Cognitive and adaptive functioning after liver transplantation for maple syrup urine disease: A case series
- 8 October 2010
- journal article
- Published by Wiley in Pediatric Transplantation
- Vol. 15 (1), 58-64
- https://doi.org/10.1111/j.1399-3046.2010.01411.x
Abstract
Shellmer DA, DeVito Dabbs A, Dew MA, Noll RB, Feldman H, Strauss KA, Morton DH, Vockley J, Mazariegos GV. Cognitive and adaptive functioning after liver transplantation for maple syrup urine disease: A case series.Pediatr Transplantation 2011: 15:58–64. © 2010 John Wiley & Sons A/S. MSUD is a complex metabolic disorder that has been associated with central nervous system damage, developmental delays, and neurocognitive deficits. Although liver transplantation provides a metabolic cure for MSUD, changes in cognitive and adaptive functioning following transplantation have not been investigated. In this report, we present data from 14 patients who completed cognitive and adaptive functioning testing pre- and one yr and/or three yr post-liver transplantation. Findings show either no significant change (n = 8) or improvement (n = 5) in IQ scores pre- to post-liver transplantation. Greater variability was observed in adaptive functioning scores, but the majority of patients evidenced no significant change (n = 8) in adaptive scores. In general, findings indicate that liver transplantation minimizes the likelihood of additional central nervous system damage, providing an opportunity for possible stabilization or improvement in neurocognitive functioning.Keywords
This publication has 19 references indexed in Scilit:
- Assessment of Psychoeducational Outcomes After Pediatric Liver TransplantAmerican Journal of Transplantation, 2009
- Social outcome in adults with maple syrup urine disease (MSUD)Journal of Inherited Metabolic Disease, 2007
- Neuropsychometric outcome predictors for adults with maple syrup urine diseaseJournal of Inherited Metabolic Disease, 2006
- Cognitive performance of children who have undergone liver transplantationTransplantation, 2003
- Cognitive function in children who receive organ transplantation.Health Psychology, 1994
- Cognitive function in children who receive organ transplantation.Health Psychology, 1994
- Intellectual performance of children with maple syrup urine diseaseEuropean Journal of Pediatrics, 1993
- Developmental profile of patients with maple syrup urine diseaseJournal of Inherited Metabolic Disease, 1991
- Intellectual outcome in children with maple syrup urine diseaseThe Journal of Pediatrics, 1991
- Mental and motor development, social competence, and growth one year after successful pediatric liver transplantationThe Journal of Pediatrics, 1989