Abstract

Infants with peroxisomal disorders, including Zellweger's syndrome and infantile adrenoleukodystrophy, commonly have liver disease because of impaired peroxisomal metabolism and accumulation of bile acid intermediates. Using techniques of mass spectrometry, electron microscopy and MRI-spectroscopy, this study is evaluating the role of bile acid therapy (cholic acid) as therapy for the liver disease in peroxisomal disorders of infancy.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
5M01RR008084-03
Application #
5225639
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
3
Fiscal Year
1996
Total Cost
Indirect Cost

  Publications

Natarajan, Girija; Shankaran, Seetha; Laptook, Abbot R et al. (2018) Association between sedation-analgesia and neurodevelopment outcomes in neonatal hypoxic-ischemic encephalopathy. J Perinatol 38:1060-1067
Askie, Lisa M; Darlow, Brian A; Finer, Neil et al. (2018) Association Between Oxygen Saturation Targeting and Death or Disability in Extremely Preterm Infants in the Neonatal Oxygenation Prospective Meta-analysis Collaboration. JAMA 319:2190-2201
DiFrancesco, Mark W; Shamsuzzaman, Abu; McConnell, Keith B et al. (2018) Age-related changes in baroreflex sensitivity and cardiac autonomic tone in children mirrored by regional brain gray matter volume trajectories. Pediatr Res 83:498-505
Autmizguine, Julie; Tan, Sylvia; Cohen-Wolkowiez, Michael et al. (2018) Antifungal Susceptibility and Clinical Outcome in Neonatal Candidiasis. Pediatr Infect Dis J 37:923-929
Jilling, Tamas; Ambalavanan, Namasivayam; Cotten, C Michael et al. (2018) Surgical necrotizing enterocolitis in extremely premature neonates is associated with genetic variations in an intergenic region of chromosome 8. Pediatr Res 83:943-953
Hahn, Andrew D; Higano, Nara S; Walkup, Laura L et al. (2017) Pulmonary MRI of neonates in the intensive care unit using 3D ultrashort echo time and a small footprint MRI system. J Magn Reson Imaging 45:463-471
Kingery, Kathleen M; Narad, Megan E; Taylor, H Gerry et al. (2017) Do Children Who Sustain Traumatic Brain Injury in Early Childhood Need and Receive Academic Services 7 Years After Injury? J Dev Behav Pediatr 38:728-735
Glauser, Tracy A; Holland, Katherine; O'Brien, Valerie P et al. (2017) Pharmacogenetics of antiepileptic drug efficacy in childhood absence epilepsy. Ann Neurol 81:444-453
Durber, Chelsea M; Yeates, Keith Owen; Taylor, H Gerry et al. (2017) The family environment predicts long-term academic achievement and classroom behavior following traumatic brain injury in early childhood. Neuropsychology 31:499-507
Hung, Anna H; Cassedy, Amy; Schultz, Hanna M et al. (2017) Predictors of Long-Term Victimization After Early Pediatric Traumatic Brain Injury. J Dev Behav Pediatr 38:49-57

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