bluuebunny/crossref_metadata_2025_split huggingface dataset:
State of the art – how I manage immune thrombocytopenia
Authors: Cooper Nichola 
Published: March 2017

SummaryThe management of patients with immune thrombocytopenia (ITP) is rapidly evolving. Over the last 15 years, a number of novel treatments have improved practice, with many steroid‐sparing agents and a reduction in the progression to splenectomy. Although this has improved clinical care, many therapeutic challenges remain. There is no diagnostic test, no biomarkers to direct treatment and few comparative studies to help management decisions. Development of up to date guidelines is difficult with little high‐grade evidence. First line treatment continues to be steroids and intravenous immunoglobulins (IVIG) although both are often poorly tolerated and not curative. Common second line treatments include rituximab, immunosuppressive agents, such as azathioprine and mycophenolate mofetil, and the thrombopoietin receptor agonists romiplostim and eltrombopag. There are no comparative studies to decide between these agents and treatment is generally individualized, depending on comorbidity. Use of splenectomy has declined and is generally reserved for patients with chronic disease, although the exact position of splenectomy is subject to debate. Further understanding of the cause of disease in individual patients may help guide treatment. Randomized controlled studies of common treatments and novel treatments for refractory patients are urgently needed.
Home and the social worlds beyond: exploring influences in the lives of children of mothers with intellectual disability
Authors: Collings S. , Llewellyn G. , Grace R. 
Published: March 2017

AbstractBackgroundMothers with intellectual disability are likely to raise their children in socially disadvantaged circumstances, and many face social isolation; however, the impact of a potentially restricted home context on children's social worlds has not been examined. This study was conducted to explore influences in the social worlds of children of mothers with intellectual disability from a child's perspective.MethodsSeven children aged 7 to 11 years took part in at least two semi‐structured interviews over a year. Narrative accounts of each child's social interactions were analysed to ascertain if a pattern was present across the group.ResultsHome was found to influence the children's social interactions elsewhere by providing (or not) predictable routines and rules, and support from a significant adult other than a mother. Home environments were found to influence other social worlds by establishing a foundation for children's expectations about social interactions.ConclusionsThe social worlds of school‐aged children of mothers with intellectual disability are shaped by influences in the home that cannot be attributed exclusively to having a parent with intellectual disability. Significant adults provide an important support role and can be fulfilled by social service workers when a family‐centred approach is applied.
The polymorphic CAG repeat in exon 1 of androgen receptor is associated with level of HDL cholesterol and hypertension in Chinese middle‐aged and elderly men
Authors: Yang Deyu , Tian Jinhai , Zhang Xu , Yu Jingjing , Li Shuya , Wang Zhizhong , Ma Yuying , Liu Lan , Huang Qi , Ma Rong , Wang Jia , Li Xin , Jiang Min 
Published: March 2017

SummaryObjectiveThe length of the CAG repeats in exon 1 of the androgen receptor (AR) gene has been shown to be inversely correlated with AR transcriptional activity. This study aimed to investigate the correlations between the length of CAG repeat in AR and serum lipids and hypertension in Chinese men.Design and patientsThe relationship between length of the CAG repeat in exon 1 of AR with prevalence of hypertension and the levels of serum lipids among Chinese men (aged ≥40 years).MeasurementsThe physical condition of the subjects was examined and recorded. The concentrations of blood lipids and sex hormones were measured, and the CAG repeat lengths of the AR gene were determined.ResultsThe length of the AR CAG repeats was associated with HDL cholesterol (HDL‐C) concentration, and the stepwise multiple regression model showed that this association was independent of body mass index (BMI), triglycerides (TG) and total cholesterol (TC), although these factors influence HDL‐C concentration. Furthermore, men with <22 vs men with ≥22 CAG repeats showed higher blood pressure and higher prevalence of hypertension. Shorter CAG repeat numbers were associated with the increased risk of hypertension in a multivariate logistic regression analysis (odds ratio = 0·715; 95% confidence interval, 0·517–0·989; P = 0·043). No significant correlation of AR CAG repeat polymorphism with sex hormone levels, TG, LDL cholesterol (LDL‐C) or TC was found.ConclusionsThis study provides evidence that men carrying shorter (<22) AR CAG repeats have lower HDL‐C level and increased risk of hypertension. The androgenic activity may differ due to the polymorphic length of CAG repeats of the AR gene.
Inpatient treatment of patients with acute idiopathic peripheral facial palsy: A population‐based healthcare research study
Authors: Plumbaum K. , Volk G.F. , Boeger D. , Buentzel J. , Esser D. , Steinbrecher A. , Hoffmann K. , Jecker P. , Mueller A. , Radtke G. , Witte O.W. , Guntinas‐Lichius O. 
Published: March 2017

