bluuebunny/crossref_metadata_2025_split huggingface dataset:
Frontline therapy of acute promyelocytic leukemia: Randomized comparison of ATRA and intensified chemotherapy versus ATRA and anthracyclines
Authors: Lengfelder Eva , Görlich Dennis , Nowak Daniel , Spiekermann Karsten , Haferlach Claudia , Krug Utz , Kreuzer Karl‐Anton , Braess Jan , Schliemann Christoph , Lindemann Hans‐Walter , Horst Heinz A. , Schiel Xaver , Flasshove Michael , Hecht Anna , Schnittger Susanne , Schneider Stephanie , Wörmann Bernhard , Hofmann Wolf‐Karsten , Berdel Wolfgang E. , Bormann Eike , Sauerland Cristina , Büchner Thomas , Hiddemann Wolfgang ,   for the German Acute Myeloid Leukemia Cooperative Group (AMLCG)
Published: November 2017

AbstractObjectivesRandomized comparison of two treatment strategies in frontline therapy of acute promyelocytic leukemia (APL): all‐trans retinoic acid (ATRA) and double induction intensified by high‐dose cytosine arabinoside (HD ara‐C) (German AMLCG) and therapy with ATRA and anthracyclines (Spanish PETHEMA, LPA99).Patients and resultsEighty of 87 adult patients with genetically confirmed APL of all risk groups were eligible. The outcome of both arms was similar: AMLCG vs PETHEMA: hematological complete remission 87% vs 83%, early death 13% vs 17% (P = .76), overall survival, event‐free survival, leukemia‐free survival, cumulative incidence of relapse at 6 years 75% vs 78% (P = .92); 75% vs 68% (P = .29); 86% vs 81% (P = .28); and 0% vs 12% (P = .04, no relapse vs four relapses), respectively. The median time to achieve molecular remission (RT‐PCR negativity of PML‐RARA) was 60 days in both arms (P = .12). The AMLCG regimen was associated with a longer duration of neutropenia (P = .02) and a higher rate of WHO grade ≥3 infections.ConclusionsThe small number of patients limits the reliability of conclusions. With these restrictions, the outcomes of both approaches were similar and show the limitations of ATRA and chemotherapy. The HD ara‐C–containing regimen was associated with a lower relapse rate in high‐risk APL.
Recessive distal motor neuropathy with pyramidal signs in an Omani kindred: underlying novel mutation in the SIGMAR1 gene
Authors: Nandhagopal R. , Meftah D. , Al‐Kalbani S. , Scott P. 
Published: November 2017

Background and purposeDistal hereditary motor neuropathy (dHMN) due to sigma non‐opioid intracellular receptor 1 (SIGMAR1) gene mutation (OMIM 601978.0003) is a rare neuromuscular disorder characterized by prominent amyotrophic distal limb weakness and co‐existing pyramidal signs initially described in a Chinese family recently. We report an extended consanguineous Omani family segregating dHMN with pyramidal signs in an autosomal recessive pattern and describe a novel mutation in the SIGMAR1 gene underlying this motor phenotype. We also provide an update on the reported phenotypic profile of SIGMAR1 mutations.MethodsWe utilized homozygosity mapping and whole‐exome sequencing of leucocyte DNA obtained from three affected members of an Omani family who manifested with a length‐dependent motor neuropathy and pyramidal signs.ResultsWe identified a novel C>T transition at nucleotide position 238 (c.238C>T) in exon 2 of the SIGMAR1 gene. Sanger sequencing and segregation analysis confirmed the presence of two copies of the variant in the affected subjects, unlike the unaffected healthy parents/sibling who carried, at most, a single copy. The T allele is predicted to cause a truncating mutation (p.Gln80*), probably flagging the mRNA for nonsense‐mediated decay leading to a complete loss of function, thereby potentially contributing to the disease process.ConclusionsOur finding expands the spectrum of SIGMAR1 mutations causing recessive dHMN and indicates that this disorder is pan‐ethnic. SIGMAR1 mutation should be included in the diagnostic panel of a dHMN, especially if there are co‐existing pyramidal signs and autosomal recessive inheritance.
PCR for antigen receptor rearrangement (PARR) in equine veterinary medicine
Authors: Hollis A. R. 
Published: November 2017

SummaryPCR for antigen receptor rearrangement (PARR) is a technique developed to aid with the assessment of lymphoid malignancies. The technology relies on the assessment of clonality of T or B cells, and there are many pitfalls. One major advantage is that only a small amount of sample is required to detect clonality, and it can be performed on any type of sample, including effusions, formalin‐fixed paraffin‐embedded tissues and cytological samples. However, the risk of both false negative and false positive results means that PARR should not be used to diagnose a lymphoid malignancy in isolation, but that results should be considered in conjunction with a full clinical and clinicopathological assessment.
Preparing Teaching Assistants to Work With All Learners: The Impact of Accessibility Training
Authors: Gallego Muriel , Busch Carey 
Published: November 2017

AbstractThis study investigated the impact of accessibility training on incoming teaching assistants’ (TAs) awareness of and preparation to implement academic adjustments and accommodations for students with disabilities. Pre‐ and postworkshop questionnaires and data gathered from weekly in‐service group discussions indicated that TAs’ knowledge of and confidence in implementing accommodations increased, as well as their awareness concerning legal mandates and accessibility issues in general. Recommendations regarding the training and mentorship of TAs are offered.
Clinical features of pediatric autoimmune hepatitis in Japan: A nationwide survey
Authors: Sogo Tsuyoshi , Takahashi Atsushi , Inui Ayano , Fujisawa Tomoo , Ohira Hiromasa , Takikawa Hajime ,   Japan AIH Study Group (JAIHSG)
Published: November 2017

AimThe purpose of this study was to determine the characteristics of children with autoimmune hepatitis (AIH) in Japan.MethodsQuestionnaires that asked about patients newly diagnosed with AIH from 2009 to 2013 were sent to hospitals certified as training facilities for pediatrics in January 2015.ResultsA total of 35 patients were enrolled. The median age at diagnosis was 10 years (range, 3 months–15 years), and the male‐to‐female ratio was 2:3. Female patients were more prevalent among those older than 10 years and male patients were more prevalent in those younger than 10 years. Fifteen patients had jaundice as a subjective symptom, and 5 had hepatic coma grade II. Liver histology classified 20 as chronic hepatitis, 8 as acute hepatitis, and 4 as cirrhosis. Liver histology was not described in 4 patients. Among the 35 patients, 32 were treated with corticosteroids and 29 were initially treated with methylprednisolone pulse therapy. Corticosteroid therapy was effective in 27 patients and ineffective in 1 patient. Plasma exchange with continuous i.v. infusion of cyclosporine A was given to 7 patients with acute hepatitis. Of these, 4 patients presented with fulminant hepatitis and received high‐flow, continuous hemodiafiltration.ConclusionsThis survey clarified that the clinical profile of pediatric AIH in Japan is not only different from that of adult AIH in Japan but is also different from that of pediatric AIH in other countries.
Changes in liver function and body composition by direct‐acting antiviral therapy for hepatitis C virus infection
Authors: Sugimoto Ryosuke , Iwasa Motoh , Hara Nagisa , Tamai Yasuyuki , Yoshikawa Kyoko , Ogura Suguru , Tanaka Hideaki , Eguchi Akiko , Yamamoto Norihiko , Kobayashi Yoshinao , Hasegawa Hiroshi , Takei Yoshiyuki 
Published: November 2017

AimManagement of low skeletal muscle mass (LSM) is a very important topic as LSM affects patient mortality in liver diseases. Changes in body composition are unexplored in chronic hepatitis C virus (HCV) patients, including those with liver cirrhosis, who receive direct‐acting antiviral (DAA) therapy. Body composition measurements and liver function tests were carried out before and after DAA therapy.MethodsBlood examination, visceral fat area (VFA) and extremity skeletal muscle mass were measured using the multifrequency bioelectrical impedance analysis method: (i) at 24 weeks before DAA therapy; (ii) at the start of DAA therapy; (iii) at the end of DAA therapy; (iv) at 24 weeks after DAA therapy; and (v) at 48 weeks after DAA therapy.ResultsSerum albumin (Alb) levels were significantly increased at 48 weeks post DAA therapy, especially in patients with LSM. Skeletal muscle mass index (SMI) was significantly increased after DAA therapy (at 24 weeks and 48 weeks post DAA therapy) in patients with LSM (P < 0.05). An increase in SMI was associated with an increase in body weight or a decrease in VFA.ConclusionsWe continuously measured body composition in HCV‐infected patients who received DAA therapy and found that skeletal muscle mass was significantly increased, associated with an elevation of serum Alb levels and/or body weight or reduction in VFA, but only in patients who presented with LSM before DAA therapy.
Loss of skeletal muscle mass in patients with chronic liver disease is related to decrease in bone mineral density and exercise tolerance
Authors: Hayashi Fumikazu , Kaibori Masaki , Sakaguchi Tatsuma , Matsui Kosuke , Ishizaki Morihiko , Kwon A‐Hon , Iwasaka Junji , Kimura Yutaka , Habu Daiki 
Published: November 2017

AimThis study aimed to identify the relationship between loss of skeletal muscle mass and clinical factors such as osteoporosis in patients with chronic liver disease.MethodsThe subjects were 112 patients (85 men and 27 women) with hepatocellular carcinoma who were scheduled to undergo hepatectomy. Skeletal muscle reduction was diagnosed according to the cut‐off level of the skeletal mass index (SMI) for Asians (men <7.0 kg/m2, women <5.4 kg/m2). Osteoporosis was diagnosed according to T‐score ≤−2.5 standard deviation. The SMI and T‐score were assessed using the results of dual‐energy X‐ray absorption. Peak oxygen consumption (PeakVO2), an index of exercise tolerance, was evaluated using the cardiopulmonary exercise test. The characteristics of patients with low SMI (low SMI group) were compared with those of patients whose SMI was not low (control group). Outcomes are presented as median (interquartile range).ResultsThe T‐score was significantly lower in the low SMI group (control vs. low SMI −1.1 [1.8] vs. −1.6 [1.9], P = 0.049). T‐score positively correlated with SMI (r = 0.409, P < 0.0001). PeakVO2 was significantly decreased in the low SMI group (17.7 [6.3] vs. 14.4 [4.5], P = 0.006). In multivariate logistic regression analysis, T‐score (odds ratio [OR], 3.508; 95% confidence interval [CI], 1.074–11.456; P = 0.038) and PeakVO2 (OR, 3.512; 95% CI, 1.114–11.066; P = 0.032) were significantly related to SMI, independent of age and sex.ConclusionsSkeletal muscle reduction in chronic liver disease is closely related to exercise tolerance and osteoporosis, and these factors are believed to be associated with physical inactivity in daily life.
Patient and public perspectives of community pharmacies in the United Kingdom: A systematic review
Authors: Hindi Ali M. K. , Schafheutle Ellen I. , Jacobs Sally 
Published: November 2017

AbstractBackgroundThe United Kingdom has been at the forefront of enhancing pharmacist roles and community pharmacy services, particularly over the past decade. However, patient and public awareness of community pharmacy services has been limited.ObjectiveTo identify and synthesize the research literature pertaining to patient and public perspectives on: existing community pharmacy services, extended pharmacist roles and strategies to raise awareness of community pharmacy services.Search strategySystematic search of 8 electronic databases; hand searching of relevant journals, reference lists and conference proceedings.Inclusion criteriaUK studies investigating patient or public views on community pharmacy services or pharmacist roles from 2005 to 2016.Data extraction and synthesisData were extracted into a grid and subjected to narrative synthesis following thematic analysis.Main resultsFrom the 3260 unique papers identified, 30 studies were included. Manual searching identified 4 additional studies. Designs using questionnaires (n = 14, 41%), semi‐structured interviews (n = 8, 24%) and focus groups (n = 6, 18%) made up the greatest proportion of studies. Most of the studies (n = 28, 82%) were published from 2010 onwards and covered perceptions of specific community pharmacy services (n = 31). Using a critical appraisal checklist, the overall quality of studies was deemed acceptable. Findings were grouped into 2 main themes “public cognizance” and “attitudes towards services” each with 4 subthemes.Discussion and conclusionsPatients and the public appeared to view services as beneficial. Successful integration of extended pharmacy services requires pharmacists’ clinical skills to be recognized by patients and physicians. Future research should explore different approaches to increase awareness.
Tracking wetland community evolution using Diptera taxonomic, functional and phylogenetic structure
Authors: Gregoire Taillefer Amelie , Wheeler Terry A. 
Published: November 2017

AbstractDifferent processes drive spatial variation in community composition. Standard measures of composition are useful in species‐based conservation and ecology, but they may be less informative in the context of evolutionary history and functional diversity. Functional and phylogenetic approaches are increasingly used to test mechanisms driving biodiversity patterns.We studied 28 families of flies (Diptera) with a range of functional characteristics in three wetland classes (bogs, swamps, marshes) in Quebec, Canada. We examined taxonomic, phylogenetic and functional structure of communities and assessed whether rarity is deterministic or stochastic. Beta‐ and phylobeta‐diversity were also examined for relatedness to local environmental conditions, patch area, and/or surrounding landscape.Phylogenetic community structure analyses had high value and complementarity to standard measures. Environmental filtering acted on bog communities during assembly, as they emerged from a slow peat accumulation process and the plant composition is characteristic as few species can survive in these acidic and low nutrient conditions. Subsequently, community assembly happened randomly. Neutral processes of community assembly are more important in marshes and swamps, as dispersal limitation explained species abundance dynamics of small and common Diptera species. The assembly of marsh communities is a balance between neutral processes and environmental filtering, while assembly in swamps can be seen as neutral.Clustering increased with environmental extremes, indicating environmental filtering. Rare species tended to be less closely related to common species. They have unique habitat requirements, and the high diversity is maintained by temporal turnover of species with similar traits filtered by the environment.
Comparison of conventional cytomorphology, flow cytometry immunophenotyping, and automated cell counting of CSF for detection of CNS involvement in acute lymphoblastic leukemia
Authors: Jaime‐Pérez J. C. , Borrego‐López M. F. , Jiménez‐Castillo R. A. , Méndez‐Ramírez N. , Salazar‐Riojas R. , Fernández L. T. , Gómez‐Almaguer D. 
Published: November 2017

AbstractBackground and objectiveCytospin conventional cytomorphology (CCC) is the standard method for detecting lymphoblasts in cerebrospinal fluid (CSF) of patients with acute lymphoblastic leukemia (ALL) and for guiding treatment decisions. We evaluated flow cytometry immunophenotyping (FCI) performance for improving detection of central nervous system (CNS) involvement in ALL.MethodsThis prospective study included analysis of consecutive CSF samples of patients of all ages with ALL at 3 clinical stages: new diagnosis, relapse suspicion, and after relapse treatment. Manual, cytospin, automated, and FCI methods were compared and their performance statistically assessed. Using FCI as the reference method, optimal CSF cutoff cell count that better correlated with presence of lymphoblasts was established by receiver operating characteristic (ROC) curve analysis.ResultsSeventy‐seven CSF samples were investigated, 35 (45.4%) from newly diagnosed cases, 30 (39%) suspicion of relapse, and 12 (15.6%) after treatment for relapse. Median manual WBC count in patients with CNS involvement detected by FCI was 3.75 cells/μL (0.0‐1280), and this was also the count that best correlated with CNS infiltration (sensitivity, 50.0%; specificity, 82.2%). Compared with FCI, CCC sensitivity and specificity were 28.6% and 100%. Automated CSF WBC count in patients with CNS involvement detected by FCI was 5 (0.0‐1578). For automated count, optimal WBC cutoff was 4.5 cells/μL (sensitivity, 62.5%; specificity, 70.5%).ConclusionFlow cytometry immunophenotyping complements conventional cytospin analysis for detection of lymphoblasts in the CSF of ALL patients at any clinical stage.
Bleeding score in Type 1 von Willebrand disease patients using the ISTH‐BAT questionnaire
Authors: Pathare A. , Al Omrani S. , Al Hajri F. , Al Obaidani N. , Al Balushi B. , Al Falahi K. 
Published: November 2017

AbstractBackgroundBleeding assessment tools have evolved in the last decade to standardize the assessment of the severity of bleeding symptom in a consistent way. In 2010, the International Society on Thrombosis and Hemostasis‐Bleeding Assessment Tool (ISTH‐BAT) was developed and validated.AimsOur aim was to administer ISTH‐BAT questionnaire to the Omani patients with type 1 VWD and obtain the bleeding score (BS). We also studied the severity of their bleeding symptoms and correlated it with the BS as well as with the laboratory parameters.MethodsForty‐eight type I VWD index cases and 52 normal subjects were interviewed and the ISTH‐BAT questionnaire administered. The BS was calculated based on a history of bleeding symptoms from 12 different sites according to the standard ISTH‐BAT questionnaire. Laboratory parameters were obtained from patient's medical records.ResultsThe mean age of this cohort was 27 years (range, 6‐49) with 60% being females. The median time to administer this questionnaire was 10 minutes with an interquartile range (IQR) from 8 to 17 minutes. Overall, the median BS was 7 (IQR; 2,11) although individual scores ranged between 0 and 36. The BS was negatively correlated with VWF: Ag, VWF: RCo, and VWF: CB and the Spearman's correlation coefficient “rho” was, respectively, ‐0.15, ‐0.08, and ‐0.22.ConclusionThe ISTH‐BAT BS is designed to reflect the severity of bleeding. Our results demonstrate the inherent variability of this bleeding pattern. We also found that the ISTH‐BAT BS significantly correlated with VWF: Ag and VWF: CB.
A leg ulcer with pulsating varicose veins – from the legs to the heart
Authors: Swoboda Sabrina Johanna , Schumann Hauke , Kiritsi Dimitra 
Published: November 2017

