Chest
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Case Reports
Subclavian Artery Branch Pseudoaneurysm Rupture With Massive Hemothorax in a Patient With Neurofibromatosis Type 1.
Neurofibromatosis type 1 is a rare disorder that occurs secondary to pathogenic variants in the NF1 tumor suppressor gene on chromosome 17. Characteristic clinical manifestations include multiple hyperpigmented macules, axillary and inguinal freckling, optic gliomas, and numerous skin neurofibromas. ⋯ Aneurysms in these patients are often asymptomatic, and most patients with this complication appear for treatment after vessel rupture. We describe a 33-year-old man with neurofibromatosis type 1 who presented with chest pain and was ultimately found to have a ruptured left subclavian artery branch pseudoaneurysm leading to a large hemothorax.
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Clinical characteristics of the international population with severe asthma are unknown. Intercountry comparisons are hindered by variable data collection within regional and national severe asthma registries. We aimed to describe demographic and clinical characteristics of patients treated in severe asthma services in the United States, Europe, and the Asia-Pacific region. ⋯ Using a common data set and definitions, this study describes severe asthma characteristics of a large patient cohort included in multiple severe asthma registries and identifies country differences. Whether these are related to underlying epidemiological factors, environmental factors, phenotypes, asthma management systems, treatment access, and/or cultural factors requires further study.
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Bronchial epithelial reticular basement membrane (RBM) thickening occurs in diseases with both eosinophilic (allergic bronchial asthma [BA]) and neutrophilic (cystic fibrosis [CF] and primary ciliary dyskinesia [PCD]) chronic airway inflammation; however, the lung function and airway remodeling relation remains unclear. The aim of this study was to test whether ventilation inhomogeneity is related to RBM thickening. ⋯ Lung function impairment in relation to RBM thickness was milder in BA than in CF and PCD. In asthma, ventilation inhomogeneity did not correlate with RBM thickness, whereas it did in CF and PCD. This outcome suggests a different structure-function relation in these diseases.
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Age has been implicated as a factor in the plateau of long-term survival after lung transplant. ⋯ The youngest and oldest adult recipients experienced the lowest posttransplant survival through divergent pathways that may present opportunities for intervention to improve survival after lung transplant.