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Archives of Disease in Childhood 2006;91:610-611; doi:10.1136/adc.2006.094268
Copyright © 2006 BMJ Publishing Group Ltd & Royal College of Paediatrics and Child Health.

SHORT REPORT

Survival probability in ataxia telangiectasia

T O Crawford1,2, R L Skolasky1, R Fernandez3, K J Rosquist2, H M Lederman2

1 Department of Neurology, The Johns Hopkins Hospital, Baltimore, Maryland, USA
2 Department of Pediatrics, The Johns Hopkins Hospital
3 Ataxia Telangiectasia Children’s Project, Deerfield Beach, Florida, USA

Correspondence to:
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.

Abbreviations: ATCC, Ataxia Telangiectasia Clinical Center at Johns Hopkins Hospital; ATCP, Ataxia Telangiectasia Children’s Project

Keywords: survival analysis; cancer; ataxia telangiectasia


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