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Non-invasive ventilation for the treatment of hypercapnic respiratory failure in cystic fibrosis

Correspondence to:
Peadar G Noone, Pulmonary Division, CB# 7020, UNC Chapel Hill, NC 27599-7020, USA; pnoone@med.unc.edu

The first 150 words of the full text of this article appear below.

Median survival for patients with cystic fibrosis (CF) has improved steadily over the past several decades as a result of a multifaceted treatment approach to the disease.¹ Despite this aggressive care, many patients with classic disease eventually develop respiratory failure from progressive airways obstruction and bronchiectasis.² At this stage, treatment strategies may focus on alleviating both the symptomatic and physiological effects of respiratory failure. Initially respiratory failure may be mainly hypoxic (type 1: arterial oxygen pressure (PaO₂) <8 kPa (or 60 mm Hg), with normal arterial carbon dioxide pressure (PaCO₂)), and supplemental oxygen during sleep and/or exercise may be effective. However, when the disease becomes more severe, hypercapnia may ensue (type 2: PaCO₂ >6 kPa (or 45 mm Hg)). Such patients with chronic respiratory failure usually have obvious clinical signs and symptoms, and blood gas analysis demonstrates a compensated respiratory acidosis.³ Thus it is important . . . [Full text of this article]