摘要
background Biliary atresia(BA)is one of the most challenging hepatobiliary diseases in children.Notwithstanding the reasonable outcome of liver transplantation(LT),portoenteral anastomosis(Kasai operation)is still the first choice of treatment for patients with BA.How to improve the survival rate of the patients with BA in the era of LT is a new challenge.Data sources Based on recent original publications and the experience with the BA in China,we review many factors that influence BA survival situation,including early diagnosis and screen plan,defects of early treatment,Kasai operation,and LT and indicate present questions about BA diagnosis and treatment in China.results BA diagnosis may also be delayed due to insufficient understanding of BA and lack of jaundice monitoring methods at different levels of the hospitals in China.Further education of the physicians at smaller city hospitals about BA,neonatal jaundice and cholestasis would be helpful in improving early diagnosis of BA.Early surgical intervention is still the only guarantee to improve the survival rate of BA with native liver.Conclusions In the era of LT,especially in China,Kasai operation can provide waiting time for living donor LT and improve the success rate of LT and minimize the rapid deterioration of liver function of the children with BA and decrease the mortality in patients with BA.
基金
This study was supported by National Natural Fund:81570471.Tianjin Institutes of Health grants:14KG129
