Open AccessLong‐term Effects of Cholestatic Liver Disease in Childhood on Neuropsychological Outcomes and Neurochemistry
Author(s) -
Talcott Joel B.,
Beath Sue V.,
Patel Tulpesh,
Griffiths Gareth,
Kelly Deirdre A.
Publication year - 2019
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.206
H-Index - 131
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0000000000002380
Subject(s) - medicine , liver transplantation , transplantation , liver disease , immunosuppression , chronic liver disease , subclinical infection , disease , hepatic encephalopathy , gastroenterology , pediatrics , cirrhosis
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.