bluuebunny/crossref_metadata_2025_split huggingface dataset:
Tyrosine Kinase Inhibitors Short Exposure in Pregnant Female Patients with Chronic Myeloid Leukemia
Authors: Zu Yingling , Zhao Huifang , Chen Jianling , Dang Huibing , Shi Yanrong , Liang Lixin , Mei Shuhao , Lu Wenyi , Zhou Jian , Song Yongping , Zhang Yanli 
Published: December 2024

Introduction In the era of tyrosine kinase inhibitor (TKI), improving the quality of life has become the main therapeutic goal for patients with chronic myeloid leukemia (CML). Unintended pregnancy for female patients with chronic myeloid leukemia (CML) raises the discussion of treatment choices due to the teratogenicity of tyrosine kinase inhibitor (TKI). Method We reported 51 accidental pregnant CML chronic phase (CP) patients with TKI withdrawal immediately after pregnancy from December 2010 to February 2024 to observe the effect of short exposure to TKI on the fetus and the infant outcomes.
Results 59 pregnancies resulted in 100% normal childbirth without birth abnormalities. The median TKI exposure duration was 4 (4-20) weeks in 58 pregnancies, and one pregnancy avoided TKI exposure due to treatment discontinuation of the patient with treatment-free remission (TFR). All newborns had normal birth weight except one premature infant with low birth weight less than the 10th percentile. Up to now, all the children are in good health. 13 (25.5%) and 30 (58.8%) patients had achieved major molecular response (MMR) and deep molecular response (DMR) at pregnancy, respectively. After TKI discontinuation, loss of MMR and complete hematologic response occurred in 6 (11.8%) and 2 (3.9%) patients at delivery, respectively. 38 patients resumed TKI treatment after delivery, and 13 patients sustained TFR after delivery. The median time to regain MMR and DMR were 3 (2-6) months and 6 (1-28) months, respectively.
Conclusion These results demonstrate that TKI discontinuation during pregnancy is feasible for CML-CP patients, and short TKI exposure of pregnant patients has little influence on children's growth and development.
Clinical Outcomes for Female Patients Following Hematopoietic Stem Cell Transplant: An Analysis of the National Inpatient Sample
Authors: Thor Danielle C , Elias Tony , Polineni Vineet , Brik Ben 
Published: December 2024

Background: As hematopoietic stem cell transplant (HSCT) grows in accessibility and utility, it remains imperative to understand outcomes for its recipients. Limited data is available on the sex-based correlations for this steadily increasing patient population. We sought to examine the National Inpatient Sample (NIS) database to describe in-hospital outcomes among the female population who have received a HSCT.
Methods: Data were extracted from the NIS Database from 2019 and 2020. The NIS was searched for hospitalizations of all patients greater than 18 years old who had previously received a HSCT and were hospitalized thereafter. We then analyzed the outcomes of female patients. Multivariate logistic regression analysis was used to adjust for confounders; SPSS software was used for statistical analysis. The primary outcome was inpatient mortality and secondary outcomes are listed below.
Results: This study included 8,302 patients who had received a HSCT, of which 3636 (43.8%) were identified as female. Female recipients of a HSCT had a slightly higher prevalence of obesity (7.5% versus 7.0%, p&lt;0.001), as well as lower prevalence of smoking (16.5% versus 20.5%, p&lt;0.001), hypertension (21.8% versus 23.7%, p&lt;0.001), and chronic kidney disease (19.3% versus 21.3%, p&lt;0.001). Multivariate regression demonstrated that female recipients of a HSCT had higher inpatient mortality (OR 1.721, CI 1.572-1.884, p&lt;0.001). On secondary analysis, female HSCT recipients were found to be more likely to have anemia (OR 1.790, CI 1.746-1.836, p&lt;0.001), thrombocytopenia (OR 1.692, CI 1.621-1.765, p&lt;0.001), hypertension (OR 1.674, CI 1.615-1.736, p&lt;0.001), acute kidney failure (OR 1.698, CI 1.635-1.764, p&lt;0.001), all-cause arrhythmias (OR 1.588, CI 1.502-1.680, p&lt;0.001), all-cause heart block (OR 1.441, CI 1.323-1.570, p&lt;0.001), all-cause sepsis (OR 1.859, CI 1.789-1.932, p&lt;0.001), all-cause coagulopathies (OR 1.744, CI 1.605-1.895, p&lt;0.001), and acute heart failure (OR 1.734, CI 1.658-1.815, p&lt;0.001).
Conclusion: In this nationally-representative, population-based retrospective cohort study, female patients who have received a HSCT and were subsequently hospitalized were associated with higher mortality and worse outcomes.
Efficacy and Safety of Mepolizumab in Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta-Analysis
Authors: Ejaz Hassan , Sajjad Maha , Hasan Sajjad Ul , Khan Malik W.Z. , Hussain Amna , Ali Aizaz , Azeem Touba , Afridi Abdullah , Sajjad Ayema , Javed Muhammad Arsham , Qudrat Salma , Khan Salman , Niazi Muhammad Raphay , Yetiskul Ekrem , Ali Shandana 
Published: December 2024

