Health Related Quality of Life and Neurocognitive Outcomes in the First Year after Pediatric Acute Liver Failure

J Pediatr. 2018 May:196:129-138.e3. doi: 10.1016/j.jpeds.2018.01.030. Epub 2018 Mar 16.

Abstract

Objective: To determine health-related quality of life (HRQoL) and neurocognitive impairment in survivors of pediatric acute liver failure (PALF).

Study design: A longitudinal prospective study was conducted. At 6 and 12 months after PALF presentation, surveys of HRQoL were completed for 2- to 19-year-olds and executive functioning for ages 2-16 years. At 12 months, patients 3-16 years of age completed neurocognitive testing. HRQoL scores were compared with a healthy, matched sample. Neurocognitive scores were compared with norms; executive functioning scores were examined categorically.

Results: A total of 52 parent-report HRQoL surveys were completed at 6 months, 48 at 12 months; 25 patients completed neurocognitive testing. The median age at 6 months was 7.9 years (range 3.5-15.0), and final diagnosis was indeterminate for 46.2% (n = 24). Self and parent-report on Pediatric Quality of Life Inventory Generic and Multidimensional Fatigue scales fell below the healthy sample at 6 months and 12 months (almost all P < .001). Children reported lower mean scores on cognitive fatigue at 12 months (60.91 ± 22.99) compared with 6 months (73.61 ± 27.49, P = .006) . The distribution of Behavior Rating Inventory of Executive Function scores was shifted downward on parent-report (preschool) for all indices at 6 months (n = 14, P ≤ .003); Global Executive Composite and Emergent Metacognition at 12 months (n = 10, P = .03). Visual Motor Integration (VMI-6) Copying (mean = 90.3 ± 13.8, P = .0002) and VMI-6 Motor Coordination (mean = 85.1 ± 15.2 P = .0002) fell below norms, but full scale IQ (Wechsler Scales) and Attention (Conners' Continuous Performance Test) did not.

Conclusions: Survivors of PALF appear to show deficits in motor skills, executive functioning, HRQoL, and evidence for worsening cognitive fatigue from 6 to 12 months following PALF presentation.

Keywords: cognition disorders; cognitive fatigue; executive functioning; pediatric liver disease; pediatric liver transplantation.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Attention
  • Child
  • Child, Preschool
  • Cognition
  • Cognitive Dysfunction / diagnosis*
  • Cross-Sectional Studies
  • Depression / complications
  • Executive Function
  • Fatigue
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Intelligence Tests*
  • Liver Failure, Acute / psychology*
  • Liver Failure, Acute / therapy*
  • Longitudinal Studies
  • Male
  • Mental Status and Dementia Tests
  • Motor Skills*
  • Prospective Studies
  • Quality of Life*
  • Stress Disorders, Post-Traumatic / complications
  • Treatment Outcome
  • Young Adult