bluuebunny/crossref_metadata_2025_split huggingface dataset:
Extrajudicial border enforcement against LGBTIQ+ asylum seekers
Authors: Espinosa Adriana , Hampton Kathryn , Nathwani Nishin , Powell Kimahli , Sereneo Monique , Wackett Curtis 
Published: June 2024

AbstractRecent scholarship has highlighted how states differentially restrict the movement of persons who are divergently racialized, gendered, sexualized, abled, and aged. This paper explores the phenomenon of extrajudicial border enforcement—instances where airline officials act on behalf of states to prevent the cross-border movement of marginalized persons. Based on a qualitative analysis of fifty-two cases of failed travel of at-risk lesbian, gay, bisexual, transgender, intersex, and queer (LGBTIQ+) persons attempting to cross an international border between April 2018 and April 2022, this paper offers an intersectional lens into how airline officials, whom the coauthors term extended state agents, deny boarding to individuals through an arbitrary and discretionary process, despite the individual’s compliance with the entry requirements of transit and destination countries. Because state penalties incentivize these boarding denials, implementation of carrier sanctions should be understood as a byproduct of law rather than an exemption from it. These expulsions without due process are in sharp contrast to the LGBTIQ+ rights affirming rhetoric of the expelling states, which we characterize as a form of pinkwashing. In this analysis, coauthors question the responsibility of states in human rights violations consequent to denial of boarding under carrier sanctions regimes.
Over 4 Years of Safety and Efficacy with Voxelotor in Patients with Sickle Cell Disease
Authors: Brown Clark , Davis Mark , Achebe Maureen , Hassab Hoda , Al-Kindi Salam , Telfer Paul , Biemond Bart J , Lipato Thokozeni , Gray Sarah , Gordeuk Victor R 
Published: June 2024

Abstract

Presentation Date: 6/9/2024
Presentation Start Time: 1:33:00 PM

Background
In patients with sickle cell disease (SCD), polymerization of sickle hemoglobin (HbS) results in red blood cell sickling and leads to hemolysis, chronic anemia, and vaso-occlusive crises. Voxelotor, a first-in-class HbS polymerization inhibitor, is approved in the United States and Europe for the treatment of patients with SCD aged ≥4 years and ≥12 years, respectively. To assess the safety and efficacy of long-term voxelotor use, we report an updated interim analysis of an open-label extension (OLE) of the HOPE phase 3 trial (NCT03036813).

Methods
Patients who completed the HOPE trial were eligible to enroll in the multicenter, global OLE study (NCT03573882) and received ongoing treatment consisting of once daily voxelotor 1500 mg if they continued to derive clinical benefit. Adverse event (AE) data were collected through 28 days after voxelotor discontinuation, and measurements of hemoglobin and clinical markers of hemolysis were summarized through 168 weeks of treatment in the OLE. Data are based on an interim data cut (December 31, 2022).

Results
Of 199 patients who completed the HOPE trial, 178 (89.4%) were enrolled and dosed in the OLE. Median age at enrollment was 25 years. The median duration of voxelotor exposure in the OLE was 124.0 weeks, and 81 patients were treated for ≥168 weeks. Of these, 52 had received voxelotor in the HOPE trial, for a combined duration of exposure of ≥ 240 weeks. Patients previously treated with placebo in the HOPE trial had a mean (SD) hemoglobin change from baseline (start of OLE) of 1.1 (1.51) g/dL at Week 168. The mean (SD) hemoglobin change from baseline for patients who previously received voxelotor 1500 mg was 0.5 (1.49) g/dL (n = 18), indicating durability of response with continued treatment. Markers of hemolysis (indirect bilirubin, reticulocyte percentage) improved in patients who received placebo in the HOPE trial and were stable for patients who previously received 1500 mg voxelotor in the HOPE trial. Non-SCD-related treatment-emergent AEs (TEAEs) were reported in 88.2% of patients; no new safety signals were identified. Most TEAEs were grade 1 or 2 in severity. Eight deaths occurred, none deemed related to voxelotor treatment.

