Chest
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Multicenter Study
Effects of Ivacaftor in cystic fibrosis patients carrying the G551D mutation with severe lung disease.
The development of ivacaftor represents a significant advance in therapeutics for patients with cystic fibrosis (CF) who carry the G551D mutation. Patients with an FEV1 < 40% predicted represent a considerable proportion of eligible patients but were excluded from phase 3 clinical trials, and the effectiveness of the drug in this population is, therefore, unknown. ⋯ Ivacaftor was clinically effective in patients with CF who carry the G551D mutation and have severe pulmonary disease. The reductions in treatment requirements were clinically and statistically significant and have not been described in less severe populations.
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American College of Chest Physicians' (CHEST) new Living Guidelines Model will not only provide clinicians with guidance based on the most clinically relevant and current science but will also allow expert-informed guidance to fill in any gaps in the existing evidence. These guidance documents will be updated, as necessary, using one or more of three processes: (1) evidence-based guidelines, (2) trustworthy consensus statements, and (3) a hybrid of the other two. The new Living Guidelines Model will be more sustainable and will encourage maintenance of current and targeted recommendations and suggestions. ⋯ These reliable statements of guidance for health-care providers and patients are based on a rigorous methodology and transparency of process.
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The 2011 International Association for the Study of Lung Cancer (IASLC)/American Thoracic Society (ATS)/European Respiratory Society (ERS) classification of pulmonary adenocarcinomas introduces adenocarcinoma in situ and minimally invasive carcinoma and categorizes adenocarcinoma with more extensive invasion by the predominant subtype. Data have shown that wedge or segmentectomy (W/S) may be appropriate for in situ and minimally invasive adenocarcinoma, but whether sublobar resection is appropriate for tumors with more extensive invasion is unclear. The aim of this pilot study is to evaluate whether there are any trends regarding the impact of invasion and subtypes of carcinoma regarding survival in lobectomy vs W/S procedures using a comprehensive histologic evaluation. ⋯ Our findings corroborate the prognostic significance of the 2011 adenocarcinoma subtyping classification and additionally suggest that lobectomy does not offer an overall survival advantage over W/S regardless of histologic subtype. Therefore, this finding suggests that limited resection may be appropriate for small size tumors, particularly those ≤ 2 cm with invasive histology.
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Lung transplantation reduces mortality in patients with end-stage lung disease; however, only approximately 21% of lungs from potential donor patients undergo transplantation. A large number of donor lungs become categorized as unsuitable for lung transplantation as a result of lung injury around the time of brain death. Limiting this injury is key to increasing the number of successful lung procurements and subsequent transplants. This narrative review by a working group of pulmonologists, respiratory therapists, and lung transplant specialists elucidates principles of mechanical ventilatory support that can be used to limit lung injury in potential lung donor patients and examines the implementation of protocolized strategies in enhancing the procurement of donor lungs for transplantation.
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Pulmonary venoocclusive disease (PVOD) is a rare lung disease, diagnosed in 5% to 10% of patients with pulmonary hypertension (PH). The incidence, prevalence, and etiology of PVOD in children are not well defined. The mortality remains high, related, at least partly, to the limited treatment options. ⋯ PVOD is an important differential diagnosis for pediatric patients with PH. CT scanning is a valuable tool to image lung abnormalities; the definitive diagnosis can only be made by examination of lung biopsy specimens, which subjects the patient to additional risk. Early listing for lung transplantation is essential, as the mean survival time is only 14 months.