Related Experiment Video
Updated: Jul 25, 2025

A Mouse Model of Chronic Liver Fibrosis for the Study of Biliary Atresia
Published on: February 3, 2023
Early life growth and developmental trajectory in children with biliary atresia undergoing primary liver
Heping Fang1, Zehao Li2, Ruoling Xian1
1Department of Child Health Care, Children's Hospital of Chongqing Medical University, Chongqing Key Laboratory of Pediatrics, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, Chongqing, China.
Insights
Children with biliary atresia (BA) after primary liver transplantation (pLT) experience growth and developmental challenges. Motor and language delays are common post-transplant, indicating a need for ongoing monitoring and further research.
Area of Science:
- Pediatric Surgery
- Hepatology
- Developmental Pediatrics
Background:
- Biliary atresia (BA) is a severe neonatal liver disease requiring timely intervention.
- Primary liver transplantation (pLT) is a critical treatment for BA, but early outcomes require detailed characterization.
- Understanding the growth and developmental trajectory post-pLT is essential for optimizing patient care.
Purpose of the Study:
- To investigate the early growth and developmental characteristics of children with BA following pLT.
- To identify specific growth parameters and developmental milestones affected in BA-pLT patients.
- To establish a baseline for long-term outcome assessment in this population.
Main Methods:
- A prospective cohort study followed 48 children diagnosed with BA undergoing pLT.
- Growth parameters (weight-for-age, length-for-age, head circumference-for-age Z-scores) were assessed against WHO standards.
- Developmental status was evaluated using Denver Developmental Screening Tests at multiple time points post-pLT.
Main Results:
- At pLT, all growth parameters were below WHO standards, with head circumference-for-age Z-values being particularly low.
- Post-pLT, weight-for-age and head circumference-for-age Z-values improved, but length-for-age Z-values remained suboptimal.
- Significant developmental delays were observed, with 35% suspicious and 15% abnormal at 1-4 months post-pLT, progressing to motor and language delays by 1 year.
Conclusions:
- Children undergoing pLT for BA face significant early growth and developmental issues.
- Head circumference is a key concern pre-pLT, while linear growth remains a challenge post-pLT.
- Motor and language developmental delays are prevalent after pLT, necessitating further investigation into long-term outcomes and influencing factors.
Objective:
To clarify the early growth and developmental characteristics of children with biliary atresia (BA) undergoing primary liver transplantation (pLT).
Methods:
A prospective cohort study, which specifically focused on BA-pLT children, was conducted after the diagnosis of BA by following the children at the time of pLT and 1, 3, 5, 7 months and 1 year after pLT for growth and developmental monitoring. The growth parameters were calculated according to the WHO standard, and the developmental status was assessed using Denver Developmental Screening Tests.
Results:
A total of 48 BA children who received pLT at the age of 5.00 ± 0.94 months were analyzed. The weight-for-age Z-value (ZW) and length-for-age Z-value (ZL) were higher than the head circumference-for-age Z-value (ZHC) at pLT (P = 0.002 and 0.02), but they were all lower than the WHO growth standard (Z = 0) (P < 0.001). The ZW and ZHC decreased first and then returned to the population level at 1 year after pLT, while the ZL only returned to the preoperative status and was lower than the ZW and ZHC (P < 0.001). Developmental screening showed that 35% (17/48) of the children were defined as suspicious and 15% (7/48) were abnormal at 1-4 months after pLT, the most likely time to be suspected of developmental delay. At 1 year after pLT, gross motor skill delay still existed (12/45, 27%), and language skill delay began to appear (4/45, 9%).
Conclusions:
BA-pLT children suffer from growth and developmental problems. Low ZHC is the main growth problem before pLT, while low ZL is the problem after pLT. Developmental delays are significant after pLT, especially in motor and language skills. The current study suggested that further studies are warranted to clarify the long-term growth and developmental outcomes of BA-pLT children, to compare them with children undergoing the Kasai procedure and to explore their influencing factors and possible mechanisms.

