Chest
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Poor treatment adherence is common in cystic fibrosis (CF) and may lead to worse health outcomes and greater health-care use. This study evaluated associations of adherence to pulmonary medications, age, health-care use, and cost among patients with CF. ⋯ Worse adherence to pulmonary medications was associated with higher acute health-care use in a national, privately insured cohort of patients with CF. Addressing adherence may reduce avoidable health-care use.
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OSA is highly prevalent in children and usually initially treated by adenotonsillectomy. Nonsurgical alternatives for mild OSA primarily consisting of antiinflammatory approaches have emerged, but their efficacy has not been extensively assessed. ⋯ A combination of ICS + OM as initial treatment of mild OSA appears to provide an effective alternative to adenotonsillectomy, particularly in younger and nonobese children. These results support implementation of multicenter randomized trials to more definitively establish the role of ICS + OM treatment in pediatric OSA.
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Randomized Controlled Trial Comparative Study
Attentional modulation of reflex cough.
Reflex cough is a defensive response generated in the brainstem in response to chemical and mechanical stimulation of the airways. However, converging evidence shows that reflex cough is also influenced by central neural control processes. In this study, we investigate whether reflex cough can be modulated by attentional focus on either external stimuli or internal cough-related stimuli. ⋯ Reflex cough can be modulated by attentional focus. Internally focused attention may be a mechanism involved in excessive (idiopathic) cough, while an external focus may be introduced as part of treatments targeting excessive cough.
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Short telomeres are frequently identified in patients with idiopathic pulmonary fibrosis (IPF) and its inherited form, familial interstitial pneumonia (FIP). We identified a kindred with FIP with short telomeres who did not carry a mutation in known FIP genes TERT or hTR. We performed targeted sequencing of other telomere-related genes to identify the genetic basis of FIP in this kindred. ⋯ Polymerase chain reaction-based sequencing of NOP10 , TINF2 , NHP2 , and DKC1 revealed that both affected siblings shared a novel A to G 1213 transition in DKC1 near the hTR binding domain that is predicted to encode a Thr405Ala amino acid substitution. hTR levels were decreased out of proportion to DKC1 expression in the T405A DKC1 proband, suggesting this mutation destabilizes hTR and impairs telomerase function. This DKC1 variant represents the third telomere-related gene identified as a genetic cause of FIP. Further investigation into the mechanism by which dyskerin contributes to the development of lung fibrosis is warranted.
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Observational Study
Functional Impact of a Spectrum of Interstitial Lung Abnormalities in Rheumatoid Arthritis.
Approximately 10% of patients with rheumatoid arthritis (RA) have interstitial lung disease (ILD), and one-third have subclinical ILD on chest CT scan. In this study, we aimed to further characterize functional decrements in a spectrum of RA-associated ILD. ⋯ We have shown that patients with RA have varying degrees of ILAs that are associated with a spectrum of functional and physiologic decrements. Our findings suggest that improved risk stratification and detection of ILAs will provide a therapeutic window that could improve RA-ILD outcomes.