title: Neuropsychological functioning and health-related quality of life: pediatric acute liver failure study group results.
abstract: OBJECTIVE
  Pediatric acute liver failure (PALF) is a rare but serious event, with poorly understood functional outcomes. The goal was to determine the prevalence of reduced neuropsychological functioning and health-related quality of life (HRQOL) following PALF.
  
  
  METHODS
  This multicenter study examined neuropsychological functioning and HRQOL 1 to 6 (median 3.8) years after PALF. Participants ages 6 to 16 (median 9.9) years were recruited from the PALF registry and administered measures of intelligence, visual spatial/visual motor coordination, attention, executive function, depression, and adaptive skills. HRQOL and fatigue were assessed using the Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL 4.0) and PedsQL Multidimensional Fatigue Scale.
  
  
  RESULTS
  A total of 36 patients participated; 50% were boys and 67% were white. Median age at PALF was 5.6 years. A history of grade 3 or 4 hepatic encephalopathy was reported in 5/36 (14%) participants and 23/36 (64%) received a liver transplant. Visual spatial ability was significantly better than norms (P = 0.009), but motor coordination was worse (P = 0.04). Teachers (P = 0.04 to P < 0.0001) and parents (P = 0.005) reported more executive deficits versus norms, and participants had worse attention (P = 0.02). Participants did not differ significantly from norms on IQ, depression, or adaptive functioning. All of the child self-report PedsQL Generic Core and fatigue scales were significantly lower than a matched healthy sample (P = 0.001 to P < 0.0001) and parent proxy report was lower on the fatigue scales (P = 0.001 to P < 0.0001).
  
  
  CONCLUSIONS
  Long-term PALF survivors demonstrate average IQ and visual spatial ability, but greater than expected impairments in motor skills, attention, executive function, HRQOL, and fatigue.
authors.affiliation: *Department of Child & Adolescent Psychiatry †Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL ‡Department of Epidemiology, University of Pittsburgh, Pittsburgh, PA §Department of Psychology & Neuroscience, Baylor University, Waco, TX ||Department of Pediatrics, Texas A&M University, College Station ¶Department of Paediatrics, Hospital for Sick Children, Toronto, ON, Canada #Department of Pediatrics, University of Pittsburgh, Pittsburgh, PA.
authors.forename: Lisa G
authors.initials: LG
authors.lastname: Sorensen
authors.forename: Katie
authors.initials: K
authors.lastname: Neighbors
authors.forename: Song
authors.initials: S
authors.lastname: Zhang
authors.forename: Christine A
authors.initials: CA
authors.lastname: Limbers
authors.forename: James W
authors.initials: JW
authors.lastname: Varni
authors.forename: Vicky L
authors.initials: VL
authors.lastname: Ng
authors.forename: Robert H
authors.initials: RH
authors.lastname: Squires
authors.forename: Estella M
authors.initials: EM
authors.lastname: Alonso
mesh_terms: D000046:Academic Medical Centers; D058186:Acute Kidney Injury / Q000601:surgery*; D000293:Adolescent; D002170:Canada / Q000453:epidemiology; D002648:Child; D003430:Cross-Sectional Studies; D005221:Fatigue / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016030:Kidney Transplantation / Q000009:adverse effects* / Q000523:psychology; D008297:Male; D001523:Mental Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D009422:Nervous System Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D009483:Neuropsychological Tests; D015995:Prevalence; D011602:Psychophysiologic Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D011788:Quality of Life; D012042:Registries; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014481:United States / Q000453:epidemiology