Cognitive Performance in Pediatric Liver Transplant Recipients

Abstract

To date, the course of cognitive development in children after liver transplantation (Ltx) is poorly understood. Cognitive performance, however, is crucial in all developmental stages and for educational achievement. This cross‐sectional single‐center study examined the prevalence of long‐term cognitive impairment in a cohort of 64 pediatric patients after Ltx. Median age at Ltx was 12 months. The revised Wechsler Intelligence Scale IV was administered to assess cognitive performance. Patients were compared with an age‐ and gender‐matched group of children without a chronic health condition. Liver transplanted children performed significantly worse in three of four cognitive domains as well as in the Total Intelligence Quotient (Total IQ) (p = 0.017 to p = 0.005). Liver transplant recipients showed substantially more “serious delays” (IQ < 70) compared to the reference group (9.4% vs. 4.7%). Children with a genetic–metabolic disease performed worse than the other groups in three of the four WISC Indices and in the Total IQ (p = 0.05 to p = 0.01). The strongest association was revealed between height at Ltx and Verbal Comprehension (R2 = 0.21), Perceptual Reasoning (R2 = 0.30), Working Memory (R2 = 0.23) and Total IQ (R2 = 0.25). Our results indicate a high impact of primary diagnosis and height percentile at Ltx even on children's long‐term cognitive performance.

Phenotypic spectrum and diagnostic pitfalls of ABCB4 deficiency depending on age of onset

“…Although no higher education was reported in our pediatric cohort, statistic confounders and the patient's young age or socioeconomic background must be considered. Children surviving liver transplantation are at risk of prolonged cognitive and academic deficits …”

“…Children surviving liver transplantation are at risk of prolonged cognitive and academic deficits. (25,26) Patients with an initial diagnosis of ABCB4 disease in adulthood may display a clinical phenotype with a later onset, such as ICP; alternatively, clinical or laboratory signs of the disease could have been misinterpreted or overlooked. These patients typically present with a heterogeneous clinical picture.…”

Delayed transplantation may affect intellectual ability in children

“…Furthermore, Kaller et al . suggested that there might be a critical period for liver disease to affect the brain during early childhood, and that if LT is performed very early in life, neuronal plasticity might alleviate the potential toxic influences . They also showed that children who received LT within the first year of life had a normal cognitive outcome compared with the mean .…”

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“…The same study also determined that the neurologic status of children who underwent a liver transplant because of a genetic metabolic disorder was even worse. 31 Therefore, some liver transplant patients should continue their education in special education centers. Health professionals should evaluate recipients who repeat a grade, have excessive absences or drop out of school in terms of their special education needs and should refer them to special education centers.…”