Non-infectious Lung Disease in Patients with Adenosine Deaminase Deficient Severe Combined Immunodeficiency

@article{Booth2012NoninfectiousLD,
  title={Non-infectious Lung Disease in Patients with Adenosine Deaminase Deficient Severe Combined Immunodeficiency},
  author={Claire Booth and V. E. Algar and Jinhua Xu-Bayford and Lynette Dianne Fairbanks and Clarence Owens and Hubert Bobby Gaspar},
  journal={Journal of Clinical Immunology},
  year={2012},
  volume={32},
  pages={449-453}
}
Adenosine deaminase deficiency is a disorder of purine metabolism manifesting severe combined immunodeficiency (ADA-SCID) and systemic abnormalities. Increased levels of the substrate deoxyadenosine triphosphate (dATP) lead to immunodeficiency and are associated in a murine model with pulmonary insufficiency. We compared a cohort of patients with ADA-SCID and X-linked SCID and found that despite similar radiological and respiratory findings, positive microbiology is significantly less frequent… CONTINUE READING