Journal
PEDIATRIC TRANSPLANTATION
Volume 15, Issue 1, Pages 58-64Publisher
WILEY-BLACKWELL PUBLISHING, INC
DOI: 10.1111/j.1399-3046.2010.01411.x
Keywords
maple syrup urine disease; liver transplantation; pediatric transplantation; cognitive functioning; adaptive functioning
Categories
Funding
- NIDDK NIH HHS [L40 DK079757] Funding Source: Medline
- NINR NIH HHS [R01 NR010711, K01 NR013189, R01 NR010711-02S2] Funding Source: Medline
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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.
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