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Background: Comprehensive US epidemiologic data for adult pleural disease are not available.Research Question: What are the epidemiologic measures related to adult pleural disease in the United States?Study Design and Methods: Retrospective cohort study using Healthcare Utilization Project databases (2007-2016). Adults (≥ 18 years of age) with malignant pleural mesothelioma, malignant pleural effusion, nonmalignant pleural effusion, empyema, primary and secondary spontaneous pneumothorax, iatrogenic pneumothorax, and pleural TB were studied.Results: In 2016, ED treat-and-discharge (T&D) visits totaled 42,215, accounting for charges of $286.7 million. In 2016, a total of 361,270 hospitalizations occurred, resulting in national costs of $10.1 billion. A total of 64,174 readmissions contributed $1.16 billion in additional national costs. Nonmalignant pleural effusion constituted 85.5% of ED T&D visits, 63.5% of hospitalizations, and 66.3% of 30-day readmissions. Contemporary sex distribution (male to female ratio) in primary spontaneous pneumothorax (2.1:1) differs from older estimates (6.2:1). Decadal analyses of annual hospitalization rates/100,000 adult population (2007 vs 2016) showed a significant (P < .001) decrease for malignant pleural mesothelioma (1.3 vs 1.09, respectively), malignant pleural effusion (33.4 vs 31.9, respectively), iatrogenic pneumothorax (17.9 vs 13.9, respectively), and pleural TB (0.20 vs 0.09, respectively) and an increase for empyema (8.1 vs 11.1, respectively) and nonmalignant pleural effusion (78.1 vs 100.1, respectively). Empyema hospitalizations have high costs per case ($38,591) and length of stay (13.8 days). The mean proportion of readmissions attributed to a pleural cause varied widely: malignant pleural mesothelioma, 49%; malignant pleural effusion, 45%; nonmalignant pleural effusion, 31%; empyema, 27%; primary spontaneous pneumothorax, 27%; secondary spontaneous pneumothorax, 27%; and iatrogenic pneumothorax, 20%. Secondary spontaneous pneumothorax had the shortest time to readmission in 2016 (10.3 days, 95% CI, 8.8-11.8 days).Interpretation: Significant epidemiologic trends and changes in various pleural diseases were observed. The analysis identifies multiple opportunities for improvement in management of pleural diseases. [ABSTRACT FROM AUTHOR]

Organizational Culture Matters: Culture is a key driver of organizational performance and underpins strategy. As previously discussed, if the strategy is the plants and the garden plan for a garden, the culture is the soil. Without a healthy culture, nothing will grow, irrespective of how well planned the garden or how beautiful the individual flowers. Using the case of establishing the culture in an institute at the Cleveland Clinic, the article examines an approach to establishing and maintaining an organizational culture. Anchors for this process are a situational assessment of the current culture as a new leader steps in and mindfulness by the leader of how members of the institute should experience the organization. Critical success factors include open communication and establishing psychological safety as well as modeling integrity. Fundamentally, when cultures are grounded in the seven classical virtues-trust, compassion, courage, justice, wisdom, temperance, and hope-they are best positioned to unleash the discretionary effort of its members. When people expend discretionary effort, they do the right thing when nobody is watching and the performance drivers are internal motivation and alignment with mission rather than external drivers to seek reward (carrots) or to avoid punishment (sticks). [ABSTRACT FROM AUTHOR]

Because teams can accomplish goals that individuals cannot, teams matter. Indeed, teams especially matter in settings such as health care, where favorable outcomes depend critically on the contributions of many different people with diverse skills. As important as effective teambuilding is for health care, how to build teams is often not included in medical curricula, and physicians learn to build teams through "hidden curricula." In the context that we can do better, this "How I Do It" presents an approach to building a team in a common scenario for the chest physician: picking up the inpatient Pulmonary Consult Service. The approach is informed by considering the attributes of an effective team, knowledge of common team dysfunctions, and best practices for building a team. The importance of teambuilding is underscored by substantial evidence that effective teamwork produces superior clinical outcomes. [ABSTRACT FROM AUTHOR]

Emotional intelligence (EI) has become widely appreciated as an important leadership attribute, in business, education and, increasingly, in health care. Defined as "the capacity to understand your own and others' emotions and to motivate and develop yourself and others in service of improved work performance and enhanced organizational effectiveness," EI is correlated with a number of success attributes in several sectors; for example, in business, with enhanced business performance and enhanced personal career success, and in health care, with enhanced patient satisfaction, lower burnout, lower litigation risk, and enhanced leadership success. While multiple models of EI have evolved, perhaps the most popular model is framed around four general rubrics with component competencies. The general rubrics are: self-awareness, self-management, social awareness, and relationship management. EI can be measured by using available instruments, and it can be learned and taught. Indeed, teaching EI has become increasingly common in health-care organizations in service of improving health care and health-care leadership. Although more research is needed, ample evidence supports the notion that EI is a critical success element for success as a health-care leader, especially because EI competencies differ markedly from the clinical and scientific skills that are core to being a clinician and/or investigator. This review of EI presents evidence in support of the relevance of EI to health care and health-care leadership, discusses how and when EI can be developed among health-care providers, and considers remaining questions. [ABSTRACT FROM AUTHOR]

Change is a fact of life; the absence of change creates stagnation. This is perhaps especially true in health care, where progress in treating disease depends on innovation and progress. At the same time, change is often uncomfortable. Thus, it is helpful to model the change process to optimize the chances of successfully effecting change. Furthermore, how to lead change is a critical leadership competency. Three models for leading change are reviewed: the first-the eight stages of change-which was not designed for health care; the second called "switch"; and the third called Amicus, which was uniquely designed for health care. The models share many common features, with the explicit reminder in the third model that physicians should be involved in the change effort early. Although sparse, the evidence does suggest the applicability of these models to health care. Beyond having a roadmap for leading change, it is helpful to assess the worthiness of undertaking a change effort and of predicting the phasic response to change efforts, given that humans are often change-averse. In this regard, both the "payoff matrix" and the change curve, derived from the work of Kübler-Ross on grieving, are offered as tools. Finally, physicians' avidity for change is framed by two opposing vectors. On the one hand, physicians share in the general human aversion to change. On the other hand, physicians are data-reverent and also wish to do their best for patients, which encourages their embrace of ever-increasing evidence and change. [ABSTRACT FROM AUTHOR]

