elizabeth A; Esnault, Stephane; Liu, Lin Ying et al. (2017) Mepolizumab Attenuates Airway Eosinophil Numbers, but Not Their Functional Phenotype, in Asthma. Am J Respir Crit Care Med 196:1385-1395
Shen, Zhong-Jian; Hu, Jie; Kashi, Venkatesh P et al. (2017) Epstein-Barr Virus-induced Gene 2 Mediates Allergen-induced Leukocyte Migration into Airways. Am J Respir Crit Care Med 195:1576-1585
Anderson, Halie M; Lemanske Jr, Robert F; Evans, Michael D et al. (2017) Assessment of wheezing frequency and viral etiology on childhood and adolescent asthma risk. J Allergy Clin Immunol 139:692-694
Gomez, Jose L; Yan, Xiting; Holm, Carole T et al. (2017) Characterisation of asthma subgroups associated with circulating YKL-40 levels. Eur Respir J 50:
Kelly, Elizabeth A; Esnault, Stephane; Johnson, Sean H et al. (2016) Human eosinophil activin A synthesis and mRNA stabilization are induced by the combination of IL-3 plus TNF. Immunol Cell Biol 94:701-8
Bray, Bethany C; Smith, Rachel A; Piper, Megan E et al. (2016) Transitions in Smokers' Social Networks After Quit Attempts: A Latent Transition Analysis. Nicotine Tob Res 18:2243-2251
Dougherty, Ryan J; Ellingson, Laura D; Schultz, Stephanie A et al. (2016) Meeting physical activity recommendations may be protective against temporal lobe atrophy in older adults at risk for Alzheimer's disease. Alzheimers Dement (Amst) 4:14-7
Johansson, Mats W; Evans, Michael D; Crisafi, Gina M et al. (2016) Serum periostin is associated with type 2 immunity in severe asthma. J Allergy Clin Immunol 137:1904-1907.e2
Lee, Yong Gyu; Jeong, Jong Jin; Nyenhuis, Sharmilee et al. (2015) Recruited alveolar macrophages, in response to airway epithelial-derived monocyte chemoattractant protein 1/CCl2, regulate airway inflammation and remodeling in allergic asthma. Am J Respir Cell Mol Biol 52:772-84

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