A team of physicians and scientists from three medical centers has combined forces to show that an infusion of liver cells can function for more than a year to partially correct a patient's rare metabolic liver disease. The procedure, which is reported in this week's New England Journal of Medicine, suggests that cell transplantation may have broader application and be a safer and less invasive treatment than liver transplantation for some patients with disorders of the liver.
The liver cell infusion took place in April 1997 at the University of Nebraska Medical Center (UNMC) in Omaha and was performed on a 10-year-old girl suffering from Crigler-Najjar Syndrome Type I, a rare disease in which the liver does not make the enzyme that allows bilirubin, a blood cell byproduct, to be normally excreted by the body.
The team performing the procedure was headed by Ira Fox, M.D., a UNMC transplant surgeon who collaborated with J. Roy-Chowdhury, M.D. and N. Roy-Chowdury, Ph.D., liver disease experts at the Albert Einstein College of Medicine in Bronx, N.Y., and Stephen Strom, Ph.D., an expert in isolating human liver cells from the University of Pittsburgh.
"This is an important step forward," Dr. Fox said. "We now know that factors which have limited the long-term effectiveness of pancreatic islet cell transplants for the treatment of diabetes do not affect the long-term function of transplanted liver cells, at least for some liver disorders."
Because the body is unable to conjugate and normally excrete bilirubin, patients with Crigler-Najjar disease develop jaundice and must spend 12 hours a day or longer receiving phototherapy, which helps degrade the bilirubin in the skin, so it can be cleared from the body.
Other than a liver transplant, there is no cure for Crigler-Najjar
disease. The oldest living patient with this disease is 31 years old and few
Contact: Tom O'Connor
University of Nebraska Medical Center