Chest
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Cystic fibrosis (CF) is a life-shortening autosomal recessive disorder caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is an anion channel that conducts bicarbonate and chloride across cell membranes. Although defective anion transport across epithelial cells is accepted as the basic defect in CF, many of the features observed in people with CF and organs affected by CF are modulated by the nervous system. ⋯ The goal of this special feature article is to highlight the expression and function of CFTR in the nervous system. Special emphasis is placed on nervous system abnormalities described in people with CF and in animal models of CF. Finally, features of CF that may be modulated by or attributed to faulty nervous system function are discussed.
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We sought to describe the characteristics of adult patients with bronchiectasis enrolled in the US Bronchiectasis Research Registry (BRR). ⋯ Adult patients with bronchiectasis enrolled in the US BRR are described, with differences noted in demographic, radiographic, microbiological, and treatment variables based on stratification of the presence of NTM.
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Digital high-speed video microscopy (DHSV) allows analysis of ciliary beat frequency (CBF) and ciliary beat pattern (CBP) of respiratory cilia in three planes. Normal reference data use a sideways edge to evaluate ciliary dyskinesia and calculate CBF using the time needed for a cilium to complete 10 beat cycles. Variability in CBF within the respiratory epithelium has been described, but data concerning variation of CBP is limited in healthy epithelium. This study aimed to document variability of CBP in normal samples, to compare ciliary function in three profiles, and to compare CBF calculated over five or 10 beat cycles. ⋯ Our study demonstrates a lack of uniformity and consistency in manual CBP analysis of samples from healthy subjects, emphasizing the risk of automated CBP analysis in limited regions of interest and of single and limited manual CBP analysis. The toward the observer and from above profiles may be used to calculate CBF but may be less sensitive for evaluation of ciliary dyskinesia and CBP. CBF can be measured reliably by evaluation of only five-beat cycles.
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A 61-year-old woman presented for outpatient evaluation of a 1-week history of fever and upper respiratory symptoms. She denied tobacco use, weight loss, hemoptysis, chronic cough, or recent travel and was in otherwise good health. ⋯ She denied any worsening hoarseness or any other vocal changes. She did report a positive family history of squamous cell lung cancer in her father.
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A 33-year-old woman of Latin American origin was referred to our department by her primary care physician for a left lower lobe mass, which was incidentally found on a CT scan of her abdomen. The patient had complaints of abdominal pain for which she underwent imaging of her abdomen. Review of systems was negative for any respiratory complaints, and she denied any history of cigarette smoking or recreational drug use.