In the context that leadership matters and that leadership competencies differ from those needed to practice medicine or conduct research, developing leadership competencies for physicians is important. Indeed, effective leadership is needed ubiquitously in health care, both at the executive level and at the bedside (eg, leading clinical teams and problem-solving on the ward). Various leadership models have been proposed, most converging on common attributes, like envisioning a new and better future state, inspiring others around this shared vision, empowering others to effect the vision, modeling the expected behaviors, and engaging others by appealing to shared values. Attention to creating an organizational culture that is informed by the seven classic virtues (trust, compassion, courage, justice, wisdom, temperance, and hope) can also unleash discretionary effort in the organization to achieve high performance. Health care-specific leadership competencies include: technical expertise, not only in one's clinical/scientific arena to garner colleagues' respect but also regarding operations; strategic thinking; finance; human resources; and information technology. Also, knowledge of the regulatory and legislative environments of health care is critical, as is being a problem-solver and lifelong learner. Perhaps most important to leadership in health care, as in all sectors, is having emotional intelligence. A spectrum of leadership styles has been described, and effective leaders are facile in deploying each style in a situationally appropriate way. Overall, leadership competencies can be developed, and leadership development programs are signature features of leading health-care organizations. [ABSTRACT FROM AUTHOR]

The coronavirus pandemic has disrupted clinical practice, health-care organizations, and life. In the context that "a crisis is a terrible thing to waste," as disruptive as the pandemic has been to traditional practices-both clinically and educationally-opportunities have also presented. Clinical benefits have included the propulsion of clinical innovation, including such items as the development of novel vaccines and accelerated understanding of multiplex ventilation. Approaches to educating students and other learners have also changed radically, with the suspension of live teaching in most instances and a precipitous transition to virtual instruction. This perspective considers a SWOT analysis (strengths, weaknesses, opportunities, and threats) associated with the coronavirus pandemic in health care that focuses on the implications for education. Although the obvious disadvantages (weaknesses) regard the loss of face-to-face interaction with all of its consequences (eg, isolation, risks to camaraderie, loss of hands-on training opportunities, and loss of in-person celebratory events like graduations and end-of-training celebrations), there are clearly offsetting strengths. These include growing experience with virtual teaching and virtual learning strategies, the invitation to codify best virtual teaching practices, a tightening of alignment between undergraduate and graduate medical education (eg, around virtual interview strategies), and opportunities for both self-reflection and a commitment to act virtuously. On balance, the pandemic has created the opportunity, indeed the necessity, to innovate in practice and in education, making the landscape ripe for creative practice, new mastery, and the concomitant benefits to learners and to educators. [ABSTRACT FROM AUTHOR]

Rare diseases pose particular challenges to patients who are affected, to the clinicians who care for them, and to the investigators who study their conditions. Although individually uncommon, rare diseases are common in the aggregate, with approximately 7,000 described rare diseases affecting 25 to 30 million US adults. Challenges posed to affected individuals and their families largely regard being diagnosed, receiving optimal care, and affording disease-specific medications. Challenges facing clinicians who care for affected individuals include gaining knowledge and experience in caring for such patients, and the availability of local experts and of expert guidelines. Finally, challenges to investigators regard the difficulty and expense of assembling large cohorts of affected individuals for study, and garnering funding for research. Fortunately, in the face of these challenges, the steadfast resolve of patient and clinical/scientific communities to enhance care and generate new knowledge has fostered a large inventory of countermeasures to offset these challenges. Although further progress is surely needed, successes to date include the formation of powerful patient advocacy groups which have brokered collaborations between the patient, scientific communities, the government, and pharma/device communities in service of detection, optimal care, and research; procurement of funds to support research; formation of consortia of clinicians and scientists to collaborate; and general activation of the respective patient communities to perpetuate these successes. Persisting needs include enhanced detection strategies, dissemination of knowledge regarding optimal care, and research to prevent, treat, and cure disease. [ABSTRACT FROM AUTHOR]

Background: Because little attention has been given to the long-term outcomes of patients in hospital-based ventilator weaning units, we undertook this assessment of 5-year outcomes of patients discharged from the Respiratory Special Care Unit (ReSCU) of the Cleveland Clinic Hospital. Methods: The ReSCU consists of six private beds on a pulmonary specialty ward. Features of the unit include noninvasive monitoring with signal output at each bedside and at a central monitoring station. Thc unit is staffed by nurses with specific pulmonary rehabilitation expertise and has 24-h respiratory therapist supervision. Ongoing prospective data collection in the ReSCU includes monitoring weaning success, demographic features, hospital discharge status, and hospital discharge disposition. Long-term outcomes were ascertained using a review of hospital medical records and direct inquiry to patients and/or family members. Results: Between August 22, 1993, and August 22, 1996, 162 individuals were admitted to the ReSCU, with 7 persons having repeat admissions during separate hospital admissions. Seventeen percent of these persons (n = 27) died during the hospitalization, while 83% were discharged from the index hospitalization (ie, the hospital stay during which the patient was first admitted to thc ReSCU). Kaplan-Meier (KM) mortality rate estimates were as follows: 1 month, 11% (95% confidence interval [CI], 6 to 15%); 1 year, 57% (95% CI, 49 to 65%); 2 years, 68% (95% CI, 61 to 75%); 3 years, 73% (95% CI, 66 to 80%); 4 years, 76% (95% CI, 69 to 83%); and 5 years, 81% (95% CI, 75 to 87%). The 5-year KM mortality rate estimates considered by year of ReSCU admission were as follows: 1993, 92% (95% CI, 77 to 100%); 1994, 84% (95% CI, 73 to 95%); 1995, 87% (95% CI, 77 to 96%); and through August 22, 1996, 66% (95% CI, 51 to 81%). Conclusions: In this population requiring prolonged inpatient ventilatory support, moderately high acute mortality rates are consistent with data from other series. In this analysis of longer-term follow-up rates, thc 5-year survival rates are low, with higher mortality rates within the first 2 years and a slower decline in survival thereafter. [ABSTRACT FROM AUTHOR]

The article focuses on the long-term use of supplemental long-term oxygen therapy (LTOT) in chronic obstructive pulmonary disease (COPD) patients. It is noted that supplemental oxygen improves exercise performance in short-term investigations of patients with COPD. A rationale for the National Heart, Lung and Blood Institute Long-term Oxygen Treatment Trial, which aims to enroll subjects with COPD, is provided.

The article presents a clinical study of coronary artery bypass graft and valvular operations after pre-pneumonectomy. In relation to pneumonectomy and cardiac surgery, various cases have been associated with complications and postoperative deaths in several high-risk patient groups. The authors note that this condition reflects that cardiac surgery can be conducted with a logical probability of a positive outcome in patients who belong in the high-risk bracket.

The article presents the case study of an African-American woman who was admitted to a pulmonary medicine clinic for evaluation of progressive dyspnea and pulmonary hypertension. It says that erect and supine pulmonary function testing diagnosed the presence of bilateral diaphragmatic dysfunction or shrinking lung syndrome in patient. The article also discusses how glucocorticoids significantly improve symptoms and physiologic impairment in the shrinking lung syndrome.

Case Presentation: A 21-year-old male college student presented for a second opinion with low alpha-1 antitrypsin (AAT) levels and complaints of episodic dyspnea with wheezing and cough. He was a never smoker with a medical history of frequent respiratory tract infections in early childhood and allergy to dander, dust mites, peanuts, and eggs. There was no travel history outside of the continental United States. His mother had asthma. His symptoms were not controlled on inhaled corticosteroids and bronchodilators. His AAT genotype was found to be PI∗SZ, and augmentation therapy (with pooled human-plasma derived AAT) was recommended locally. [ABSTRACT FROM AUTHOR]

This article presents information on a study related to diagnosis of α1-Antitrypsin deficiency (AAT). Despite its affecting an estimated 100,000 Americans, AAT deficiency commonly goes unrecognized by clinicians. As a result, AAT-deficient individuals experience long delays between their first symptom and initial diagnosis, often with adverse medical and psychosocial sequelae. Researchers reasoned that growing awareness of AAT deficiency by clinicians might enhance clinical recognition and accelerate detection in affected individuals. Thus, they undertook the current analysis of mail survey results to assess whether individuals with AAT deficiency who received a more recent diagnosis had shorter delays in diagnosis than those with a diagnosis made earlier.

The article presents a case report of a 63-year-old woman with subcutaneous emphysema following endotracheal intubation. The physical condition of the patient was described and offers information on the medical history of the patient. The physical examination of the patient showed a mild tachycardia with regular rhythm and the abdomen was soft and nontender. The laboratory and radiography findings were provided.

Study objective: To clarify the mortality rate and causes of death of individuals with α1-antitrypsin (AAT) deficiency, the Death Review Committee (DRC) of the National Heart, Lung, and Blood Institute Registry of Individuals with Severe AAT Deficiency reviewed all available medical records regarding the deaths of study subjects during Registry follow-up (up to 7.2 years). Methods: Individual determinations by each member of the three-person DRC led to consensus judgments regarding the underlying cause and the immediate and contributing causes of death. Results: Of the 1,129 Registry subjects, 204 died (18.1%) [approximately 3%/yr]. Record availability permitted detailed review in 120 decedents, and death certificates were available in 56 of the remaining 84 subjects (67%). Emphysema and cirrhosis were the most common underlying causes of death (72% and 10%, respectively), with malignancy and diverticulitis accounting for 3% of deaths each. To assess attributable mortality, standardized mortality ratio analysis was performed and indicated that excess mortality was ascribable entirely to lung and liver disease. Conclusions: We conclude that severe AAT deficiency poses a significant threat to health, that severe airflow obstruction is a major determinant of mortality, and that liver and lung disease account for the excess mortality in affected individuals. These findings support current efforts to enhance diagnostic recognition and treatment of AAT-deficient individuals. [ABSTRACT FROM AUTHOR]

Study objectives: To describe patterns of prescribing augmentation therapy, and types and rates of adverse events in the National Heart, Lung, and Blood Institute Registry for Individuals with Severe Deficiency of Alpha[sub 1]-Antitrypsin. Design: Observational cohort study with follow-up visits every 6 to 12 months for up to 7 years. Measurements: The rate and dosing frequency with which Registry participants were prescribed to receive augmentation therapy by their managing physicians, and the type and frequency of adverse events, classified in two ways: severity of self-reported symptoms, and actions taken as a consequence of the symptom. Results: Over the course of Registry follow-up, 66% (n = 747) of the participants received augmentation therapy at some time. In keeping with recommendations made in the 1989 American Thoracic Society (ATS) statement, 75% of participants with airflow obstruction at first visit (defined as FEV[sub 1] < 80% predicted) received augmentation therapy within 3 years, though some participants with FEV[sub 1] ≥ 80% predicted (14%) also received augmentation therapy. Among those with COPD for whom augmentation therapy was not prescribed, financial constraints were the reported cause in 30%. Observed patterns also varied from approved practice, in that dosing frequencies other than the US Food and Drug Administration-approved, once-weekly regimen were frequently prescribed. The overall rate of reported adverse events was 0.02 per patient month, with 83% of participants reporting no events. This overall rate was composed of 16% considered mild events, 76% moderate events, and 9% severe events. Conclusions: We conclude that augmentation therapy was generally well tolerated and, consistent with ATS guidelines, physicians generally did not prescribe augmentation therapy for subjects with FEV[sub 1] ≥ 80% predicted. However, the large percentage of subjects with FEV[sub 1] <80% predicted not receiving augmentation therapy and the... [ABSTRACT FROM AUTHOR]

Background: Augmentation therapy with pooled human plasma-derived alpha(1)-antitrypsin (AAT) has been shown to have biochemical efficacy in restoring serum AAT levels above the protective threshold. Also, clinical efficacy has been suggested.Objective: To evaluate the bioequivalence of a new solvent detergent-treated preparation of pooled human plasma-derived AAT (proposed name Respitin; Alpha Therapeutic Corporation; Los Angeles, CA) to the commercially available preparation (Prolastin; Bayer Corporation; West Haven, CT), we conducted a randomized controlled trial.Methods: Eligible subjects were adults (> 18 years of age) who had never smoked or were ex-smokers, had severe deficiency of AAT, and had fixed airflow obstruction (ie, postbroncholdilator FEV(1) of 30 to 80% of predicted values and/or diffusing capacity of the lung for carbon monoxide [DLCO] of < 70% of predicted values with evidence of emphysema on a CT scan). Of the 28 subjects recruited, 26 completed the 12-week comparison. Participants were randomized to receive Respitin (60 mg/kg once weekly; 14 subjects) or Prolastin (60 mg/kg once weekly; 14 subjects), and recipients of Prolastin then crossed over to receive Respitin thereafter for the 24-week duration of the study.Results: The primary efficacy criteria were satisfied for equivalence to comparator (ie, the ratio of mean trough serum levels for Respitin/Prolastin at weeks 8 to 11 exceeded the efficacy criterion [0.905; p = 0.0206] as did the slope of the mean trough level over weeks 11 to 23 [-0.003 micromol per week]). In Respitin recipients, the trough serum antineutrophil elastase capacity at week 7 and at weeks 8 to 11 was also equivalent to the comparator, as was the rise in AAT levels in epithelial lining fluid from baseline to week 7. The levels of urinary elastin degradation products showed little appreciable change for > 24 weeks, and no difference between compared groups was shown through week 12. Adverse events were similarly infrequent in compared groups. Finally, neither spirometry measurements nor DLCO showed a significant change through 24 weeks.Conclusions: We conclude that this new solvent detergent-treated pooled human plasma-derived AAT (Respitin) demonstrates biochemical equivalence to Prolastin and that this new drug is well-tolerated. [ABSTRACT FROM AUTHOR]

Background: Significant challenges exist to investigating uncommon illnesses because too few patients are seen at any single clinical center to permit appropriate research studies. Recognizing this impediment to clinical research in alpha(1)-antitrypsin deficiency, the Alpha One Foundation, a patient-organized research foundation, has collaborated with clinician-scientists to organize a voluntary registry of individuals with alpha(1)-antitrypsin deficiency.Purpose: To facilitate clinical research in alpha(1)-antitrypsin deficiency by organizing a registry of affected individuals willing to be approached to participate in clinical studies.Methods: Elements of the Alpha One Foundation Research Network Registry include a Medical and Scientific Advisory Committee, composed of physician-investigators and patient advocates, designated clinical resource centers at medical institutions with expertise in the management of individuals with alpha(1)-antitrypsin deficiency, and a data coordinating center with responsibility for database management and analysis. Questionnaires requesting information about demographic features, alpha(1)-antitrypsin phenotype, smoking history, and health-care utilization were distributed to prospective registrants through the following channels: mailings from the Alpha One Foundation; mailings from the clinical resource centers; and distribution by home-care and pharmaceutical companies. Information from this questionnaire formed the basis of the initial registry database.Results: Between May 1997 and June 1999, 7,789 forms were distributed, and forms were returned by 712 unique registrants. Registrants have the following characteristics: mean (+/- SD) age, 49.3+/-13.2 years; women, 47.7%; white, 96.2%; PI*ZZ phenotype, 70.7%; ex-smokers, 73.3%; COPD patients, 87.2% (emphysema patients, 54.2%; chronic bronchitis patients, 33%); and self-reported liver disease, 6.4%. The mean number of physician visits reported by registrants in the preceding 12 months was 7.8+/-9.4, 59% reported currently receiving IV augmentation therapy, and 35% reported using supplemental oxygen at home. Examples of ongoing research studies using this unique database include: (1) a case-control study to evaluate occupational risk factors for obstructive lung disease in individuals with alpha(1)-antitrypsin deficiency and (2) a study to evaluate the health-care costs for affected individuals.Conclusions: A registry currently including 712 individuals with alpha(1)-antitrypsin deficiency has been organized through a collaboration between physician-investigators and a patient-organized research foundation. Use of the registry has already facilitated studies that were previously difficult because of the paucity of identifiable study subjects. The registry cohort promises to provide an important resource for future clinical and epidemiologic studies. [ABSTRACT FROM AUTHOR]

Having a strategic plan is important to reach organizational goals. Equally important is knowing how to develop and execute that plan. Also, such plans evolve and are executed in the context of the organization's culture, which is another critical success element. Using a garden metaphor, the arrangement of the plants in the garden is like the strategy. With a good strategy, the arrangement of the plants will be appealing. But the soil in the garden is the organizational culture. If the soil is fouled, no plants will grow, regardless of how appealing the garden plan. This "How We Do It" paper addresses the issue of developing and executing a strategy and then, in a companion piece, the related process of envisioning and cultivating an organizational culture. The strategic planning discussion invokes a "real-win-worth" paradigm to address the real-world case of assuring uniform, best-in-class ICU outcomes across multiple ICUs in a large academic medical center system. [ABSTRACT FROM AUTHOR]

Background: Deficiencies in risk assessment for patients with pulmonary nodules (PNs) contribute to unnecessary invasive testing and delays in diagnosis.Research Question: What is the accuracy of a novel PN risk model that includes plasma proteins and clinical factors? How does the accuracy compare with that of an established risk model?Study Design and Methods: Based on technology using magnetic nanosensors, assays were developed with seven plasma proteins. In a training cohort (n = 429), machine learning approaches were used to identify an optimal algorithm that subsequently was evaluated in a validation cohort (n = 489), and its performance was compared with the Mayo Clinic model.Results: In the training set, we identified an support vector machine algorithm that included the seven plasma proteins and six clinical factors that demonstrated an area under the receiver operating characteristic curve of 0.87 and met other selection criteria. The resulting risk reclassification model (RRM) was used to recategorize patients with a pretest risk of between 10% and 84%, and its performance was assessed across five risk strata (low, ≤ 10%; moderate, 10%-34%; intermediate, 35%-70%; high, 71%-84%; very high, > 85%). Stratification by the RRM decreased the proportion of intermediate-risk patients from 26.7% to 10.8% (P < .001) and increased the low-risk and high-risk strata from 16.8% to 21.9% (P < .001) and from 3.7% to 12.1% (P < .001), respectively. Among patients classified as low risk by the RRM and Mayo Clinic model, the corresponding true-negative to false-negative ratios were 16.8 and 19.5, respectively. Among patients classified as very high risk by the RRM and Mayo Clinic model, the corresponding true-positive to false-positive ratios were 28.5 and 17.0, respectively. Compared with the Mayo Clinic model, the RRM provided higher specificity at the low-risk threshold and higher sensitivity at the very high-risk threshold.Interpretation: The RRM accurately reclassified some patients into low-risk and very high-risk categories, suggesting the potential to improve PN risk assessment. [ABSTRACT FROM AUTHOR]

Background: Alpha-1 antitrypsin deficiency (AATD) is an autosomal co-dominant condition that predisposes to emphysema, cirrhosis, panniculitis, and vasculitis. Underrecognition has prompted efforts to enhance early detection and testing of at-risk individuals. Direct-to-consumer (DTC) genetic testing represents an additional method of detection.Research Question: The study addressed three questions: (1) Does a DTC testing service identify previously undetected individuals with AATD? (2) What was the interval between initial AATD-related symptoms and initial diagnosis of AATD in such individuals? and (3) What was the behavioral impact of learning about a new diagnosis of AATD through a DTC test?Study Design and Methods: In this cross-sectional study, 195,014 individuals responded to a survey within the 23andMe, Inc. research platform.Results: Among 195,014 study participants, the allele frequency for the PI∗S and PI∗Z AATD variants was 21.6% (6.5% for PI∗Z and 15.1% for PI∗S); 0.63% were PI∗ZZ, half of whom reported having a physician confirm the diagnosis. Approximately 27% of those with physician-diagnosed AATD reported first becoming aware of AATD through the DTC test. Among those newly aware participants, the diagnostic delay interval was 22.3 years. Participants frequently shared their DTC test results with health care providers (HCPs) and the reported impact of learning a diagnosis of AATD was high. For example, 51.1% of PI∗ZZ individuals shared their DTC result with an HCP. The OR for PI∗ZZ smokers to report smoking reduction as a result of receiving the DTC result was 1.7 (95% CI = 1.4-2.2) compared with those without a Z allele and for reduced alcohol consumption this was 4.0 (95% CI = 2.6-5.9).Interpretation: In this largest available report on DTC testing for AATD, this test, in combination with clinical follow-up, can help to identify previously undiagnosed AATD patients. Moreover, receipt of the DTC AATD report was associated with positive behavior change, especially among those with risk variants. [ABSTRACT FROM AUTHOR]

Background: Hypogammaglobulinemia (serum IgG levels < 7.0 g/L) has been associated with increased risk of COPD exacerbations but has not yet been shown to predict hospitalizations.Research Question: To determine the relationship between hypogammaglobulinemia and the risk of hospitalization in patients with COPD.Study Design and Methods: Serum IgG levels were measured on baseline samples from four COPD cohorts (n = 2,259): Azithromycin for Prevention of AECOPD (MACRO, n = 976); Simvastatin in the Prevention of AECOPD (STATCOPE, n = 653), Long-Term Oxygen Treatment Trial (LOTT, n = 354), and COPD Activity: Serotonin Transporter, Cytokines and Depression (CASCADE, n = 276). IgG levels were determined by immunonephelometry (MACRO; STATCOPE) or mass spectrometry (LOTT; CASCADE). The effect of hypogammaglobulinemia on COPD hospitalization risk was evaluated using cumulative incidence functions for this outcome and deaths (competing risk). Fine-Gray models were performed to obtain adjusted subdistribution hazard ratios (SHR) related to IgG levels for each study and then combined using a meta-analysis. Rates of COPD hospitalizations per person-year were compared according to IgG status.Results: The overall frequency of hypogammaglobulinemia was 28.4%. Higher incidence estimates of COPD hospitalizations were observed among participants with low IgG levels compared with those with normal levels (Gray's test, P < .001); pooled SHR (meta-analysis) was 1.29 (95% CI, 1.06-1.56, P = .01). Among patients with prior COPD admissions (n = 757), the pooled SHR increased to 1.58 (95% CI, 1.20-2.07, P < .01). The risk of COPD admissions, however, was similar between IgG groups in patients with no prior hospitalizations: pooled SHR = 1.15 (95% CI, 0.86-1.52, P =.34). The hypogammaglobulinemia group also showed significantly higher rates of COPD hospitalizations per person-year: 0.48 ± 2.01 vs 0.29 ± 0.83, P < .001.Interpretation: Hypogammaglobulinemia is associated with a higher risk of COPD hospital admissions. [ABSTRACT FROM AUTHOR]

Background: Although inhaled therapy reduces exacerbations among patients with COPD, the effectiveness of providing inhaled treatment per risk stratification models remains unclear.Research Question: Are inhaled regimens that align with the 2017 Global Initiative for Chronic Obstructive Lung Disease (GOLD) strategy associated with clinically important outcomes?Study Design and Methods: We conducted secondary analyses of Long-term Oxygen Treatment Trial (LOTT) data. The trial enrolled patients with COPD with moderate resting or exertional hypoxemia between 2009 and 2015. Our exposure was the patient-reported inhaled regimen at enrollment, categorized as either aligning with, undertreating, or potentially overtreating per the 2017 GOLD strategy. Our primary composite outcome was time to death or first hospitalization for COPD. Additional outcomes included individual components of the composite outcome and time to first exacerbation. We generated multivariable Cox proportional hazard models across strata of GOLD-predicted exacerbation risk (high vs low) to estimate between-group hazard ratios for time to event outcomes. We adjusted models a priori for potential confounders, clustered by site.Results: The trial enrolled 738 patients (73.4% men; mean age, 68.8 years). Of the patients, 571 (77.4%) were low risk for future exacerbations. Of the patients, 233 (31.6%) reported regimens aligning with GOLD recommendations; most regimens (54.1%) potentially overtreated. During a 2.3-year median follow-up, 332 patients (44.9%) experienced the composite outcome. We found no difference in time to composite outcome or death among patients reporting regimens aligning with recommendations compared with undertreated patients. Among patients at low risk, potential overtreatment was associated with higher exacerbation risk (hazard ratio, 1.42; 95% CI, 1.09-1.87), whereas inhaled corticosteroid treatment was associated with 64% higher risk of pneumonia (incidence rate ratio, 1.64; 95% CI, 1.01-2.66).Interpretation: Among patients with COPD with moderate hypoxemia, we found no difference in clinical outcomes between inhaled regimens aligning with the 2017 GOLD strategy compared with those that were undertreated. These findings suggest the need to reevaluate the effectiveness of risk stratification model-based inhaled treatment strategies. [ABSTRACT FROM AUTHOR]

The article presents the case of a 77-year-old man presented for evaluation of hemoptysis after a 7-month history of cough, progressive dyspnea, and intermittent hemoptysis, which consisted of tablespoon amounts of bright red blood and occurred up to eight times per day. Episodes of intermittent blood-streaked sputum had occurred for 18 months. A chest radiograph showed persistent left lower lobe infiltrates, and the results of blood tests consisted of a normal CBC count, normal metabolic panel, normal urinary sediment level, and normal coagulation studies. Tests for the presence of antineutrophil cytoplasmic antiglomerular basement membrane, antiphospholipid, and antinuclear antibodies were all negative.

Examines the sources of direct medical costs of alpha-antitrypsin deficiency based on survey data from individuals with human chromosome abnormalities. Range of the annual health care costs; Value of the use of intravenous augmentation therapy; List of other major cost sources of treatment.

The article presents reasons why guidelines from the American College of Chest Physicians (CHEST) cannot be applied internationally. Topics discussed include key factors that composes an optimal guideline and challenges presented by diversity in health care and patient population in developing a global guideline including the disconnect between the global guideline and medical care access in resource-poor settings.

The article focuses on a study which assessed the features of pulmonary function testing (PFT) laboratory, including equipment used, types of tests offered, volume of testing, reference equations used and interpretive strategies. The study showed variation in PFT laboratory practices, such as the choice of reference equations, criteria used to define abnormality, and strategies for interpreting tests. The implications of such variation for the consistency of result interpretation are discussed.

Background: Because α(1)-antitrypsin deficiency (AATD) is underrecognized, strategies to enhance guideline-based diagnostic testing are warranted.Methods: We studied the impact of issuing a guideline-based alert within an electronic health record (EHR) to test for AATD on the rate of testing for and detection of AATD. For patients at the Cleveland Clinic whose physician-ordered pulmonary function test results showed airflow obstruction, the rates of testing for and detection of AATD were determined during a baseline (prealert) period and again after implementing the alert.Results: During the baseline period, among 979 eligible subjects, 4.7% were tested; 8.9% of those who had phenotype testing performed were found to have AATD (serum levels < 100 mg/dL), of whom 3.2% had the PI*ZZ genotype. After the alert, among the 624 eligible subjects, the rate of testing increased severalfold (15.1%, P < .001), though neither the rate of detecting AATD (5.3%) nor PI*ZZ (2.6%) differed from the rates during the baseline period. Having an alternate diagnosis (other than asthma) that could explain airflow obstruction (eg, congestive heart failure, sarcoidosis, Langerhans cell histiocytosis) was associated with a lower rate of testing (P = .041), while carrying a diagnosis of asthma tended to increase the testing frequency (P = .15). Multivariate analysis showed that younger age and a smoking history were associated with being tested.Conclusions: Issuing an alert within an EHR was associated with a severalfold increase in the frequency of testing for α(1)-antitrypsin without a higher rate of diagnosing severe AATD. While the lack of more frequent diagnosis of AATD may reflect a high rate of baseline detection, these results prompt consideration of additional strategies to enhance detection of AATD. [ABSTRACT FROM AUTHOR]

The article explores a study on the impact of a clinical decision support system (CDS) in an electronic health record (EHR) on the detection of α1-antitrypsin deficiency (AATD). It notes that AATD patients tend to experience delays from symptom onset to the initial diagnosis. Under the study, electronic alerts urge physicians to test patients for AATD when their EHR shows airflow obstruction. Findings show that such approach does not lead to enhanced AATD detection.

Long-term use of supplemental oxygen improves survival in patients with COPD and severe resting hypoxemia. However, the role of oxygen in symptomatic patients with COPD and more moderate hypoxemia at rest and desaturation with activity is unclear. The few long-term reports of supplemental oxygen in this group have been of small size and insufficient to demonstrate a survival benefit. Short-term trials have suggested beneficial effects other than survival in patients with COPD and moderate hypoxemia at rest. In addition, supplemental oxygen appeared to improve exercise performance in small short-term investigations of patients with COPD and moderate hypoxemia at rest and desaturation with exercise, but long-term trials evaluating patient-reported outcomes are lacking. This article reviews the evidence for long-term use of supplemental oxygen therapy and provides a rationale for the National Heart, Lung, and Blood Institute Long-term Oxygen Treatment Trial. The trial plans to enroll subjects with COPD with moderate hypoxemia at rest or desaturation with exercise and compare tailored oxygen therapy to no oxygen therapy. [ABSTRACT FROM AUTHOR]

Anatomic right-to-left shunt causes hypoxemia that can pose a diagnostic challenge to clinicians. Among the many possible causes of right-to-left shunt, persistent left-sided superior vena cava (PLSVC) with an "unroofed" coronary sinus represents an uncommon congenital anomaly in which detection by saline-contrast echocardiogram (bubble echo) or contrast-enhanced CT scan requires injection of contrast in the left arm. We present the case of an elderly man with hypoxemia on the basis of a right-to-left shunt accompanying a PLSVC with unroofed coronary sinus in whom the shunt escaped initial detection following a bubble echo with contrast injected into the right arm. This case reminds pulmonary clinicians, who are frequently called on to assess the cause of hypoxemia, that specifying a contrast injection into the left arm is required in the pursuit of this specific shunt-producing anomaly. [ABSTRACT FROM AUTHOR]

The article presents a case study of an 83 year old man with hypoxemia. It states that the patient underwent COPD diagnosis and has used supplemental oxygen. Chest radiograph shows cardiomegaly, pulmonary vascular redistribution and arterial blood gas on 100% oxygen on the patient. It mentions that the patient died before left-sided injection bubble echocardiogram (EB). It suggests the importance of left arm as the default contrast injection site on pursuing specific shunt-producing anomaly.

The use of IV augmentation therapy with plasma-derived alpha1-antitrypsin (AAT) has become the standard of care for the treatment of pulmonary disease associated with the severe genetic deficiency of AAT. The Medical and Scientific Advisory Committee of the Alpha-1 Foundation has become aware that physicians are prescribing this expensive blood product for the treatment of individuals with a single abnormal AAT gene, primarily the PI*MZ genotype. We are aware of no evidence that such therapy is effective in this patient population. The most important therapeutic interventions in such patients remain smoking cessation and elimination of other risk factors for lung disease. This commentary discusses the treatment of AAT deficiency and the concerns regarding treatment of PI*MZ individuals. We conclude that clinicians should avoid prescribing augmentation therapy for this heterozygote population. [ABSTRACT FROM AUTHOR]

The article focuses on the use of plasma-derived alpha 1-antitrypsin to treat pulmonary diseases associated with severe genetic deficiency of α[sub1]-antitrypsin (AAT). Severe deficiency of AAT is a common condition that can be inclined to obstructive lung diseases. It says that augmentation therapy is the only available therapy for lung-affected patients with severe deficiency of AAT. However, it reveals that due to lack of evidence the use of this therapy is being discouraged.

The article presents a research related to the quality of chest compressions and timing of defibrillation during cardiopulmonary resuscitation. It addresses the impact of chest compressions on initial defibrillation after sudden death. According to the findings of the research that used animal model of cardiac arrest, the quality of the chest compressions was the predominant determinant of successful resuscitation rather than the priority of initial defibrillation.

The article cites a case study of a 24-year-old woman patient with pulmonary hypertension and hereditary hemorrhagic telangiectasia. The patient was followed for more than 10 years on several vasoactive medications such as nifedipine and epoprostenol. The case study reveals that the aggressive therapy in such case can lead to long-term improvement in clinical parameters and survival.

The article proposes a regression equation for estimating FVC from measured values of the forced expiratory volume in 2 s and forced expiratory volume in 3 s. In the context that spirometry is essential in assessing respiratory disorders, FVC is an important measurement to diagnose the presence of obstructive lung disease and to evaluate restrictive disease. The main findings of this study are that FEV3 can be used to estimate FVC with reasonable accuracy. Although this surrogate parameter confers benefits for interpreting spirometry results when spirometric end-of-test criteria are not satisfied, estimates of the true FVC are imperfect and use of the FEV1 estimated FVC3 does not eliminate the risk of misclassifying the presence of obstruction.

Studies the diagnostic role of flexible bronchoscopy (FB) in immunocompromised patients with pulmonary infiltrates. Prospective, observational study; Tertiary care hospital; Participation of 104 consecutive non-HIV-infected immunocompromised patients with lung infiltrates; Indication that FB has a high diagnostic yield in immunocompromised patients with pulmonary infiltrates; Recommendation for the performance of transbronchial biopsy in these patients, whenever possible.

Study Objectives: To examine the sources of the direct medical costs of alpha(1)-antitrypsin (AAT) deficiency based on survey data from affected individuals.Background: Prior research has reported the total cost of AAT deficiency but has not examined the specific components of the direct medical costs of affected individuals.Methods: In order to detail the sources of the direct medical costs, we sent surveys to 688 respondents of a previous survey. We estimated the costs in three ways, which differed in the method of managing missing values. With method 1, the group mean value of cost per unit of utilization, multiplied by the occurrences of utilization, was used to replace the missing value. Two sensitivity analyses (ie, methods 2 and 3) were conducted to test the robustness of our estimate. With method 2, values of zero were entered for all missing values. With method 3, the missing values were replaced by the group mean value. The Wilcoxon test was used to test the cost differences between patients of different phenotypes. All cost data were expressed in 1998 US dollars.Results and Conclusions: Two hundred ninety-two individuals responded to the survey. The annual total health-care costs were high (mean range, $36,471 to $46,114 US dollars; median range, $12,485 to $37,100 US dollars [according to the method for managing missing data]) for AAT deficiency. The total costs for individuals with the PI*ZZ phenotype exceeded those for individuals with a non-PI*ZZ phenotype. The use of IV augmentation therapy accounted for more than half of all direct medical costs for the respondents. Besides the costs for therapy with alpha(1)-proteinase inhibitor (Prolastin; Bayer; West Haven, CT), other major cost sources were prescription drugs other than alpha(1)-proteinase inhibitor, hospitalization, health insurance, and physician visits. [ABSTRACT FROM AUTHOR]

Study Objectives: To describe patterns of prescribing augmentation therapy, and types and rates of adverse events in the National Heart, Lung, and Blood Institute Registry for Individuals with Severe Deficiency of Alpha(1)-Antitrypsin.Design: Observational cohort study with follow-up visits every 6 to 12 months for up to 7 years.Measurements: The rate and dosing frequency with which Registry participants were prescribed to receive augmentation therapy by their managing physicians, and the type and frequency of adverse events, classified in two ways: severity of self-reported symptoms, and actions taken as a consequence of the symptom.Results: Over the course of Registry follow-up, 66% (n = 747) of the participants received augmentation therapy at some time. In keeping with recommendations made in the 1989 American Thoracic Society (ATS) statement, 75% of participants with airflow obstruction at first visit (defined as FEV(1) < 80% predicted) received augmentation therapy within 3 years, though some participants with FEV(1) > or = 80% predicted (14%) also received augmentation therapy. Among those with COPD for whom augmentation therapy was not prescribed, financial constraints were the reported cause in 30%. Observed patterns also varied from approved practice, in that dosing frequencies other than the US Food and Drug Administration-approved, once-weekly regimen were frequently prescribed. The overall rate of reported adverse events was 0.02 per patient-month, with 83% of participants reporting no events. This overall rate was composed of 16% considered mild events, 76% moderate events, and 9% severe events.Conclusions: We conclude that augmentation therapy was generally well tolerated and, consistent with ATS guidelines, physicians generally did not prescribe augmentation therapy for subjects with FEV(1) > or = 80% predicted. However, the large percentage of subjects with FEV(1) <80% predicted not receiving augmentation therapy and the frequent use of 2- to 3-week or monthly dosing reflects variation of practice from suggested treatment guidelines. [ABSTRACT FROM AUTHOR]

