Cholangiodestruction of bile ducts leads to
biliary atresia, a
rare disease characterized by intrahepatic and extrahepatic biliary
inflammation. If the intrahepatic biliary tree is unaffected, surgical reconstruction by the
Kasai procedure of
hepatoportoenterostomy of the extra hepatic biliary tract is possible. Untreated, this condition leads to
cirrhosis and death within the first year of the life. If the atresia is complete,
liver transplantation is the only option. As a result of the shortage of donor livers, hepatocytes have been infused over the past two decades, providing proof of the concept that
cell therapy can be effective for the treatment of
liver diseases. In the present study, we report a confirmed case of a girl of 1 year of age with increased
bilirubin of 28.5 mg/dL and pediatric
end-stage liver disease score 20. Biochemical liver function tests showed
cholestasis (
elevated cholesterol and gamma-GTs) and increased ALT, total
bilirubin, conjugated
bilirubin, and ALP. The patient was treated with hepatic progenitor cell infusion through the hepatic artery. The total
bilirubin and conjugated
bilirubin started decreasing during the first month after cell infusion. The level of total
bilirubin maintained a threefold decrease after months of cell infusion. The conjugated
bilirubin was 16.35 mg/dL before cell infusion, decreasing to eightfold after cell infusion. After 2 months of cell infusion, hepatobiliary scintigraphy showed increased liver cell function. This case demonstrated the efficacy and functionality of hepatic progenitor cells for the management of
biliary atresia. Further, as there was a decrease in serum
bilirubin, it showed that there was some percentage of the engraftment of the infused cells. As the procedure is simple and the patient has tolerated the infusion
therapy, it might be repeated to manage
biliary atresia.