International Collaborative Network Research Articles

Financial Development and Climate Change: A Detailed Bibliometric Investigation

This paper presents a detailed bibliometric analysis of the interaction between financial development and climate change, with the main aim of elucidating the current state of research in this area, identifying existing gaps, and guiding future researchers interested in this rapidly expanding field. The study used VOSviewer software version 1.6.18 to analyze the bibliometric data, facilitating the mapping of co-author networks, institutional collaborations, and the identification of main research directions. Through this tool, 730 papers from the Web of Science database covering the period 2010–2024 were extracted and analyzed. The study highlights the authors and institutions that have made significant contributions to the investigation of the relationship between financial development and climate change. The identification of key contributors and international collaborative networks provides a solid foundation for future research and policy initiatives. In addition, the study identifies the most prolific journals and assesses the quality and impact of the research. This allows for a deeper understanding of current research directions and potential future developments. The study not only clarifies the current state of research but also opens up new opportunities for investigating innovative and sustainable solutions aimed at improving the quality of life and protecting the environment.

BCL11A intellectual developmental disorder: defining the clinical spectrum and genotype-phenotype correlations.

An increasing number of individuals with intellectual developmental disorder (IDD) and heterozygous variants in BCL11A are identified, yet our knowledge of manifestations and mutational spectrum is lacking. To address this, we performed detailed analysis of 42 individuals with BCL11A-related IDD (BCL11A-IDD, a.k.a. Dias-Logan syndrome) ascertained through an international collaborative network, and reviewed 35 additional previously reported patients. Analysis of 77 affected individuals identified 60 unique disease-causing variants (30 frameshift, 7 missense, 6 splice-site, 17 stop-gain) and 8 unique BCL11A microdeletions. We define the most prevalent features of BCL11A-IDD: IDD, postnatal-onset microcephaly, hypotonia, behavioral abnormalities, autism spectrum disorder, and persistence of fetal hemoglobin (HbF), and identify autonomic dysregulation as new feature. BCL11A-IDD is distinguished from 2p16 microdeletion syndrome, which has a higher incidence of congenital anomalies. Our results underscore BCL11A as an important transcription factor in human hindbrain development, identifying a previously underrecognized phenotype of a small brainstem with a reduced pons/medulla ratio. Genotype-phenotype correlation revealed an isoform-dependent trend in severity of truncating variants: those affecting all isoforms are associated with higher frequency of hypotonia, and those affecting the long (BCL11A-L) and extra-long (-XL) isoforms, sparing the short (-S), are associated with higher frequency of postnatal microcephaly. With the largest international cohort to date, this study highlights persistence of fetal hemoglobin as a consistent biomarker and hindbrain abnormalities as a common feature. It contributes significantly to our understanding of BCL11A-IDD through an extensive unbiased multi-center assessment, providing valuable insights for diagnosis, management and counselling, and into BCL11A's role in brain development.

Exploring innovative approaches to fundraising for palliative care

Palliative care is a holistic approach aimed at improving the quality of life for those facing life-limiting illnesses and their families. Palliative care services often face financial constraints that restrict their ability to reach all those in need. There are difficulties accessing traditional funding mechanisms; therefore, innovative fundraising strategies are required to sustain and expand palliative care initiatives. In this paper, diverse fundraising approaches for palliative care are described, based on group discussions and collective brainstorming sessions by clinicians undergoing a Fellowship in Palliative Care. Fundraising approaches that were deemed innovative, yet feasible to apply in each locality, were shortlisted and described, with their strengths and challenges outlined in detail. These approaches include art and photography exhibitions, “piggy-bank” projects, community-based fundraising, charity shops, lottery and lucky draws, shuttle bus services, grants from international organizations and collaborative networks, the use of tap-on technology, festive events, and restaurant partnerships. It is hoped that through these innovative approaches, collaborative efforts, strategic planning, and resource mobilization, it would be possible to generate funds to develop and provide palliative care services for those in need to fulfill a fundamental human right.

