bluuebunny/crossref_metadata_2025_split huggingface dataset:
What is in a name: defining pediatric refractory ITP
Authors: Nakano Taizo A. , Grimes Amanda B. , Klaassen Robert J. , Lambert Michele P. , Neunert Cindy , Rothman Jennifer A. , Shimano Kristin A. , Amend Christina , Askew Megan , Badawy Sherif M. , Baker Jillian M. , Breakey Vicky , Crary Shelley , Davini Monica , Fritch Lilla Stephanie , Gilbert Megan , Hays Taru , Hege Kerry , Hillier Kirsty , Jacobson-Kelly Amanda , Kaicker Shipra , Kim Taylor O. , Kochhar Manpreet , Leblanc Thierry , Martinelli Marie , Nunez Mara , Remiker Allison , Schultz Corrina , Sharma Ruchika , Grace Rachael F. 
Published: July 2024

Abstract
There are no agreed upon terminology to define “refractory” pediatric immune thrombocytopenia (ITP). Guidelines are therefore limited to arbitrary and outdated definitions. The Pediatric ITP Consortium of North America held a meeting in 2023 to define this entity. With 100% agreement, the faculty established that pediatric ITP that is refractory to emergent therapy could be defined as no platelet response after treatment with all eligible emergent pharmacotherapies. With 100% agreement, the working group established that pediatric patients with ITP that continue to demonstrate high disease burden and/or no platelet response despite treatment with multiple classes of disease-modifying therapies represent a challenging subset of ITP. These patients are at higher risk of ongoing disease burden and merit additional investigation as well as consideration for clinical trials or novel therapies. Future efforts to define disease burden and disease response will be completed in collaboration with the ITP International Working Group.
Early respiratory system reactance predicts respiratory outcomes in preterm infants: a retrospective, multicentre study
Authors: Zannin Emanuela , Rigotti Camilla , Schulzke Sven M. , Sindelar Richard , Werther Tobias , Lavizzari Anna , Neumann Roland P. , Wallström Linda , Mosca Fabio , Ventura Maria Luisa , Dellacà Raffaele L. , Veneroni Chiara 
Published: July 2024

BackgroundThis multicentre, international, retrospective study aimed to investigate whether respiratory system reactance (Xrs) assessed by respiratory oscillometry on day 7 of life is associated with respiratory outcomes in preterm infants below 32 weeks gestational age (GA).MethodsSinusoidal pressure oscillations (2–5 cmH2O peak-to-peak, 10 Hz) were superimposed on the positive end-expiratory pressure. We assessed the association ofXrsz-score with the duration of respiratory support using linear regression and with bronchopulmonary dysplasia (BPD) using logistic regression. We used the likelihood ratio test to evaluate whetherXrsz-score adds significantly to clinical predictors, including GA, birthweight (BW) and the National Institute of Child Health and Human Development (NICHD) BPD prediction model.Results137 infants (median (interquartile range) 28.43 (26.11–30.29) weeks GA) were included; 44 (32%) developed BPD.Xrsz-score was significantly associated with the duration of respiratory support (R2=0.35).Xrsz-score was significantly higher in infants who developed BPD (p<0.001); the optimal cut-off value was 2.6, associated with 77% sensitivity and 80% specificity. In univariable analysis, per z-score increase inXrs, the odds ratio for BPD increased by 60% and the respiratory support by 8 days. In multivariable analysis,Xrsz-score added significantly to the NICHD model and to GA and BW z-score to predict respiratory support duration (p=0.016 and p=0.014, respectively) and BPD development (p=0.003 and p<0.001, respectively).ConclusionXrsz-score on the 7th day after birth improves the prediction of respiratory outcome in preterm infants.
Chronic thromboembolic pulmonary hypertension is an uncommon complication of COVID-19: UK national surveillance and observational screening cohort studies
Authors: Reddy S. Ashwin , Newman Joseph , Leavy Olivia C. , Ghani Hakim , Pepke-Zaba Joanna , Cannon John E. , Sheares Karen K. , Taboada Dolores , Bunclark Katherine , Lawrie Allan , Sudlow Cathie L. , Berry Colin , Wild James M. , Mitchell Jane A. , Quint Jennifer , Rossdale Jennifer , Price Laura , Howard Luke S. , Wilkins Martin , Sattar Naveed , Chowienczyk Philip , Thompson Roger , Wain Louise V. , Horsley Alexander , Ho Ling-Pei , Chalmers James D. , Marks Michael , Poinasamy Krisnah , Raman Betty , Harris Victoria C. , Houchen-Wolloff Linzy , Brightling Christopher E. , Evans Rachael A. , Toshner Mark R. 
Published: July 2024

