bluuebunny/crossref_metadata_2025_split huggingface dataset:
Efficacy of romiplostim in treatment of thrombocytopenia in children with Wiskott–Aldrich syndrome
Authors: Khoreva Anna , Abramova Irina , Deripapa Elena , Rodina Yulia , Roppelt Anna , Pershin Dmitry , Larin Sergey , Voronin Kirill , Maschan Alexey , Novichkova Galina , Shcherbina Anna 
Published: November 2020

SummaryWiskott–Aldrich syndrome (WAS) is a life‐threatening primary immunodeficiency associated with bleeding of variable severity due to thrombocytopenia. Correction of the thrombocytopenia is of paramount importance for most WAS patients. We report a retrospective analysis of the safety and efficacy of romiplostim treatment in reducing thrombocytopenia and bleeding tendency in 67 children (median age 1·3 years) with genetically confirmed WAS, followed in eight months (range, 1–12 months). Complete or partial primary responses regarding platelet counts were observed in 22 (33%) and 18 (27%) subjects, respectively. Yet, even in the non‐responder group, the risk of haemorrhagic events decreased significantly, to 21%, after the first month of treatment. The responses tended to be durable and stable over time, with no significant fluctuations in platelets counts. The results of this retrospective study of a large cohort of WAS patients demonstrates that romiplostim can be used to increase platelet counts and reduce the risks of life‐threatening bleeding in WAS patients awaiting haematopoietic stem cell transplantation or forgoing the procedure for various reasons.
Basophil activation test for Staphylococcus aureus enterotoxins in severe asthmatic patients
Authors: Flora Martina , Perna Francesco , Abbadessa Salvatore , Garziano Federica , Maffucci Rosalba , Maniscalco Mauro , Mollica Mariano , Pelaia Corrado , Tremante Eugenio , Maffei Marianna , Calabrese Cecilia 
Published: November 2020

AbstractBackgroundSeveral studies have shown an association between severe asthma and serum immunoglobulins E (IgE) against Staphylococcus aureus enterotoxins (SEs). SEs—the prototypes being types A (SEA), B (SEB) and toxic shock syndrome toxin 1 (TSST‐1)—can induce both polyclonal and specific IgE responses.ObjectiveThe aim of the study was to evaluate the ability of SEs to induce basophil activation in severe asthmatic patients using the basophil activation test (BAT).Methods57 severe asthmatic patients were enrolled. BAT in response to SEA, SEB and TSST‐1 was performed in all patients, while serum IgE to SEA, SEB and SEC was available in 49 patients. BAT was considered positive when CD203c+ basophils to SEs were ≥5%, and the stimulation index (SI, ratio between % of CD203c+ basophils to SEs and to negative control) was >2. Two threshold values (>0.1 kU/L and >0.35 kU/L, respectively) were used to assess serum SEsIgE.Results36.8% of severe asthmatic patients had a BAT positive for at least one SE (BAT SEs+). Serum SEsIgE >0.35 kU/L (SEs IgE+) was associated with BAT SEs positivity. Among patients with negative skin prick test, 35% were BAT SEs+, 30% SEs IgE+, 55% BAT or IgE− SEs+. A negative correlation between SI of BAT to SEs and both clinical (ACT score) and functional parameters was observed, together with a positive correlation of BAT with asthma exacerbations.ConclusionsThe positivity of BAT for SEs in a subgroup of severe asthmatic patients further supports the pathogenic role of Staphylococcus aureus in severe asthma.
An X‐linked syndrome with severe neurodevelopmental delay, hydrocephalus, and early lethality caused by a missense variation in the OTUD5 gene
Authors: Tripolszki Kornelia , Sasaki Erina , Hotakainen Ronja , Kassim Abdul Halim , Pereira Catarina , Rolfs Arndt , Bauer Peter , Reardon William , Bertoli‐Avella Aida M. 
Published: November 2020

