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Updated: Jun 15, 2026

A Mouse Model of Chronic Liver Fibrosis for the Study of Biliary Atresia
Published on: February 3, 2023
Language and motor skills are impaired in infants with biliary atresia before transplantation
Susan E Caudle1, Jennifer M Katzenstein1, Saul J Karpen2
1Psychology Service, Texas Children's Hospital and Department of Pediatrics, Baylor College of Medicine, Houston, TX.
Insights
Infants with biliary atresia show developmental delays in motor and language skills before liver transplant. Early intervention and nutrition are crucial for neurocognitive development in these children.
Area of Science:
- Pediatric Gastroenterology
- Neurodevelopmental Pediatrics
- Hepatology
Background:
- Biliary atresia is a serious neonatal liver disease.
- Potential neurocognitive deficits in affected infants require investigation.
Purpose of the Study:
- To determine if cognitive deficits are present in infants with biliary atresia before liver transplantation.
- To characterize the developmental profile in this population.
Main Methods:
- Utilized the Mullen Scales of Early Learning (Mullen) to assess 15 infants with biliary atresia (mean age 7.8 months).
- Correlated Mullen scores with clinical and biochemical parameters, including international normalized ratio and growth parameters.
- Examined the impact of Kasai procedure timing, hospitalization duration, and feeding methods.
Main Results:
- Infants demonstrated significant delays in gross motor and language skills.
- Fine motor and visual reasoning skills were relatively preserved.
- Inverse correlation found between international normalized ratio and motor skills; positive correlation between growth parameters and expressive language.
Conclusions:
- Very young children with biliary atresia exhibit a distinct pattern of early developmental deficits prior to transplantation.
- Findings support early intervention and aggressive nutritional management to mitigate neurocognitive effects.
Objective:
To test the hypothesis that in very young patients with biliary atresia, cognitive deficits are apparent before transplantation.
Study Design:
With the Mullen Scales of Early Learning (Mullen), we examined 15 infants (mean age, 7.8 months) with biliary atresia, correlating Mullen scores with standard clinical and biochemical parameters.
Results:
Overall, participants displayed significant delays in gross motor and language skills, and fine motor and visual reasoning skills were relatively preserved. The international normalized ratio correlated inversely with gross (P < .01) and fine (P < .05) motor skills. Growth parameters correlated positively with expressive language ability, but length of hospitalization and mode of feeding did not. Age at performance of the Kasai procedure was found to correlate with receptive language performance (P < .05).
Conclusions:
Very young children with biliary atresia display a characteristic profile of early developmental deficits before transplantation. These findings suggest that early intervention and aggressive nutritional management should be the standard of care to minimize neurocognitive effects.
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