AbstractObjectivesTo determine the inpatient management for patients with acute idiopathic facial palsy (IFP) in Thuringia, Germany.DesignPopulation‐based study.SettingAll inpatients with IFP in all hospitals with departments of otolaryngology and neurology in 2012, in the German federal state, Thuringia.Main outcome measuresPatients’ characteristics and treatment were compared between departments, and the probability of recovery was tested.ResultsA total of 291 patients were mainly treated in departments of otolaryngology (55%) and neurology (36%). Corticosteroid treatment was the predominant therapy (84.5%). The probability to receive a facial nerve grading (odds ratio [OR=12.939; 95% confidence interval [CI]=3.599 to 46.516), gustatory testing (OR=6.878; CI=1.064 to 44.474) and audiometry (OR=32.505; CI=1.485 to 711.257) was significantly higher in otolaryngology departments, but lower for cranial CT (OR=0.192; CI=0.061 to 0.602), cerebrospinal fluid examination (OR=0.024; CI=0.006 to 0.102). A total of 131 patients (45%) showed a recovery to House‐Brackmann grade≤II. A pathological stapedial reflex test (Hazard ratio [HR]=0.416; CI=0.180 to 0.959) was the only independent diagnostic predictor of worse outcome. Prednisolone dose >500 mg (HR=0.579; CI 0.400 to 0.838) and no adjuvant physiotherapy (HR=0.568; CI=0.407 to 0.794) were treatment‐related predictors of worse outcome.ConclusionsInpatient treatment of IFP seems to be highly variable in daily practice, partly depending on the treating discipline and despite the availability of evidence‐based guidelines. The population‐based recovery rate was worse than reported in clinical trials.
Residual pyruvate kinase activity in PKLR‐deficient erythroid precursors of a patient suffering from severe haemolytic anaemia
Authors: Klei Thomas R.L. , Kheradmand Kia Sima , Veldthuis Martijn , Beuger Boukje M. , Geissler Judy , Dehbozorgian Javad , Karimi Mehran , van Bruggen Robin , van Zwieten Rob 
Published: March 2017

AbstractObjectiveHere, we present a 7‐year‐old patient suffering from severe haemolytic anaemia. The most common cause of chronic hereditary non‐spherocytic haemolytic anaemia is red blood cell pyruvate kinase (PK‐R) deficiency. Because red blood cells rely solely on glycolysis to generate ATP, PK‐R deficiency can severely impact energy supply and cause reduction in red blood cell lifespan. We determined the underlying cause of the anaemia and investigated how erythroid precursors in the patient survive.MethodsPK activity assays, Western blot and Sanger sequencing were employed to determine the underlying cause of the anaemia. Patient erythroblasts were cultured and reticulocytes were isolated to determine PK‐R and PKM2 contribution to glycolytic activity during erythrocyte development.ResultsWe found a novel homozygous mutation (c.583G>A) in the PK‐R coding gene (PKLR). Although this mutation did not influence PKLR mRNA production, no PK‐R protein could be detected in the red blood cells nor in its precursors. In spite of the absence of PK‐R, the reticulocytes of the patient exhibited 20% PK activity compared with control. Western blotting revealed that patient erythroid precursors, like controls, express residual PKM2.ConclusionsWe conclude that PKM2 rescues glycolysis in PK‐R‐deficient erythroid precursors.
Pathophysiological aspects of red blood cells in end‐stage renal disease patients resistant to recombinant human erythropoietin therapy
Authors: Georgatzakou Hara T. , Tzounakas Vassilis L. , Kriebardis Anastasios G. , Velentzas Athanassios D. , Papageorgiou Effie G. , Voulgaridou Artemis I. , Kokkalis Apostolos C. , Antonelou Marianna H. , Papassideri Issidora S. 
Published: March 2017

