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The cognitive functioning of identical twins, discordant for prune belly syndrome and end stage renal disease, was evaluated longitudinally over a 6‐year period. Assessments were performed at the onset of hemodialysis of the renal twin and at 1 month, 1 year, 3 years, and 5 years after successful transplantation. Identical measures of intellectual ability, academic achievement, problem solving, memory, and attention were used for the first four evaluations. At 5 years posttransplant, intellectual ability was reassessed in conjunction with an extended battery of neuropsychological tests. Results revealed a clinically significant difference in intellectual ability which has remained stable over the past 6 years. The more comprehensive battery documented additional deficits in memory, attention, motor speed, visuomotor integration, and organizational ability in the transplanted twin. Verbal abilities appear to be preserved relative to severe deficits in analytic and organizational skills with visuospatial material. The paper concludes with a discussion of the nature, permanence, and potential mechanisms of neuropsychological deficits associated with early onset renal insufficiency.
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