Dictionary of Human Evolution and Biology

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Adenosine Deaminase Deficiency

An autosomal dominant condition, one of the primary immunodeficiency diseases (PIDs) characterized by skeletal and neurological abnormalities; frequent infections, fatal if untreated. Death usually occurs by the age of 7months owing to infection. ADA deficiency accounts for about 15% of all severe combined immune deficiency (SCID) cases. There are no T cells, and B cells do not produce antibodies. An ADAdeficient SCID girl was selected as the first person with a genetic disorder to be treated by somatic gene therapy, using a viral vector, in September 1990.

Cf. agammaglobulinemia.

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