Introduction:
Eosinophilic granulomatosis with polyangiitis (EGPA), known previously as Churg-Strauss syndrome, is a systemic vasculitis involving small to medium-sized vessels. Corticosteroids and immunosuppressive therapies historically managed EGPA, but these therapies are often associated with adverse effects and a high likelihood of relapse. Recently, mepolizumab (MPZ), a monoclonal antibody that targets interleukin-5 (IL-5) has shown promising results, especially for those EGPA patients who are refractory to conventional corticosteroid therapy.
Methods:
A systematic literature search was conducted on PubMed, Cochrane Library, Science Direct, Google Scholar, ClinicalTrials.gov, and the American Society of Hematology (ASH) from inception to June 01, 2024, using MeSH terms and keywords used for “Churg-Strauss syndrome”, “EGPA”, “Eosinophilic Granulomatosis with Polyangiitis”, “Allergic Granulomatosis”, and “Mepolizumab”. Clinical remission and adverse effects were the primary outcomes. Secondary outcomes were prednisolone-free remission, serious adverse effects, relapses, and asthma exacerbations. The random-effects model with the Freeman Tukey double arcsine transformation was used to pool overall prevalence with 95% CI for dichotomous variables in MetaXL version 5.3.
Results:
Seventeen studies were included (n = 1,075 patients). Nine studies (178 out of 311 patients) reported data on clinical remission with a pooled prevalence of 65% (95% CI: 49%-79%, I2=86%). Twelve studies (385 out of 853 patients) reported data on any adverse effects after treatment with a pooled prevalence of 52% (95% CI: 25%-77%, I2 =98%). Three studies reported remission in both 100 mg and 300 mg subgroups with a pooled prevalence of 50% (95% CI: 22%-78%, I2 =85%) and 72% (95% CI: 56%-86%, I2 =7%) respectively. Seven studies (73 out of 238 patients) reported prednisolone-free remission with a pooled prevalence of 32% (95% CI: 21%-43%, I2=65%).
Conclusion:
The systematic review and meta-analysis suggests that MPZ is effective and safe for treating patients with EGPA. Subgroup analysis by dosage regimen indicated that 100 mg could be more effective for inducing prednisolone-free remission with fewer serious adverse effects and fewer relapses.
Retention of Young Adults with Sickle Cell Disease in Outpatient Adult Care
Authors: Pavkov Isabela , Kutlar Abdullah , Gollan Siera 
Published: December 2024

Introduction
As the life expectancy of people with sickle cell disease (SCD) increases (Payne 2020), focus has been on preparing the transition from pediatric to adult care, with success measured as attendance at the first adult appointment. The handoff to adult care typically marks the end of transition support. However, people with SCD aged 25-35 have the highest rates of healthcare utilization (Benenson 2017). Little is known about how to retain young adults in adult care in the years following transition. Our objective is to examine current literature for strategies used by adult clinics to support young adults with SCD to remain engaged in specialist care after the first adult appointment.
Methods
Following guidelines established by Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) (Tricco 2018), we reviewed the literature for interventions aimed at retaining young adults (age 18-24) with SCD in outpatient care after their first adult appointment following transition from pediatric care. We queried multiple online databases (PubMed, CINAHL, Web of Science) for articles published between January 2010 to April 2024. Articles did not need to include success of intervention. The initial search with focus on SCD returned limited findings, prompting us to broaden the review and include care continuity of young adults with any chronic illness.
Results
The scoping review identified several studies which employ an intervention to retain young adults with chronic illnesses in adult specialist care. Some studies show success with co-located adult and pediatric care for retention of patients with SCD and human immunodeficiency virus. Other institutions employed a transition navigator who provides pre-transition support and limited post-transition follow-up. A study including young adults with any chronic physical illness used a mobile app to foster engagement with adult healthcare services.
Conclusions
The number of studies documenting interventions for retaining young adults with SCD in outpatient care following the initial adult visit is severely limited. While it is possible that more interventions exist that are not published, it is not unreasonable to consider applying interventions from other chronic illnesses to SCD. However, the approach with the most success, that of the co-location model, may not be feasible for most institutions. Technology-based interventions, such as mobile applications, would likely be more widely implementable. More interventions to ensure young adults engage with adult outpatient specialty care are much needed. Continuity of care in young adults with any chronic illness is important, and it is especially necessary in the SCD population. Adults with SCD require long-term specialist care to minimize the devastating complications that characterize the natural course of their disease and elevate quality of life.
Dynamic regulation of proximal tubular autophagy from injury to repair after ischemic kidney damage
Authors: Gong Yuhong , Zhu Wei , Li Yongqiang , Lu Tao , Tan Jiexing , He Changsheng , Yang Luodan , Zhu Yufeng , Gong Li 
Published: December 2024

Abstract
Background
The role of proximal tubular autophagy in repairing kidney injury following ischemia remains unclear.

Methods
In this study, we utilized mice with conditional deletion of the Atg5 gene in proximal tubules and monitored the long-term dynamic regulation of autophagy following ischemic acute kidney injury (AKI).

Results
The results showed that Atg5-deficient proximal tubule epithelial cells exhibited damaged mitochondria, concentric membranes, and lysosomal accumulation 24 h after ischemia/reperfusion. However, 28 days after ischemia/reperfusion, concentric membrane bodies remained, but lysosomal accumulation was no longer observed. Notably, the absence of Atg5 in renal tubular epithelial cells impaired renal function and led to increased tubular cell proliferation and oxidative stress in the early stage of injury. However, during the repair period following AKI, Atg5 deficiency exhibited no significant difference in the expression of proliferating cell nuclear antigen (PCNA) and 4-hydoxynonenal (4HNE), suggesting that the improvement in renal fibrosis associated with Atg5 deficiency is unlikely to result from its effect on cell proliferation or reactive oxygen species levels. Additionally, Atg5 deficiency inhibits the secretion of profibrotic factor fibroblast growth factor 2 (FGF2) from the early stage of renal injury to the recovery stage of AKI, indicating that autophagy-specific regulation of FGF2 secretion is a dynamic process overlapping with other stages of injury. Furthermore, increased co-localization of ATG5 with 4HNE and FGF2 was observed in patient samples.

Conclusion
In summary, our results suggest that the dynamic regulation of autophagy on key molecules involved in kidney injury and repair varies with the stage of kidney injury.

Graphical Abstract
A natural history study of pediatric patients with early onset of GM1 gangliosidosis, GM2 gangliosidoses, or gaucher disease type 2 (RETRIEVE)
Authors: Héron Bénédicte , Batzios Spyros , Mengel Eugen , Giugliani Roberto , Patterson Marc , Gautschi Matthias , Cornelisse Peter , Trokan Luba , Schwierin Barbara , Rohrbach Marianne 
Published: December 2024

Abstract
Background
The GM1 and GM2 gangliosidoses and type 2 Gaucher disease (GD2) are inherited lysosomal storage disorders with most cases having symptom onset in infancy and reduced life expectancy. The conditions are rare, and there is therefore a need for accurate and up to date information concerning the disease course and survival to assist in the design of clinical trials. RETRIEVE is a natural history study aiming to: (1) collect data on the survival of patients with early-onset (onset of first neurological manifestation before 24 months of age) GM1, GM2, or GD2; (2) collect data that could constitute a historical control group for future clinical trials; and (3) evaluate whether the conditions can be assessed together in a single interventional clinical trial. Group A included patients who were deceased or with unknown survival status at enrollment and was thus limited to retrospective data. Group B included patients who were alive at enrollment, who were followed prospectively with additional retrospective data collection.

Results
Group A included 185 patients (60 with GM1, 78 with GM2, and 47 with GD2), and Group B included 40 patients (18 with GM1, 16 with GM2, and 6 with GD2). Mean and median age at diagnosis and age at onset of first neurological manifestation were youngest in patients with GD2 and oldest in patients with GM2 in both groups. In Group A, median (95% CI) survival was 19.0 (18.0, 22.0), 44.0 (37.0, 51.9) and 14.0 (10.0, 16.0) months in patients with GM1, GM2 and GD2, respectively. In Group B, hypotonia was experienced by most patients with GM1 (17/18, 94.4%), and was less common in patients with GM2 (12/16, 75.0%) and GD2 (4/6, 66.7%). Strabismus and splenomegaly were reported in all six patients with GD2.

Conclusions
RETRIEVE is one of the largest natural history studies of GM1, GM2, and GD2. Results were generally consistent with the published literature, with differences potentially due to variation in inclusion criteria. The difference in median survival between patients with early-onset GM1, GM2, and GD2 reported in this study suggests that the three diseases should not be pooled for study in clinical trials.
Global longitudinal strain in pre-symptomatic patients with mutation for transthyretin amyloidosis
Authors: Canciello Grazia , Tozza Stefano , Todde Gaetano , Nolano Maria , Borrelli Felice , Palumbo Giovanni , Lombardi Raffaella , Cassano Emanuele , Acampa Wanda , Esposito Giovanni , Manganelli Fiore , Losi Maria Angela 
Published: December 2024

Abstract
Background
Hereditary transthyretin (ATTRv) amyloidosis is rare, autosomal dominant disease with a fatal outcome if left untreated. Early stages detection is crucial for intervention. We aimed identifying early indexes of cardiac involvement and their eventual correlation with neurological indexes, in pre-symptomatic subjects with TTR gene mutation.

Methods
Sixteen TTR-mutation carriers (mean age 51 ± 9 years, 6 males, 7 with Val30Met and 9 with Phe64Leu mutation) without left ventricular hypertrophy were studied. Predicted Age of Disease Onset (PADO) and time to PADO (Time-to PADO = PADO-age at evaluation) were computed. Subjects underwent: cardiological and echocardiographic assessment including global longitudinal strain (GLS); tactile and thermal quantitative sensory testing (QST); Perugini score by bone scintigraphy.

Results
Time to PADO was 30 ± 15 years. Nine subjects showed abnormal GLS (> −20%), unrelated to age, LVMi, MWT, E/e’, NT-proBNP or Time-to PADO. QST findings were abnormal in most subjects. At a worse cold pain threshold corresponded a worse GLS (r = 0.786, p < 0.001). Perugini score was positive in 1 subject.

Conclusions
GLS and QST findings support an early involvement of heart and small nerve fibers even many years before PADO. Interestingly, cardiac impairment seems to parallel that of small, nerve fibers, at least in the earliest stage of disease.
A child opportunity index in Italy: a pilot proposal
Authors: Ferrara Pietro , Cipolla Domenico , Corsello Giovanni , Lagalla Luca M. , Tantillo Matilde , Galione Giusyelisa , Martorana Chiara , Mazzone Teresa , Zona Margherita , Cammisa Ignazio 
Published: December 2024

Abstract
Background
The Child Opportunity Index (COI) is a new and innovative tool designed to assess the environment in which children grow up, offering a broad evaluation of the opportunities available to them in different neighborhoods. This initiative aims to ensure improvements in children’s living conditions and future health outcomes.

Methods
The study was performed in the cities of Palermo and Rome. Our Italian COI consists of three main domains: education, health and environment, and economy, each subdivided into specific indicators. We collected information, when available, useful for our indicators from institutional sites and municipal archives. Furthermore, in the city of Rome, we distributed a questionnaire through local pediatricians, collecting data in 2 randomly chosen neighborhoods with questions on children’s health and quality of life, proposing an initial approach that, when implemented using data provided by the government and public and private health institutions, aims to evaluate the correlation between socio-economic opportunities and the psycho-physical health of children, as demonstrated in the literature.

Results
As a result, many aspects, such as the rate of air pollution or the illegal occupation of houses, were not taken into consideration. We therefore consider our COI proposal only a starting model that will have to be implemented once all the necessary information has been obtained. However, what can be deduced from this first descriptive study is how the opportunities in different neighborhoods are not the same for all children. The number of educational opportunities as well as the number of environmental opportunities differs between the various districts and is not homogeneous between different cities or within the same city.

Conclusions
In conclusion, it is not simple to analyze in a scientific manner the child’s health impact of living in different areas. The COI could be a useful and simple tool that can give us this information. Pediatricians could collaborate with institutions to implement intervention plans and to reduce existing differences, social and health inequalities. Future studies will have to implement this pilot study to create and validate an Italian model of COI to be used as a useful tool in children’s assistance.
Assessment of vascular invasion of pancreatic ductal adenocarcinoma based on CE-boost black blood CT technique
Authors: Lin Yue , Liu Tongxi , Hu Yingying , Xu Yinghao , Wang Jian , Guo Sijia , Xie Sheng , Sun Hongliang 
Published: December 2024

Abstract
Objectives
To explore the diagnostic efficacy of advanced intelligent clear-IQ engine (AiCE) and adaptive iterative dose reduction 3D (AIDR 3D), combination with and without the black blood CT technique (BBCT), for detecting vascular invasion in patients diagnosed with nonmetastatic pancreatic ductal adenocarcinoma (PDAC).

Methods
A total of 35 consecutive patients diagnosed with PDAC, proceeding with contrast-enhanced abdominal CT scans, were enrolled in this study. The arterial and portal venous phase images were reconstructed using AiCE and AIDR 3D. The corresponding BBCT images were established as AiCE–BBCT and AIDR 3D–BBCT, respectively. Two observers scored the image quality independently. Cohen’s kappa (k) value or intraclass correlation coefficient (ICC) was used to analyze consistency. The diagnostic performance of four algorithms in detecting vascular invasion in PDAC patients was assessed using the area under the curve (AUC).

Results
The AiCE and AiCE–BBCT groups demonstrated superior image noise and diagnostic acceptability compared with AIDR 3D and AIDR 3D–BBCT groups (all p < 0.001), and the k value was 0.861–0.967 for both reviewers. In terms of diagnostic capability for vascular invasion in PDAC, the AiCE–BBCT group exhibited higher specificity (95.0%) and sensitivity (93.3%) compared to the AIDR 3D and AIDR 3D–BBCT groups, with an AUC of 0.942 (95% CI: 0.849–1.000, p < 0.05). Furthermore, all vascular evaluations conducted using AiCE–BBCT demonstrated better consistency (ICC: 0.847–0.935).

Conclusion
The BBCT technique in conjunction with AiCE could lead to notable enhancements in both the image quality of PDAC images and the diagnostic performance for tumor vascular invasion.

Critical relevance statement
Better diagnostic accuracy of vascular invasion of PDAC based on BBCT in combination with an AiCE is a critical factor in determining treatment strategies and patient outcomes.

Key Points

Identifying vascular invasion of PDAC is important for prognostication.

Combined images provide improved image quality and higher diagnostic accuracy.

Combined images can excellently display the vascular wall and invasion.

Graphical Abstract
Prevalence and molecular characterization of ESBL-producing Enterobacteriaceae in Egypt: a systematic review and meta-analysis of hospital and community-acquired infections
Authors: Azzam Ahmed , Khaled Heba , Samer Dareen , Nageeb Wedad M. 
Published: December 2024

Abstract
Background
ESBL-producing Enterobacteriaceae (ESBL-PE) represent a significant global health threat. In response to this growing concern and the lack of a surveillance system for ESBL-PE infections in Egypt, we conducted this meta-analysis. In this study, we aimed to quantify the prevalence of ESBL-PE based on the source of infection and characterize their molecular dissemination. Additionally, we sought to uncover temporal trends to assess the spread of ESBL-PE over time.

Methods
A comprehensive literature search was conducted in PubMed, Scopus, Google Scholar, Web of Science, and the Egyptian Knowledge Bank to identify studies that: (1) report the prevalence of ESBL-PE in Egypt; (2) use valid detection methods; (3) involve clinical specimens; and (4) were published between 2010 and 2024. The quality of the included studies was evaluated using the “Joanna Briggs Institute Critical Appraisal Checklist”. Meta-analysis was performed using the R meta package, reporting pooled prevalence with 95% confidence intervals (CI) via a random effects model.

Results
This meta-analysis included 34 studies with 4,528 isolates, spanning 2007 to 2023. The overall prevalence of ESBL-PE in Egypt was 60% (95% CI: 54–65). The leave-one-out meta-analysis demonstrated the absence of influential outliers and Egger’s test indicated no evidence of publication bias (P = 0.25). The prevalence of ESBL-PE was 62% (95% CI: 55–68) in nosocomial infections and 65% (95% CI: 52–75) in community-acquired infections, with no statistically significant difference (P = 0.68). The prevalence of ESBL producers in E. coli (64%) and K. pneumoniae (63%) is higher than in Proteus mirabilis (46%) (P = 0.06). Temporal analysis showed a stable ESBL prevalence over time. Moreover, in phenotypically confirmed ESBL-producing, E. coli harboring blaCTX−M was most prevalent (73%), followed by blaTEM (60%) and blaSHV (22%), with significant differences (P < 0.01). Subsequent analysis identified blaCTX−M−15 as the predominant variant of the blaCTX−M gene.

Conclusions
The prevalence of ESBL-PE in Egypt is alarmingly high at 60%. The observed high rates in both hospital and community-acquired infections underscore the need for public health strategies targeting both settings. One limitation of this study is the high heterogeneity, which partly attributed to regional and institutional variations in antibiotic use and stewardship practices.