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Showing papers in "Clinics in Chest Medicine in 2016"


Journal ArticleDOI
TL;DR: VILI prevention strategies must balance risk of lung injury with untoward side effects from the preventive effort, and may be most effective when targeted to subsets of patients at increased risk.

199 citations


Journal ArticleDOI
TL;DR: There is early onset of lung disease in PCD with abnormal airflow mechanics and radiographic abnormalities detected in infancy and early childhood.

172 citations


Journal ArticleDOI
TL;DR: All possible causes of eosinophilia, including drug, toxin, fungus related etiologies, must be thoroughly investigated andExtrathoracic manifestations should raise the suspicion of eOSinophilic granulomatosis with polyangiitis.

140 citations


Journal ArticleDOI
TL;DR: It is now appreciated that CF airways typically harbor complex microbial communities, and that changes in the structure and activity of these communities have a bearing on patient clinical condition and lung disease progression.

121 citations


Journal ArticleDOI
TL;DR: The diagnosis of PAP-causing diseases in secondary PAP requires further studies, and whole-lung lavage is the current standard therapy and promising new pharmacologic therapies are in development.

117 citations


Journal ArticleDOI
TL;DR: CF now serves as a model for chronic illness management, continuous quality improvement via registry data, and a seamless link between basic science research, translational studies, clinical trials, and outcomes research to enable rapid expansion of treatment options.

111 citations


Journal ArticleDOI
TL;DR: The article discusses the known factors associated with increased risk for developing sepsis and the limitations of the current clinical definition and the clinical correlations of theCurrent epidemiology.

101 citations


Journal ArticleDOI
TL;DR: Although several treatments have been shown to protect the microcirculation during sepsis, they have not improved patient outcomes when applied indiscriminately and future outcomes-oriented studies are needed to test sepsi therapeutics when personalized to a patient's microcirculatory dysfunction.

90 citations


Journal ArticleDOI
TL;DR: HPS pulmonary fibrosis shows many of the clinical, radiologic, and histologic features found in idiopathic pulmonary Fibrosis, but occurs at a younger age.

77 citations


Journal ArticleDOI
TL;DR: The current understanding of immune dysregulation in CF is summarized, which may drive hyperinflammation and impaired host defense.

72 citations


Journal ArticleDOI
TL;DR: A dysfunction of the brainstem, amygdala, and hippocampus might account for the increased mortality, psychological disorders, and cognitive impairment in SAE.

Journal ArticleDOI
TL;DR: Computed tomography of the chest in patients with LIP may reveal ground-glass opacities, centrilobular and subpleural nodules, and randomly distributed thin-walled cysts, which are the key to differentiating LIP from lymphoma.

Journal ArticleDOI
TL;DR: Aggressive use of antipseudomonal antibiotics is the standard of care for acute pulmonary exacerbations in cystic fibrosis, and providers must take into account specific patient characteristics when making treatment decisions related to antibiotic selection, route and duration of administration, and site of care.

Journal ArticleDOI
TL;DR: Given the marked changes in metabolism in sepsis and the association of worse prognosis in patients with severe metabolic derangements, this work summarizes the seminal trials conducted to optimize nutrition in the ICU.

Journal ArticleDOI
TL;DR: The clinical features of the hyper-IgE syndromes caused by mutations in STAT3, DOCK8, and PGM3 are discussed with a particular focus on the pulmonary manifestations and discussion of the genetics, pathogenesis, and approaches to therapy.

Journal ArticleDOI
TL;DR: Emerging data strongly suggest that cigarette smoke and its components can lead to acquired cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction, which is associated with chronic bronchitis and may contribute to smoking-related systemic diseases.

Journal ArticleDOI
TL;DR: There is great phenotypic variability in the clinical features of BHD, and patients can present with any combination of skin, pulmonary, or renal findings.

Journal ArticleDOI
TL;DR: In this article, the central role of intravenous fluid in sepsis management, fundamental questions regarding which fluid and in what amount remain unanswered, despite advances in understanding the physiologic response to fluid administration, and large clinical studies examining resuscitation strategies, fluid balance after resuscitation, colloid versus crystalloid solutions, and high- versus low-chloride crystalloids, inform the current approach to septic fluid management.

Journal ArticleDOI
TL;DR: If transplant evaluation has been completed, patients with CF and respiratory failure requiring mechanical ventilation and extracorporeal membrane oxygenation may remain viable candidates for transplant with outcomes comparable to other individuals with CF.

Journal ArticleDOI
TL;DR: Clinicians and laboratories alike must appreciate key factors influencing the appropriate use and potential impact of rapid molecular methods for the diagnosis of sepsis and their potential to enhance the success of antimicrobial stewardship programs.

Journal ArticleDOI
TL;DR: The underlying pathophysiology of cardiac dysfunction is emphasized and recent evidence related to diagnosis is explored, including the utility of biomarkers, the role of echocardiography, and management goals and treatment.

Journal ArticleDOI
TL;DR: Asymptomatic patients with a confirmed diagnosis of BML, may be followed conservatively without treatment, and, in general, most patients have a favorable prognosis.

Journal ArticleDOI
Marie E. Egan1
TL;DR: Understanding specific CF genotypes is essential for providing state-of-the art care to patients and new therapies are being developed to target mutant CFTR and restore CFTR function.

Journal ArticleDOI
TL;DR: This review highlights the relevance of hyperglycemia, hypoglyceia, and glycemic variability in patients with sepsis with an emphasis on a rational approach to management.

Journal ArticleDOI
TL;DR: An overview of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH), two disorders that challenge clinicians, radiologists, and pathologists because they often mimic pulmonary arterial hypertension (PAH).

Journal ArticleDOI
TL;DR: This article summarizes the literature on the complexity of changes at the molecular level for the casual reader on the basis of clinical criteria, prognostication, and clinical practice.

Journal ArticleDOI
TL;DR: Current evidence does not support routine use of sepsis alert systems in clinical practice, and continuous improvement in the afferent and efferent aspects will help translate theoretic advantages into measurable patient benefit.

Journal ArticleDOI
TL;DR: The most common GI complications of CF, including reflux, pancreatic insufficiency, small bowel intestinal overgrowth, distal intestinal obstruction syndrome, and GI malignancy are discussed, with emphasis on clinical presentation and management.

Journal ArticleDOI
TL;DR: The recent development of a laboratory animal model has revealed several promising treatment approaches for future trials, including supplemental oxygen therapy and lung transplantation, for patients with PAM.

Journal ArticleDOI
TL;DR: The thoracic lymphatic disorders are a heterogeneous group of uncommon conditions that are associated withThoracic masses, interstitial pulmonary infiltrates, and chylous complications and new imaging techniques to characterize lymphatic flow are redefining approaches to disease classification and therapy.