title: The critically ill patient with ataxia-telangiectasia: a case series.
abstract: OBJECTIVE
  To describe the presentation, clinical course, and outcomes of critically ill patients with ataxia-telangiectasia.
  
  
  METHODS
  Retrospective case series.
  
  
  METHODS
  Adult and pediatric intensive care units at an urban tertiary academic center.
  
  
  METHODS
  Seven consecutive patients with confirmed diagnosis of ataxia-telangiectasia had nine intensive care admissions between January 1995 and December 2009.
  
  
  METHODS
  None.
  
  
  RESULTS
  Mean age at time of admission 15.9 yrs (median, 13.9 yrs; range, 7.3-33.9 yrs). Mean duration of intensive care unit stay was 17 days (median, 9 days; range, 2-39 days). The most common admitting diagnosis was respiratory distress (six of seven patients). There was no difference in ventilator settings or duration of intensive care unit stay between survivors and nonsurvivors (p > .05). Forty-three percent (three of seven patients) survived to intensive care unit discharge with a 3-yr survival that was 14% (one of seven patients).
  
  
  CONCLUSIONS
  Critically ill patients with ataxia-telangiectasia have complex, multisystem diseases. In this case series, the most common intensive care unit admission diagnosis was respiratory failure. Suspected or confirmed bacterial infections were prevalent. Neuropathologic autopsy findings were similar to those previously reported. Special considerations for the critical care of patients with ataxia-telangiectasia are discussed.
authors.affiliation: Department of Anesthesiology, Johns Hopkins Hospital, Baltimore, MD, USA.
authors.forename: Justin L
authors.initials: JL
authors.lastname: Lockman
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Iskander
authors.forename: Melania
authors.initials: M
authors.lastname: Bembea
authors.forename: Thomas O
authors.initials: TO
authors.lastname: Crawford
authors.forename: Howard M
authors.initials: HM
authors.lastname: Lederman
authors.forename: Sharon
authors.initials: S
authors.lastname: McGrath-Morrow
authors.forename: R Blaine
authors.initials: RB
authors.lastname: Easley
mesh_terms: D000293:Adolescent; D000328:Adult; D001260:Ataxia Telangiectasia / Q000150:complications / Q000401:mortality / Q000628:therapy*; D002648:Child; D016638:Critical Illness; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D007362:Intensive Care Units / Q000706:statistics & numerical data; D008297:Male; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult