bluuebunny/crossref_metadata_2025_split huggingface dataset:
Sex-BiasedZRSR2Mutations in Myeloid Malignancies Impair Plasmacytoid Dendritic Cell Activation and Apoptosis
Authors: Togami Katsuhiro , Chung Sun Sook , Madan Vikas , Booth Christopher A.G. , Kenyon Christopher M. , Cabal-Hierro Lucia , Taylor Justin , Kim Sunhee S. , Griffin Gabriel K. , Ghandi Mahmoud , Li Jia , Li Yvonne Y. , Angelot-Delettre Fanny , Biichle Sabeha , Seiler Michael , Buonamici Silvia , Lovitch Scott B. , Louissaint Abner , Morgan Elizabeth A. , Jardin Fabrice , Piccaluga Pier Paolo , Weinstock David M. , Hammerman Peter S. , Yang Henry , Konopleva Marina , Pemmaraju Naveen , Garnache-Ottou Francine , Abdel-Wahab Omar , Koeffler H. Phillip , Lane Andrew A. 
Published: January 2022

AbstractBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive leukemia of plasmacytoid dendritic cells (pDC). BPDCN occurs at least three times more frequently in men than in women, but the reasons for this sex bias are unknown. Here, studying genomics of primary BPDCN and modeling disease-associated mutations, we link acquired alterations in RNA splicing to abnormal pDC development and inflammatory response through Toll-like receptors. Loss-of-function mutations in ZRSR2, an X chromosome gene encoding a splicing factor, are enriched in BPDCN, and nearly all mutations occur in males. ZRSR2 mutation impairs pDC activation and apoptosis after inflammatory stimuli, associated with intron retention and inability to upregulate the transcription factor IRF7. In vivo, BPDCN-associated mutations promote pDC expansion and signatures of decreased activation. These data support a model in which male-biased mutations in hematopoietic progenitors alter pDC function and confer protection from apoptosis, which may impair immunity and predispose to leukemic transformation.Significance:Sex bias in cancer is well recognized, but the underlying mechanisms are incompletely defined. We connect X chromosome mutations in ZRSR2 to an extremely male-predominant leukemia. Aberrant RNA splicing induced by ZRSR2 mutation impairs dendritic cell inflammatory signaling, interferon production, and apoptosis, revealing a sex- and lineage-related tumor suppressor pathway.This article is highlighted in the In This Issue feature, p. 275
Uma visita aos índios do rio Trombetas
Authors: Polykrates Gottfried , Victor Marcelo 
Published: January 2022

Sendo um dos produtos de um mapeamento etnográfico feito ao longo do rio Trombetas e afluentes, nesse texto de 1957, Polykrates trata de alguns aspectos da vida e cultura do povo Katxuyana, à época identificado como Kashuiéna, e autodenominado Uarikiana. Além de abordar o modo de vida e cultura material do povo visitado, Polykrates descreve a festa Kurínguri, a qual pode presenciar durante a sua visita a uma de suas aldeias no rio Cachorro. Neste relato de viagem também são mencionados os seguintes povos como então habitantes do rio Trombetas e vizinhanças: Kahiána (ou Kahianá), Tunajéna, Pianacotó.
Intermittent Occlusion of the Superior Vena Cava to Improve Hemodynamics in Patients With Acutely Decompensated Heart Failure: The VENUS-HF Early Feasibility Study
Authors: Kapur Navin K. , Kiernan Michael S. , Gorgoshvili Irakli , Yousefzai Rayan , Vorovich Esther E. , Tedford Ryan J. , Sauer Andrew J. , Abraham Jacob , Resor Charles D. , Kimmelstiel Carey D. , Benzuly Keith H. , Steinberg Daniel H. , Messer Julie , Burkhoff Daniel , Karas Richard H. 
Published: January 2022

Background:
Reducing congestion remains a primary target of therapy for acutely decompensated heart failure. The VENUS-HF EFS (VENUS-Heart Failure Early Feasibility Study) is the first clinical trial testing intermittent occlusion of the superior vena cava with the preCARDIA system, a catheter mounted balloon and pump console, to improve decongestion in acutely decompensated heart failure.

Methods:
In a multicenter, prospective, single-arm exploratory safety and feasibility trial, 30 patients with acutely decompensated heart failure were assigned to preCARDIA therapy for 12 or 24 hours. The primary safety outcome was a composite of major adverse cardiovascular and cerebrovascular events through 30 days. Secondary end points included technical success defined as successful preCARDIA placement, treatment, and removal and reduction in right atrial and pulmonary capillary wedge pressure. Other efficacy measures included urine output and patient-reported symptoms.

Results:

Thirty patients were enrolled and assigned to receive the preCARDIA system. Freedom from device- or procedure-related major adverse events was observed in 100% (n=30/30) of patients. The system was successfully placed, activated and removed after 12 (n=6) or 24 hours (n=23) in 97% (n=29/30) of patients. Compared with baseline values, right atrial pressure decreased by 34% (17±4 versus 11±5 mm Hg,
P
<0.001) and pulmonary capillary wedge pressure decreased by 27% (31±8 versus 22±9 mm Hg,
P
<0.001). Compared with pretreatment values, urine output and net fluid balance increased by 130% and 156%, respectively, with up to 24 hours of treatment (
P
<0.01).

Conclusions:
We report the first-in-human experience of intermittent superior vena cava occlusion using the preCARDIA system to reduce congestion in acutely decompensated heart failure. PreCARDIA treatment for up to 24 hours was well tolerated without device- or procedure-related serious or major adverse events and associated with reduced filling pressures and increased urine output. These results support future studies characterizing the clinical utility of the preCARDIA system.

Registration:

URL:
https://www.clinicaltrials.gov
; Unique identifier: NCT03836079.
Frontier Technology,  Trade,  FDI,  and TFP in the Electrical and Electronic Industries: Exporting or Processing Trade?
Authors: Khalifah Noor Aini 
Published: January 2022

Abstract
Does “openness” determine “catching-up” of establishments to frontier technology and total factor productivity (TFP) in Malaysia's electrical and electronic (E&E) industries? We contribute to this debate by applying a new measurement of processing trade intensity. Utilizing stochastic frontier analysis and Levinsohn and Pertrin (LP) TFP,  we investigate determinants of technical efficiency (TE) and TFP. The results show that processing trade intensity and not export intensity determines TE and TFP for the overall sample and subsample of foreign establishments. In the processing trade subsample, export intensity is negatively related to TE and unrelated to TFP, obtaining an unconventional result that exporters are inefficient and not associated with TFP. The results show that higher foreign ownership shares of establishments are negatively associated with LP TFP.
Chinese Silk and Globalization along the Hispanic American Road, from the Sixteenth to Eighteenth Centuries
Authors: Bonialian Mariano 
Published: January 2022

Abstract
This essay analyzes one of the main elements of the economic relationship between China and colonial Latin America: the Chinese Silk Road. The road demonstrated a bipolarity of early globalization, and its impact across the Pacific enhanced the material culture of Hispanic American society from Mexico to Buenos Aires. The route of circulation was largely informal, due to prohibitions imposed by metropolitan Spain designed to guarantee the growth of European economic connections across the Atlantic Ocean. Quality variations and affordable prices made Chinese silks among the most valuable articles in Latin American markets before the nineteenth century, when British-Indian cotton emerged as the main textile of the global era.
Low-Frequency Atmospheric Variability Patterns and Synoptic Types Linked to Large Floods in the Lower Ebro River Basin
Authors: Peña J. C. , Balasch J. C. , Pino D. , Schulte L. , Barriendos M. , Ruiz-Bellet J. L. , Prohom M. , Tuset J. , Mazon J. , Castelltort X. 
Published: January 2022

Abstract
This study analyzes the atmospheric variability that caused the largest floods affecting the town of Tortosa, Spain, in the mouth of the Ebro River (northeast Iberian Peninsula). The Tortosa flood database and flood marks in the nearby town of Xerta are used to define the more relevant flooding episodes (discharges >2900 m3 s−1) of the 1600–2005 period. We explore the atmospheric variability based on low-frequency patterns and synoptic types applying a multivariable analysis to grids at sea level pressure and geopotential at 500 hPa provided by the twentieth-century V3 Reanalysis Project for the instrumental period (since 1836). Output from the Last Millennium Ensemble Project was used to analyze the sea level pressure over the pre-instrumental period (before 1836). Our analysis includes 33 flood episodes. Four synoptic types are related to floods in Tortosa since 1836, characterized by low pressure systems that interact with the Mediterranean warm air mass and promote atmospheric destabilization. Flooding in Tortosa is related to relative high values of solar activity, positive Northern Hemisphere temperature anomalies, and NAO in positive phase. This result indicates that the major floods are related to zonal atmospheric circulations (west-to-east cyclone transfer). During winter, the main impact of the floods is located at the western part of the basin, and the Pyrenean subbasins are affected during autumn. The major finding is that similar flood behavior is detected since 1600, improving our understanding of past climates, enhancing the knowledge base for some aspects and impacts of climate change, and reducing uncertainty about future outcomes.

