bluuebunny/crossref_metadata_2025_split huggingface dataset:
Self-criticisms toward a socially responsible science in the field of management
Authors: de Frutos-Belizón Jesús , Martín-Alcázar Fernando , Sánchez-Gardey Gonzalo 
Published: December 2021

Management scholarship should be placed in a unique position to develop relevant scientific knowledge because business and management organizations are deeply involved in most global challenges. However, different critical voices have recently been raised in essays and editorials, and reports have questioned research in the management field, identifying multiple deficiencies that can limit the growth of a relatively young field. Based on an analysis of published criticisms of management research, we would like to shed light on the current state of management research and identify some limitations that should be considered and should guide the growth of this field of knowledge. This work offers guidance on the main problems of the discipline that should be addressed to encourage the transformation of management research to meet both scientific rigor and social relevance. The article ends with a discussion and a call to action for directing research toward the possibility and necessity of reinforcing “responsible research” in the management field. JEL CLASSIFICATION: M00, M10
Direct oral anticoagulant (DOAC) interference in hemostasis assays
Authors: Moser Karen A. , Smock Kristi J. 
Published: December 2021

Abstract
Direct oral anticoagulants (DOACs) are a group of direct coagulation factor inhibitors including both direct thrombin inhibitors and direct factor Xa inhibitors. These medications may cause hemostasis assay interference by falsely increasing or decreasing measured values, depending on the analyte. Considering the potential for DOAC interference in a variety of hemostasis assays is essential to avoid erroneous interpretation of results. Preanalytic strategies to avoid DOAC interference include selecting alternatives to clot-based hemostasis assays in patients taking DOACs when possible and sample collection timed when the patient is off anticoagulant therapy or at the expected drug trough. Clinical laboratories may also provide educational materials that clearly describe possible interferences from DOAC, develop testing algorithms to aid in detection of DOAC in submitted samples, use DOAC-neutralizing agents to remove DOACs before continuing with testing, and write interpretive comments that explain the effects of DOAC interference in hemostasis tests. Using a combination of the described strategies will aid physicians and laboratorians in correctly interpreting hemostasis and thrombosis laboratory tests in the presence of DOACs.
The clinical and laboratory evaluation of patients with suspected hypocellular marrow failure
Authors: Keel Siobán , Geddis Amy 
Published: December 2021

AbstractThe overlap in clinical presentation and bone marrow features of acquired and inherited causes of hypocellular marrow failure poses a significant diagnostic challenge in real case scenarios, particularly in nonsevere disease. The distinction between acquired aplastic anemia (aAA), hypocellular myelodysplastic syndrome (MDS), and inherited bone marrow failure syndromes presenting with marrow hypocellularity is critical to inform appropriate care. Here, we review the workup of hypocellular marrow failure in adolescents through adults. Given the limitations of relying on clinical stigmata or family history to identify patients with inherited etiologies, we outline a diagnostic approach incorporating comprehensive genetic testing in patients with hypocellular marrow failure that does not require immediate therapy and thus allows time to complete the evaluation. We also review the clinical utility of marrow array to detect acquired 6p copy number-neutral loss of heterozygosity to support a diagnosis of aAA, the complexities of telomere length testing in patients with aAA, short telomere syndromes, and other inherited bone marrow failure syndromes, as well as the limitations of somatic mutation testing for mutations in myeloid malignancy genes for discriminating between the various diagnostic possibilities.
When to worry about inherited bone marrow failure and myeloid malignancy predisposition syndromes in the setting of a hypocellular marrow
Authors: Narla Anupama 
Published: December 2021

Abstract
With our increasing understanding of inherited marrow failure and myeloid malignancy predisposition syndromes, it has become clear that there is a wide phenotypic spectrum and that these diseases must be considered in the differential diagnosis of both children and adults with unexplained defects in hematopoiesis. Moreover, these conditions are not as rare as previously believed and may present as aplastic anemia, myelodysplastic syndrome, or malignancy over a range of ages. Establishing the correct diagnosis is essential because it has implications for treatment, medical management, cancer screening, and family planning. Our goal is to highlight insights into the pathophysiology of these diseases, review cryptic presentations of these syndromes, and provide useful references for the practicing hematologist.
Double-hit lymphoma: optimizing therapy
Authors: Dunleavy Kieron 
Published: December 2021

