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

Lisa G Sorensen; Katie Neighbors; Regina M Hardison; Kathleen M Loomes; James W Varni; Vicky L Ng; Robert H Squires; Estella M Alonso; Pediatric Acute Liver Failure Study Group

doi:10.1016/j.jpeds.2018.01.030

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.

Authors

Lisa G Sorensen¹, Katie Neighbors², Regina M Hardison³, Kathleen M Loomes⁴, James W Varni⁵, Vicky L Ng⁶, Robert H Squires⁷, Estella M Alonso²; Pediatric Acute Liver Failure Study Group

Collaborators

Pediatric Acute Liver Failure Study Group:
Kathryn Bukauskas⁸, Madeline Schulte⁸, Michael R Narkewicz⁹, Michelle Hite⁹, Elizabeth B Rand¹⁰, David Piccoli¹⁰, Deborah Kawchak¹⁰, Christa Seidman¹⁰, Rene Romero¹¹, Saul Karpen¹¹, Liezl de la Cruz-Tracy¹¹, Kelsey Hunt¹², Girish C Subbarao¹³, Ann Klipsch¹³, Sarah Munson¹³, Susan Kelly¹⁴, Philip J Rosenthal¹⁴, Shannon Fleck¹⁴, Mike A Leonis¹⁵, John Bucuvalas¹⁵, Tracie Horning¹⁵, Norberto Rodriguez Baez¹⁶, Shirley Montanye¹⁶, Margaret Cowie¹⁶, Simon P Horslen¹⁷, Karen Murray¹⁷, Melissa Young¹⁷, Heather Nielson¹⁷, Jani Klein¹⁷, David A Rudnick¹⁸, Ross W Shepherd¹⁸, Kathy Harris¹⁸, Saul J Karpen¹⁹, Alejandro De La Torre¹⁹, Dominic Dell Olio²⁰, Deirdre Kelly²⁰, Carla Lloyd²⁰, Steven J Lobritto²¹, Sumerah Bakhsh²¹, Maureen Jonas²², Scott A Elifoson²², Roshan Raza²², Kathleen B Schwarz²³, Wikrom W Karnsakul²³, Mary Kay Alford²³, Anil Dhawan²⁴, Emer Fitzpatrick²⁴, Nanda N Kerkar²⁵, Brandy Haydel²⁵, Sreevidya Narayanappa²⁵, M James Lopez²⁶, Victoria Shieck²⁶

Affiliations

¹ Department of Child & Adolescent Psychiatry, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL.
² Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL.
³ Department of Epidemiology, University of Pittsburgh, Pittsburgh, PA.
⁴ Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA.
⁵ Department of Pediatrics and Department of Landscape Architecture and Urban Planning, Texas A&M University, College Station, TX.
⁶ Department of Paediatrics, Hospital for Sick Children, University of Toronto, Ontario, Canada.
⁷ Department of Pediatrics, University of Pittsburgh, Pittsburgh, PA.
⁸ Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.
⁹ Children's Hospital Colorado, Aurora, CO.
¹⁰ Children's Hospital of Philadelphia, Philadelphia, PA.
¹¹ Emory University, Atlanta, GA.
¹² Hospital for Sick Children, Toronto, Ontario, Canada.
¹³ Indiana University Riley Hospital, Indianapolis, IN.
¹⁴ University of California San Francisco, San Francisco, CA.
¹⁵ University of Cincinnati, Cincinnati, OH.
¹⁶ University of Texas Southwestern, Dallas, TX.
¹⁷ University of Washington, Seattle, WA.
¹⁸ Washington University, St. Louis, MO.
¹⁹ Baylor College of Medicine, Houston, TX.
²⁰ Birmingham Children's Hospital, Birmingham, United Kingdom.
²¹ Columbia University, New York, NY.
²² Harvard Medical School, Boston, MA.
²³ Johns Hopkins University, Baltimore, MD.
²⁴ King's College Hospital, London, United Kingdom.
²⁵ Mt. Sinai School of Medicine, New York, NY.
²⁶ University of Michigan, Ann Arbor, MI.

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

Grants and funding

U01 DK072146/DK/NIDDK NIH HHS/United States