BackgroundPulmonary embolism (PE) is a well-recognised complication of coronavirus disease 2019 (COVID-19) infection, and chronic thromboembolic pulmonary disease with and without pulmonary hypertension (CTEPD/CTEPH) are potential life-limiting consequences. At present the burden of CTEPD/CTEPH is unclear and optimal and cost-effective screening strategies yet to be established.MethodsWe evaluated the CTEPD/CTEPH referral rate to the UK national multidisciplinary team (MDT) during the 2017–2022 period to establish the national incidence of CTEPD/CTEPH potentially attributable to COVID-19-associated PE with historical comparator years. All individual cases of suspected CTEPH were reviewed by the MDT for evidence of associated COVID-19. In a separate multicentre cohort, the risk of developing CTEPH following hospitalisation with COVID-19 was calculated using simple clinical parameters at a median of 5 months post-hospital discharge according to existing risk scores using symptoms, ECG and N-terminal pro-brain natriuretic peptide.ResultsBy the second year of the pandemic, CTEPH diagnoses had returned to the pre-pandemic baseline (23.1versus27.8 cases per month; p=0.252). Of 334 confirmed CTEPD/CTEPH cases, four (1.2%) patients were identified to have CTEPH potentially associated with COVID-19 PE, and a further three (0.9%) CTEPD without PH. Of 1094 patients (mean age 58 years, 60.4% male) hospitalised with COVID-19 screened across the UK, 11 (1.0%) were at high risk of CTEPH at follow-up, none of whom had a diagnosis of CTEPH made at the national MDT.ConclusionA prioririsk of developing CTEPH following COVID-19-related hospitalisation is low. Simple risk scoring is a potentially effective way of screening patients for further investigation.
Characterizing the journey of Rett syndrome among females in the United States: a real-world evidence study using the Rett syndrome natural history study database
Authors: May Damian , Kponee-Shovein Kalé , Neul Jeffrey L. , Percy Alan K. , Mahendran Malena , Downes Nathaniel , Chen Grace , Watson Talissa , Pichard Dominique C. , Kennedy Melissa , Lefebvre Patrick 
Published: July 2024

Abstract
Background
With the advent of the first targeted therapy for Rett Syndrome (RTT), a comprehensive assessment of the journey of RTT is needed to elucidate on present unmet needs in this population. This study characterized females with RTT in the United States and their disease journey with respect to longitudinal treatment patterns, RTT-related outcomes, and changes in disease severity.

Methods
This retrospective cohort study used registry data of females with RTT from the 5211 RTT Natural History Study (RNHS) (November 2015–July 2021). Pharmacological and supportive therapy use, RTT-related outcomes, and RTT severity, as measured by the Clinical Severity Scale and Motor Behavioral Assessment scale, were evaluated following the first RNHS visit. Analyses were conducted overall and in subgroups by RTT type (classic and atypical RTT) and age at first visit (pediatric and adult).

Results
A total of 455 females with RTT were included in the study, of whom 90.5% had classic RTT and 79.8% were pediatric individuals. Over a median follow-up of 4 years, use of pharmacological therapies, including prokinetic agents (42.7% vs. 28.3%), and supportive therapies, including physical therapy (87.3% vs. 40.2%) and speech-language therapy (86.8% vs. 23.9%), were more common in pediatric than adult individuals (all p < 0.05). Nearly half (44.6%) of all individuals had a hospital or emergency room visit, with a higher proportion of visits in individuals with classic RTT than atypical RTT and pediatric than adult individuals (both p = 0.001). An increasing trend in clinical severity was observed in pediatric individuals (mean change per year: 0.24; 95% confidence interval [CI]: 0.03, 0.44), while an increasing trend in motor-behavioral dysfunction was observed in pediatric individuals (mean change per year: 1.12; 95% CI: 0.63, 1.60) and those with classic RTT (mean change per year: 0.97; 95% CI: 0.53, 1.41).

