Neurodevelopmental Outcomes in Children With Inherited Liver Disease and Native Liver.
J Pediatr Gastroenterol Nutr
; 74(1): 96-103, 2022 01 01.
Article
en En
| MEDLINE
| ID: mdl-34694263
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 nâ=â70, PFIC nâ=â43, A1AT nâ=â102); median age was 7.6âyears (3.0-16.9). Mean FSIQ in ALGS was lower than A1AT (94 vs 101, Pâ=â0.01). Frequency of FSIQâ<â85 (>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%, Pâ=â0.003). 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.
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Colestasis
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Colestasis Intrahepática
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Síndrome de Alagille
Tipo de estudio:
Clinical_trials
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Prognostic_studies
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Risk_factors_studies
Límite:
Child
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Child, preschool
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Humans
Idioma:
En
Revista:
J Pediatr Gastroenterol Nutr
Año:
2022
Tipo del documento:
Article