Significance Statement
A total of 33 large floods (>2900 m3 s−1) were registered since 1600 in Tortosa, Spain, located at the mouth of the Ebro River (northeast Iberian Peninsula). They occur associated with low pressure systems that interact with the Mediterranean warm air mass promoting atmospheric destabilization. The floods in Tortosa are also associated with other important processes occurring at significantly longer time scales: high values of solar activity, positive Northern Hemisphere temperature anomalies, and NAO in positive phase, indicating that the major floods are related to zonal atmospheric circulations. The major finding is that we detect similar flood behaviors since 1600, improving our understanding of past climates, enhancing the knowledge base for some aspects and impacts of climate change, and reducing uncertainty about future outcomes.
Understanding the Extratropical Liquid Water Path Feedback in Mixed-Phase Clouds with an Idealized Global Climate Model
Authors: Frazer Michelle E. , Ming Yi 
Published: January 2022

AbstractA negative shortwave cloud feedback associated with higher extratropical liquid water content in mixed-phase clouds is a common feature of global warming simulations, and multiple mechanisms have been hypothesized. A set of process-level experiments performed with an idealized global climate model (a dynamical core with passive water and cloud tracers and full Rotstayn–Klein single-moment microphysics) show that the common picture of the liquid water path (LWP) feedback in mixed-phase clouds being controlled by the amount of ice susceptible to phase change is not robust. Dynamic condensate processes—rather than static phase partitioning—directly change with warming, with varied impacts on liquid and ice amounts. Here, three principal mechanisms are responsible for the LWP response, namely higher adiabatic cloud water content, weaker liquid-to-ice conversion through the Bergeron–Findeisen process, and faster melting of ice and snow to rain. Only melting is accompanied by a substantial loss of ice, while the adiabatic cloud water content increase gives rise to a net increase in ice water path (IWP) such that total cloud water also increases without an accompanying decrease in precipitation efficiency. Perturbed parameter experiments with a wide range of climatological LWP and IWP demonstrate a strong dependence of the LWP feedback on the climatological LWP and independence from the climatological IWP and supercooled liquid fraction. This idealized setup allows for a clean isolation of mechanisms and paints a more nuanced picture of the extratropical mixed-phase cloud water feedback than simple phase change.
The Life Cycle and Variability of Antarctic Weak Polar Vortex Events
Authors: Shen Xiaocen , Wang Lin , Osprey Scott , Hardiman Steven C. , Scaife Adam A. , Ma Ji 
Published: January 2022

Abstract
Motivated by the strong Antarctic sudden stratospheric warming (SSW) in 2019, a survey on the similar Antarctic weak polar vortex events (WPVs) is presented, including their life cycle, dynamics, seasonality, and climatic impacts. The Antarctic WPVs have a frequency of about four events per decade, with the 2002 event being the only major SSW. They show a similar life cycle to the SSWs in the Northern Hemisphere but have a longer duration. They are primarily driven by enhanced upward-propagating wavenumber 1 in the presence of a preconditioned polar stratosphere (i.e., a weaker and more contracted Antarctic stratospheric polar vortex). Antarctic WPVs occur mainly in the austral spring. Their early occurrence is preceded by an easterly anomaly in the middle and upper equatorial stratosphere in addition to the preconditioned polar stratosphere. The Antarctic WPVs increase the ozone concentration in the polar region and are associated with an advanced seasonal transition of the stratospheric polar vortex by about one week. Their frequency doubles after 2000 and is closely related to the advanced Antarctic stratospheric final warming in recent decades. The WPV-resultant negative phase of the southern annular mode descends to the troposphere and persists for about three months, leading to persistent hemispheric-scale temperature and precipitation anomalies.

Significance Statement
The Antarctic weak polar vortex events (WPVs) are similar to the sudden stratospheric warming (SSW), but many of their characteristics remain unclear. Their climatology is presented as a benchmark based on high-quality reanalysis datasets. WPVs have a life cycle that is similar to that of Arctic SSWs but has a longer duration. They occur due to the amplified tropospheric wave forcing in the presence of a preconditioned polar stratosphere. Its seasonality is partly controlled by the equatorial stratospheric easterly in addition to the polar stratosphere. Its occurrence is closely related to the advanced breakdown of the Antarctic polar vortex and can reduce the size of the Antarctic ozone hole. Moreover, it further causes persistent hemispheric-scale climate anomalies in the troposphere, which provides a prediction potential for surface weather and climate.
How I treat relapsed multiple myeloma
Authors: Kastritis Efstathios , Terpos Evangelos , Dimopoulos Meletios A. 
Published: January 2022

Abstract
Despite recent advances, multiple myeloma remains an incurable disease for most patients, and initial remission will be followed by relapses requiring therapy. For many, there will be several remissions and relapses until resistance develops to all available therapies. With the introduction of several new agents, myeloma treatment has changed drastically, and there are new options for the management of relapsed or refractory disease, including new drug classes with distinct mechanisms of action and cellular therapies. However, resistance to major drug classes used in first-line remains the most critical factor for the choice of treatment at relapse. Continuous lenalidomide-based therapy is used extensively at first-line, and resistance to lenalidomide has become the key factor for the choice of salvage therapy. Daratumumab is increasingly used in first-line, and soon patients that relapse while on daratumumab will become a common challenge. Three-drug regimens are the standard approach to manage relapsed disease. Adding drugs with new mechanisms of activity can improve outcomes and overcomes class resistance, but, until now, while biology is important, it can offer only limited guidance for the choice of therapy.
Autologous stem cell transplantation for large B-cell lymphoma with secondary central nervous system involvement
Authors: Akin Serkan , Hosing Chitra , Khouri Issa , Ahmed Sairah , Alousi Amin , Fowler Nathan , Joseph Jacinth , Truxillo Jonathan , Ramdial Jeremy L. , Maadani Farzaneh , Rondon Gabriela , Daher May , Im Jin S. , Steiner Raphael , Westin Jason , Iyer Swaminathan P. , Dabaja Bouthaina , Anderlini Paolo , Popat Uday R. , Qazilbash Muzaffar H. , Flowers Christopher R. , Shpall Elizabeth , Champlin Richard E. , Nieto Yago , Srour Samer A. 
Published: January 2022

Abstract
Secondary central nervous system large B-cell lymphoma (SCNSL) is rare, with a generally poor prognosis. There is limited data about the role of autologous stem cell transplantation (ASCT) in these high-risk patients. We explored in this study treatment outcomes and prognostic factors for patients with SCNSL who underwent ASCT. We included all consecutive patients who underwent ASCT at our institution. Primary endpoints were progression-free survival (PFS) and overall survival (OS). One-hundred two patients were identified. Median age at transplant was 56 (range, 21-71) years. With a median follow-up of 56 (range, 1-256) months, the median PFS and OS were 40 and 88 months, respectively. The 4-year PFS and OS were 48% and 57%, respectively. In univariate analysis, complete remission (CR) at transplant, prior lines of therapy (≤2), normal lactate dehydrogenase, and parenchymal involvement were significantly associated with improved PFS. For OS, only CR at transplant and ≤2 prior lines of therapy were associated with improved survival. On multivariable analysis for PFS, CR at transplant (hazard ratio [HR], 0.278; 95% CI, 0.153-0.506; P ≤ .0001) and ≤2 prior lines of therapy (HR, 0.485; 95% CI, 0.274-0.859; P = .0131) were significantly associated with superior PFS. Similarly, CR at transplant (HR, 0.352; 95% CI, 0.186-0.663; P = .0013) and ≤2 prior lines of therapy (HR, 0.476; 95% CI, 0.257-0.882; P = .0183) were associated with improved survival. In the largest single-center study, our findings indicate that ASCT is associated with durable responses and prolonged survival in patients with SCNSL. Patients in CR at transplant and those who received ≤2 lines of therapy have particularly excellent outcomes.
Clofarabine with topotecan, vinorelbine, and thiotepa reinduction regimen for children and young adults with relapsed AML
Authors: Ramaswamy Kavitha , Steinherz Peter G. , Agrawal Anurag K. , Forlenza Christopher J. , Mauguen Audrey , Roshal Mikhail , Trippett Tanya , Kernan Nancy A. , Sulis Maria Luisa , Shukla Neerav 
Published: January 2022

Abstract
Effective reinduction regimens are needed for children with relapsed and refractory acute myeloid leukemia (AML), as outcomes remain poor. Therapeutic options are limited in this heavily pretreated patient population, many of whom have reached lifetime recommended doses of anthracycline chemotherapy. The development of effective non-anthracycline–based salvage regimens is crucial to these patients who are at significant risk of life-threatening cardiotoxicity. We previously reported results of a phase 2 trial of a clofarabine-based regimen with topotecan, vinorelbine, and thiotepa (TVTC) in patients with relapsed acute leukemias. Here we report on an expanded bicenter cohort of 33 patients, &lt;25 years of age, with relapsed/refractory AML treated with up to 2 cycles of the TVTC reinduction regimen from 2007 to 2018. The overall response rate, defined as complete remission or complete remission with partial recovery of platelet count, was 71.4% (95% confidence interval [CI], 41.9-91.6) for those patients in first relapse (n = 14) and 47.4% (95% CI, 24.4-71.1) for patients in second or greater relapse or with refractory disease. Responses were seen across multiple high-risk cytogenetic and molecular subtypes, with 84% of responders successfully bridged to allogeneic stem cell transplantation. The 5-year overall survival for patients in first relapse was 46.2% (95% CI, 19.1-73.3) and 50.0% (95% CI, 26.9-73.1) for patients who responded to TVTC. For pediatric and young adult patients with relapsed/refractory AML, TVTC reinduction compares favorably with currently used salvage regimens and warrants further exploration.
The bisphenol F and bisphenol S and cardiovascular disease: results from NHANES 2013–2016
Authors: Wang Ruihua , Fei Qiaoyuan , Liu Shan , Weng Xueqiong , Liang Huanzhu , Wu Yingying , Wen Lin , Hao Guang , Cao Guangwen , Jing Chunxia 
Published: January 2022

Abstract
Background
Bisphenol F (BPF) and bisphenol S (BPS) have replaced bisphenol A (BPA) in the manufacturing of products containing polycarbonates and epoxy resins; however, the effects of these substitutes on the risk of cardiovascular disease (CVD), including congestive heart failure, coronary heart disease, angina pectoris, heart attack, and stroke, have not been assessed.

Objective
To examine the association of urinary BPS and BPF with CVD risk in a U.S. representative U.S. population.

Methods
Cross-sectional data from 1267 participants aged 20–80 years from the 2013–2016 National Health and Nutrition Examination Survey (NHANES) were analyzed. Survey-weighted multiple logistic regression was used to assess the association between BPA, BPF, BPS and CVD. The Bayesian kernel machine regression (BKMR) model was applied to assess the mixture effect.

Results
A total of 138 patients with CVD were identified. After adjusting for potential confounding factors, the T3 tertile concentration of BPS increased the risk of total CVD (OR: 1.99, 95% CI 1.16–3.40). When stratified by age, we found that BPS increased the risk of CVD in the 50–80 age group (OR: 1.40, 95% CI 1.05–1.87). BPS was positively associated with the risk of coronary heart disease, and the T3 tertile concentration of BPS increased the coronary heart disease risk by 2.22 times (95% CI 1.04–4.74). No significant association was observed between BPF and CVD. Although the BKMR model did not identify the mixed exposure effect of BPS, the risk of CVD increased with increasing compound concentration.

Conclusion
Our results suggest that BPS may increase the risk of total CVD and coronary heart disease in the US population, and prospective studies are needed to confirm the results.
Brucellosis relapse causing thoracic aortic ulcers and aneurysm formation: a case report
Authors: Li Shuai , Wang Qiang 
Published: January 2022

Abstract
Background
Brucellosis is an infectious disease caused by Brucella spp, which can involve the cardiovascular, digestive, and respiratory systems. Cardiovascular involvement is a rare occurrence, it has an extremely high mortality rate.

Case presentation
A 67-year-old Chinese man presented with thoracic aortic multiple ulcers and partial aneurysm formation that caused symptoms of left waist and left buttock pain. The man was admitted to our hospital due to abdominal aorta pseudoaneurysms 5 years ago. The diagnosis was made by thoracic computed tomography angiography (CTA), previous history, and positive culture of Brucella, and the patient was successfully treated by thoracic aortic covered stent-graft implantation and specific medical treatment.

Conclusions
People who have a history of contact with cattle and sheep, should beware of the possibility of Brucella infection. If chest and abdominal pain occur, timely medical treatment is recommended, aortic aneurysm, the disease with a high risk of death, can be identified or excluded by CTA. Early treatment and prevention of disease progression are more beneficial to patients.