AbstractObjectiveModified, bioreactive red blood cells (RBCs) and RBC‐derived microvesicles (MVs) likely contribute to the hematological and cardiovascular complications in end‐stage renal disease (ESRD). This study assesses the physiological profile of RBCs in patients with ESRD receiving standard or high doses of recombinant human erythropoietin (rhEPO).MethodBlood samples from twenty‐eight patients under sustained hemodialysis, responsive, or not to standard rhEPO administration were examined for RBC morphology, fragility, hemolysis, redox status, removal signaling, membrane protein composition, and microvesiculation before and after dialysis. Acute effects of uremic plasma on RBC features were examined in vitro through reconstitution experiments.ResultsOverall, the ESRD RBCs were characterized by pathological levels of shape distortions, surface removal signaling, and membrane exovesiculation, but reduced fragility compared to healthy RBCs. Irreversible transformation of RBCs was found to be a function of baseline Hb concentration. The more toxic uremic context in non‐responsive patients compared to rhEPO responders was blunted in part by the antioxidant, antihemolytic, and anti‐apoptotic effects of high rhEPO doses, and probably, of serum uric acid. A selective lower expression of RBC membrane in complement regulators (CD59, clusterin) and of CD47 “marker‐of‐self” was detected in non‐responders and responders, respectively. Evidence for different short‐term dialysis effects and probably for a different erythrocyte vesiculation mechanism in rhEPO responsive compared to non‐responsive patients was also revealed.ConclusionDeregulation of RBC homeostasis might involve diverse molecular pathways driving erythrocyte signaling and removal in rhEPO non‐responders compared to responsive patients.
Habitat filtering not dispersal limitation shapes oceanic island floras: species assembly of the Galápagos archipelago
Authors: Carvajal‐Endara Sofía , Hendry Andrew P. , Emery Nancy C. , Davies T. Jonathan 
Published: March 2017

AbstractRemote locations, such as oceanic islands, typically harbour relatively few species, some of which go on to generate endemic radiations. Species colonising these locations tend to be a non‐random subset from source communities, which is thought to reflect dispersal limitation. However, non‐random colonisation could also result from habitat filtering, whereby only a few continental species can become established. We evaluate the imprints of these processes on the Galápagos flora by analysing a comprehensive regional phylogeny for ~ 39 000 species alongside information on dispersal strategies and climatic suitability. We found that habitat filtering was more important than dispersal limitation in determining species composition. This finding may help explain why adaptive radiation is common on oceanic archipelagoes – because colonising species can be relatively poor dispersers with specific niche requirements. We suggest that the standard assumption that plant communities in remote locations are primarily shaped by dispersal limitation deserves reconsideration.
Location of New Firms: Influence of Commuting Behaviour
Authors: Backman Mikaela , Karlsson Charlie 
Published: March 2017

AbstractIn the entrepreneurship literature, it is generally assumed that an individual establishes a new firm in a location in which they have strong ties, normally in the municipality of residence or employment. We scrutinise this general assumption and show that firm location depends on individual characteristics, such as the commuting experience. Our results show that commuting influences the firm location choice. The probability of establishing a firm in the work municipality increases if the entrepreneur is a commuter, holding constant the type of region and unobservable and observable individual features.
Ageing and consumption in Finland: The effect of age and life course stage on ecological, economical and self‐indulgent consumption among late middle‐agers and young adults between 1999 and 2014
Authors: Kuoppamäki Sanna‐Mari , Wilska Terhi‐Anna , Taipale Sakari 
Published: March 2017

AbstractPrevious studies on ageing consumers have mainly focused on chronological age and generational values or studied ageing and consumption with cross‐sectional data. Few quantitative studies exist that examine the effect of age together with life course on consumption using longitudinal data. To bridge this gap, the article examines ageing and attitudes towards consumption in Finland, focusing particularly on late middle‐agers (46–60 year‐olds) in comparison with young adults (18–30 year‐olds) between 1999 and 2014. The article explores three consumption patterns based on attitudinal statements: ecological, economical and self‐indulgent consumption. Through analysis of a nationally representative survey study in Finland (N = 8,543), the article reveals that in all years under examination, late middle‐agers reported more ecological attitudes towards consumption than young adults. In 1999 and 2004, the attitudes of late middle‐agers appeared more economical, but age‐related differences in economical attitudes disappeared between 2009 and 2014. In each year, late middle‐agers reported less self‐indulgent attitudes than young adults, and these age‐related differences did not remarkably change between 1999 and 2014. The results indicate that in 1999 and 2004, ecological and economical attitudes towards consumption were best predicted by age at the year of the study. In later years, ecological attitudes were more closely determined by life course stage, that is household type and other socio‐demographic determinants. Regarding economical attitudes, generational or cohort effects were pronounced among late middle‐agers in 1999. In contrast, the significance of age remained throughout the years for self‐indulgent attitudes, indicating the absence of generational or cohort effects.
The emotional toll of out‐migration on mothers and fathers left behind in Mexico
Authors: Fuller Heather R. 
Published: March 2017

AbstractA growing body of research examines how families are affected by international migration; yet, implications for family members who remain behind are less well understood. Recent studies highlight implications for wives and young children when Mexican men migrate to the US, but have not yet adequately addressed the unique perspectives across the intergenerational family system, including the impact on parents when their adult children migrate. The current qualitative study explores the perceptions of transnational family ties among five focus groups (N=28) consisting of mothers and fathers of migrants in Mexico. An inductive, comparative method was used to identify emerging themes related to the emotional toll that parents of migrants experience. Salient themes included sadness, longing, guilt, and worry. The perceptions and expression of these emotions varied between mothers and fathers. Findings highlight the unique impact and distinct policy implications of Mexican familial transnationalism on parents who remain behind.
Expression of the limited‐transpiration trait under high vapour pressure deficit in peanut populations: Runner and virginia types
Authors: Shekoofa A. , Sinclair T. R. , Aninbon C. , Holbrook C. C. , Isleib T. G. , Ozias‐Akins P. , Chu Y. 
Published: March 2017

AbstractDrought can be a critical limitation on peanut yield. A physiological trait that may help to ameliorate drought is limited transpiration (TRlim), defined as a limitation on further increases in transpiration rate (TR) under high vapour pressure deficit (VPD) conditions. The advantage of the TRlim trait is that it allows plant water conservation to increase soil water availability for use during late‐season drought. While this trait has been identified in peanut, there is no information of how readily the trait may be transfer to progeny lines. The objective of this study was to obtain preliminary information on the expression of the TRlim trait in two peanut progeny populations. One population was runner type of 88 RILs derived from the mating of Tifrunner × NC 3033. The second set was selected for the virginia‐type phenotype of large pods obtained from mating of PI 585005 (ICGV 86015) and N0808olJCT, both of which expressed the TRlim trait. A two‐tier screen was applied to both populations. The initial screen was based on exposure of de‐rooted shoots to silver ions. Fifteen runner type and 12 virginia type were selected for direct measures of transpiration response to varying VPD. The results from each of the two populations showed that an effective expression of the TRlim trait occurred in about 30% of the progeny in each population. While these results do not offer a definitive index of inheritance, they do indicate that there appears to be a strong possibility of transferring the TRlim trait to progeny genotypes.
Genetic parameters for fur quality graded on live animals and dried pelts of American mink (Neovison vison)
Authors: Thirstrup J.P. , Jensen J. , Lund M.S. 
Published: March 2017

