Duke University Medical Center physicians have reported in the Aug. 1, 2003 issue of the journal Blood, successfully treating the immune disorder complete DiGeorge Syndrome in seven of 12 children who underwent an experimental thymus transplantation procedure. As many as one in 4,000 children in the United States are born each year with varying degrees of DiGeorge Syndrome, a condition in which the body does not produce adequate quantities of T cells, the cells that help the body fight infections. Between five and 10 children are born in the United States each year with complete DiGeorge Syndrome, a condition in which babies' immune systems do not develop at all because they are born without a thymus.
All 12 patients in the Duke study were diagnosed with complete DiGeorge Syndrome. The 12 children were treated between 1993 and 2001 at Duke University Medical Center, the only center in the world currently offering the experimental thymus transplantation procedure.
The thymus rests on the heart and functions as a "schoolhouse" for immune cells. As cells pass through the thymus they are trained to become T cells, white blood cells that fight infection. A person without a thymus does not produce these T cells and, therefore, is at great risk for developing infections. By the time humans reach puberty, the thymus has completed most of its role in the body, shrinks in physical size and becomes dormant.
Without intervention, few children with complete DiGeorge Syndrome live to age 1, and none survive past 3 years of age. The seven surviving Duke patients are all well and living at home two to 10 years after receiving their transplants. Five patients in this study died, all from underlying congenital problems.
Contact: Laura Sawyer Brinn
Duke University Medical Center