AbstractWe describe an X‐linked syndrome in 13 male patients from a single family with three generations affected. Patients presented prenatally or during the neonatal period with intrauterine growth retardation, ventriculomegaly, hydrocephalus, hypotonia, congenital heart defects, hypospadias, and severe neurodevelopmental delay. The disease is typically fatal during infancy, mainly due to sepsis (pneumonias). Female carriers are asymptomatic. We performed genome sequencing in four individuals and identified a unique candidate variant in the OTUD5 gene (NM_017602.3:c.598G > A, p.Glu200Lys). The variant cosegregated with the disease in 10 tested individuals. OTUD5 was considered as a candidate gene based on two previous missense variants detected in patients with intellectual disability. In conclusion, we define a syndrome associated with OTUD5 defects and add compelling evidence of genotype–phenotype association. This finding ended the long diagnostic odyssey of this family.
Early technical pancreas failure in Simultaneous Pancreas–Kidney Recipients does not impact renal allograft outcomes
Authors: Das Devika M. , Huskey Janna L. , Harbell Jack W. , Heilman Raymond L. , Singer Andrew L. , Mathur Amit , Neville Matthew R. , Morgan Paige , Reddy Kunam S. , Jadlowiec Caroline C. 
Published: November 2020

AbstractEarly pancreas loss in simultaneous pancreas–kidney (SPK) transplants has been associated with longer perioperative recovery and reduced kidney allograft function. We assessed the impact of early pancreas allograft failure on transplant outcomes in a contemporary cohort of SPK patients (n = 218). Early pancreas allograft loss occurred in 12.8% (n = 28) of recipients. Delayed graft function (DGF) was more common (21.4% vs. 7.4%, p = 0.03) in the early pancreas loss group, but there were no differences in hospital length of stay (median 6.5 vs. 7.0, p = 0.22), surgical wound complications (p = 0.12), or rejection episodes occurring in the first year (p = 0.87). Despite differences in DGF, both groups had excellent renal function at 1 year post‐transplant (eGFR 64.1 ± 20.8 vs. 65.8 ± 22.9, p = 0.75). There were no differences in patient (HR 0.58, 95% CI 0.18–1.87, p = 0.26) or kidney allograft survival (HR 0.84, 95% CI 0.23–3.06, p = 0.77). One‐ and 2‐year protocol kidney biopsies were comparable between the groups and showed minimal chronic changes; the early pancreas loss group showed more cv changes at 2 years (p = 0.04). Current data demonstrate good outcomes and excellent kidney allograft function following early pancreas loss.
Youth and non‐European ethnicity are associated with increased loss of publicly funded insulin pump access in New Zealand people with type 1 diabetes
Authors: Hennessy Lorelei D. , De Lange Michel , Wiltshire Esko J. , Jefferies Craig , Wheeler Benjamin J. 
Published: November 2020

AbstractAimsContinuous subcutaneous insulin infusion (CSII) has been publicly funded in New Zealand for people living with type 1 diabetes since 2012. The aim of the current study was to investigate the loss of access, once obtained, to public‐funded CSII. The frequency and socio‐demographics of access, and loss, to CSII spanning the period 2012 to 2018 were examined.MethodsNationally held data collections including the New Zealand Virtual Diabetes Register were used to calculate the overall and subgroup proportions using and ceasing CSII. A logistic regression model was used to estimate odds ratios for pump use for the predictor variables (sex, age group, ethnicity and deprivation index) and to calculate odds ratios for pump cessation for the same demographic factors.ResultsOnce CSII access is obtained, approximately 4% per year cease CSII in a subsequent year. This cessation of publicly funded CSII was not distributed equally among the population, showing over‐representation in youth (aged 10–29 years) and non‐Europeans, in particular Māori and Pasifika. Compounding this, it remains less likely for people with diabetes to initially access publicly funded CSII in New Zealand if they are non‐European and more socio‐economically deprived.ConclusionsIn New Zealand, Māori and Pasifika, as well as youth, are over‐represented in the cessation of CSII in comparison with Europeans and all other age groups. These groups are also less likely to gain initial access to public funding. Efforts to understand and reduce these disparities are needed, including review of current public funding access criteria.
Diagnostic utility of mitral annular displacement by speckle tracking echocardiography in predicting significant coronary artery disease in suspected chronic stable angina pectoris
Authors: Sharma Jai Bharat , Deora Surender , Choudhary Rahul , Kaushik Atul 
Published: November 2020

