Chest
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Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and remains one of the most common life-shortening genetic diseases affecting the lung and other organs. CFTR functions as a cyclic adenosine monophosphate-dependent anion channel that transports chloride and bicarbonate across epithelial surfaces, and disruption of these ion transport processes plays a central role in the pathogenesis of CF. These findings provided the rationale for pharmacologic modulation of ion transport, either by targeting mutant CFTR or alternative ion channels that can compensate for CFTR dysfunction, as a promising therapeutic approach. ⋯ The approval of the first potentiator ivacaftor for the treatment of patients with specific CFTR mutations and, more recently, the corrector lumacaftor in combination with ivacaftor for patients homozygous for the common F508del mutation, were major breakthroughs on the path to causal therapies for all patients with CF. The present review focuses on recent developments and remaining challenges of CFTR-directed therapies, as well as modulators of other ion channels such as alternative chloride channels and the epithelial sodium channel as additional targets in CF lung disease. We further discuss how patient-derived precision medicine models may aid the translation of emerging next-generation ion channel modulators from the laboratory to the clinic and tailor their use for optimal therapeutic benefits in individual patients with CF.
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In specialty clinics, a staff physician is often required to direct patient flow through the clinic and performs all documentation for coding/billing. In response to the workload created by increased patient volume, many specialty clinics have implemented protocols for both disease treatment and coordination of clinic flow. In this article, we review the literature on using mobile technology to assist with patient care, clinic flow, disease treatment, and documentation/billing. We also describe the development and implementation of a mobile application in our pulmonary clinic designed to automate patient flow, assist the physician in documentation/billing, and gather research data including review of initial user data and lessons learned.
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Refractory shock is a lethal manifestation of cardiovascular failure defined by an inadequate hemodynamic response to high doses of vasopressor medications. Approximately 7% of critically ill patients will develop refractory shock, with short-term mortality exceeding 50%. Refractory vasodilatory shock develops from uncontrolled vasodilation and vascular hyporesponsiveness to endogenous vasoconstrictors, causing failure of physiologic vasoregulatory mechanisms. ⋯ Novel vasopressor agents, such as synthetic human angiotensin II, can increase BP and reduce the need for high doses of catecholamine vasopressors in severe or refractory vasodilatory shock. Few effective rescue therapies exist for established refractory shock, which emphasizes the importance of aggressive intervention before refractory shock develops, including the earlier initiation of rational combination vasopressor therapy. The present review discusses the diagnosis and management of refractory shock to offer guidance for management of this important clinical problem and to provide a framework for future research.
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Compared with pulmonary sarcoidosis, sarcoidosis without lung involvement may involve other immunopathologic mechanisms and be associated with other demographic and clinical features. ⋯ Significant demographic and sex differences were observed between patients with pulmonary and nonpulmonary sarcoidosis. These differences reflect previous data concerning differences between patients with skin and lung sarcoidosis because the skin was the major organ involved with NPS. Although the lungs are likely the primary site of exposure in pulmonary sarcoidosis, the high prevalence of skin involvement in NPS suggests the skin is the most conducive site of antigen capture outside of the respiratory tract.
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Lung ultrasound examination is becoming an even more important part of pulmonologists' clinical routine. As indicated in the literature, the coordinates of any findings on lung parenchyma are based on surface landmarks or conventional quadrants. In our experience we have noticed that lung fissures are clearly detectable as interruptions of the pleural line, but this has never been investigated previously. The aim of this study was to evaluate whether lung fissures are detectable under normal conditions in routine clinical practice. ⋯ Lung fissures may be detected with ultrasound once adequate training is provided. This may allow the clinician a more precise anatomical delineation of pathology identified by lung ultrasound.