Conclusions
In this updated analysis from the HOPE OLE, long-term use of voxelotor was effective at improving hemoglobin and clinical markers of hemolysis in patients with SCD. The safety profile in the OLE was consistent with findings from the HOPE trial, and no new safety signals were identified with exposure through a combined 240 weeks of treatment.
© American Society of Hematology (2023). Reused with permission.
Incidence of Thromboembolic Events in Patients with Sickle Cell Disease Taking Voxelotor
Authors: Ansari Muneeb , Zhang Xu , Rizvi Insia , Farooqui Marwah 
Published: June 2024

Abstract

Presentation Date: 6/9/2024
Presentation Start Time: 2:09:00 PM

Background
Voxelotor was approved for patients with sickle cell in 2019 and acts to increase the hemoglobin in patients with severe hemolysis. Given hemoglobin’s contribution to hypercoagulable states, there is a concern that medications increasing hemoglobin concentrations may increase viscosity and contribute to thromboembolic events. This concern was furthered by a case report of a pulmonary embolism in a patient immediately after starting voxelotor.
Methods : This retrospective cohort study analyzed adult and pediatric patients ages 7 to 66 years of age with HbSS and HbSβ0 type sickle cell disease at the University of Illinois Health Sickle Cell Center started on voxelotor between January 2020 and October 2023. The charts of these patients were reviewed for cerebrovascular accident, pulmonary embolism, or deep vein thrombosis post voxelotor initiation.
Labs at the time of the thromboembolic event were recorded and the time between starting voxelotor and the event was calculated.

Results
In total, 84 patient charts were assessed. 24 were exclusively on voxelotor without hydroxyurea, L-glutamine, or crizanlizumab disease-modifying therapy. 10 patients (6 men and 4 women) had thromboembolic events (12%). Of those patients, 4 (2 men and 2 women) were in the voxelotor-only group.

Conclusion
Out of 84 adult and pediatric patients with sickle cell disease on voxelotor, 10 experienced thromboembolic events ranging from 1-29 months after starting the medication. Advanced age and history of a previous VTE were significantly associated with an increased risk of a subsequent VTE. The majority were unprovoked. Further research into characterizing the medication’s risk is warranted.
Systematic Review of Emergency Department Management of Vaso-occlusive Crisis
Authors: Hutcherson Timothy C , Cieri-Hutcherson Nicole , Rowlee Paige , Abeles Jennifer 
Published: June 2024

Abstract

Presentation Date: 6/9/2024
Presentation Start Time: 3:45:00 PM

Purpose
The objective of this systematic review was to characterize the management of vaso-occlusive crisis (VOC) in adult patients with sickle cell disease (SCD) in the emergency department (ED).

Methods
A literature search was conducted spanning origin through May 31, 2023, using Embase, MEDLINE ALL, and International Pharmaceutical Abstracts databases. Primary literature reporting on adult patients with SCD in a SCD-specific pathway or protocol in an ED setting was included. Data extracted included study design characteristics, patient demographics, descriptors of the pathway/protocol, and outcomes related to patient pain, pain medications, and hospital length of stay. The Cochrane Risk of Bias 2.0 tool was used to assess risk of bias (ROB) in randomized controlled trials (RCTs). The Risk of Bias for Non-Randomized Studies of Interventions was used to assess ROB in nonrandomized trials (NRTs). Certainty of evidence was assessed via the Grading of Recommendations, Assessment, Development, and Evaluations tool. This systematic review was not registered or funded.

Results
A total of 9 studies met eligibility criteria: 2 RCTs, 7 observational NRTs. RCTs had low ROB; of the 7 NRT studies, 5 had moderate ROB, and 2 had serious ROB. Improvements in time to first opioid, ED pain scores, length of ED treatment time, overall treatment time (length of stay), and inpatient readmission improved with policies or protocols for the management of VOC in the ED setting. These five outcomes had a moderate certainty of evidence.

Conclusion
Institutions should consider implementing protocols or pathways to improve management of VOC in the ED.
Menopause in Women with Sickle Cell Disease
Authors: Adedeji Monica , Andrews Elizabeth , Riddick-Burden Gaye , Rizk Sanaa 
Published: June 2024

Abstract

Presentation Date: 6/9/2024
Presentation Start Time: 4:15:00 PM

Background
Sickle cell disease (SCD) is the most common genetic disorder globally. With improved SCD management over the decades, more focus has been directed on long-term survival, and age-related complications. While menarche has been studied in adolescents with SCD and seems to be delayed, since women with SCD previously did not live long enough to experience menopause, there remains a paucity of data on menopause so far. This study sought to illicit the menopause experience in women with SCD including the age of onset and symptom severity. We hypothesized that women with SCD may have earlier onset menopause compared to women without SCD as reports have mentioned ovarian sickling as a proposed mechanism of premature ovarian failure, and iron overload as a possible cause of endocrinopathy--leading to impaired ovarian reserve.

Methods
Within the Jefferson Health Network, 37 menopausal Black women with SCD were interviewed using a standardized questionnaire inquiring about their menopause experience. Mean, median and standard deviation were calculated using basic statistics.

Results
Women with SCD, irrespective of genotype, underwent menopause at a median age of 48 years compared to 52 years--the national median age of menopause in Black women. Furthermore, women not on hydroxyurea had an increased incidence of menopausal symptoms including weight gain, hot flashes, sleep disturbances, vaginal dryness and mood swings, compared to women taking hydroxyurea.

Conclusions
While this study’s results are limited due to small sample size, it sheds light on the menopause experience in women with SCD and further highlights the need for further research on this topic.
Clinical Data of Menopause in Women with Sickle cell disease (SCD) With and Without Hydrea Therapy
Nontraumatic Epidural Hematoma in Sickle Cell Disease
Authors: Paros Alexandra 
Published: June 2024

Abstract

Presentation Date: 6/8/2024
Presentation Start Time: 6:00:00 PM

Background
Non-traumatic epidural hematoma is an extremely rare finding in patients with sickle cell disease. Spontaneous intracranial epidural hemorrhage has been associated with coagulopathy, malignancy, and infection, but few cases secondary to sickle cell disease have been reported in the literature. The mechanism of hemorrhage in these patients is often unclear, although previous cases have suggested associations with vaso-occlusive pain crises, bone infarction, and/or vascular malformations.We report a 9 year old male with sickle cell disease type SS who presented with right shoulder pain, headaches, emesis, and no neurological deficits. CT head revealed large mixed density left frontoparietal epidural hematoma leading to complete left lateral ventricle effacement and 0.5 cm left to right midline shift.

Methods
Chart review was performed through the electronic medical record to obtain information about the patient and case.

Results
The patient underwent a left parietal craniotomy for evacuation of epidural hematoma from which he recovered well.

Conclusions
Although rare, atraumatic epidural hematoma in SCD is associated with significant risk of morbidity and mortality. The prevalence and proposed pathologic process behind this rare complication will be discussed.
Sudden Death Caused by Plastic Bronchitis in a Child with Sickle Cell Disease
Authors: Morgan Epley , Lau Jaime , Hanna Cherry , Ahmed Rafat , Ostrowicki Renata , Krulish Sean , Nathan Anita 
Published: June 2024

Abstract

Presentation Date: 6/8/2024
Presentation Start Time: 6:00:00 PM

Background
Plastic bronchitis is a rare and poorly described complication of sickle cell disease and acute chest syndrome. It is defined as the formation of casts made of various materials, which obstruct lung bronchi. Plastic bronchitis has been diagnosed in several disease states including asthma, cystic fibrosis, and congenital heart disease following Fontan procedure. This case describes a case of a 5-year-old male with Sickle Cell SS Disease, admitted for pain crisis and Acute Chest Syndrome, which rapidly progressed to cardiovascular decompensation and death secondary to diffuse lung infiltration and obstruction due to plastic bronchitis.

Methods
Single case report

Results
A 5-year-old male with Sickle Cell SS Disease and moderate persistent asthma was admitted to the pediatric ward for generalized abdominal pain and extremity pain consistent with vaso-occlusive pain crisis. Subsequently, the patient developed a fever associated with respiratory distress and pain at his port-a-cath site. He was started on broad-spectrum antibiotics for Acute Chest Syndrome, given a new lung infiltrate in the right lower lobe on chest x-ray, and possible central line-associated bloodstream infection (CLABSI). He was initiated on high-flow nasal cannula (HFNC) and transferred to the pediatric intensive care unit. His respiratory status stabilized on HFNC with moderate supplemental oxygen and intermittent albuterol; however, he continued to have persistent tachycardia, tachypnea, and worsening lung infiltrates on repeat chest x-ray. Several hours later, he went into sudden cardiac arrest with asystole. Despite immediate CPR, intubation, and resuscitative measures, the patient expired. The autopsy revealed plastic bronchitis with fibrinous casts with 95% airway occlusion from the carina to the bronchioles in both lung fields.

Conclusions
Plastic bronchitis, a rare complication, can progress to rapid deterioration and death in pediatric patients with acute chest syndrome and sickle cell disease. The presentation of plastic bronchitis in sickle cell disease is unique compared to other underlying disease states. Clinicians should be on high alert for signs and symptoms indicating this potential complication, especially worsening chest radiographs in a patient with acute chest syndrome.
Mammography in Women with Sickle Cell Disease
Authors: Elmileik Eiman , Farooqui Marwah , Zhang Xu , Hussain Faiz , Haqqani Rayyan , Sung Kaitlin , Saraf Santosh L 
Published: June 2024

Abstract

Presentation Date: 6/8/2024
Presentation Start Time: 6:00 PM

Background
Sickle cell disease (SCD) is one of the most common inherited blood disorders and is associated with significant morbidity and reduced life expectancy. With advances in disease-modifying medications, morbidity and mortality are improved, and research focusing on cancer screenings becomes more relevant. This study aims to identify patterns regarding breast cancer screening, breast composition, and whether guidelines for the general population are appropriate for SCD patients.

Methods
This is a single-center retrospective study in which medical records of 156 individuals with SCD between 2018 and 2022 were analyzed.

Results
The average age of screening mammography was 44. Of the total individuals included in this study, 56% had screening mammography within the past two years (2021 and 2022); when compared to most recent CDC data from 2019, 70.8% of non-Hispanic Black women aged 40 and over had screening mammography within the past two years (2018 and 2019). With a binomial test, the difference was not statistically significant. 54% of those screened had dense breasts. Regression analysis data revealed an inverse relationship between BMI and screening age, and breast density; however, only the relationship between BMI and breast density was statistically significant.

Conclusion
This study was limited by sample size and its retrospective nature. Despite these constraints, this research contributes valuable insights into breast cancer screening practices and breast composition in individuals with SCD. Prospective studies with larger cohorts are recommended to enhance generalizability and foster a comprehensive understanding of cancer risks and screening patterns in this vulnerable patient population.
Damages and Their Quantification
Authors: Caro de Sousa Pedro 
Published: June 2024

AbstractThis chapter discusses the quantification of damages. It explains that the quantification of harm in competition cases is subject to considerable limits due to the degree of certainty and precision that can be achieved. In practice, the amount that a claimant may obtain in damages proceedings can depend on different factors, such as the impact of interest, and the relationship between the harm suffered and the amount of damages awarded. The chapter then elaborates further on the differences between compensatory damages, punitive damages, and exemplary damages. It also considers the efforts to promote public enforcement affecting the extent of the liability for damages of some participants in anti-competitive conduct.
Safeguarding Financial Stability in China
Authors: Li Bo , He Pengyu 
Published: June 2024

Abstract
This chapter covers the legal and institutional framework for safeguarding financial stability in China. In recent years, based upon China’s domestic circumstances and international best practices, the Chinese government has substantially reformed China’s financial stability institutional framework by (i) strengthening the overall coordination of financial stability work; (ii) improving the macroprudential policy framework; (iii) further rationalizing the financial regulatory systems and (iv) enhancing resolution mechanisms. The proposed "Draft Financial Stability Law" would further bolster China’s legal and institutional framework for financial stability by (i) strengthening the legal foundation of the coordination mechanism for financial stability; (ii) putting the central bank’s critical role in the macroprudential policy framework on a firmer legal footing and (iii) upgrading the legal framework for systemic risk resolution. Future reforms should continue to improve China’s financial regulatory systems and promote the appropriate separation of prudential supervision and conduct supervision.
The Evolution of the Regulatory Framework Governing Climate Change and Sustainable Finance in the EU and UK
Authors: Gortsos Christos V , Blair William 
Published: June 2024

Abstract
This chapter focuses on the evolution of the regulatory framework governing climate change and sustainable finance in the EU and the UK. It clarifies that sustainable finance is not limited to the climate change issue. The European Union (EU) has established a detailed rules-based sustainable finance legal structure (including a unique ‘Taxonomy framework’), while the United Kingdom (UK) developed an approach based on principles. The chapter tackles how the regulatory system is taking shape despite having no global consensus over the issue correlating to climate change and sustainable finance. According to the United Nations Conference on Trade and Development (UNCTAD), if the sector continues to grow, the sustainable investment market needs to address greenwashing concerns, the niche market risk, so that sustainable investment becomes a market norm, and the geographical imbalance of investments transforming the market from a developed-country phenomenon to a global market.
Legal Framework for Monetary Integration in West Africa—AfCFTA
Authors: Akin-Olugbade Adesegun 
Published: June 2024

Abstract
This chapter focuses on the African Continental Free Trade Agreement (AfCFTA), which is an agreement between 54 African countries to create a common market of over 1.2 billion people. AfCFTA was enacted as a direct solution to some of the seven aspirations of Agenda 2063, including social and economic development, integration, governance, peace, and security. Moreover, the agreement was expected to merge the markets by advocating for the free flow of people and goods. The chapter then elaborates on Agenda 2063, a 50-year development plan expected to result in an integrated Africa. It describes the attempts at monetary integration in West Africa prior to the AfCFTA and posits that the AfCFTA could finally provide the basis for achieving monetary integration in West Africa.
The UTMOST-NS: a fully digital, wide-field transient search facility operating at a centre frequency of 831 MHz
Authors: Mandlik A , Deller A T , Flynn C , Bailes M , Bateman T , Campbell-Wilson D , Day C K , Dunn L , Green A , Gupta V , Jameson A , Lee Y S C , Plant K , Price Danny C , Sekhri R , Sutherland A , Torr G , Urquhart G 
Published: June 2024

ABSTRACT
The Molonglo Cross was first commissioned in 1965, as a transit radio (408 MHz) interferometer with the largest collecting area in the Southern hemisphere. In 1981, the telescope was redeveloped as an Earth-rotation synthesis interferometer using only the East–West arm (843 MHz), known as the Molonglo Observatory Synthesis Telescope. While the East–West arm was revitalized in the 2010s, the (slightly larger) North–South (NS) arm, which consists of two co-linear paraboloid cylindrical reflectors spanning 2 × 778 m × 12.7 m, had not been used for over 40 yr. Re-fitting this 19 800-m2 collecting area with modern electronics is a cost-effective way of producing a significant survey instrument. The upgrades made to the entire signal chain of the NS arm from the antenna through the transport, digitization, and digital signal processing are described, along with the subsequent performance of the system. The instrument was designed to undertake pulsar timing and searching for dispersed single pulses [e.g. from fast radio bursts (FRBs)]. The upgraded system operated across the 806.25–856.25-MHz frequency range, and had a primary beam that spans 12.7 × 2.5 deg. It had dual linear polarization capability and a lower system temperature and wider bandwidth compared with the East–West system. The digital signal processing was performed on servers with graphics processing units, which enabled low-latency, high-speed data processing, and made use of pipelines built from existing and custom codes. It timed around 70 pulsars per day while running concurrent FRB searches at nearly 100 per cent duty cycle during its operation.
Ensemble X-ray variability of optically selected QSOs: dependence on black hole mass and Eddington ratio
Authors: Georgakakis A , Buchner J , Ruiz A , Boller T , Akylas A , Paolillo M , Salvato M , Merloni A , Nandra K , Dwelly T 
Published: June 2024

ABSTRACT
Although flux variability is one of the defining properties of accretion flows on to supermassive black holes, its dependence on physical parameters such as the mass of the compact object and the Eddington ratio remains under discussion. In this paper, we address this issue using the structure function statistic to measure the variability at X-ray wavelengths of a sample of optically selected QSOs with available black hole masses and Eddington ratios. We present a new Bayesian methodology for estimating the structure function tailored to the Poisson nature of the X-ray data. This is applied to 15 548 SDSS DRQ16 QSOs with repeat observations in the XMM–Newton archive and/or the SRG/eROSITA All Sky Survey. The X-ray structure function monotonically increases to time intervals of about 10–15 yrs, consistent with scenarios in which instabilities of the accretion disc contribute to the X-ray variability on long time-scales. Additionally, there is evidence that the amplitude of the stochastic X-ray flux variations rises with decreasing black hole mass and Eddington ratio. This finding imposes stringent constraints on empirical models of Active Galactic Nuclei variability derived from local samples, emphasizing the significance of high-redshift population studies for comprehending the stochastic flux variations in active black holes.