Early death in Schimke immune-osseous dysplasia often results from renal Fa
ilure and/or cell-mediated immunodeficiency. Kidney transplants have improv
ed renal function, but effective therapy for the immunodeficiency has not y
et been reported. We describe markedly improved marrow function 2 years aft
er bone marrow transplantation in a boy with Schimke immunoosseous dysplasi
a.