Chest
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In COPD, a decreased inspiratory capacity to total lung capacity ratio (IC/TLC) is associated with dynamic hyperinflation and poor exercise capacity. The association with upper-extremity force measured by handgrip strength (HGS) and 6-min walk distance (6MWD) has not been previously described. We hypothesized that IC/TLC affects muscle strength in the upper and lower extremities, affecting HGS and 6MWD. ⋯ HGS and 6MWD are reduced in patients with COPD, particularly in those with hyperinflation and evidence of longitudinal deterioration not seen in control subjects. This finding suggests that resting hyperinflation may exert a detrimental effect on cardiac function and plays a role in reduced exercise performance in patients with COPD.
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Lymphangioleiomyomas occur in 38% of patients with sporadic lymphangioleiomyomatosis (LAM) and may cause pain and increased abdominal girth, mimicking the presence of a malignancy. Lymphatic involvement in LAM is closely associated with elevated serum levels of vascular endothelium growth factor-D (VEGF-D). Because lymphangioleiomyomas undergo diurnal variation in volume, we hypothesized that daytime ingestion of food, by increasing chyle formation and lymphatic flow, is the cause of an increase in lymphangioleiomyoma volume. ⋯ Lymphangioleiomyoma volume decreased during the fasting state. Conversely, a combination of food intake and decreased chyle flow through lymphatics partially obstructed by LAM cells may account for increases in lymphangioleiomyoma size. Imaging studies performed under fasting conditions may help in determining whether an abdominal tumor is a result of LAM or malignancy.
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Interstitial lung disease (ILD) is a heterogeneous group of rare diseases that primarily affect the pulmonary interstitium. Studies have implicated a role for telomere length (TL) maintenance in ILD, particularly in idiopathic interstitial pneumonia (IIP). Here, we measure TL in a wide spectrum of sporadic and familial cohorts of ILD and compare TL between patient cohorts and control subjects. ⋯ The results show telomere shortening across all ILD diagnoses. The difference in TL between the FIP-TERT and FIP-SFTP groups indicates the distinction between acquired and innate telomere shortening. Short TL in the IPF and FIP-no mutation groups is indicative of an innate telomere-biology defect, while a stress-induced, acquired telomere shortening might be the underlying process for the other ILD diagnoses.