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Ataxia‐Telangiectasia and mechanical ventilation: A word of caution
Author(s) -
Verhagen Mijke M.M.,
Yntema JanBart,
Weemaes Corry M.R.,
Willemsen Michèl A.A.P.,
Verhagen Mijke M.M.,
van Deuren Marcel,
Van der Hoeven Hans J.,
Neeleman Chris,
Heijdra Yvonne F.
Publication year - 2009
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20957
Subject(s) - medicine , pediatric neurology , pediatrics
Ataxia-telangiectasia (A-T) is a rare autosomal recessive multisystem disorder with a short life expectancy. During adoles- cence pulmonary complications are common in A-T patients, and respiratory failure is the most important cause of death. Therefore me- chanical ventilation is often considered in these patients. In this study we retrospectively evaluated the clinical characteristics and ICU course of three consecutive adolescent A-T patients who were mechanically ventilated for respiratory failure. None of these patients had a history of respiratory infection. In all patients volume-pressure ratios indicated severe restrictive lung dysfunction. This was supported by data on pre-admission lung function testing. All three patients died of hypercapnic respiratory failure during ICU admission. The study shows that severe restrictive lung function is a common finding in adolescent A-T patients which may seriously complicate ventilatory treatment. Preadmission lung function testing is essential for the optimal medical management of these patients.