AbstractBackgroundMore than 50% of patients currently referred for coronary angiography (CAG) with suspected chronic stable angina pectoris (CSAP) shows normal or nonobstructive CAD. Mitral annular displacement (MAD) is a function of global shortening deformation of left ventricle (LV) and mirrors sub‐endocardial ischemia in CAD. The aim of this study was to ascertain if MAD can predict the presence of CAD in patient presenting with suspected CSAP.MethodsThis cross‐sectional study was conducted on patients with suspected CSAP (n = 140) and preserved LV function without apparent wall motion abnormalities during the study period. We excluded patients with prior heart disease (valvular, ischemic), presenting with acute coronary syndrome, arrhythmia, heart failure, and poor imaging. All the patients were subjected to speckle tracking echocardiography (STE) and CAG. Average MAD and normalized MAD were calculated by automated function imaging (AFI), and receiver operating characteristic (ROC) curve was plotted for presence of significant CAD considering CAG as gold standard.ResultsThe results showed that both average MAD (4 segment) and normalized MAD were significantly reduced (P = <.001) in patients with significant CAD (n = 81). In ROC analysis, area under curve (AUC) for predicting significant CAD in patients of CSAP was better for normalized MAD (0.88) compared to average MAD (4 segment, 0.85).The optimal cutoff of normalized MAD and average MAD for detection of significant CAD were ≤15.3% (sensitivity 90.1%, specificity 79.7%) and ≤11.19 (sensitivity 84%, specificity 69.5%), respectively. Normalized MAD showed week inverse correlation with SYNTAX score (−0.24, R2 = 0.058) and Hs CRP (−0.23, R2 = 0.057).ConclusionMAD is a simple and rapid noninvasive diagnostic tool which accurately predicted the presence of significant CAD in patients with CSAP.
Frailty assessment, hip fracture and long‐term clinical outcomes in older adults
Authors: Pizzonia Monica , Giannotti Chiara , Carmisciano Luca , Signori Alessio , Rosa Gianmarco , Santolini Federico , Caffa Irene , Montecucco Fabrizio , Nencioni Alessio , Monacelli Fiammetta 
Published: November 2020

AbstractBackgroundThe primary aim of the study was determining the validation of the modified 19‐item Frailty Index (mFI‐19), based on the standard procedure for creating a frailty index scoring in the accumulation deficit theory of Rockwood and comparing it with the gold standard comprehensive geriatric assessment (CGA) in old age patients with hip fracture. As a secondary aim, we compared prognostic accuracies of mFI‐19 and CGA in predicting long‐term mortality after surgery.Materials and MethodsA total of 364 older patients with hip fractures, each a candidate for surgery, were consecutively enrolled. All were subjected to CGA and mFI‐19 at baseline and time to death (years from hip surgery) were collected prospectively.ResultsMean patient age was 86.5 (SD: 5.65) years. The most common clinical phenotype (77%) was frail. Both CGA and mFI‐19 performed similarly in predicting long‐term mortality (Harrell's C‐index: 0.66 and 0.68, respectively).ConclusionsThe mFI‐19 was validated, compared to the gold standard CGA, based on a systematic process for creating a frailty index in relation to the accumulation deficit. This is one of few prospective studies addressing long‐term mortality in older adults with hip fractures, invoking a methodologically robust frailty screening assessment.
C‐Reactive Protein: Marker of risk for post‐traumatic stress disorder and its potential for a mechanistic role in trauma response and recovery
Authors: Friend Samantha F. , Nachnani Rahul , Powell Susan B. , Risbrough Victoria B. 
Published: November 2020

AbstractIncreasing evidence indicates that inflammation plays a role in PTSD and stress disorder pathophysiology. PTSD is consistently associated with higher circulating inflammatory protein levels. Rodent models demonstrate that inflammation promotes enduring avoidance and arousal behaviors after severe stressors (e.g., predator exposure and social defeat), suggesting that inflammation may play a mechanistic role in trauma disorders. C‐reactive protein (CRP) is an innate acute phase reactant produced by the liver after acute infection and chronic disease. A growing number of investigations report associations with PTSD diagnosis and elevated peripheral CRP, CRP gene mutations, and CRP gene expression changes in immune signaling pathways. CRP is reasonably established as a potential marker of PTSD and trauma exposure, but if and how it may play a mechanistic role is unclear. In this review, we discuss the current understanding of immune mechanisms in PTSD with a particular focus on the innate immune signaling factor, CRP. We found that although there is consistent evidence of an association of CRP with PTSD symptoms and risk, there is a paucity of data on how CRP might contribute to CNS inflammation in PTSD, and consequently, PTSD symptoms. We discuss potential mechanisms through which CRP could modulate enduring peripheral and CNS stress responses, along with future areas of investigation probing the role of CRP and other innate immune signaling factors in modulating trauma responses. Overall, we found that CRP likely contributes to central inflammation, but how it does so is an area for further study.
Fish and hyperoxia—From cardiorespiratory and biochemical adjustments to aquaculture and ecophysiology implications
Authors: McArley Tristan J. , Sandblom Erik , Herbert Neill A. 
Published: November 2020

AbstractHyperoxia occurs when water oxygen (O2) levels exceed normal atmospheric pressure (i.e., >100% air saturation). Fish can experience hyperoxia in shallow environments due to photosynthesis or in aquaculture through O2 supplementation. This review provides a comprehensive synthesis of the effects of hyperoxia on fish, spanning influences on cardiorespiratory function, acid‐base balance, oxidative stress and whole animal performance (e.g., thermal tolerance and growth). Fish hypoventilate in hyperoxia, but arterial and venous blood oxygenation increases in spite of reduced convection. Persistently high levels of blood oxygenation in hyperoxia do not commonly result in reduced blood O2 carrying capacity, but assessments in undisturbed fish are required to clarify this. Hypoventilation also causes the retention of carbon dioxide, hence respiratory acidosis. Another consequence of hyperoxia is increased levels of oxidative stress and concomitant changes to antioxidant defence systems. Despite these changes, however, the bulk of evidence shows no effect of hyperoxia on growth. Hyperoxia does impact the aerobic metabolic rate of fish with either no effect or elevated resting metabolic rate and substantial increases in maximum metabolic rate. There is also evidence that hyperoxia increases aerobic capacity improves cardiac performance and mitigates anaerobic stress during acute warming. Along with improved upper thermal tolerance in some species, these findings collectively suggest that hyperoxia might provide fish a metabolic refuge during acute warming. Since hyperoxia occurs in shallow aquatic habitats, further research establishing the ecophysiological implications of concomitant heat stress and hyperoxia is pertinent, particularly with a changing climate.
Quality of different rice cultivars and factors influencing consumer willingness‐to‐purchase rice
Authors: Charoenthaikij Phantipha , Chaovanalikit Arusa , Uan‐On Tanat , Waimaleongora‐ek Pamarin 
Published: November 2020

SummaryPhysicochemical properties and descriptive profiles of four rice samples including white rice (RD31 and KDML105), brown rice (BKDML) and a brown/red rice mix (BR + HMD) were investigated. Consumer acceptance and factors influencing rice consumption and purchase intent were also determined (n = 112). The unpolished rice samples were more flavourful, harder and lower in retrogradation rate than the polished ones. The RD31 with 36.81% apparent amylose content exhibited the lowest peak viscosity (133.5 RVU) with the highest retrogradation rate (59.7%) resulting in the lowest consumer acceptance. The KDML105 obtained the highest acceptance score (6.9) with the softest texture (23.8 N) and the lowest cooking time (18 min). The preference mapping revealed that consumer acceptance was highly associated with the peak viscosity and vice versa for the amylose content. The factor analysis indicated that the accessibility, eating behaviour, rice variety, preference as well as rice quality and health benefits, respectively, were the factor‐driven consumers’ attitudes towards rice consumption and purchase intention.
Characterization of ecdysteroid biosynthesis in the pond wolf spider, Pardosa pseudoannulata
Authors: Yang Z.‐M. , Yu N. , Wang S.‐J. , Korai S. K. , Liu Z.‐W. 
Published: November 2020

AbstractEcdysteroids, as the key growth hormones, regulate moulting, metamorphosis and reproduction in arthropods. Ecdysteroid biosynthesis is catalysed by a series of cytochrome P450 monooxygenases (CYP450s) encoded by Halloween genes, including spook (spo), phantom (phm), disembodied (dib), shadow (sad) and shade (shd). The ecdysteroid biosynthesis in insects is clear with 20‐hydroxyecdysone (20E) as the main ecdysteroid. However, the information on the major ecdysteroids in arachnids is limited. In this study, Halloween genes spo, dib, sad and shd, but not phm, were identified in the pond wolf spider, Pardosa pseudoannulata. Phylogenetic analysis grouped arachnid and insect Halloween gene products into two CYP450 clades, the CYP2 clan (spo and phm) and the mitochondrial clan (dib, sad, and shd). In P. pseudoannulata, the temporal expression profile of the four Halloween genes in concurrence with spiderling moulting with steady increase in the course of the 2nd instar followed by a rapid dropdown once moulting was completed. Spatially, the four Halloween genes were highly expressed in spiderling abdomen and in the ovaries of female adults. In parallel, ponasterone A (PA), but not 20E, was detected by LC–MS/MS analysis in P. pseudoannulata, and it was demonstrated as a functional ecdysteroid in the spider by accelerating of moulting with PA addition. The present study revealed the different ecdysteroid biosynthesis pathways in spiders and insects.
Effect of donor age on adult unrelated donor haemopoietic cell transplant outcome: the Australian experience
Authors: Nivison‐Smith Ian , Bajel Ashish , Dodds Anthony J. , Gottlieb David , Hamad Nada , Kennedy Glen , Kerridge Ian , Ma David D. F. , Milliken Samuel , Moore John , Purtill Duncan , Szer Jeff 
Published: November 2020

AbstractBackgroundResults have been varied regarding the effect of donor age on the outcome of unrelated donor haemopoietic cell transplantation (HCT).AimsTo determine the influence of donor age on adult unrelated donor HCT outcome in Australia.MethodsPatients were included in the study if they were aged 16 years or above and underwent first allogeneic unrelated donor HCT in Australia for the indications of acute lymphoblastic leukaemia (ALL), acute myelogenous leukaemia (AML), chronic myelogenous leukaemia (CML) or myelodysplastic syndromes (MDS) between the years of 2001 and 2014 inclusive. The main outcome measure was overall survival (OS), which was tested against independent variables using univariate Kaplan–Meier methods and multivariate Cox regression.ResultsA total of 1158 unrelated donor HCT were represented in the data. Cumulative incidences of engraftment, transplant related mortality (TRM), acute graft‐versus‐host disease (GvHD), chronic GvHD and relapse were not significantly affected by donor age. OS probability at 5 years post‐transplant was 48.3%. In multivariate analysis of OS, year of transplant 2001–2007, recipient age 40 years or greater, poor risk disease, human leukocyte antigen (HLA) match less than 6/6 and poor performance status at transplant (Karnofsky scale) were independently significant adverse OS risk factors. Donor age was not a significant risk factor for OS in univariate or multivariate analysis.ConclusionsThe conclusion from this study was that donor age (up to 59 years) did not influence post‐transplant outcome among adult unrelated donor HCT performed in Australia for haematologic malignancies.
Zoledronate promotes inflammatory cytokine expression in human CD14‐positive monocytes among peripheral mononuclear cells in the presence of γδ T cells
Authors: Takimoto Reiko , Suzawa Tetsuo , Yamada Atsushi , Sasa Kiyohito , Miyamoto Yoichi , Yoshimura Kentaro , Sasama Yuji , Tanaka Motohiro , Kinoshita Mitsuhiro , Ikezaki Kaori , Ichikawa Makoto , Yamamoto Matsuo , Shirota Tatsuo , Kamijo Ryutaro 
Published: November 2020

SummaryBisphosphonates distributed to bone exert toxic effects specifically towards osteoclasts. On the other hand, intravenous administration of a nitrogen‐containing bisphosphonate (N‐BP) such as zoledronate induces acute‐phase reactions (APRs), including influenza‐like fever 1 day later, indicating an interaction with immunocompetent cells circulating blood. Although it has been reported that activation of γδ T cells is pivotal to induce an APR following treatment with zoledronate, downstream events, including the production of inflammatory cytokines after activation of γδ T cells, remain obscure. We investigated the effects of zoledronate on inflammatory cytokine expression in human peripheral blood mononuclear cells (PBMCs) in vitro. While zoledronate induced mRNA expressions of tumour necrosis factor‐α (TNF‐α), interleukin (IL)‐1β, IL‐6 and interferon‐γ (IFN‐γ) in PBMC, depletion of γδ T cells abolished that zoledronate‐induced expression of those cytokines, indicating the necessity of γδ T cells for expression induction by zoledronate. However, which types of cells were responsible for the production of those cytokines in blood remained unclear. As it is generally accepted that monocytes and macrophages are primary sources of inflammatory cytokines, CD14+ cells from PBMC were exposed to zoledronate in the presence of PBMC, which resulted in induced expression of mRNAs for IL‐1β, IL‐6 and IFN‐γ, but not for TNF‐α. These results indicate that CD14+ cells are responsible, at least in part, for the production of IL‐1β, IL‐6 and IFN‐γ in blood exposed to zoledronate. This suggests that CD14+ cells play an essential role in the occurrence of APRs following N‐BP administration.
Tailoring carboxyl tubular carbon nanofibers/MnO2 composites for high‐performance lithium‐ion battery anodes
Authors: Huyan Yu , Chen Junjie , Yang Ke , Zhang Qiuyu , Zhang Baoliang 
Published: November 2020

AbstractThree kinds of novel carboxyl modification tubular carbon nanofibers (CMTCFs) and MnO2 composites materials (CMTCFs/MnO2) are prepared by combining hyper‐crosslinking, liquid phase oxidation and hydrothermal technology. The complex morphology and crystal phase of MnO2 in CMTCFs/MnO2 are effectively regulated by adjusting the hydrothermal reaction time. The δ‐MnO2 nanosheet‐wrapped CMTCFs (CMTCFs@MNS) are used as anode and compared with the other two CMTCFs/MnO2. Electrochemical analysis shows that CMTCFs@MNS electrode exhibits a large reversible capacity of 1497.1 mAh g−1 after 300 cycles at 1000 mA g−1 and a long cycling reversible capacity of 400.8 mAh g−1 can be maintained after 1000 cycles at 10 000 mA g−1. CMTCFs@MNS manifests an average reversible capacity of 256.32 mAh g−1 at 10 000 mA g−1 after twelve changes in current density. In addition, the structural superiority of CMTCFs@MNS electrode is clarified by characterizing the microscopic morphology and crystal phase of the electrode after electrochemical performance test.