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. 2004 Feb;144(2):278-80.
doi: 10.1016/j.jpeds.2003.10.055.

Bilateral sensorineural deafness in adenosine deaminase-deficient severe combined immunodeficiency

Wendy Albuquerque  1 , Hubert B Gaspar
Affiliations

Bilateral sensorineural deafness in adenosine deaminase-deficient severe combined immunodeficiency

Wendy Albuquerque et al. J Pediatr. 2004 Feb.

Abstract

Adenosine deaminase deficiency presents with severe combined immunodeficiency and is treatable by bone marrow transplantation. With improved survival, the nonimmunologic manifestations of this condition are becoming apparent. We report a high incidence of bilateral sensorineural deafness in transplanted patients, which highlights the systemic nature of the disease.

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