Bone marrow transplants from relatives can extend the lives of children born with an extremely weak immune system, a rare genetic disorder that once was considered almost always fatal, a study found.
Doctors have known since 1968 that bone marrow transplants could help children with SCIDS, or Severe Combined Immunodeficiency Syndrome, but there was scant information on the longer-term effects.
Babies with the disease can't produce T-cells that ward off infection, and can die from something as minor as a cold. The best-known victim was David, the Houston "Bubble Boy" who lived in a germ-proof enclosure.
In a study in Thursday's New England Journal of Medicine, Dr. Rebecca H. Buckley and colleagues at the Duke University Medical Center showed that children who received bone marrow transplants from relatives benefited, even if the donor was not an identical match.
They examined 89 infants who received transplants between 1982 and 1998. Among them, 72, or 81 percent, were still alive three months to 16´ years after transplantation.
In an accompanying editorial, Dr. Alain Fischer of the Necker Hospital in Paris called the study's results impressive.
"Long-term follow-up, which found the children in good general health, validates these results. These outcomes are by far the best that have been reported," Fischer wrote.
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