Chest
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Unintentional leaks, patient-ventilatory asynchrony, and obstructive or central events (either residual or induced by noninvasive positive pressure ventilation [NPPV]) occur in patients treated with NPPV, but the impact of ventilator settings on these disturbances has been little explored. The objective of this study was to investigate the impact of backup respiratory rate (BURR) settings on the efficacy of ventilation, sleep structure, subjective sleep quality, and respiratory events in a group of patients with obesity hypoventilation syndrome (OHS). ⋯ In a homogenous group of patients treated with long-term NPPV for obesity-hypoventilation, changing BURR from an S/T mode with a high or low BURR to an S mode was associated with the occurrence of a highly significant increase in respiratory events, of mainly central and mixed origin.
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Patent foramen ovale (PFO) may contribute to nocturnal desaturation in patients with obstructive sleep apnea (OSA), and the effect of PFO closure in OSA is unknown. Our study tested the hypotheses that: (1) patients with severe OSA have a higher prevalence of PFO compared with healthy control subjects, (2) patients with severe OSA with clinically significant PFO experience more nocturnal desaturation than those without, and (3) PFO closure reduces nocturnal desaturation. ⋯ Patients with severe OSA have a higher prevalence of PFO with large shunts compared with control subjects. The ODI/AHI ratio is increased in patients with OSA with clinically significant shunts. PFO closure does not reduce nocturnal desaturation.
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The occurrence and mechanisms of nocturnal hypoxemia in precapillary pulmonary hypertension (PH) are not clearly defined. ⋯ The occurrence of nocturnal hypoxemia is high in PH and should be screened for systematically. Further studies are needed to determine the impact of nocturnal hypoxemia on the outcome of patients with PH.
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Cystic fibrosis (CF) is a life-shortening inherited disease caused by mutations in the CF transmembrane conductance regulator gene (CFTR), which encodes for the CF transmembrane conductance regulator (CFTR) ion channel that regulates chloride and water transport across the surface of epithelial cells. Ivacaftor, a drug recently approved by the US Food and Drug Administration, represents the first mutation-specific therapy for CF. It is a CFTR channel modulator and improves CFTR function in patients with CF who have a G551D mutation. ⋯ However, a decrease in sweat chloride did not correlate with improvement in FEV(1), nor did there appear to be a threshold level for change in sweat chloride above which an improvement in FEV(1) was apparent. The lack of correlation of sweat chloride with improvement in FEV(1) speaks to the multiplicity of factors, physiologic, environmental, and genetic, that likely modulate CF disease severity. Future clinical trials of drugs that are directed to the defective CFTR will need take into account the uncertainty of using even established measurements, such as sweat chloride, as clinical end points.
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Bullous pemphygoid is the most common blistering skin disease, characterized by an autoantibody response against two major hemidesmosomal antigens within the dermo-epidermal junction. We describe a proven case of bullous pemphigoid with extensive tracheobronchial involvement and with the only bronchoscopic images available in the published literature, to our knowledge. The patient, a 73-year-old woman with a medical history of bullous pemphigoid, was admitted to our hospital for dyspnea, productive cough, and blood-streaked sputum. ⋯ Pathologic evaluation, direct immunofluorescence microscopy examination, and enzyme-linked immunosorbent assay led to a definitive diagnosis of bullous pemphigoid. Due to the potential confounding presence of bacterial superinfection, the real prevalence of such manifestation of this disease is still unknown. Our experience should alert clinicians about this possible localization of bullous pemphigoid.