Abstract
Aggressive B-cell lymphoma is a heterogeneous entity with disparate outcomes based on clinical and pathological characteristics. While most tumors in this category are diffuse large B-cell lymphoma (DLBCL), the recognition that some cases have high-grade morphology and frequently harbor MYC and BCL2 and/or BCL6 translocations has led to their separate categorization. These cases are now considered distinct from DLBCL and are named “high-grade B-cell lymphoma” (HGBL). Most are characterized by distinct rearrangements, but others have high-grade morphological features without these and are called HGBL-not otherwise specified. Studies have demonstrated that this group of diseases leads to poor outcomes following standard rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone therapy; retrospective and recent single-arm, multicenter studies suggest they should be approached with dose-intense treatment platforms. As yet, this has not been validated in randomized trial settings due to the rarity of these diseases. In the relapsed and refractory setting, novel approaches such as anti-CD19 chimeric antigen receptor T cells and antibodies against CD19 have demonstrated high efficacy in this subgroup. Recently, genomic studies have made much progress in investigating some of the molecular underpinnings that drive their lymphomagenesis and have paved the way for testing additional novel approaches.
Allogeneic hematopoietic cell transplantation for older patients
Authors: Lin Richard J. , Artz Andrew S. 
Published: December 2021

Abstract
Hematologic malignances are more common and often higher risk in older patients. Allogeneic hematopoietic cell transplantation (alloHCT) best enables long-term disease control for patients with poor risk or relapsed/refractory hematologic malignancies such as acute myeloid leukemia, myelodysplastic syndromes, or myelofibrosis. Rates of alloHCT among older patients, while still relatively low compared with younger patients, have risen sharply over the past decade. Accumulating evidence supports alloHCT for patients ≥60 years of age relative to non-HCT therapies based on improved overall and disease-free survival. However, a significant proportion of older adults have limitations characterized by geriatric assessment. A systematic process to evaluate and optimize older patients may improve decision making, transplant outcomes, and alloHCT access. We present case-based studies to illustrate a stepwise and rational approach to proper older patient evaluation, pretransplant optimization, and posttransplant care with attention to important geriatric issues and quality of life.
Diagnosis and clinical management of enzymopathies
Authors: Luzzatto Lucio 
Published: December 2021

AbstractAt least 16 genetically determined conditions qualify as red blood cell enzymopathies. They range in frequency from ultrarare to rare, with the exception of glucose-6-phosphate dehydrogenase deficiency, which is very common. Nearly all these enzymopathies manifest as chronic hemolytic anemias, with an onset often in the neonatal period. The diagnosis can be quite easy, such as when a child presents with dark urine after eating fava beans, or it can be quite difficult, such as when an adult presents with mild anemia and gallstones. In general, 4 steps are recommended: (1) recognizing chronic hemolytic anemia; (2) excluding acquired causes; (3) excluding hemoglobinopathies and membranopathies; (4) pinpointing which red blood cell enzyme is deficient. Step 4 requires 1 or many enzyme assays; alternatively, DNA testing against an appropriate gene panel can combine steps 3 and 4. Most patients with a red blood cell enzymopathy can be managed by good supportive care, including blood transfusion, iron chelation when necessary, and splenectomy in selected cases; however, some patients have serious extraerythrocytic manifestations that are difficult to manage. In the absence of these, red blood cell enzymopathies are in principle amenable to hematopoietic stem cell transplantation and gene therapy/gene editing.
CHIP: is clonal hematopoiesis a surrogate for aging and other disease?
Authors: Gondek Lukasz P. 
Published: December 2021

