Alagille Syndrome
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Progressive Familial Intrahepatic Cholestasis

ALGS may lead to lower Full Scale IQ

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Patients with Alagille syndrome (ALGS) are at an increased risk of lower Full Scale IQ (FSIQ) whereas patients with progressive familial intrahepatic cholestasis (PFIC) and alpha 1 antitrypsin deficiency (A1AT) are not, according to a study.

Deficits in working memory and processing speed were noted in patients with ALGS and A1AT.

This longitudinal, multicenter study examined the FSIQ of 70 patients with ALGS, 43 with PFIC, and 102 with A1AT.

Mean FSIQ in patients ALGS was 94 compared to 101 in patients with A1AT (P = 0.01).

Patients with ALGS had the highest frequency of FSIQ <85 at 29% followed by patients with PFIC at 18.6% and patients with A1AT at 12.8%. The frequency was greater than expected in ALGS.

Patients with ALGS scored significantly lower than normal in most composites whereas patients with A1AT scored lower on Working Memory and Processing Speed.

Total bilirubin, alkaline phosphatase, albumin, hemoglobin, and parental education were significantly associated with FSIQ.

Leung DH, Sorensen LG, Ye W, Hawthorne K, et al; Childhood Liver Disease Research Network (ChiLDReN). Neurodevelopmental Outcomes in Children with Inherited Liver Disease and Native Liver. J Pediatr Gastroenterol Nutr. 2021;doi: 10.1097/MPG.0000000000003337. Epub ahead of print. PMID: 34694263.