6482. A 19-year-old man with relapsing bilateral pneumothorax, hemoptysis, and intrapulmonary cavitary lesions diagnosed with vascular Ehlers-Danlos syndrome and a novel missense mutation in COL3A1.
A 19-year-old sportsman experienced a right-sided pneumothorax and hemoptysis after having had an intermittent cough and blood-tinged sputum for 2 months. A chest CT scan revealed small cavitary lesions in both lungs. The relapsing pneumothorax was treated with a chest tube twice, as well as surgically after the second relapse. Two months after surgery, the patient developed a cough, fever, and high C-reactive protein levels. At that time, large consolidations had developed in the right lung, while the left lung subsequently collapsed due to pneumothorax. The patient's physical appearance and anamnestic information led us to suspect a genetic connective tissue disease. A sequencing analysis of the COL3A1 gene identified a novel, de novo missense mutation that confirmed the diagnosis of vascular Ehlers-Danlos syndrome (EDS). This atypical presentation of vascular EDS with intrathoracic complications shows that enhanced awareness is required and demonstrates the usefulness of the genetic analyses that are clinically available for several hereditary connective tissue disorders.
6485. Starting a lung transplant program: a roadmap for long-term excellence.
作者: Julia Klesney-Tait.;Michael Eberlein.;Lois Geist.;John Keech.;Joseph Zabner.;Peter J Gruber.;Mark D Iannettoni.;Kalpaj Parekh.
来源: Chest. 2015年147卷5期1435-1443页
Lung transplantation is an effective therapy for many patients with end-stage lung disease. Few centers across the United States offer this therapy, as a successful lung transplant program requires significant institutional resources and specialized personnel. Analysis of the United Network of Organ Sharing database reveals that the failure rate of new programs exceeds 40%. These data suggest that an accurate assessment of program viability as well as a strategy to continuously assess defined quality measures is needed. As part of strategic planning, regional availability of recipient and donors should be assessed. Additionally, analysis of institutional expertise at the physician, support staff, financial, and administrative levels is necessary. In May of 2007, we started a new lung transplant program at the University of Iowa Hospitals and Clinics and have performed 101 transplants with an average recipient 1-year survival of 91%, placing our program among the top in the country for the past 5 years. Herein, we review internal and external factors that impact the viability of a new lung transplant program. We discuss the use of four prospectively identified quality measures: volume, recipient outcomes, financial solvency, and academic contribution as one approach to achieve programmatic excellence.
6486. Mobile health: assessing the barriers.
Mobile health (mHealth) combines the decentralization of health care with patient centeredness. Mature mHealth applications (apps) and services could provide actionable information, coaching, or alerts at a fraction of the cost of conventional health care. Different categories of apps attract diverse safety and privacy regulation. It is too early to tell whether these apps can overcome questions about their use cases, business models, and regulation.
6487. The effect of OSA on work disability and work-related injuries.
OSA is a common yet underdiagnosed respiratory disorder characterized by recurrent upper airway obstruction during sleep. OSA results in sleep fragmentation and repetitive hypoxemia and is associated with a variety of adverse consequences including excessive daytime sleepiness, reduced quality of life, cardiovascular disease, decreased learning skills, and neurocognitive impairment. Neurocognitive impairments that have been linked to poor sleep include memory deficits, decreased learning skills, inability to concentrate, and decreased alertness. Furthermore, the societal and economic costs of OSA are substantial; for example, patients with OSA have a significantly greater risk of motor vehicle crashes, consume more health-care resources, and have associated annual costs in the billions of dollars per year. It is increasingly recognized that OSA may also have substantial economic consequences. Specifically, there is accumulating evidence implicating OSA as an important contributor to work disability (including absenteeism, presenteeism) and work-related injuries. This review summarizes the current state of knowledge in these two areas.
6488. Antimicrobial resistance in hospital-acquired gram-negative bacterial infections.
Aerobic gram-negative bacilli, including the family of Enterobacteriaceae and non-lactose fermenting bacteria such as Pseudomonas and Acinetobacter species, are major causes of hospital-acquired infections. The rate of antibiotic resistance among these pathogens has accelerated dramatically in recent years and has reached pandemic scale. It is no longer uncommon to encounter gram-negative infections that are untreatable using conventional antibiotics in hospitalized patients. In this review, we provide a summary of the major classes of gram-negative bacilli and their key mechanisms of antimicrobial resistance, discuss approaches to the treatment of these difficult infections, and outline methods to slow the further spread of resistance mechanisms.
6489. Diagnosing and staging lung cancer involving the mediastinum.
The purpose of this article is to provide an update on evidence-based methods for mediastinal staging in patients with lung cancer. This is a review of the recently published studies and a summary of relevant guidelines addressing the role of CT scan, PET scan, endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA), and mediastinoscopy as pertinent to lung cancer staging and restaging. The focus is on how these diagnostic methods fit into the best algorithm for patients with chest imaging abnormalities suspected of malignant disease. Several studies, meta-analyses, and systematic reviews specifically targeted the role of PET scan, EBUS-TBNA, and mediastinoscopy for detecting mediastinal lymph node involvement in patients suffering from lung cancer. Based on the recommendations from the currently published guidelines, algorithms of care are proposed for staging and restaging of the mediastinum.
6490. Predischarge bundle for patients with acute exacerbations of COPD to reduce readmissions and ED visits: a randomized controlled trial.
作者: Jeffrey H Jennings.;Krishna Thavarajah.;Michael P Mendez.;Michael Eichenhorn.;Paul Kvale.;Lenar Yessayan.
来源: Chest. 2015年147卷5期1227-1234页
Hospital readmissions for acute exacerbations of COPD (AECOPDs) pose burdens to the health-care system and patients. A current gap in knowledge is whether a predischarge screening and educational tool administered to patients with COPD reduces readmissions and ED visits.
6491. Ambulatory extracorporeal membrane oxygenation as a bridge to lung transplantation: walking while waiting.
The proportion of critically ill patients awaiting lung transplantation has increased since the implementation of the Lung Allocation Score (LAS) in 2005. Critically ill patients comprise a sizable proportion of wait-list mortality and are known to experience increased posttransplant complications. These critically ill patients have been successfully bridged to lung transplantation with extracorporeal membrane oxygenation (ECMO), but historically these patients have required excessive sedation, been immobile, and have had difficult functional recovery in the posttransplant period and high mortality. One solution to the deconditioning often seen in critically ill patients is the implementation of rehabilitation and ambulation while awaiting transplantation on ECMO. Ambulatory ECMO programs of this nature have been developed in an attempt to provide rehabilitation, physical therapy, and minimization of sedation prior to lung transplantation to improve both surgical and posttransplant outcomes. Favorable outcomes have been reported using this novel approach, but how and where this strategy should be implemented remain unclear. In this commentary, we review the currently available literature for ambulation and rehabilitation during ECMO support as a bridge to lung transplantation, discuss future directions for this technology, and address the important issues of resource allocation and regionalization of care as they relate to ambulatory ECMO.
6500. Workplace Blame and Related Concepts: An Analysis of Three Case Studies.
Blame has been thought to affect quality by decreasing error reporting. Very little is known about the incidence, characteristics, or consequences of the distress caused by being blamed. Blame-related distress (B-RD) may be related to moral distress, but may also be a factor in burnout, compassion fatigue, lateral violence, and second-victim syndrome. The purpose of this article is to explore these related concepts through a literature review applied to three index critical care clinician cases.
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