For a study, researchers sought to assess the neurodevelopmental state of children with inherited cholestatic liver disorders who have a native liver and the factors that predict impairment. Participants in a longitudinal, multicenter study with Alagille syndrome (ALGS), progressive familial intrahepatic cholestasis (PFIC), and alpha 1 antitrypsin deficiency (A1AT) completed the Wechsler Preschool and Primary Scale of Intelligence-III or the Intelligence Scale for Children-IV. The Full-Scale Intelligence Quotient (FSIQ) was studied continuously and categorically (>100, 85–99, 70–84, 70). The connection between FSIQ and risk variables, stratified by disease, was investigated using univariate linear regression. About 215 people were tested (ALGS n=70, PFIC n=43, A1AT n=102), with a median age of 7.6 years (3.0–16.9). ALGS had a lower mean FSIQ than A1AT (94 vs 101, P=0.01). ALGS had the highest frequency of FSIQ, less than 85 (>1 SD below average) (29%), compared to 18.6% in PFIC and 12.8% in A1AT, and was higher than expected based on the normal distribution (29% vs 15.9%, P=0.003). In almost all Wechsler composites, ALGS scored significantly lower than test norms; A1AT scored lower on Working Memory and Processing Speed, and PFIC did not differ from test norms. Total bilirubin, alkaline phosphatase, albumin, hemoglobin, and parental education were all linked to FSIQ in a meaningful way. Patients with ALGS have a higher chance of having a poorer FSIQ, although the outcomes imply that A1AT and PFIC were not. Working memory and processing speed deficiencies in A1AT and ALGS were symptomatic of attention/executive function impairment. Malnutrition, the severity of liver illness, and sociodemographic variables all appear to be linked to FSIQ impairments, suggesting possible targets for early intervention.

Source:journals.lww.com/jpgn/Fulltext/2022/01000/Neurodevelopmental_Outcomes_in_Children_With.17.aspx