Neurocognitive outcomes at kindergarten entry after liver transplantation at <3 yr of age

Abstract

This prospective inception cohort study determines kindergarten-entry neurocognitive abilities and explores their predictors following liver transplantation at age <3 yr. Of 52 children transplanted (1999-2008), 33 (89.2%) of 37 eligible survivors had psychological assessment at age 54.7 (8.4) months: 21 with biliary atresia, seven chronic cholestasis, and five acute liver failure. Neurocognitive scores (mean [s.d.], 100 [15]) as tested by a pediatric-experienced psychologist did not differ in relation to age group at transplant (≤12 months and >12 months): FSIQ, 93.9 (17.1); verbal (VIQ), 95.3 (16.5); performance (PIQ), 94.3 (18.1); and VMI, 90.5 (15.9), with >70% having scores ≥85, average or above. Adverse predictors from the pretransplant, transplant, and post-transplant (30 days) periods using univariate linear regressions for FSIQ were post-transplant use of inotropes, p = 0.029; longer transplant warm ischemia time, p = 0.035; and post-transplant highest serum creatinine, (p = 0.04). For PIQ, they were pretransplant encephalopathy, p = 0.027; post-transplant highest serum creatinine, p = 0.034; and post-transplant inotrope use, p = 0.037. For VMI, they were number of post-transplant infections, p = 0.019; post-transplant highest serum creatinine, p = 0.025; and lower family socioeconomic index, p = 0.039. Changes in care addressing modifiable predictors, including reducing acute post-transplant illness, pretransplant encephalopathy, transplant warm ischemia times, and preserving renal function, may improve neurocognitive outcomes.

Kindergarten‐age neurocognitive, functional, and quality‐of‐life outcomes after liver transplantation at under 6 years of age

“…For VMI and GAC, the average scores in our study were more than one‐half SD of population norms, suggesting that as a group, these children have difficulties. The VMI scores may herald difficulties with copying and pencil skills, reported also after survival of prematurity and linked to right brain insult and low ability in mathematics . The GAC scores may predict difficulties in realistic, independent behaviors of patients, and the effectiveness of interaction with others.…”

“…Some data on severe complications post‐operatively were obtained retrospectively as described in a previous publication examining predictors of early post‐operative outcomes in these children . Definitions of variables have been described previously . “Any severe complication” was defined as any one of the following: hepatic artery thrombosis, portal vein thrombosis, bile leak, bowel perforation, intra‐abdominal infection, re‐transplant, or death during the primary hospitalization.…”

“…We previously reported neurocognitive outcomes at kindergarten entry in 33 children who had liver transplant at age under 3 years at our center . That report was limited by the small number of patients that precluded multiple regression analyses for predictors of outcomes and lack of data for HRQL and functional outcomes.…”

Academic potential and cognitive functioning of long‐term survivors after childhood liver transplantation

“…We are not the only group to report normal mean IQ in transplanted patients. Two recent North American studies have reported their cohorts to have full‐scale IQ in the normal range . Robertson et al.…”

Pre‐school neurocognitive and functional outcomes after liver transplant in children with early onset urea cycle disorders, maple syrup urine disease, and propionic acidemia: An inception cohort matched‐comparison study

“…Fourth, the matched comparison group included patients with heterogeneous causes of liver disease, and it is possible that specific subgroups of non‐IEM patients may have similarly poor outcomes to the IEM group. Nevertheless, in our follow‐up program the outcomes of acute liver failure patients transplanted at age <3 years include FSIQ of 92, suggesting that the IEM group does more poorly than even this high‐risk subgroup of non‐IEM patients. Fifth, the small sample size precluded our testing for predictors of adverse outcome in the IEM cohort.…”