Sex Differences in Frequency, Severity, and Distribution of Cerebral Microbleeds

Cerebral small vessel disease (SVD) is associated with various cerebrovascular outcomes, but data on sex differences in SVD are scarce. To investigate whether the frequency, severity, and distribution of cerebral microbleeds (CMB), other SVD markers on magnetic resonance imaging (MRI), and outcomes differ by sex. This cohort study used pooled individual patient data from the Microbleeds International Collaborative Network, including patients from 38 prospective cohort studies in 18 countries between 2000 and 2018, with clinical follow-up of at least 3 months (up to 5 years). Participants included patients with acute ischemic stroke or transient ischemic attack with available brain MRI. Data were analyzed from April to December 2023. Outcomes of interest were presence of CMB, lacunes, and severe white matter hyperintensities determined on MRI. Additionally, mortality, recurrent ischemic stroke, and intracranial hemorrhage during follow-up were assessed. Multivariable random-effects logistic regression models, Cox regression, and competing risk regression models were used to investigate sex differences in individual SVD markers, risk of recurrent cerebrovascular events, and death. A total of 20 314 patients (mean [SD] age, 70.1 [12.7] years; 11 721 [57.7%] male) were included, of whom 5649 (27.8%) had CMB. CMB were more frequent in male patients, and this was consistent throughout different age groups, locations, and in multivariable models (female vs male adjusted odds ratio [aOR], 0.86; 95% CI, 0.80-0.92; P < .001). Female patients had fewer lacunes (aOR, 0.82; 95% CI, 0.74-0.90; P < .001) but a higher prevalence of severe white matter hyperintensities (aOR, 1.10; 95% CI, 1.01-1.20; P = .04) compared with male patients. A total of 2419 patients (11.9%) died during a median (IQR) follow-up of 1.4 (0.7-2.5) years. CMB presence was associated with a higher risk of mortality in female patients (hazard ratio, 1.15; 95% CI, 1.02-1.31), but not male patients (hazard ratio, 0.95; 95% CI, 0.84-1.07) (P for interaction = .01). A total of 1113 patients (5.5%) had recurrent ischemic stroke, and 189 patients (0.9%) had recurrent intracranial hemorrhage, with no sex differences. This cohort study using pooled individual patient data found varying frequencies of individual SVD markers between female and male patients, indicating potential pathophysiological differences in manifestation and severity of SVD. Further research addressing differences in pathomechanisms and outcomes of SVD between female and male patients is required.

Mapping the evolution and impact of ketogenic diet research on diabetes management: a comprehensive bibliometric analysis from 2005 to 2024.

The ketogenic diet (KD) has been explored for diabetes management; however, a quantitative synthesis of its specific effects on diabetes has not yet been conducted. This study aims to examine the current status and research hotspots of KD in diabetes management from 2005 to 2024, providing a reference for future research. We retrieved articles published between 2005 and 2024 from the Web of Science database and analyzed them using R software, VOSviewer, and CiteSpace. This study includes 432 relevant publications. From 2005 to 2024, the volume of literature in this field has shown a steady upward trend, with a notable increase from 2017 to 2021, and a slight decline observed from 2021 to 2023. The United States is the leading country in terms of the number of publications, followed by China, Australia, and Canada. The United States not only leads in publication volume but also maintains a broader international collaboration network. Nutrients and the American Journal of Clinical Nutrition are the most frequently published and cited journals. Current research hotspots primarily focus on the impact of KD on blood glucose control, insulin resistance, and lipid metabolism in diabetic patients. Mechanistic studies on KD in diabetes management concentrate on aspects such as the "regulation of genes by β-hydroxybutyrate," "anti-inflammatory effects," and "oxidative stress." The role of the gut microbiome is also emerging as an important research area. Currently, exploring the application of KD in managing different age groups and types of diabetes has become a significant research trend. As an emerging dietary intervention, KD is gradually attracting widespread attention from researchers around the world and is expected to become a major research focus in the future for diabetes management and control. This paper provides a systematic review and analysis of the current research status and hotspots of KD in diabetes management, offering important references and insights for future research in related fields.