ABSTRACTWe present the case of a 58‐year‐old man who presented to our wound care centre with a 7 ‐month history of a progressive unilateral leg ulcer. He reported intermittent, heavy bleeding at the ulcer. The duplex sonography showed a bilateral incompetence of the saphenofemoral junctions and the entire course of the great saphenous veins and, surprisingly, also a bilateral reversed arterial‐like pulsating flow in the great and small saphenous veins as well as in the deep veins of the lower extremities. During cardiac examination by auscultation and echocardiography, we detected a previously undescribed severe tricuspid insufficiency with strongly reduced systolic ejection of both the left and the right ventricle and repercussions on the peripheral venous system as a cause for the pulsating veins. This case report highlights that, although infrequent, the presence of pulsating varicose veins points to the presence of cardiac abnormalities, especially severe tricuspid insufficiency, and should direct clinicians to initiate a thorough cardiological examination. Our case highlights that consequent compression therapy can be sufficient for wound healing in such cases and should be initially considered. These patients are at risk of severe bleeding, and therefore, interventions should be carefully planned.
Factors predicting Registered Nurses’ intentions to leave their organization and profession: A job demands‐resources framework
Authors: Moloney Willoughby , Boxall Peter , Parsons Matthew , Cheung Gordon 
Published: November 2017

AbstractAimsTo develop a comprehensive model of nursing turnover intention by examining the effects of job demands, job resources, personal demands and personal resources on burnout and work engagement and subsequently on the intention to leave the organization and profession.BackgroundThe ageing population and a growing prevalence of multimorbidity are placing increasing strain on an ageing nursing workforce. Solutions that address the anticipated nursing shortage should focus on reducing burnout and enhancing the engagement of Registered Nurses (RNs) to improve retention.DesignA cross‐sectional survey design.MethodData were collected in 2014–2015 via an e‐survey from 2,876 RNs working in New Zealand. Data were analysed with structural equation modelling.ResultsHigher engagement results in lower intention to leave the organization and profession. Burnout has significant effects on intentions to leave through lower engagement. While most of the demands and resources’ variables (except professional development) have effects on intentions to leave, greater workload and greater work‐life interference result in higher burnout and are the strongest predictors of intentions to leave. Greater emotional demands (challenges) and greater self‐efficacy also have strong effects in lowering intentions to leave through higher engagement.ConclusionsEmployee burnout and work engagement play an important role in transmitting the impacts of job demands, job resources, personal demands and personal resources into RN intention to leave the organization and profession. Work‐life interference and high workloads are major threats to nursing retention while challenge demands and higher levels of self‐efficacy support better retention.
The Female Self‐Advocacy in Cancer Survivorship Scale: A validation study
Authors: Hagan Teresa L. , Cohen Susan M. , Rosenzweig Margaret Q. , Zorn Kristin , Stone Clement A. , Donovan Heidi S. 
Published: November 2017

AbstractAimTo develop and psychometrically test the validity of the Female Self‐Advocacy in Cancer Survivorship Scale.BackgroundFemale cancer survivors need to self‐advocate to overcome challenges associated with cancer yet no valid measure of self‐advocacy exists.DesignInstrument development. Mixed‐mode cross‐sectional survey design.ParticipantsWe recruited adult females (18+ years; N = 317) with a history of invasive cancer from local and national tumour registries and advocacy organizations to complete online or paper questionnaires.MethodsBetween July 2014 ‐ March 2015 to evaluate the construct validity based on evidence of the scale's: (1) internal structure consistent with the underlying model of self‐advocacy; (2) sensitivity to differences between groups known to differ in self‐advocacy skills; (3) relationships between self‐advocacy and key potential predictors (openness and conscientiousness; information engagement; social support) and outcomes (symptom burden and healthcare utilization); (4) relationships between self‐advocacy and related concepts (patient activation; self‐advocacy within another patient population); and (5) relationships between self‐advocacy and criterion measures. Analyses included an exploratory factor analysis, t tests, and bivariate correlations using validated, reliable measures for constructs.ResultsEvidence from all five hypotheses supported the construct validity of the Female Self‐Advocacy in Cancer Survivorship Scale. The factor analysis confirmed the three underlying dimensions of self‐advocacy resulting in a 20‐item measure with strong internal consistency that explained almost half of response variance.ConclusionThe Female Self‐Advocacy in Cancer Survivorship Scale is a valid, reliable measure of how well adult female cancer survivors can get their needs met in the face of adversity.