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Intravenous Deoxycytidine Therapy in a Patient with Adenosine Deaminase Deficiency

  • Morton J. Cowan
  • David W. MartinJr.
  • Diane W. Wara
  • Arthur J. Ammann
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 165)

Abstract

Adenosine deaminase (ADA) deficiency usually results in severe combined immunodeficiency disease. Without therapy, these children usually die from overwhelming infections. The most successful therapy remains bone marrow transplantation from a histocompatible sibling donor. Efforts at a biochemical approach have focused on enzyme replacement using repeated transfusions from ADA-positive donors (Polmar et al., 1976). Unfortunately, only a relatively few patients with ADA deficiency have histocompatible siblings to provide a bone marrow transplant and less than 50% of patients with ADA deficiency show a significant response to red cell transfusions. In addition, there are significant risks of repeated red cell transfusions, including iron overload, transfusion reactions, and viral infections.

Keywords

Adenosine Deaminase Deoxycytidine Deaminase Severe Combine Immunodeficiency Disease Adenosine Deaminase Activity Ribonucleotide Reductase Inhibition 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

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Copyright information

© Plenum Press, New York 1984

Authors and Affiliations

  • Morton J. Cowan
    • 1
  • David W. MartinJr.
    • 1
  • Diane W. Wara
    • 1
  • Arthur J. Ammann
    • 1
  1. 1.Departments of Pediatrics and MedicineUniversity of CaliforniaSan FranciscoUSA

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