• Justin L Lockman, Andrew J Iskander, Melania Bembea, Thomas O Crawford, Howard M Lederman, Sharon McGrath-Morrow, and R Blaine Easley.
• Department of Anesthesiology, Johns Hopkins Hospital, Baltimore, MD, USA.
• Pediatr Crit Care Me. 2012 Mar 1; 13 (2): e84-90.

ObjectiveTo describe the presentation, clinical course, and outcomes of critically ill patients with ataxia-telangiectasia.DesignRetrospective case series.SettingAdult and pediatric intensive care units at an urban tertiary academic center.PatientsSeven consecutive patients with confirmed diagnosis of ataxia-telangiectasia had nine intensive care admissions between January 1995 and December 2009.InterventionsNone.Measurements And Main ResultsMean 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).ConclusionsCritically 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.

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