Long-term Effects of Cholestatic Liver Disease in Childhood on Neuropsychological Outcomes and Neurochemistry


OBJECTIVES: Children with liver disease have increased risk of long-term cognitive deficits. We differentiated between the effects of chronic liver disease from that associated with transplantation by recruiting children with cholestatic liver disease (CLD) with and without transplantation. METHODS: Psychometric measures and magnetic resonance spectroscopy were obtained for 3 groups of children: stable liver disease without transplantation; CLD from birth with transplantation; and individuals healthy to 18 months of age, before transplantation for acute liver failure. RESULTS: Cognitive outcomes between children with different disease histories were significantly associated with the duration of liver disease but not the effects of transplantation, including that of immunosuppression. Lower intellectual ability was most frequently observed in the CLD group, whereas all of the acute liver failure group scored within the normal range. Myoinositol and glutamate/glutamine concentrations in cortex were significantly associated with disease duration across the cohort. Neurometabolite profiles in stable liver disease were consistent with subclinical encephalopathy. Impaired growth in early childhood was associated with later cognitive performance. CONCLUSION: Children with prolonged liver disease had the poorest cognitive outcomes despite successful transplantation, suggesting that prolonged cholestasis before transplantation adversely affects neurodevelopment, and reinforces the need for timely interventions.

Publication DOI: https://doi.org/10.1097/MPG.0000000000002380
Divisions: College of Health & Life Sciences
College of Health & Life Sciences > School of Psychology
College of Health & Life Sciences > Aston Institute of Health & Neurodevelopment (AIHN)
College of Health & Life Sciences > Clinical and Systems Neuroscience
College of Health & Life Sciences > School of Optometry > Vision, Hearing and Language
College of Engineering & Physical Sciences > School of Infrastructure and Sustainable Engineering > Chemical Engineering & Applied Chemistry
College of Engineering & Physical Sciences
Additional Information: © 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology. This is the accepted manuscript of the article: Long-Term Effects of Cholestatic Liver Disease in Childhood on Neuropsychological Outcomes and Neurochemistry Talcott, Joel B.; Beath, Sue V.; Patel, Tulpesh; Griffiths, Gareth; Kelly, Deirdre A. Journal of Pediatric Gastroenterology and Nutrition: May 6, 2019
Publication ISSN: 1536-4801
Last Modified: 10 Jun 2024 07:34
Date Deposited: 20 Jun 2019 08:35
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Related URLs: https://journal ... e_in.96426.aspx (Publisher URL)
PURE Output Type: Article
Published Date: 2019-08-01
Published Online Date: 2019-05-06
Accepted Date: 2019-04-18
Authors: Talcott, Joel B (ORCID Profile 0000-0001-7958-8369)
Patel, Tulpesh
Griffiths, Gareth (ORCID Profile 0000-0002-7507-6945)
Kelly, Deirdre
Beath, Sue V.



Version: Accepted Version

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