Cognitive outcomes in paediatric liver transplant survivors

PURPOSE: To investigate the impact of liver disease on cognitive ability in children with liver disease in the context of covariates such as the age of onset and transplant (Tx) intervention. METHOD: This cross-sectional study used standard Wechsler psychometric assessment batteries to evaluate neuropsychological function (Full-scale IQ and Information Processing Speed (IPS)). We studied 23 children wish liver diseases, of whom 13 had indergone liver Tx, and compared their performance to 9 healthy age-matching controls, 17 patients had cholestatic liver disease from birth (early onset liver disease (EOLD); pre-Tx n=10, post-Tx n=7), 6 were well until they developed fulminant liver failure after3 year of age (late onset liver disease (LOLD); post-Tx n=6. RESULTS: Chronic liver disease bad significant negative effect on cognitive development, with age at the onset of disease an important moderator of this effect. Significant difference in IPS were observed between the EOLD, post-Tx group (IPS=81.6) and controls (mean IPS=104.7) p= .017; (d)=1.36), with 31% of the variance in IPS accoutered for by EOLD coupled with transplantation. Differences in IPS between the EOLD post-Tx patients and EOLD pre-Tx patients (mean IPS=91.7) approached statistical significance (p=.06; effect size (d)=1.26). LOLD (fulminant liver failure) post-Tx patients (mean IPS=102.5) performed no differently to controls on any psychometric measures. CONCLUSION: EOLD has a significant impact on cognitive outcomes, possibly through the disruption of early underdevelopment processes. Survivors of fulminant liver failure however, have performances similar to that of the healthy controls suggesting that the transplant procedure itself did not affect cognitive outcome. Enrolling patients in a large-scale, longitudinal study would help illuminate the extent and persistence of the cognitive deficits observed in paediatric liver disease.