A bibliometric analysis of research on the variability of precipitation over the Antarctic Peninsula

AbstractRegional warming rates experienced in the Antarctic Peninsula since the mid-twentieth century, linked to global climate change, have been amongst the world's fastest. The majority of studies of change in this region have focused on temperature, and while precipitation is also predicted to change (both in form and quantity) in the models, fewer studies have set out to document and test this prediction. In this study, we examined trends in research publications on precipitation variability over the Antarctic Peninsula from 1990 to 2023 using the Web of Science Core Collection database. A total of 86 relevant papers were retained and used to identify patterns in scientific outputs. VOSviewer and Bibliometrix software packages were used to illustrate the subject content of and trends in publications retrieved by key word analysis. Our findings revealed a positive trend in the number of papers published by year. Within the analysed period, research on precipitation variability in the Antarctic Peninsula region was initiated by a study of Turner and colleagues from 1997. The UK and US research communities were the two largest contributors to this field of Antarctic research globally, with their researchers also holding strong positions within international collaborative networks.

Research trends and hot topics of wearable sensors in wound care over past 18 years: A bibliometric analysis

ObjectiveThis study determined the development trends, analyzed collaboration networks, and identified research hotspots in the field of wearable sensors for wound care from 2007 to 2024 using a rigorous bibliometric analysis approach. MethodsBibliometric and scientometric analyses were performed utilizing data sourced from the Web of Science Core Collection database. This study examined publication trends, contributions from various countries and institutions, author productivity, keyword prevalence, and citation patterns to discern research hotspots and potential future avenues in the application of wearable sensors for wound care. ResultsThis study included 1177 articles, which demonstrated a marked increase in publications since 2016 and underscores the burgeoning interest in wearable sensors for wound care. China and the United States have emerged as prominent contributors to the research field, exhibiting numerous international collaborations. An analysis of keywords and citation bursts highlighted wound healing, hydrogels, and sensors as the key research foci with recent trends shifting towards the integration of wearable technology with advanced materials and artificial intelligence for advanced wound management. The research landscape is characterized by a diverse network of international collaborations and an emphasis on interdisciplinary approaches that integrate materials science, sensor technology, and clinical applications. ConclusionThe utilization of wearable sensors in wound care constitutes a rapidly progressing area of research, garnering significant interest and promising avenues for future advances. The integration of wearable sensors with advanced materials and AI technologies presents a frontier of opportunity for innovating wound care methodologies, enhancing patient outcomes, and optimizing the allocation of healthcare resources.

Multilateral collaborative governance of Arctic shipping safety: Examining the impact of the Arctic Council via collaborative network analysis

Global climate change has dramatically reduced Arctic sea ice, opening new potential shipping routes, which has garnered international attention. This study explores the role of the Arctic Council in the collaborative governance network focused on Arctic shipping safety. For this purpose, a collection of 49 collaborative events initiated by international organisations and the Arctic Council was used as the data sample. These events are divided into two groups, one containing all the events and the other excluding those initiated by the Arctic Council. As a result, two collaborative networks are developed and evaluated based on network density, average path length, network transitivity, centrality indices (degree, closeness, betweenness), and clustering analysis. The results demonstrate that the Arctic Council significantly enhances the network density and transitivity of the international collaboration network, and reshapes core-periphery dynamics and cohesive subgroup structures. For instance, under the influence of the Arctic Council, Canada, France, and Japan surpass the United Kingdom in centrality (degree, closeness, betweenness). Results of the clustering coefficients reveal the Arctic Council's impact on connections among states, leading to shifts in collaboration patterns. Additionally, subgroup cohesion and density coefficients confirm the Arctic Council's role in promoting collaboration among states and restructuring governance networks.

Methods for causality assessment of idiosyncratic drug-induced liver injury.

The diagnosis of idiosyncratic drug-induced liver injury (DILI) is a challenging task due to the lack of specific features or definitive diagnostic tools. A minimum of clinical and pharmacological information is required, together with laboratory and imaging tests to exclude other causes of liver injury. Several standardized methods have been developed to support clinical judgement and establish causality assessment, the most widely used being the Roussel Uclaf Causality Assessment Method-RUCAM-and structured Expert Opinion. More recently, an evidence-based, revised RUCAM, Electronic Causality Assessment Method-RECAM-has been developed and, although still a work in progress, may replace RUCAM scoring in the future. International collaborative networks and ongoing research efforts are key to advancing biomarker qualification and validation and developing new invitro patient-based methods that will help improve DILI diagnosis and move towards a personalized medicine approach.

Factors influencing the research impact in cancer research: a collaboration and knowledge network analysis

BackgroundCancer is a major public health challenge globally. However, little is known about the evolution patterns of cancer research communities and the influencing factors of their research capacity and impact, which is affected not only by the social networks established through research collaboration but also by the knowledge networks in which the research projects are embedded.MethodsThe focus of this study was narrowed to a specific topic – 'synthetic lethality’ – in cancer research. This field has seen vibrant growth and multidisciplinary collaboration in the past decade. Multi-level collaboration and knowledge networks were established and analysed on the basis of bibliometric data from ‘synthetic lethality’-related cancer research papers. Negative binomial regression analysis was further applied to explore how node attributes within these networks, along with other potential factors, affected paper citations, which are widely accepted as proxies for assessing research capacity and impact.ResultsOur study revealed that the synthetic lethality-based cancer research field is characterized by a knowledge network with high integration, alongside a collaboration network exhibiting some clustering. We found significant correlations between certain factors and citation counts. Specifically, a leading status within the nation-level international collaboration network and industry involvement were both found to be significantly related to higher citations. In the individual-level collaboration networks, lead authors’ degree centrality has an inverted U-shaped relationship with citations, while their structural holes exhibit a positive and significant effect. Within the knowledge network, however, only measures of structural holes have a positive and significant effect on the number of citations.ConclusionsTo enhance cancer research capacity and impact, non-leading countries should take measures to enhance their international collaboration status. For early career researchers, increasing the number of collaborators seems to be more effective. University–industry cooperation should also be encouraged, enhancing the integration of human resources, technology, funding, research platforms and medical resources. Insights gained through this study also provide recommendations to researchers or administrators in designing future research directions from a knowledge network perspective. Focusing on unique issues especially interdisciplinary fields will improve output and influence their research work.

Dispersal of a wolf with complex ancestry from the Dinaric–Alpine region to Germany demonstrates the value of transboundary monitoring networks

AbstractLarge carnivores such as wolves (Canis lupus) at times disperse distances of several hundred kilometers, which may enable gene flow over vast distances. Such long‐distance dispersal events, however, often remain undetected, and documenting long‐distance dispersers and their movements typically depend on strong transboundary collaborations. Here, we report the dispersal of a wolf (MSV0FF) from the Alpine region of northern Slovenia to Bavaria in southern Germany, a straight‐line distance of around 300 km. The disperser originated in a pack with a genetically diverse breeding pair, where the father exhibited ancestry from the Dinaric and Italian Alpine populations. Genetic analysis of the mother indicated that she was an immigrant from a divergent population further south in the Dinaric‐Balkan region. Such a varied ancestry augments the probability of immigrants being genetically misclassified as wolf‐dog hybrids in their area of arrival, which increases the risk of unfavorable management decisions toward individuals that would be genetically highly valuable for the recipient population. Wolf MSV0FF therefore demonstrates the benefits of international collaborative monitoring networks and the value of sharing samples and analytical approaches for the monitoring of wide‐ranging species.

Research on acupuncture and glial cells: A bibliometric analysis.

There are a growing number of studies on the effect of acupuncture on glial cells in the central nervous system; however, there are few related bibliometric analyses in this area. Therefore, the purpose of this bibliometric study was to visualize the literature on acupuncture-regulated glial cells. On November 23, 2022, regular and review articles on acupuncture and glial cell-related research were retrieved from the Web of Science Core Collection database. The R package "bibliometrix" was used to summarize the main findings, count the occurrences of the top keywords, visualize the international collaboration network, and generate a 3-field plot. The VOSviewer software was used to conduct both co-authorship and co-occurrence analyses. CiteSpace was used to identify the best references and keywords with the highest citation rates. Overall, 348 publications on acupuncture and glial cells were included. The publications were primarily from China, Korea, and the United States of America. The majority of publications were found in relevant journals. Apart from "acupuncture" and "glial cells," the most frequently used keywords were "neuroinflammation," "hyperalgesia," and "pain." This bibliometric study mapped a fundamental knowledge structure comprising countries, institutions, authors, journals, and articles in the research fields of acupuncture and glial cells over the last 3 decades. These results provide a comprehensive perspective on the wider landscape of this research area.

Mapping the Environmental Co-Benefits of Reducing Low-Value Care: A Scoping Review and Bibliometric Analysis.

Reducing low-value care (LVC) and improving healthcare's climate readiness are critical factors for improving the sustainability of health systems. Care practices that have been deemed low or no-value generate carbon emissions, waste and pollution without improving patient or population health. There is nascent, but growing, research and evaluation to inform practice change focused on the environmental co-benefits of reducing LVC. The objective of this study was to develop foundational knowledge of this field through a scoping review and bibliometric analysis. We searched four databases, Medline, Embase, Scopus and CINAHL, and followed established scoping review and bibliometric analysis methodology to collect and analyze the data. A total of 145 publications met the inclusion criteria and were published between 2013 and July 2023, with over 80% published since 2020. Empirical studies comprised 21%, while commentary or opinions comprised 51% of publications. The majority focused on healthcare generally (27%), laboratory testing (14%), and medications (14%). Empirical publications covered a broad range of environmental issues with general and practice-specific 'Greenhouse gas (GHG) emissions', 'waste management' and 'resource use' as most common topics. Reducing practice-specific 'GHG emissions' was the most commonly reported environmental outcome. The bibliometric analysis revealed nine international collaboration networks producing work on eight key healthcare areas. The nineteen 'top' authors were primarily from the US, Australia and Canada.

Trends and hotspots in gastrointestinal neoplasms risk assessment: A bibliometric analysis from 1984 to 2022.

Gastrointestinal neoplasm (GN) significantly impact the global cancer burden and mortality, necessitating early detection and treatment. Understanding the evolution and current state of research in this field is vital. To conducts a comprehensive bibliometric analysis of publications from 1984 to 2022 to elucidate the trends and hotspots in the GN risk assessment research, focusing on key contributors, institutions, and thematic evolution. This study conducted a bibliometric analysis of data from the Web of Science Core Collection database using the "bibliometrix" R package, VOSviewer, and CiteSpace. The analysis focused on the distribution of publications, contributions by institutions and countries, and trends in keywords. The methods included data synthesis, network analysis, and visualization of international collaboration networks. This analysis of 1371 articles on GN risk assessment revealed a notable evolution in terms of research focus and collaboration. It highlights the United States' critical role in advancing this field, with significant contributions from institutions such as Brigham and Women's Hospital and the National Cancer Institute. The last five years, substantial advancements have been made, representing nearly 45% of the examined literature. Publication rates have dramatically increased, from 20 articles in 2002 to 112 in 2022, reflecting intensified research efforts. This study underscores a growing trend toward interdisciplinary and international collaboration, with the Journal of Clinical Oncology standing out as a key publication outlet. This shift toward more comprehensive and collaborative research methods marks a significant step in addressing GN risks. This study underscores advancements in GN risk assessment through genetic analyses and machine learning and reveals significant geographical disparities in research emphasis. This calls for enhanced global collaboration and integration of artificial intelligence to improve cancer prevention and treatment accuracy, ultimately enhancing worldwide patient care.

Growth and Trends of Geometrical Optics Research Using Scientometrics Indicators

ABSTRACT Geometric Optics is a rapidly-evolving field with implications for a broad range of applications, from biomedical imaging to advanced materials engineering. This comprehensive bibliometric analysis presents an in-depth examination of global research in Geometric Optics from 2012–2021, using data from the Scopus database. The analysis reveals a total of 5,388 publications authored by 15,277 individuals, with a steady annual growth rate of 3.7% and an impressive citation impact of 11.99% per paper. The top 20 productive authors are identified, who contributed to 18.5% of global publications and 11.1% of citations, and the international collaboration networks that have emerged around key research areas are mapped. The study also reveals an expanding research landscape, with emerging areas such as nanophotonics and metamaterials gaining traction. These findings provide a comprehensive overview of the global research landscape in Geometric Optics, which can be used to guide future research and collaborative efforts. The study represents a valuable resource for researchers, practitioners, and policymakers alike, seeking to advance the field and unlock its full potential.

Anti-T-lymphocyte globulin improves GvHD-free and relapse-free survival in myelofibrosis after matched related or unrelated donor transplantation

Acute and chronic graft-versus-host disease (GvHD) are major complications of allogeneic hematopoietic cell transplantation (alloHCT). In vivo T-cell depletion with anti-T-lymphocyte globulin (ATLG) as part of the conditioning regimen prior to alloHCT is frequently used as GvHD prophylaxis, but data on its role in myelofibrosis is scarce. We took advantage of an international collaborative network to investigate the impact of ATLG in myelofibrosis undergoing first alloHCT. We included 707 patients (n = 469 ATLG and n = 238 non-ATLG prophylaxis). The cumulative incidence of acute GvHD grade II-IV was 30% for the ATLG group vs. 56% for the non-ATLG group (P < 0.001). Acute GvHD grade III-IV occurred in 20% vs. 25%, respectively (P = 0.01). Incidence of mild-to-severe chronic GvHD was 49% vs. 50% (P = 0.52), while ATLG showed significantly lower rates of severe chronic GvHD (7% vs. 18%; P = 0.04). GvHD-free and relapse-free survival (GRFS) at 6 years was 45% for the ATLG group vs. 37% for the non-ATLG group (P = 0.02), driven by significantly improved GRFS of ATLG in matched related and matched unrelated donors. No significant differences in risk for relapse, non-relapse mortality, and overall survival were observed. Multivariable modeling for GRFS showed a 48% reduced risk of GvHD, relapse, or death when using ATLG.

The influence of collaborators and knowledge diversity on innovation performance of tourism and hospitality academia network

In the knowledge economy era, there is a growing global emphasis on the impact of international scientific collaboration networks (SCNs) on academic innovation. Based on a cross-level analysis perspective, this study constructs a dual-stage cross-level moderated mediation model and tests the hypotheses related to scientific collaboration, knowledge diversity, and innovation performance. The data were collected from the tourism and hospitality academia publications from 1983 to 2018, involving 79 groups of 303 academics from China. This study indicates that knowledge diversity positively mediates the relationship between the number of collaborators and tourism academics’ innovation performance. Furthermore, in complex SCNs, network relationship scale and team collaboration density have cross-level moderated effects on this process.

Global research trends in Total Body Irradiation: a bibliometric analysis.

This manuscript presents a bibliometric and visualization analysis of Total Body Irradiation (TBI) research, aiming to elucidate trends, gaps, and future directions in the field. This study aims to provide a comprehensive overview of the global research landscape of TBI, highlighting its key contributions, evolving trends, and potential areas for future exploration. The data for this study were extracted from the Web of Science Core Collection (WoSCC), encompassing articles published up to May 2023. The analysis included original studies, abstracts, and review articles focusing on TBI-related research. Bibliometric indicators such as total publications (TP), total citations (TC), and citations per publication (C/P) were utilized to assess the research output and impact. Visualization tools such as VOS Viewer were employed for thematic mapping and to illustrate international collaboration networks. The analysis revealed a substantial body of literature, with 7,315 articles published by 2,650 institutions involving, 13,979 authors. Full-length articles were predominant, highlighting their central role in the dissemination of TBI research. The authorship pattern indicated a diverse range of scholarly influences, with both established and emerging researchers contributing significantly. The USA led in global contributions, with significant international collaborations observed. Recent research trends have focused on refining TBI treatment techniques, investigating long-term patient effects, and advancing dosimetry and biomarker studies for radiation exposure assessments. TBI research exhibits a dynamic and multifaceted landscape, driven by global collaboration and innovation. It highlights the clinical challenges of TBI, such as its adverse effects and the need for tailored treatments in pediatric cases. Crucially, the study also acknowledges the fundamental science underpinning TBI, including its effects on inflammatory and apoptotic pathways, DNA damage, and the varied sensitivity of cells and tissues. This dual focus enhances our understanding of TBI, guiding future research toward innovative solutions and comprehensive care.

Inborn Errors of Immunity in Jordan: First Report from a Tertiary Referral Center.

Inborn errors of immunity (IEI) are a heterogeneous group of diseases with variable clinical phenotypes. This study was conducted to describe the epidemiology, clinical presentations, treatment, and outcome of IEI in Jordanian children. A retrospective data analysis was conducted for children under 15 years diagnosed with IEI from the pediatric Allergy, Immunology, and Rheumatology Division-based registry at Queen Rania Children's Hospital, Amman, Jordan, between 2010 and 2022. A total of 467 patients, 263 (56.3%) males and 204 (43.7%) females, were diagnosed with IEI. The mean age at symptom onset was 18 months (1 week to 144 months), a positive family history of IEI was reported in 43.5%, and the consanguinity rate was 47.9%. The most common IEI category was immunodeficiencies affecting cellular and humoral immunity at 33.2%, followed by predominantly antibody deficiencies at 16.9%. The overall median diagnostic delay (range) was 6 (0-135) months; patients with a positive family history of IEI had a statistically significant shorter diagnostic delay. Pulmonary and gastrointestinal clinical features were the most common at 55.2% and 45.6%, respectively. The overall mortality was 33.2%; the highest rate was reported in severe combined immunodeficiency at 56.2%. The high minimal estimated IEI prevalence at 16.2/100,000 Jordanian children compared to the regional and worldwide data, with the diversities in clinical presentation and distribution of IEI categories in our cohort point to unique features of IEI in Jordanian children, call for national registry establishment, regional and international collaborative networks.

Association between arterial oxygen and mortality across critically ill patients with hematologic malignancies: results from an international collaborative network.

Patients with hematological malignancies are at high risk for life-threatening complications. To date, little attention has been paid to the impact of hyperoxemia and excess oxygen use on mortality. The aim of this study was to investigate the association between partial pressure of arterial oxygen (PaO2) and 28-day mortality in critically ill patients with hematologic malignancies. Data from three international cohorts (Europe, Canada, Oceania) of patients who received respiratory support (noninvasive ventilation, high-flow nasal cannula, invasive mechanical ventilation) were obtained. We used mixed-effect Cox models to investigate the association between day one PaO2 or excess oxygen use (inspired fraction of oxygen ≥ 0.6 with PaO2 > 100mmHg) on day-28 mortality. 11,249 patients were included. On day one, 5716 patients (50.8%) had normoxemia (60 ≤ PaO2 ≤ 100mmHg), 1454 (12.9%) hypoxemia (PaO2 < 60mmHg), and 4079 patients (36.3%) hyperoxemia (PaO2 > 100mmHg). Excess oxygen was used in 2201 patients (20%). Crude day-28 mortality rate was 40.6%. There was a significant association between PaO2 and day-28 mortality with a U-shaped relationship (p < 0.001). Higher PaO2 levels (> 100mmHg) were associated with day-28 mortality with a dose-effect relationship. Subgroup analyses showed an association between hyperoxemia and mortality in patients admitted with neurological disorders; however, the opposite relationship was seen across those admitted with sepsis and neutropenia. Excess oxygen use was also associated with subsequent day-28 mortality (adjusted hazard ratio (aHR) [95% confidence interval (CI)]: 1.11[1.04-1.19]). This result persisted after propensity score analysis (matched HR associated with excess oxygen:1.31 [1.20-1.1.44]). In critically-ill patients with hematological malignancies, exposure to hyperoxemia and excess oxygen use were associated with increased mortality, with variable magnitude across subgroups. This might be a modifiable factor to improve mortality.