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Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis
L S Aboussouan1, S U Khan, D P Meeker
1Division of Pulmonary and Critical Care Medicine, Wayne State University School of Medicine, Harper Hospital, Detroit, MI 48201, USA.
Background:
Noninvasive positive-pressure ventilation may benefit patients with amyotrophic lateral sclerosis and respiratory insufficiency.
Objective:
To determine 1) whether patients tolerant of noninvasive positive-pressure ventilation have better survival than intolerant patients and 2) whether bulbar symptoms account for intolerance of noninvasive positive-pressure ventilation.
Design:
Observational cohort study.
Setting:
Tertiary care referral center.
Patients:
39 patients with amyotrophic lateral sclerosis who were treated with noninvasive positive-pressure ventilation.
Intervention:
Noninvasive positive-pressure ventilation was started for patients with new orthopnea, new hypercapnia, or both. Patients were divided into two groups: those tolerant of and those intolerant of noninvasive positive-pressure ventilation.
Results:
The risk for death from onset of respiratory insufficiency was higher for intolerant patients than for tolerant patients (relative risk, 3.1 [95% CI, 1.8 to 9.6]). Moderate or severe bulbar symptoms were more prevalent among intolerant patients than among tolerant patients (67% compared with 33%; P = 0.04).
Conclusions:
Among patients with amyotrophic lateral sclerosis, those who are tolerant of noninvasive positive-pressure ventilation have better survival than do those who are intolerant. Bulbar symptoms partially account for intolerance of noninvasive positive-pressure ventilation.
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