Management of hyperbilirubinemia in biliary atresia by hepatic progenitor cell transplantation through hepatic artery: a case report.

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.
AuthorsA A Khan, N Parveen, V S Mahaboob, A Rajendraprasad, H R Ravindraprakash, J Venkateswarlu, P Rao, G Pande, M Lakshmi Narusu, M N Khaja, R Pramila, A Habeeb, C M Habibullah
JournalTransplantation proceedings (Transplant Proc) Vol. 40 Issue 4 Pg. 1153-5 (May 2008) ISSN: 0041-1345 [Print] United States
PMID18555137 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Alanine Transaminase
  • Bilirubin
  • Alanine Transaminase (blood)
  • Bilirubin (blood)
  • Female
  • Fetal Tissue Transplantation (methods)
  • Hepatic Artery
  • Hepatocytes (transplantation)
  • Humans
  • Hyperbilirubinemia (blood, enzymology, surgery)
  • Infant
  • Liver (enzymology)
  • Liver Function Tests
  • Stem Cell Transplantation (methods)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research network!

Choose Username:
Verify Password: