bluuebunny/crossref_metadata_2025_split huggingface dataset:
Incidence, Risk Factors, and Outcome of Life-Threatening Ventricular Arrhythmias in Giant Cell Myocarditis
Authors: Ekström Kaj , Lehtonen Jukka , Kandolin Riina , Räisänen-Sokolowski Anne , Salmenkivi Kaisa , Kupari Markku 
Published: December 2016

Background—
Ventricular tachyarrhythmias are characteristic of giant cell myocarditis, but their true incidence, predictors, and outcome are unknown.

Methods and Results—

Our work involved 51 patients with giant cell myocarditis (35 women) aged 52±12 years. Their medical records were reviewed for history, results of laboratory and imaging studies, and occurrence of serious cardiac events, including life-threatening ventricular tachyarrhythmias. Sudden cardiac death (fatal or aborted) was the primary end point of our analyses, whereas the composite of sudden cardiac death and ventricular tachycardia requiring treatment constituted the secondary end point. Giant cell myocarditis presented as nonfatal ventricular tachyarrhythmia in 10 patients and as a fatal cardiac arrest in 1 patient. Overall, 14 of 50 patients suffered a sudden cardiac death during follow-up, with a cumulative incidence of 22% at 1 year and 26% at 5 years from presentation. The composite incidence of sudden cardiac death or ventricular tachycardia was 41% at 1 year and 55% at 5 years. The incidence of arrhythmias was associated with high plasma concentrations of troponin-T and N-terminal brain natriuretic propeptide, as well as with moderate-to-severe fibrosis on myocardial biopsy and history of ventricular tachyarrhythmias at presentation (
P
<0.05 for all). An intracardiac cardioverter defibrillator was implanted in 31 patients, of whom 17 had altogether 114 appropriate antiarrhythmic therapies by the device and none suffered an arrhythmic death.

Conclusions—
In giant cell myocarditis, the risk of life-threatening ventricular arrhythmias exceeds 50% at 5 years from admission, being related to the presenting clinical manifestation and markers of myocardial injury and scarring.
Evaluation of polymorphic variants in apoptotic genes and their role in susceptibility and clinical progression to systemic lupus erythematosus
Authors: Glesse N , Vianna P , Paim L M G , Matte M C C , Aguiar A K K , Palhano P L , Monticielo O A , Brenol C V , Xavier R M , Chies J A B 
Published: December 2016

Background Systemic lupus erythematosus (SLE) is an autoimmune disease marked by the disruption of the immune homeostasis. Patients exhibit a wide range of clinical manifestations, and environmental and genetic factors are involved in SLE pathogenesis. Evidence suggests that abnormalities in the cellular and molecular events that coordinate apoptosis may favour the generation of autoantigens involved in autoimmunity. In this way, the apoptotic deregulation may be affected by polymorphic variants in apoptotic-related genes. Methods We analyzed FAS, FASL, BCL-2 and BAX polymorphisms in order to correlate to SLE susceptibility and clinical features. A total of 427 SLE patients from the Hospital de Clínicas de Porto Alegre and 543 controls from southern Brazil were evaluated. Results We observed higher frequencies of the FASL –844CC genotype and –844C allele, as well as of the FASL-844C/IVS2nt-124A haplotype in African-derived SLE patients when compared to controls ( P < 0.001). FASL –844C, which is related to high FasL expression, could contribute to increased apoptosis and to the breakdown of immunological tolerance, favouring autoantibody production and inflammation. On the other hand, the BAX –248GA genotype and the –248A allele , related to low protein expression, were observed as a protective factor against SLE in this same population. The rate of apoptosis and cell death was evaluated in peripheral lymphocytes, and SLE patients presented a higher percentage of dead lymphocytes (CD3+Annexin V+ 7-AAD+) compared to the control group. Conclusion Our data support a role for apoptosis in SLE susceptibility.
The Role of Constituency, Party, and Industry in Pennsylvania's Act 13
Authors: Bishop Bradford H. , Dudley Mark R. 
Published: December 2016

AbstractWhile a large body of research exists regarding the role of industry money on roll-call voting in the U.S. Congress, there is surprisingly little scholarship pertaining to industry influence on state politics. This study fills this void in an analysis of campaign donations and voting during passage of Act 13 in Pennsylvania during 2011 and 2012. After collecting information about natural gas production in state legislative districts, we estimate a series of multivariate models aimed at uncovering whether campaign donations contributed to a more favorable policy outcome for industry. Our findings indicate that campaign donations played a small but systematic role in consideration of the controversial legislation, which represented one of the first and most important state-level regulatory reforms for the hydraulic fracturing industry.
Below-Knee Amputation Failure and Poor Functional Outcomes Are Higher Than Predicted in Contemporary Practice
Authors: Columbo Jesse A. , Nolan Brian W. , Stucke Ryland S. , Rzucidlo Eva M. , Walker Karen L. , Powell Richard J. , Suckow Bjoern D. , Stone David H. 
Published: December 2016

Objective: The perceived functional benefit of below-knee amputation (BKA) must be carefully weighed against the need for potential reinterventions. This study sought to examine the contemporary clinical and functional outcomes of patients undergoing BKA in the endovascular era. Methods: All patients who underwent BKA from January 2008 to December 2014 at a single tertiary medical center were retrospectively reviewed. Demographics, comorbidities, ambulation status, and transcutaneous oximetry (TcPO2) values were recorded. Study end points included freedom from conversion to above-knee amputation (AKA), freedom from conversion to AKA or death, BKA healing, and ambulation. Statistical modeling was performed to determine associations with BKA failure. Results: Over the study interval, 130 limbs underwent BKA in 120 patients. Transcutaneous oximetry studies were obtained in 65% (n = 85). Thirty-eight percent (n = 46) of all BKA patients went on to heal and ambulate. Twenty-five percent (n = 33) required reintervention, 24 with conversion to AKA, and 9 with BKA revision. One-year freedom from conversion to AKA was 76% and was decreased among those with lower TcPO2 levels (60% TcPO2 <40 vs 81% TcPO2 ≥40; P = .04). One-year composite freedom from conversion to AKA/death was 60% and was decreased among those with lower TcPO2 readings (39% TcPO2 <40 vs 69% TcPO2 ≥40; P = .01). Conclusion: Despite a perceived functional bias toward knee salvage at the time of major amputation, most patients failed to postoperatively ambulate. Those with decreased TcPO2 levels (<40 mm Hg) have a 2-fold higher risk of AKA conversion or death, while nearly one-fourth of all BKA patients will succumb to the same fate irrespective of TcPO2. This suggests that many BKA patients in the endovascular era fail to derive the perceived benefit of knee salvage at the time of their index amputation. These findings highlight the need for careful patient selection and for a shared decision-making model in this frail population.
Exploring the Chronic Illness Experience of Rural-Dwelling Latinos of Mexican Origin
Authors: Guzman Anissa 
Published: December 2016

Introduction: Chronic illness is a complex and ever-growing phenomenon that is affecting millions of Americans every day, and it is disproportionately experienced by Latinos of Mexican origin. Method: In this quantitative study, the specific aims were to evaluate perceptions of chronic illness(es), locus of control (health and God), health status, and cultural orientation of rural-dwelling Latinos of Mexican origin in Colorado who have one or more chronic illnesses and to explore the relationships existing between these concepts. Results: A sample of 102 varied from overall national statistics for this population in the United States by gender, mean annual income, and education completed. Conclusion: As Latinos of Mexican origin move toward becoming the largest minority population in the United States, it is important to improve the body of nursing science that targets this population.
Accuracy of outpatient service data for activity-based funding in New South Wales, Australia
Authors: Munyisia Esther N , Reid David , Yu Ping 
Published: December 2016

Background: Despite increasing research on activity-based funding (ABF), there is no empirical evidence on the accuracy of outpatient service data for payment. Objective: This study aimed to identify data entry errors affecting ABF in two drug and alcohol outpatient clinic services in Australia. Methods: An audit was carried out on healthcare workers’ (doctors, nurses, psychologists, social workers, counsellors, and aboriginal health education officers) data entry errors in an outpatient electronic documentation system. Results: Of the 6919 data entries in the electronic documentation system, 7.5% (518) had errors, 68.7% of the errors were related to a wrong primary activity, 14.5% were due to a wrong activity category, 14.5% were as a result of a wrong combination of primary activity and modality of care, 1.9% were due to inaccurate information on a client’s presence during service delivery and 0.4% were related to a wrong modality of care. Conclusion: Data entry errors may affect the amount of funding received by a healthcare organisation, which in turn may affect the quality of treatment provided to clients due to the possibility of underfunding the organisation. To reduce errors or achieve an error-free environment, there is a need to improve the naming convention of data elements, their descriptions and alignment with the national standard classification of outpatient services. It is also important to support healthcare workers in their data entry by embedding safeguards in the electronic documentation system such as flags for inaccurate data elements.
A cross-sectional study to assess the patient safety culture in the Palestinian hospitals: a baseline assessment for quality improvement
Authors: Elsous Aymen , Akbari Sari Ali , Rashidian Arash , Aljeesh Yousef , Radwan Mahmoud , AbuZaydeh Hatem 
Published: December 2016

Objectives To measure and establish a baseline assessment of the patient safety culture in the Palestinian hospitals. Design A cross-sectional descriptive study using the Arabic version of the Safety Attitude Questionnaire (Short Form 2006). Participants A total of 339 nurses and physicians returned the questionnaire out of 370 achieving a response rate of 91.6%. Setting Four public general hospitals in the Gaza Strip, Palestine. Methods Nurses and physicians were randomly selected using a proportionate random sampling. Data analysis performed using Statistical Package for the Social Sciences software version 20, and p value less than 0.05 was statistically significant. Main outcomes measures Current status of patient safety culture among healthcare providers and percentage of positive attitudes. Results Male to female ratio was 2.16:1, and mean age was 36.5 ± 9.4 years. The mean score of Arabic Safety Attitude Questionnaire across the six dimensions on 100-point scale ranged between 68.5 for Job Satisfaction and 48.5 for Working Condition. The percentage of respondents holding a positive attitude was 34.5% for Teamwork Climate, 28.4% for Safety Climate, 40.7% for Stress Recognition, 48.8% for Job Satisfaction, 11.3% for Working Conditions and 42.8% for Perception of Management. Healthcare workers holding positive attitudes had better collaboration with co-workers than those without positive attitudes. Conclusion Findings are useful to formulate a policy on patient safety culture and targeted a specific safety culture dimension to improve the safety of patients and improve the clinical outcomes within healthcare organisations.
Inherited thrombophilia: a double-edged sword
Authors: Middeldorp Saskia 
Published: December 2016

AbstractInherited thrombophilia is a blood coagulation disorder that increases the risk for venous thromboembolism (VTE). During the last decades, the practice of testing has evolved from testing selected populations, leading to high perceived risks, to broad testing for various conditions that included VTE, arterial thrombosis, and pregnancy complications. Because results of such tests usually do not guide treatment decisions, not testing patients with VTE for inherited thrombophilia is on the “Choosing Wisely” list endorsed by multiple specialty societies, including ASH. Inherited thrombophilia can be regarded a double-edged sword, as despite the rationale not to test, it is still being performed frequently. Another way of seeing inherited thrombophilia as a double-edged sword lies in its 2-sided association with reproduction, both in men and in women. Current areas of research are whether women with inherited thrombophilia and pregnancy complications benefit from anticoagulant therapy with regard to improving the chance of a successful pregnancy. Potential effects of inherited thrombophilia, most notably factor V Leiden, on improved embryo implantation in women and sperm counts in men are intriguing, but are currently poorly understood.
Thrombosis and anticoagulation in the setting of renal or liver disease
Authors: Ribic Christine , Crowther Mark 
Published: December 2016

AbstractThrombosis and bleeding are among the most common causes of morbidity and mortality in patients with renal disease or liver disease. The pathophysiology underlying the increased risk for venous thromboembolism and bleeding in these 2 populations is distinct, as are considerations for anticoagulation. Anticoagulation in patients with kidney or liver disease increases the risk of bleeding; this risk is correlated with the degree of impairment of anticoagulant elimination by the kidneys and/or liver. Despite being in the same pharmacologic category, anticoagulant agents may have varied degrees of renal and liver metabolism. Therefore, specific anticoagulants may require dose reductions or be contraindicated in renal impairment and liver disease, whereas other drugs in the same class may not be subject to such restrictions. To minimize the risk of bleeding, while ensuring an adequate therapeutic effect, both appropriate anticoagulant drug choices and dose reductions are necessary. Renal and hepatic function may fluctuate, further complicating anticoagulation in these high-risk patient groups.
Venous thromboembolism and stroke in pregnancy
Authors: McLean Kelley , Cushman Mary 
Published: December 2016

Abstract
Pregnancy and the postpartum period substantially increase the risk for thrombotic events. Although the absolute risk for thrombosis is low, these events comprise a significant portion of maternal morbidity and mortality. The vast majority of such events are venous, although the risk for ischemic stroke also appears to be increased in pregnancy. This review will explore the overlapping and unique risk factors for venous and arterial thrombosis in pregnancy. Diagnosis and prevention will be discussed, and treatment will be briefly touched on. The benefit of using a multidisciplinary model in caring for pregnant women who have had a thrombotic event or who are at increased risk for thrombosis is a major focus of the review. Using the experience of our own Hematology and Obstetrics/Maternal Fetal Medicine shared care model, we discuss specific examples of when the use of such an approach is particularly valuable.
Introduction to cancer genetic susceptibility syndromes
Authors: McGee Rose B. , Nichols Kim E. 
Published: December 2016

AbstractThe last 30 years have witnessed tremendous advances in our understanding of the cancer genetic susceptibility syndromes, including those that predispose to hematopoietic malignancies. The identification and characterization of families affected by these syndromes is enhancing our knowledge of the oncologic and nononcologic manifestations associated with predisposing germ line mutations and providing insights into the underlying disease mechanisms. Here, we provide an overview of the cancer genetic susceptibility syndromes, focusing on aspects relevant to the evaluation of patients with leukemia and lymphoma. Guidance is provided to facilitate recognition of these syndromes by hematologists/oncologists, including descriptions of the family history features, tumor genotype, and physical or developmental findings that should raise concern for an underlying cancer genetic syndrome. The clinical implications and management challenges associated with cancer susceptibility syndromes are also discussed.
Novel agents and strategies in transplant-eligible patients with relapsed and refractory Hodgkin lymphoma
Authors: Moskowitz Craig 
Published: December 2016

Abstract
The majority of patients with Hodgkin lymphoma are cured with frontline therapy; however, 10% to 15% with early-stage disease and 20% to 30% with advanced stage require second-line therapy that includes a potentially curative transplant, of which an additional 50% to 55% are cured. Those with multiply relapsed disease traditionally would receive novel agents on a clinical trial or combination chemotherapy as a potential bridge to an allogeneic stem cell transplant. This treatment paradigm has changed with the availability of brentuximab vedotin, an antibody drug conjugate used pre- and post-ASCT, as well as for palliation. With the availability of the checkpoint inhibitors, nivolumab and pembrolizumab, there will be another shift in treatment, with these agents being used for palliation and potentially replacing allogeneic stem cell transplantation in certain patient populations. Finally, up-front management is also changing and this will have an impact on how patients in the relapsed and refractory setting will be treated.
Management of primary central nervous system lymphoma in children
Authors: Giulino-Roth Lisa , Abla Oussama , Batchelor Tracy T. 
Published: December 2016

Abstract
A 14-year-old boy with no significant past medical history presents with headaches and vomiting and is found to have a 2 × 3-cm left parietal lobe mass. A stereotactic biopsy reveals diffuse large B-cell lymphoma (DLBCL). Cerebrospinal fluid cytology, as well as bone marrow biopsies are negative, and a whole-body positron emission tomography/computed tomography scan does not demonstrate other areas of disease. The primary medical team asks how you would treat this patient.
Diagnosis of suspected venous thromboembolism
Authors: Kearon Clive 
Published: December 2016

Abstract
The primary goal of diagnostic testing for venous thromboembolism (VTE) is to identify all patients who could benefit from anticoagulant therapy. Test results that identify patients as having a ≤2% risk of VTE in the next 3 months are judged to exclude deep vein thrombosis (DVT) or pulmonary embolism (PE). Clinical evaluation, with assessment of: (1) clinical pretest probability (CPTP) for VTE; (2) likelihood of important alternative diagnoses; and (3) the probable yield of D-dimer and various imaging tests, guide which tests should be performed. The combination of nonhigh CPTP and negative D-dimer testing excludes DVT or PE in one-third to a half of outpatients. Venous ultrasound of the proximal veins, with or without examination of the distal veins, is the primary imaging test for leg and upper-extremity DVT. If a previous test is not available for comparison, the positive predictive value of ultrasound is low in patients with previous DVT. Computed tomography pulmonary angiography (CTPA) is the primary imaging test for PE and often yields an alternative diagnosis when there is no PE. Ventilation-perfusion scanning is associated with less radiation exposure than CTPA and is preferred in younger patients, particularly during pregnancy. If DVT or PE cannot be “ruled-in” or “ruled-out” by initial diagnostic testing, patients can usually be managed safely by: (1) withholding anticoagulant therapy; and (2) doing serial ultrasound examinations to detect new or extending DVT.
Risk stratification and management of acute pulmonary embolism
Authors: Becattini Cecilia , Agnelli Giancarlo 
Published: December 2016

Abstract
The clinical management of patients with acute pulmonary embolism is rapidly changing over the years. The widening spectrum of clinical management strategies for these patients requires effective tools for risk stratification. Patients at low risk for death could be candidates for home treatment or early discharge. Clinical models with high negative predictive value have been validated that could be used to select patients at low risk for death. In a major study and in several meta-analyses, thrombolysis in hemodynamically stable patients was associated with unacceptably high risk for major bleeding complications or intracranial hemorrhage. Thus, the presence of shock or sustained hypotension continues to be the criterion for the selection of candidates for thrombolytic treatment. Interventional procedures for early revascularization should be reserved to selected patients until further evidence is available. No clinical advantage is expected with the insertion of a vena cava filter in the acute-phase management of patients with acute pulmonary embolism. Direct oral anticoagulants used in fixed doses without laboratory monitoring showed similar efficacy (odds ratio [OR], 0.89; 95% confidence interval [CI], 0.70-1.12) and safety (OR, 0.89; 95% CI, 0.77-1.03) in comparison with conventional anticoagulation in patients with acute pulmonary embolism. Based on these results and on their practicality, direct oral anticoagulants are the agents of choice for the treatment of the majority of patients with acute pulmonary embolism.
Treatment of the elderly patient with mantle cell lymphoma
Authors: Soubeyran Pierre , Gressin Rémy 
Published: December 2016

Abstract
Treatment options for mantle cell lymphomas have expanded considerably over recent years, offering hematologists solutions for older patients with an appropriate risk-to-benefit ratio. Indeed, unfit older patients are exposed to a higher risk of toxicity with a standard treatment. Although new treatments have generally good safety profiles, they may lead to unexpected consequences in unfit older patients. Involving geriatricians and a comprehensive geriatric assessment in patient care could help hematologists address these vulnerabilities. The geriatric evaluation process is time-consuming but can be simplified, and its potential to help hematologists foresee unexpected consequences of treatment has now been demonstrated.
Primary immune deficiencies with defects in neutrophil function
Authors: Dinauer Mary C. 
Published: December 2016

AbstractImmune deficiencies resulting from inherited defects in neutrophil function have revealed important features of the innate immune response. Although sharing an increased susceptibility to bacterial and fungal infections, these disorders each have distinctive features in their clinical manifestations and characteristic microbial pathogens. This review provides an update on several genetic disorders with impaired neutrophil function, their pathogenesis, and treatment strategies. These include chronic granulomatous disease, which results from inactivating mutations in the superoxide-generating nicotinamide dinucleotide phosphate oxidase. Superoxide-derived oxidants play an important role in the control of certain bacterial and fungal species, and also contribute to the regulation of inflammation. Also briefly summarized are updates on leukocyte adhesion deficiency, including the severe periodontal disease characteristic of this disorder, and a new immune deficiency associated with defects in caspase recruitment domain–containing protein 9, an adaptor protein that regulates signaling in neutrophils and other myeloid cells, leading to invasive fungal disease.