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Ataxia-Telangiectasia: A Neuro-Endocrine-Immune Disease? Alternative Models of Pathogenesis

  • Richard A. Gatti

Abstract

Ataxia-telangiectasia (AT) is a disease characterized by early onset of a progressive cerebellar ataxia, sinopulmonary infections and dilated capillaries over the ears and eyes which are called “telangiectases”. AT affects approximately 1 in 30,000, however, this incidence varies from one ethnic group to another. Patients seldom live beyond twenty years although this pattern has been gradually extended, perhaps due to better pulmonary hygiene and antibiotics. Despite an early demise, approximately 1 in 8 children with this disease develop a maligancy, usually lymphoid. Such children also show signs of premature aging (Boder and Sedgwick, 1958; Gatti and Walford, in press). There is no effective treatment to halt the steady deterioration.

Keywords

Down Syndrome Spinal Muscular Atrophy Common Variable Immunodeficiency Progressive Cerebellar Ataxia Sinopulmonary Infection 
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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Copyright information

© Plenum Press, New York 1983

Authors and Affiliations

  • Richard A. Gatti
    • 1
  1. 1.Department of PathologyUCLA School of MedicineLos AngelesUSA

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