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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Acute bronchiolitis is common in young children, and some children develop chronic cough after their bronchiolitis. We thus undertook systematic reviews based on key questions (KQs) using the PICO (Population, Intervention, Comparison, Outcome) format. The KQs were: Among children with chronic cough (> 4 weeks) after acute viral bronchiolitis, how effective are the following interventions in improving the resolution of cough?: (1) Antibiotics. If so what type and for how long? (2) Asthma medications (inhaled steroids, beta2 agonist, montelukast); and (3) Inhaled osmotic agents like hypertonic saline? ⋯ The panel made several consensus-based suggestions and identified directions for future studies to advance the field of managing chronic cough post-acute bronchiolitis in children.
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A growing body of evidence supports a bidirectional relationship between posttraumatic stress disorder (PTSD) and sleep disturbances. Fragmented sleep induced by sleep-related breathing disorders, insomnia, and nightmares impacts recovery and treatment outcomes and worsens PTSD symptoms. Despite recent attention, management of these disorders has been unrewarding in the setting of PTSD. ⋯ Imagery rehearsal therapy has shown significant reduction in nightmare intensity and frequency. The success of noradrenergic blocking agents has not been consistent among studies, with one-half reporting treatment failure. An integrated stepped care approach that includes components of both behavioral and pharmacologic interventions customized to patients' sleep-maladaptive behaviors may offer a solution to delivering accessible, effective, and efficient services for individuals with PTSD.
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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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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.