Between 5 and 10 children are born in the US each year with the immune disorder complete DiGeorge Syndrome, in which the thymus – the organ responsible for generating T-cells and fighting infection – is absent.

Without medical intervention, most children with the condition die before they are one year old. None survive beyond their third birthday.

The team of researchers from the Duke University Medical Center, in North Carolina, say they have used an experimental thymus transplant procedure to successfully treat 7 out of 12 children who have the disease.

Dr Louise Markert, associate professor of pediatrics at the university, and colleagues implanted the discarded thymus tissue of infants who had undergone cardiac surgery into the quadricep muscles of both legs of the DiGeorge babies aged 33-133 days.

The infants were kept in isolation before and for several months after the transplant, and monitored intensively during the first year after transplant.

Dr Makert said that, because the infants have no immune system, their bodies could not reject new organs. “Implanting thymus tissue early enough in life can provide these children with a chance to create a new immune system,” she explained.

One year after the transplant, the surviving seven children had T-cell counts of 479-1,580 per cubic millimetre of blood compared to the normal infant count of more than 1,500.

Two to ten years after receiving their transplants the children have suffered few infections or other immune-related disorders.

The researchers claim the survival rates and immunity levels in the treated children provide good arguments for offering the transplant procedure as a standard treatment for immune-deficient children with complete DiGeorge Syndrome.

“The results we’re reporting are better than with any other therapy and are the best one would expect, given the heart and other congenital problems in these infants,” they concluded.

© HMG Worldwide 2003
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