Study Objectives: To describe asthma features in a cohort with alpha(1)-antitrypsin (AAT) deficiency, and determine the impact of asthma on FEV(1) decline.Background: Asthma may be common in those with AAT deficiency, and may lead to accelerated airflow obstruction.Design: Analysis of data obtained from a 5-year, prospective National Heart, Lung, and Blood Institute registry.Setting: A multicenter registry consisting of 37 clinical centers, a central phenotyping laboratory, and a data analysis center.Participants: A cohort of 1,052 subjects with AAT deficiency.Measurements and Results: Asthma was defined as reversible airflow obstruction, recurrent attacks of wheezing, and a reported diagnosis of asthma or allergy with or without an elevated serum IgE level. FEV(1) decline was calculated by least-square means with adjustments for covariables. Asthma was present in 21% of the cohort and in 12.5% of those with a normal FEV(1). Attacks of wheezing were reported in 66%, the first attack occurring at a mean +/- SD age of 31 +/- 16 years. Allergy and asthma was reported in 29% and 38%, respectively. An elevated IgE level occurred in 17% and was significantly associated with signs and symptoms of asthma and an allergy history. Unadjusted FEV(1) decline was less in the group without asthma and a normal IgE level (- 48.5 mL/yr) vs the groups with asthma features (> or = 64 mL/yr) [p = 0.002]. Multivariable analysis showed that bronchodilator response, age, and smoking were significant predictors for FEV(1) decline but not asthma.Conclusions: Symptoms and signs of asthma are common in AAT deficiency and may start at the age of most rapid FEV(1) loss. Adjusting for other risk factors such as bronchodilator response, asthma as defined does not lead to an accelerated FEV(1) decline. In AAT deficiency, augmentation therapy is not more effective in preventing the loss of lung function in those with asthma compared to those without. [ABSTRACT FROM AUTHOR]

Study objectives: To describe asthma features in a cohort with α[sub 1]-antitrypsin (AAT) deficiency, and determine the impact of asthma on FEV[sub 1] decline. Background: Asthma may be common in those with AAT deficiency, and may lead to accelerated airflow obstruction. Design: Analysis of data obtained from a 5-year, prospective National Heart, Lung, and Blood Institute registry. Setting: A multicenter registry consisting of 37 clinical centers, a central phenotyping laboratory, and a data analysis center. Participants: A cohort of 1,052 subjects with AAT deficiency. Measurements and results: Asthma was defined as reversible airflow obstruction, recurrent attacks of wheezing, and a reported diagnosis of asthma or allergy with or without an elevated serum IgE level. FEV[sub 1] decline was calculated by least-square means with adjustments for covariables. Asthma was present in 21% of the cohort and in 12.5% of those with a normal FEV[sub 1]. Attacks of wheezing were reported in 66%, the first attack occurring at a mean ± SD age of 31 ± 16 years. Allergy and asthma was reported in 29% and 38%, respectively. An elevated IgE level occurred in 17% and was significantly associated with signs and symptoms of asthma and an allergy history. Unadjusted FEV[sub 1] decline was less in the group without asthma and a normal IgE level (- 48.5 mL/yr) vs the groups with asthma features (≥ 64 mL/yr) [p = 0.002]. Multivariable analysis showed that bronchodilator response, age, and smoking were significant predictors for FEV[sub 1] decline but not asthma. Conclusions: Symptoms and signs of asthma are common in AAT deficiency and may start at the age of most rapid FEV[sub 1] loss. Adjusting for other risk factors such as bronchodilator response, asthma as defined does not lead to an accelerated FEV[sub 1] decline. In AAT deficiency, augmentation therapy is not more effective in preventing the loss of lung function in those with asthma compared to those without. [ABSTRACT FROM AUTHOR]

Health-care professionals (HCPs) can provide protocol-based care that has a measurable impact on critically ill patients beyond their liberation from mechanical ventilation (MV). Randomized controlled trials have demonstrated that protocols for liberating patients from MV driven by nonphysician HCPs can reduce the duration of MV. The structure and features of protocols should be adapted from published protocols to incorporate patient-specific needs, clinician preferences, and institutional resources. As a general approach, shortly after patients demonstrate that their condition has been stabilized on the ventilator, a spontaneous breathing trial (SBT) is safe to perform and is indicated. Ventilator management strategies for patients who fail a trial of spontaneous breathing include the following: (1) consideration of all remediable factors (such as electrolyte derangements, bronchospasm, malnutrition, patient positioning, and excess secretions) to enhance the prospects of successful liberation from MV; (2) use of a comfortable, safe, and well-monitored mode of MV (such as pressure support ventilation); and (3) repeating a trial of spontaneous breathing on the following day. For patients who pass the SBT, the decision to extubate must be guided by clinical judgment and objective data to minimize the risk of unnecessary reintubations and self-extubations. Protocols should not represent rigid rules but, rather, guides to patient care. Moreover, the protocols may evolve over time as clinical and institutional experience with them increases. Useful protocols aim to safely and efficiently liberate patients from MV, reducing unnecessary or harmful variations in approach. [ABSTRACT FROM AUTHOR]

Background: For individuals with emphysema because of severe α[sub 1]-antitrypsin deficiency, specific therapy called IV augmentation therapy has been available since 1989. Such therapy consists of IV infusion of pooled human plasma α[sub 1]-antiprotease. Methods: To assess the direct medical costs of having α[sub 1]-antitrypsin deficiency, the current study surveyed members of the Alpha One Foundation Registry for Individuals With α[sub 1]-Antitrypsin Deficiency regarding their annual expenditures for treatment of this disease. Data regarding demographic features, α[sub 1]-antitrypsin status, and health-resource utilization were collected from a self-administered questionnaire. Respondents were asked to provide total health-care expenditures, but costs by specific items of care (eg, drugs, physician visits, etc) were not available. Results: Mean annual cost estimates were higher for PI[sup *]ZZ-phenotype individuals ($30,948, n = 292) than for non-PI[sup *]ZZ-phenotype individuals ($20,673, n = 53; p = 0.049). Among PI[sup *]ZZphenotype individuals, self-reported costs of health-care services were further analyzed for those 288 individuals whose α[sub 1]-antiprotease use status was reported. For the 185 current α[sub 1]antiprotease users, the mean annual cost was $40,123 (median, $36,000). Conclusions: Annual health-care expenditures by individuals with α[sub 1]-antitrypsin deficiency are very high, whether or not they are currently receiving augmentation therapy. Augmentation therapy adds substantial costs, especially for heavier individuals who are receiving weekly infusions. [ABSTRACT FROM AUTHOR]

A right-to-left shunt (RLS) is an uncommon complication of a patent foramen ovale (PFO) that may cause hypoxemia from venous admixture and ischemic complications from paradoxic embolization. This report presents the third described patient whose RLS through a PFO and profound hypoxemia developed in association with right hemidiaphragm dysfunction (but without a pressure gradient driving the right-to-left flow). In addition to extending the available experience with this unusual clinical event, we report on the successful closure of the PFO by a catheter-deployed double-umbrella device, after the positioning of which the patient's oxygenation normalized. [ABSTRACT FROM AUTHOR]