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Survival probability in ataxia telangiectasia
  1. T O Crawford1,2,
  2. R L Skolasky1,
  3. R Fernandez3,
  4. K J Rosquist2,
  5. H M Lederman2
  1. 1Department of Neurology, The Johns Hopkins Hospital, Baltimore, Maryland, USA
  2. 2Department of Pediatrics, The Johns Hopkins Hospital
  3. 3Ataxia Telangiectasia Children’s Project, Deerfield Beach, Florida, USA
  1. Correspondence to:
    Dr Thomas O Crawford
    Pediatric Neurology, Johns Hopkins Hospital, Jefferson 123, 600 N Wolfe Street, Baltimore, MD 21287, USA; tcrawfo{at}jhmi.edu

Abstract

Ataxia telangiectasia is a rare, multiorgan neurodegenerative disorder with enhanced vulnerability to cancer and infection. Median survival in two large cohorts of patients with this disease, one prospective and one retrospective, is 25 and 19 years, with a wide range. Life expectancy does not correlate well with severity of neurological impairment.

  • ATCC, Ataxia Telangiectasia Clinical Center at Johns Hopkins Hospital
  • ATCP, Ataxia Telangiectasia Children’s Project
  • survival analysis
  • cancer
  • ataxia telangiectasia

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Footnotes

  • We wish to express our appreciation to the patients and families who have contributed their time and energies to ataxia telangiectasia research. This study was supported by the Ataxia-Telangiectasia Children’s Project, Deerfield Beach, FL and the Pediatric Clinical Research Center, The Johns Hopkins Hospital, Baltimore, MD (grant RR00052, Division of Research Resources, National Institutes of Health).

  • Competing interests: none declared

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