Conclusions
Findings from this study highlight the considerable burden of RTT across disease subtype and age. Despite reliance on supportive therapies and healthcare encounters, individuals with RTT experience increasing disease severity and motor-behavioral dysfunction in childhood and adolescence, underscoring the unmet needs of this population and the value of early intervention to manage RTT in the long-term.
Estimation of genetic variation in vitiligo associated genes: Population genomics perspective
Authors: Bharti Neeraj , Banerjee Ruma , Achalare Archana , Kasibhatla Sunitha Manjari , Joshi Rajendra 
Published: July 2024

Abstract
Background
Vitiligo is an auto-immune progressive depigmentation disorder of the skin due to loss of melanocytes. Genetic risk is one of the important factors for development of vitiligo. Preponderance of vitiligo in certain ethnicities is known which can be analysed by understanding the distribution of allele frequencies across normal populations. Earlier GWAS identified 108 risk alleles for vitiligo in Europeans and East Asians. In this study, 64 of these risk alleles were used for analysing their enrichment and depletion across populations (1000 Genomes Project and IndiGen) with reference to 1000 Genomes dataset. Genetic risk scores were calculated and Fisher’s exact test was performed to understand statistical significance of their variation in each population with respect to 1000 Genomes dataset as reference. In addition to SNPs reported in GWAS, significant variation in allele frequencies of 1079 vitiligo-related genes were also analysed. Two-tailed Chi-square test and Bonferroni’s multiple adjustment values along with fixation index (≥ 0.5) and minimum allele frequency (≥ 0.05) were calculated and used to prioritise the variants based on pairwise comparison across populations.

Results
Risk alleles rs1043101 and rs10768122 belong to 3 prime UTR of glutamate receptor gene SLC1A2 are found to be highly enriched in the South Asian population when compared with the ‘global normal’ population. Intron variant rs4766578 (ATXN2) was found to be deleted in SAS, EAS and AFR and enriched in EUR and AMR1. This risk allele is found to be under positive selection in SAS, AMR1 and EUR. From the ancillary vitiligo gene list, nonsynonymous variant rs16891982 was found to be enriched in the European and the Admixed American populations and depleted in all others. rs2279238 and rs11039155 belonging to the LXR-α gene involved in regulation of metalloproteinase 2 and 9 (melanocyte precursors) were found to be associated with vitiligo in the North Indian population (in earlier study).

Conclusion
The differential enrichment/depletion profile of the risk alleles provides insight into the underlying inter-population variations. This would provide clues towards prioritisation of SNPs associated with vitiligo thereby elucidating its preponderance in different ethnic groups.
Silver nanoparticle with potential antimicrobial and antibiofilm efficiency against multiple drug resistant, extensive drug resistant Pseudomonas aeruginosa clinical isolates
Authors: Kamer Amal M. Abo , El Maghraby Gamal M. , Shafik Maha Mohamed , Al-Madboly Lamiaa A. 
Published: July 2024

Abstract
Background
The study aims to investigate the effect of combining silver nanoparticles (AGNPs) with different antibiotics on multi-drug resistant (MDR) and extensively drug resistant (XDR) isolates of Pseudomonas aeruginosa (P. aeruginosa) and to investigate the mechanism of action of AGNPs.

Methods
AGNPs were prepared by reduction of silver nitrate using trisodium citrate and were characterized by transmission electron microscope (TEM) in addition to an assessment of cytotoxicity. Clinical isolates of P. aeruginosa were collected, and antimicrobial susceptibility was conducted. Multiple Antibiotic Resistance (MAR) index was calculated, and bacteria were categorized as MDR or XDR. Minimum inhibitory concentration (MIC) of gentamicin, ciprofloxacin, ceftazidime, and AGNPs were determined. The mechanism of action of AGNPs was researched by evaluating their effect on biofilm formation, swarming motility, protease, gelatinase, and pyocyanin production. Real-time PCR was performed to investigate the effect on the expression of genes encoding various virulence factors.

