Background
Biliary atresia (BA) is a serious disease in infants that leads to liver cirrhosis and failure. Most patients die within 2 years of age in the absence of surgery. Therefore, we aimed to evaluate the cognitive function and explore influencing factors in young children with BA after primary living donor liver transplantation (BA-pLDLT) during infancy.
Methods
Children with BA were recruited 6 months after pLDLT at Children's Hospital of Chongqing Medical University (2018–2022). Demographic and clinical data were collected from the health information system. Cognitive function was assessed using the Chinese version of the Griffiths Mental Development scale (GMDS-C). Multivariate linear regression were used to analyze the influencing factors of cognitive function.
Results
In total, 57 children with BA -pLDLT, aged 5.00(3.90–9.30) months at transplantation and 25.00(14.00-60.80) months at evaluation were included. The general developmental quotient (89.02 ± 12.07) and motor, language, eye-hand coordination, performance, and practical reasoning quotients were significantly lower than the normative mean values of GMDS-C(P < 0.05). Of the 57 children, 16 (28.07%) had borderline developmental delay (70–84), 3 (5.26%) had developmental delay (< 70), and 11(19.29%) had language delay. Reoperation for biliary or vascular complications after transplantation was a risk factor for decreased general and motor developmental quotients (AQ) and low ZW at assessment was associated with decline AQ.
Conclusion
Children with BA-pLDLT have varying degrees of developmental delays during early life. Reoperation and nutritional deficiencies had adverse effects on cognition.