Neurodevelopmental Outcomes in Children With Inherited Liver Disease and Native Liver.

ABSTRACT:

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, A

SUBMITTER: Leung DH 

PROVIDER: S-EPMC8673857 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature