Open AccessNeurodevelopmental Outcomes in Children With Inherited Liver Disease and Native Liver
Author(s) -
Leung Daniel H.,
Sorensen Lisa G.,
Ye Wen,
Hawthorne Kieran,
Ng Vicky L.,
Loomes Kathleen M.,
Fredericks Emily M.,
Alonso Estella M.,
Heubi James E.,
Horslen Simon P.,
Karpen Saul J.,
Molleston Jean P.,
Rosenthal Philip,
Sokol Ronald J.,
Squires Robert H.,
Wang Kasper S.,
Kamath Binita M.,
Magee John C.
Publication year - 2022
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.206
H-Index - 131
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0000000000003337
Subject(s) - medicine , univariate analysis , alagille syndrome , wechsler adult intelligence scale , gastroenterology , intelligence quotient , cholestasis , liver disease , pediatrics , psychiatry , multivariate analysis , cognition
Objective: To evaluate neurodevelopmental status among children with inherited cholestatic liver diseases with native liver and variables predictive of impairment. Methods: Participants with Alagille syndrome (ALGS), progressive familial intrahepatic cholestasis (PFIC), and alpha 1 antitrypsin deficiency (A1AT) enrolled in a longitudinal, multicenter study and completed the Wechsler Preschool and Primary Scale of Intelligence‐III or Intelligence Scale for Children‐IV. Full Scale Intelligence Quotient (FSIQ) was analyzed continuously and categorically (>100, 85–99, 70–84, <70). Univariate linear regression was performed to study association between FSIQ and risk factors, stratified by disease. Results: Two hundred and fifteen completed testing (ALGS n = 70, PFIC n = 43, A1AT n = 102); median age was 7.6 years (3.0–16.9). Mean FSIQ in ALGS was lower than A1AT (94 vs 101, P = 0.01). Frequency of FSIQ < 85 (>1 standard deviation [SD] below average) was highest in ALGS (29%) versus 18.6% in PFIC and 12.8% in A1AT, and was greater than expected in ALGS based on normal distribution (29% vs 15.9%, P = 0.003). ALGS scored significantly lower than test norms in almost all Wechsler composites; A1AT scored lower on Working Memory and Processing Speed; PFIC was not different from test norms. Total bilirubin, alkaline phosphatase, albumin, hemoglobin, and parental education were significantly associated with FSIQ. Conclusions: Patients with ALGS are at increased risk of lower FSIQ, whereas our data suggest A1AT and PFIC are not. A1AT and ALGS appear vulnerable to working memory and processing speed deficits suggestive of attention/executive function impairment. Malnutrition, liver disease severity, and sociodemographic factors appear related to FSIQ deficits, potentially identifying targets for early interventions.