Abstract
Somatic mutations are an unavoidable consequence of aging tissues. Even though most mutations are functionally silent, some may affect genes critical to proper tissue self-renewal and differentiation, resulting in the outgrowth of affected cells, also known as clonal expansion. In hematopoietic tissue such clonal dominance is known as clonal hematopoiesis (CH). Sporadic CH is frequent in aging and affects over 10% of individuals beyond the fifth decade of life. It has been associated with an increased risk of hematologic malignancies and cardiovascular disease. In addition to aging, CH has been observed in other hematologic conditions and confers an adaptation of hematopoietic stem cells (HSCs) to various environmental stressors and cell-intrinsic defects. In the presence of extrinsic stressors such as genotoxic therapies, T-cell-mediated immune attack, or inflammation, somatic mutations may result in augmentation of HSC fitness. Such attuned HSCs can evade the environmental insults and outcompete their unadapted counterparts. Similarly, in inherited bone marrow failures, somatic mutations in HSCs frequently lead to the reversion of inherited defects. This may occur via the direct correction of germline mutations or indirect compensatory mechanisms. Occasionally, such adaptation may involve oncogenes or tumor suppressors, resulting in malignant transformation. In this brief article, we focus on the mechanisms of clonal dominance in various clinical and biological contexts.
Mechanisms of somatic transformation in inherited bone marrow failure syndromes
Authors: Choijilsuren Haruna Batzorig , Park Yeji , Jung Moonjung 
Published: December 2021

AbstractInherited bone marrow failure syndromes (IBMFS) cause hematopoietic stem progenitor cell (HSPC) failure due to germline mutations. Germline mutations influence the number and fitness of HSPC by various mechanisms, for example, abnormal ribosome biogenesis in Shwachman-Diamond syndrome and Diamond-Blackfan anemia, unresolved DNA cross-links in Fanconi anemia, neutrophil maturation arrest in severe congenital neutropenia, and telomere shortening in short telomere syndrome. To compensate for HSPC attrition, HSPCs are under increased replication stress to meet the need for mature blood cells. Somatic alterations that provide full or partial recovery of functional deficit implicated in IBMFS can confer a growth advantage. This review discusses results of recent genomic studies and illustrates our new understanding of mechanisms of clonal evolution in IBMFS.
When are idiopathic and clonal cytopenias of unknown significance (ICUS or CCUS)?
Authors: Osman Afaf E. W. G. 
Published: December 2021

Abstract
Rapid advances in sequencing technology have led to the identification of somatic mutations that predispose a significant subset of the aging population to myeloid malignancies. Recently recognized myeloid precursor conditions include clonal hematopoiesis of indeterminate potential (CHIP) and clonal cytopenia of unknown significance (CCUS). These conditions can present diagnostic challenges and produce unwarranted anxiety in some instances. While the risk of progression to myeloid malignancies is very low in CHIP, true CCUS confers an exponential increase in risk. Idiopathic cytopenia of unknown significance (IDUS) lacks the predisposing genetic mutations and has a variable course. In this review we define the early myeloid precursor conditions and their risk of progression. We present our diagnostic approach to patients with unexplained cytopenias and discuss the clinical consequences of CHIP and CCUS.
Running interferon interference in treating PV/ET: meeting unmet needs
Authors: Traxler Elizabeth A. , Hexner Elizabeth O. 
Published: December 2021

AbstractEnthusiasm about interferons for the treatment of myeloproliferative neoplasms has recently arisen. How does a nontargeted therapy selectively target the malignant clone? Many foundational questions about interferon treatment are unanswered, including who, when, and for how long do we treat. Using an individual case, this review touches on gaps in risk assessment in polycythemia vera (PV) and essential thrombocythemia (ET) and the history of treatment with interferons. How is it that this proinflammatory cytokine effectively treats ET and PV, themselves proinflammatory states? We summarize existing mechanistic and clinical data, the molecular context as a modifier for treatment response, the establishment of treatment goals, and the challenges that lie ahead.
Rebalanced hemostasis in liver disease: a misunderstood coagulopathy
Authors: Roberts Lara N. 
Published: December 2021

