Bile Acid Therapy/Peroxisomal Disorders Affecting Bile Acid Metabolism

Heubi, James E.

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 #: 5M01RR008084-02
• Application #: 3745001
• Support Year: 2
• Fiscal Year: 1995

Institution

• Name: Cincinnati Children's Hospital Medical Center
• DUNS #: 071284913
• City: Cincinnati
• State: OH
• Country: United States
• Zip Code: 45229

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

Lin, Shan; Luo, Roger T; Shrestha, Mahesh et al. (2017) The full transforming capacity of MLL-Af4 is interlinked with lymphoid lineage commitment. Blood 130:903-907

Puopolo, Karen M; Mukhopadhyay, Sagori; Hansen, Nellie I et al. (2017) Identification of Extremely Premature Infants at Low Risk for Early-Onset Sepsis. Pediatrics 140:

Lin, Shan; Ptasinska, Anetta; Chen, Xiaoting et al. (2017) A FOXO1-induced oncogenic network defines the AML1-ETO preleukemic program. Blood 130:1213-1222

James, Jennifer; Munson, David; DeMauro, Sara B et al. (2017) Outcomes of Preterm Infants following Discussions about Withdrawal or Withholding of Life Support. J Pediatr 190:118-123.e4

Peralta-Carcelen, Myriam; Carlo, Waldemar A; Pappas, Athina et al. (2017) Behavioral Problems and Socioemotional Competence at 18 to 22 Months of Extremely Premature Children. Pediatrics 139: