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Objectives: Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized by dry cough, fatigue, and progressive exertional dyspnea.Lung parenchyma and architecture is destroyed, compliance is lost, and gas exchange is compromised in this debilitating condition that leads inexorably to respiratory failure and death within 3-5 years of diagnosis. This review discusses treatment approaches to IPF in current use and those that appear promising for future development.Data Source: The data were obtained from the Randomized Controlled Trials and scientific studies published in English literature. We used search terms related to IPF, antifibrotic treatment, lung transplant, and management. Results: Etiopathogenesis of IPF is not fully understood, and treatment options are limited. Pathological features of IPF include extracellular matrix remodeling, fibroblast activation and proliferation, immune dysregulation, cell senescence, and presence of aberrant basaloid cells. The mainstay therapies are the oral

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The role of oscillatory potentials occurring near the threshold for the fast sodium current (ThVos) in the induction of spontaneous and repetitive activity was studied in sheep Purkinje fibers superfused in vitro. In low extracellular potassium concentration, the steepness and amplitude of diastolic depolarization increased and ThVos appeared during quiescence. ThVos amplitude increased progressively until its depolarizing phase reached the threshold potential for the initiation of the action potential. Drive increased the amplitude of diastolic depolarization and of ThVos, and longer drives induced faster and longer-lasting repetitive activity ("overdrive excitation"). In quiescent fibers, barium depolarized the resting membrane and initiated spontaneous discharge through ThVos. Acetylcholine had similar actions. Cesium hyperpolarized the membrane, thereby suppressing ThVos and related spontaneous activity. Tetrodotoxin and lidocaine also suppressed ThVos, but not the driven action potentials. In low extracellular potassium plus high extracellular calcium concentrations, drive induced ThVos as well as the oscillatory potentials related to calcium overload (Vos), but caused overdrive excitation through ThVos, even when caffeine was present. We conclude from our results that in Purkinje "dominant" pacemaker fibers (i) diastolic depolarization initiates spontaneous activity by attaining the threshold for the upstroke of the action potential through the depolarizing phase of a ThVos; (ii) the depolarizing phase of ThVos is caused by a tetrodotoxin-sensitive Na+ component; (iii) ThVos is voltage dependent in that a small depolarization of the resting membrane induces it and a small hyperpolarization suppresses it; (iv) ThVos can induce overdrive excitation; and (v) ThVos occurs in the absence of calcium overload and has distinguishing characteristics from the Vos induced by calcium overload.

Although pulmonary rehabilitation results in improvement in multiple outcome areas, relatively few studies in the United States have evaluated its effect on healthcare utilization. This study compared aspects of healthcare utilization during the year before to the year after outpatient pulmonary rehabilitation in patients with chronic obstructive pulmonary disease referred to 11 hospital-based centers in Connecticut and New York. Utilization data from 128 of 132 patients who originally gave informed consent were evaluated; their mean age was 69 years and their forced expiratory volume in 1 second was 44% of predicted. Forty-five percent had 1 or more hospitalizations in the year before beginning pulmonary rehabilitation. In the year after pulmonary rehabilitation, there were 0.25 fewer total hospitalizations (P = .017) and 2.18 fewer hospital days (P = .015) per patient and 271 fewer hospital days for the group. Hospitalizations for respiratory reasons also decreased significantly. Most of the reduction in hospital utilization was due to a decrease in intensive care unit days. The number of physician visits decreased by 2.4 in the year after pulmonary rehabilitation (P < .0001); most of this reduction was due to decreased visits to primary care providers. The estimated costs/charges for the aspects of healthcare utilization that we studied decreased by a mean of 4,694 dollars and a median of 390 dollars (P = .0002). This study suggests that pulmonary rehabilitation leads to a reduction in healthcare utilization.

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The possible link between gastroesophageal reflux and nontuberculous mycobacterial lung disease, and the microbiology and resistance patterns of bacteria observed in these patients were clarified. A large study of inhaled tobramycin for exacerbations was inconclusive, but macrolide therapy and hyperosmolar agents hold promise.

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