Results
TEM revealed the spherical shape of AGNPs with an average particle size of 10.84 ± 4.64 nm. AGNPS were safe, as indicated by IC50 (42.5 µg /ml). The greatest incidence of resistance was shown against ciprofloxacin which accounted for 43% of the bacterial isolates. Heterogonous resistance patterns were shown in 63 isolates out of the tested 107. The MAR indices ranged from 0.077 to 0.84. Out of 63 P. aeruginosa isolates, 12 and 13 were MDR and XDR, respectively. The MIC values of AGNPs ranged from 2.65 to 21.25 µg /ml. Combination of AGNPs with antibiotics reduced their MIC by 5–9, 2–9, and 3-10Fold in the case of gentamicin, ceftazidime, and ciprofloxacin, respectively, with synergism being evident. AGNPs produced significant inhibition of biofilm formation and decreased swarming motility, protease, gelatinase and pyocyanin production. PCR confirmed the finding, as shown by decreased expression of genes encoding various virulence factors.

Conclusion
AGNPs augment gentamicin, ceftazidime, and ciprofloxacin against MDR and XDR Pseudomonas isolates. The efficacy of AGNPs can be attributed to their effect on the virulence factors of P. aeruginosa. The combination of AGNPs with antibiotics is a promising strategy to attack resistant isolates of P. aeruginosa.
Mitigating cold stress in rice: a study of genotype performance and sowing time
Authors: El-Refaee Yasser Z. , Gharib Hany S. , Badawy Shimaa A. , Elrefaey Eman M. , El-Okkiah Samira A.F. , Okla Mohamed K. , Maridueña-Zavala María Gabriela , AbdElgawad Hamada , El-Tahan Amira M. 
Published: July 2024

AbstractRice (Oryza sativa L.) is an essential food for half of the global population and is vital in maintaining global food security. Climate change, increasing population and recent incident of COVID pandemic has generated financial burden and threaten the global food security. Due to theses factors rice cultivation also has to face significant challenges. frequent weather changes pose a considerable challenge to agricultural planning, which was previously relaying on consistent seasonal variations. In this context, rice cultivation is particularly sensitive to cold, where its development and productivity inhibited by low temperatures (< 18 °C). Developing rice varietes with low temprature tolerence and good yield potential is one of the major goals of current breeding efforts of plant scientists. For this purpose, short duration and early rice varieties are most favorable to avoid cold stress and yield more in less number of days. this study was designed to investigate the effect of low temperatures on different rice varieties. the study was designed to identify low temprature tolerent genotypes with early and regular cultivation. For this, thirty-four genotypes were evaluated in two gorwing seasons (2018–2019) with four different sowing times. Statistically sowing time showed significant interaction between all yield contributing parameters. The data indicate that exposure to low temperatures during the reproductive phase prolongs the maturation period of the crop, also length of the panicle and the fertility of the spikelets drops, resulting in a significant decrease in the production of sensitive varieties. Some varieties are more sensitive to cold stress compared to others. In the Egyptian context, Giza176, Sakha104, and Sakha107 are recommended for early cultivation, while the genotypes Giza 179, Sakha101, Sakha104, and GZ 9730-1-1-1-1 are indicated for the normal cultivation period. The Sakha104 variety is particularly notable, as it is recommended for both purposes. In addition, the data obtained in this study provide valuable information for selecting rice varieties suitable for double cropping in the North Delta of Egypt. This study also contributes to the existing literature, providing insights into the resilience of rice cultivation in the face of climate change.
Long-term outcomes of catheter ablation for ventricular arrhythmias: comparing techniques with and without intracardiac echocardiography - what matters?
Authors: Lin Mingjie , Ma Chuanzhen , Rong Bing , Zhang Kai , Chen Tongshuai , Wang Juntao , Han Wenqiang , Zhong Jingquan , Wu Lin 
Published: July 2024

Abstract
Background
The increasing use of intracardiac echocardiography (ICE) in the ablation of premature ventricular complexes (PVCs) has raised questions about its true efficacy and safety.