Abstract
The combination of frequently abnormal hemostatic markers and catastrophic bleeding as seen with variceal hemorrhage has contributed to the longstanding misperception that chronic liver disease (CLD) constitutes a bleeding diathesis. Laboratory studies of hemostasis in liver disease consistently challenge this with global coagulation assays incorporating activation of the protein C pathway demonstrating rebalanced hemostasis. It is now recognized that bleeding in CLD is predominantly secondary to portal hypertension (rather than a coagulopathy) and additionally that these patients are at increased risk of venous thrombosis, particularly in the portal venous system. This narrative review describes the current understanding of hemostasis in liver disease, as well as the periprocedural management of hemostasis and anticoagulation for management of venous thromboembolism in patients with CLD.
Updates in chronic graft-versus-host disease
Authors: Hamilton Betty K. 
Published: December 2021

Abstract
Chronic graft-versus-host disease (GVHD) is the leading cause of late morbidity and mortality after allogeneic hematopoietic cell transplantation. Symptoms and manifestations of chronic GVHD are heterogeneous and pleomorphic, and there are no standard treatments beyond corticosteroids. Therapy is typically prolonged, and chronic GVHD and its treatment are associated with adverse effects that have a significant impact on long-term quality of life and functional status. Several advances have been made over the last 2 decades to define the diagnosis of chronic GVHD as well as its severity and response criteria for clinical trials. Further understanding into the biologic mechanisms of the development of chronic GVHD has led to the investigation of several novel immunomodulatory and targeted therapies. Multi-institutional collaboration and pharmaceutical support in the development of therapies based on sound biologic mechanisms and clinical trials with defined end points and responses have led to several promising agents on the horizon of approval for treatment of chronic GVHD. This article reviews advances in our knowledge of chronic GVHD and its biologic framework to improve approaches to prevention and treatment.
Indications for transfusion in the management of sickle cell disease
Authors: Han Hyojeong , Hensch Lisa , Tubman Venée N. 
Published: December 2021

Abstract
The transfusion of red blood cells (RBCs) is a crucial treatment for sickle cell disease (SCD). While often beneficial, the frequent use of transfusions is associated with numerous complications. Transfusions should be offered with specific guidelines in mind. Here we present updates to the indications for transfusion of RBCs in SCD. We review recent publications and include expert perspectives from hematology and transfusion medicine. For some clinical indications, such as ischemic stroke, the role of transfusion has been well studied and can be applied almost universally. For many other clinical scenarios, the use of transfusion therapy has less conclusive data and therefore must be tailored to individual needs. We highlight the roles of RBC transfusions in preventing or mitigating neurological disease, in reducing perioperative complications, in managing acute chest syndrome, and in optimizing pregnancy outcomes in SCD. We further highlight various transfusion techniques and when each might be considered. Potential complications of transfusion are also briefly discussed.
Association of mental demands in the workplace with cognitive function in older adults at increased risk for dementia
Authors: Zülke Andrea E. , Luppa Melanie , Röhr Susanne , Weißenborn Marina , Bauer Alexander , Samos Franziska-Antonia Zora , Kühne Flora , Zöllinger Isabel , Döhring Juliane , Brettschneider Christian , Oey Anke , Czock David , Frese Thomas , Gensichen Jochen , Haefeli Walter E. , Hoffmann Wolfgang , Kaduszkiewicz Hanna , König Hans-Helmut , Thyrian Jochen René , Wiese Birgitt , Riedel-Heller Steffi G. 
Published: December 2021

Abstract
Objectives
Growing evidence suggests a protective effect of high mental demands at work on cognitive function in later life. However, evidence on corresponding associations in older adults at increased risk for dementia is currently lacking. This study investigates the association between mental demands at work and cognitive functioning in the population of the AgeWell.de-trial.

Methods
Cross-sectional investigation of the association between global cognitive functioning (Montreal Cognitive Assessment) and mental demands at work in older individuals at increased risk for dementia (Cardiovascular Risk Factors, Aging, and Incidence of Dementia (CAIDE)score ≥ 9; n = 941, age: 60–77 years). Occupational information was matched to Occupational Information Network (O*NET)-descriptors. Associations between cognitive function and O*NET-indices executive, verbal and novelty were investigated using generalized linear models.

Results
Higher values of index verbal (b = .69, p = .002) were associated with better cognitive function when adjusting for covariates. No association was observed for indices executive (b = .37, p = .062) and novelty (b = .45, p = .119). Higher education, younger age, and employment were linked to better cognitive function, while preexisting medical conditions did not change the associations. Higher levels of depressive symptomatology were associated with worse cognitive function.