SummaryFur quality and skin size are integral qualities in the mink industry and are main determinants of sales price and subsequent income for mink fur producers. Parental animals of future generations are selected based on quality grading from live animals, but selection response is obtained from dried skins sold after pelting. In this study, we evaluated traits assessed during live grading and pelt traits examined on dried skins to determine correlation between live and pelt traits. Grading traits and body weight were measured during live animal grading for 9,539 Brown American mink, and pelt quality traits and skin size were evaluated on 8,385 dried mink skins after pelting. Data were sampled from 2 yearly production cycles. Genetic parameters were estimated using the REML method implemented in the DMU package. Heritabilities and proportions of litter variance were calculated from estimated variance components for all traits, and genetic and phenotypic correlations between all traits were estimated in a series of bivariate analyses. Heritability estimates for live grading traits ranged from 0.06 to 0.28, heritability estimates for pelt quality traits ranged from 0.20 to 0.30, and finally heritability estimates for body size traits ranged from 0.43 to 0.48. Skin size and body weight were regarded as different traits for the two sexes and were therefore analysed for each sex separately. Genetic correlations between grading traits exhibited a range of 0.30–0.99 and genetic correlations between pelt quality traits ranged from 0.38 to 0.86. Genetic correlations between quality, wool density and silky appearance evaluated during live animal grading and on dried skin after pelting were 0.74, 0.41 and 0.33, respectively. Skin size and body weight were negatively correlated with pelt quality traits and ranged from −0.55 to −0.25. Using standard selection index theory and combined information from both live grading and skin evaluation increase of reliability of selection ranged from 0.6% to 14%. Due to moderate genetic correlations between traits evaluated during live grading and on dried skins, and negative correlations between pelt quality traits and body size, we concluded that traits should be selected simultaneously.
Chronic urticaria in the real‐life clinical practice setting in Sweden, Norway and Denmark: baseline results from the non‐interventional multicentre AWARE study
Authors: Thomsen S.F. , Pritzier E.C. , Anderson C.D. , Vaugelade‐Baust N. , Dodge R. , Dahlborn A.‐K. , Vestergaard C. 
Published: March 2017

AbstractBackgroundChronic urticaria (CU) is characterized by the recurrence of itchy hives and/or angioedema for more than 6 weeks. AWARE (A World‐wide Antihistamine‐Refractory Chronic Urticaria Patient Evaluation) is a multinational study designed to document the real‐life treatment situation, burden of disease and clinical resource usage of H1‐antihistamine‐refractory CU patients.ObjectiveTo examine baseline data from Scandinavian AWARE patients.MethodsAWARE is a prospective, non‐interventional, multinational, umbrella design study, which includes adults (≥18 years) with a confirmed CU diagnosis (>2 months) that is refractory to H1‐antihistamines. Baseline patient characteristics, disease activity (urticaria control test [UCT]), pharmacological treatment, comorbidities and healthcare usage were documented by the treating physician. Quality of life (QoL; dermatology life quality index [DLQI]; chronic urticaria quality of life questionnaire [CU‐Q2oL; Danish patients only]) and work productivity and activity impairment (WPAI) scores were also assessed.ResultsOverall, 158 CU patients from seven centres in Denmark (n = 80), Norway (n = 50) and Sweden (n = 28) were included in this baseline analysis. Mean age and BMI were 40.3 years and 26.5 kg/m2, respectively. The majority of patients were female (69.6%), had uncontrolled CU (75.6%; UCT score <12) and had a ‘spontaneous’ component to their CU (61.4% CSU; 20.3% both CSU and chronic inducible urticaria). Common comorbidities included asthma (19.6%), allergic rhinitis (16.5%) and food allergies (8.2%). Overall, 60.1% of patients reported using treatments for CU including non‐sedative H1‐antihistamines (40.5%), corticosteroids (19%), montelukast (14.6%) and omalizumab (8.2%). Pharmacological treatment rates increased to 96.2% during the baseline visit. On average, patient QoL was moderately affected (mean DLQI score 7.7) and healthcare resource usage was high.ConclusionAdult Scandinavian H1‐antihistamine‐refractory CU patients reported high rates of healthcare usage and QoL impairment. Rates of pharmacological treatment use were low before study enrolment but increased to almost 100% during the baseline visit.