Methods
This retrospective study collected the periprocedural complications and PVC burden post ablation. The risk factors of PVC recurrence was further explored.

Results
The study included patients treated without ICE (control group, n = 451) and with ICE (ICE group, n = 155) from May 2019 to July 2022. The ICE group demonstrated significantly lower fluoroscopy times and X-ray doses. There were no major complications in the ICE group, and the difference in the occurrence of periprocedural complications between the groups was not statistically significant (p = 0.072). The long-term success rates were similar for the control and ICE groups (89.6% and 87.1%, respectively). The origin of PVCs was identified as the independent factor for ablation success.

Conclusions
The use of ICE did not confer an advantage with regard to long-term success in PVCs ablation. To thoroughly evaluate the safety and effectiveness of ICE in PVCs ablation, a prospective, multicenter, randomized study is warranted.
From the periphery to inclusion within the health system: promoting community health worker empowerment as a way forward
Authors: Stansert Katzen Linnea , Reid Steve , Laurenzi Christina , Tomlinson Mark 
Published: July 2024

Abstract
Background
Community health worker programmes have the potential to contribute critically towards universal health coverage. However, CHWs globally have often continued to operate on the periphery of the health care system, viewed as a non-essential cadre. This results in a workforce that often remains disempowered and under-supported. This paper presents evidence from a study conducted in a rural part of South Africa, to better understand issues of CHW prioritisation, integration, and empowerment.

Methods
We applied an analytical lens based on empowerment theory and conducted a secondary analysis of qualitative data emerging from a sub-study of a cRCT evaluating the effectiveness of supportive supervision for CHWs within a large-scale national CHW programme. The cRCT was conducted between 2017 and 2022, and 39 CHWs were included in the study.

Results
We organised our findings across the four domains of structural empowerment; information, resources, support, and opportunity, and mapped these domains against the domains of psychological empowerment. Our findings show how CHWs are still working in the periphery of the healthcare system. Without sufficient prioritisation, high level-support from national and district governments, and sufficient investments in programmatic domains—such as training, equipment, and supportive supervision—it is likely that the CHW cadre will continue to be seen as informal health care workers.

Conclusions
CHW empowerment could be a lever to potentially transform the current health system towards universal coverage; however, this process can only happen with sufficient high-level prioritization and investment.
Association of physical activity trajectories over 8 years and risk of knee replacement: data from the osteoarthritis initiative
Authors: Wang Yining , Shen Ziyuan , Xing Xing , Ge Liru , Pan Faming , Cai Guoqi 
Published: July 2024

Abstract
Background
To identify physical activity (PA) trajectories in adults with or at risk of knee osteoarthritis and to evaluate the association of PA trajectories with incident knee replacement (KR).

Methods
This study used data from the Osteoarthritis Initiative. The Physical Activity Scale for the Elderly and the KR were assessed annually from baseline to 9 years. Individuals were included if they did not undergo KR surgery at baseline and had data on PA at ≥ 1 visit before KR. Latent class growth mixture Modeling was used to identify the optimal trajectories of PA before KR. Log-binomial regression models were used to assess the association between PA trajectories and the risk of KR. Data analyses were conducted in all individuals and those with radiographic osteoarthritis (ROA) and significant knee pain (Western Ontario and McMaster Osteoarthritis Index pain score of ≥ 5 on a 0–20 scale) at baseline, respectively.

Results
Of 4731 participants (mean age 61.1 years, 58.5% female), four distinct and slightly declined PA trajectories were identified. Compared to individuals with a “Low” PA trajectory, those with “Medium-low”, “Medium-high”, or “High” PA trajectories were not significantly associated with the risk of KR (risk ratios: 0.97–1.19, all p > 0.05). Similar PA trajectories and associations with the risk of KR were observed in the subgroups of individuals with radiographic osteoarthritis and those with significant knee pain at baseline, respectively.

Conclusion
In participants with or at risk of knee osteoarthritis, PA slightly declines over time and may play no role in the risk of KR.