Conclusions
Higher levels of verbal demands at work were associated with better cognitive function for older adults with increased dementia risk. This suggests an advantage for older persons in jobs with high mental demands even after retirement and despite prevalent risk factors. Longitudinal studies are warranted to confirm these results and evaluate the potential of workplaces to prevent cognitive decline through increased mental demands.
PG-SGA SF in nutrition assessment and survival prediction for elderly patients with cancer
Authors: Zhang Qi , Li Xiang-Rui , Zhang Xi , Ding Jia-Shan , Liu Tong , Qian Liang , Song Meng-Meng , Song Chun-Hua , Barazzoni Rocco , Tang Meng , Wang Kun-Hua , Xu Hong-Xia , Shi Han-Ping ,   Investigation on Nutrition Status and its Clinical Outcome of Common Cancers (INSCOC) Group
Published: December 2021

Abstract
Background
This study was sought to report the prevalence of malnutrition in elderly patients with cancer. Validate the predictive value of the nutritional assessment tool (Patient-Generated Subjective Global Assessment Short Form, PG-SGA SF) for clinical outcomes and assist the therapeutic decision.

Methods
This is a secondary analysis of a multicentric, observational cohort study. Elderly patients with cancer older than 65 years were enrolled after the first admission. Nutritional status was identified using the PG-SGA SF.

Results
Of the 2724 elderly patients included in the analysis, 65.27% of patients were male (n = 1778); the mean age was 71.00 ± 5.36 years. 31.5% of patients were considered malnourished according to PG-SGA SF. In multivariate analysis, malnutrition(PG-SGA SF > 5) was significantly associated with worse OS (HR: 1.47,95%CI:1.29–1.68), affects the quality of life, and was related to more frequent nutrition impact symptoms. During a median follow-up of 4.5 years, 1176 death occurred. The mortality risk was 41.10% for malnutrition during the first 12 months and led to a rate of 323.98 events per-1000-patient-years. All nutritional assessment tools were correlated with each other (PG-SGA SF vs. PG-SGA: r = 0.98; PG-SGA SF vs. GLIM[Global Leadership Initiative on Malnutrition]: r = 0.48, all P < 0.05). PG-SGA SF and PG-SGA performed similarly to predict mortality but better than GLIM. PG-SGA SF improves the predictive ability of the TNM classification system for mortality in elderly patients with cancer, including distinguishing patients’ prognoses and directing immunotherapy.

Conclusions
The nutritional status as measured by PG-SGA SF which is a prognostic factor for OS in elderly cancer patients and could improve the prognostic model of TNM.
High-frequency radio wave electrocautery in modified Hotz operations for epiblepharon correction
Authors: Lee Hyunkyu , Youn Jimin , Baek Sehyun 
Published: December 2021

AbstractTo report the experience and advantageous effects of high-frequency radio wave electrocautery in modified Hotz operations for epiblepharon, We reviewed the records of all pediatric patients who underwent a modified Hotz operation with the use of high-frequency radio wave electrocautery (Ellman Surgitron Dual Frequency RF S5; Ellman International, Inc, Hewlett, NY) for epiblepharon between March 2016 and September 2019 at Korea University Guro Hospital. We evaluated the success rate, complications, recurrence rate and degree of satisfaction of our technique. Information from the medical records was collected, including demographics, ocular symptoms, severity of keratopathy, operation time, success/recurrence rate, and complications. 133 patients (98.52%) showed good correction of epiblepharon without complications or unpleasant cosmetic problems during 3 months of median follow-up period. Two patients (1.48%) showed recurrent corneociliary touch, but the degree was very mild and re-operation was not performed. One patient showed mild ectropion on his left lower eyelid, but the patient recovered well without operation. For complications, suture abscess and granulation were the most common, 3 cases in each, but all of those were temporary and resolved with conservative management. The approach with electrocautery for epiblepharon allows precise and fast incision of the lower eyelid, little bleeding, and minimal scarring. Surgical outcomes associated with the modified Hotz operation with electrocautery were consistent with previous studies.