P0100 PP SCHOOL PERFORMANCE AND IQ SCORES OF CHILDREN WITH BILIARY ATRESIA 6, 8, AND 10 YEARS FOLLOWING LIVER TRANSPLANTATION

Abstract

Introduction: Liver transplantation is now accepted as the definitive therapy for various forms of end-stage liver disease in children. Two diseases, biliary atresia and neonatal hepatitis account for the majority (80%) of pediatric transplantations. These diseases emerge in the newborn period which is a time of critical brain growth with glial proliferation and rapid myelination. Consequently, severe liver disease requiring transplantation has the potential to significantly interfere with the developing brain, future neurodevelopmental functioning and subsequent educational performance. The aims of this study are to: 1) determine the long-term neurodevelopmental status of children transplanted prior to 3 years of age; 2) to compare neurodevelopmental status with school performance. Methods: The neurodevelopmental status of fifty children diagnosed with biliary atresia receiving extrahepatic liver transplantation <3 years of age was tested at 6, 8, & 10 years post transplantation. Neurodevelopmental status was determined by the WPSSI-R or WISC-3, the two most commonly used tests to measure IQ in children 5–12 yrs. of age. School performance was organized by a descriptive statistic of normal, at-risk or delayed. Children in the normal group were performing satisfactorily in school and receiving no academic assistance. Children in the delayed group were deemed eligible for special education services while at-risk children received some type of academic assistance (e.g. tutoring, therapy) but did not qualify for special education services. Results: At 6 years post transplantation IQ scores were near normal (99+/−10). But at 8 years post tranplantation, there was a significant increase in IQ scores (110+/−15) that was maintained at 10 years post tranplantation. However, when IQ scores were compared to school performance it was found that IQ scores and school performance were not significantly correlated (.18). Even though this group of children achieved near normal IQ scores, over 40% of the study population received educational support services. Three primary school performance complaints were noted: problems with short term memory; slower cognitive performance; and disorganized approach to tasks. Conclusion: These data suggest that, for children diagnosed with biliary atresia and transplanted in the first 3 years of life, mean IQ scores are in the normal range by 6 years of post transplantation. These near normal IQ scores are sustained over time until 10 years post transplantation. However, IQ scores do not predict for school performance and may even mask learning difficulties associated with either liver disease or the post transplant drug regimen.