Chest
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Renal disease is a well-recognized manifestation of cystic fibrosis (CF) and people with CF are at increased risk of nephrolithiasis. Lithotripsy is the preferred treatment but has occasionally been associated with pulmonary complications. Here we report the case of a person with CF who developed a pneumothorax soon after lithotripsy and discuss the potential mechanism of injury. We hope this case highlights some of the additional considerations clinicians should take into account when managing patients with advanced pulmonary disease in CF.
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A 48-year-old man was referred for evaluation of an abnormal chest shadow noted on a routine chest radiograph during physical examination. He was asymptomatic and had no significant medical history and occupational exposure. ⋯ He had smoked approximately 20 cigarettes daily for 20 years. He had no illicit drug use and no animal-rearing history.
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The deficiency of α1-antitrypsin (AAT) is secondary to misfolding and polymerization of the abnormal Z-AAT in liver cells and is associated with lung emphysema. Alveolar macrophages (AMs) produce AAT; however, it is not known whether Z-AAT can polymerize in AMs, further decreasing lung AAT and promoting lung inflammation. Our intention was to investigate whether AAT polymerizes in human AMs and to study the possible relation between polymerization and degree of lung inflammation. ⋯ Polymerization of AAT in alveolar macrophages occurs in the lungs of individuals with AATD but also in smokers with normal AAT levels with or without COPD. Our findings highlight the similarities in the pathophysiology of COPD in individuals with and without AATD, adding a potentially important step to the mechanism of COPD.
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Observational Study
Poor Subpleural Perfusion Predicts Failure After Balloon Pulmonary Angioplasty for Nonoperable Chronic Thromboembolic Pulmonary Hypertension.
Poor subpleural perfusion (PSP) in the capillary phase of pulmonary angiography predicts worse outcomes following pulmonary endarterectomy in operable chronic thromboembolic pulmonary hypertension (CTEPH). Balloon pulmonary angioplasty (BPA) has emerged as a treatment for nonoperable CTEPH. The goal of the present article was to assess the association between PSP and BPA failure. ⋯ PSP in the capillary phase, suggesting the presence of small vessel disease with diffuse distal thrombosis, is a predictor of BPA failure. PSP was also associated with less developed bronchial arteries, which suggests a key role of bronchial-pulmonary anastomoses in maintaining the pulmonary capillary bed open downstream of the pulmonary arterial obstruction. PSP affected approximately 15% of patients with nonoperable CTEPH who underwent BPA.
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Parenteral prostanoids are considered the treatment of choice for patients with severe pulmonary arterial hypertension (PAH). Prognostic studies for patients treated in the modern era are limited. ⋯ In patients with PAH receiving treatment with a parenteral prostanoid, survival was significantly associated with the number of guideline-recommended lower risk and higher risk criteria achieved at first follow-up.