bluuebunny/crossref_metadata_2025_split huggingface dataset:
Validation of the PEDiatric Behçet’s Disease classification criteria: an evidence-based approach
Authors: Matucci-Cerinic Caterina , Palluy Helene , Al-Mayouf Sulaiman M , Brogan Paul A , Cantarini Luca , Gul Ahmet , Kasapcopur Ozgur , Kuemmerle-Deschner Jasmin , Ozen Seza , Saadoun David , Shahram Farhad , Bovis Francesca , Mosci Eugenia , Ruperto Nicolino , Gattorno Marco , Kone-Paut Isabelle 
Published: November 2023

Abstract

Objectives
To validate the PEDiatric Behçet’s Disease classification criteria (PEDBD) with an evidence-based approach.

Methods
A total of 210 pediatric patients [70 Behçet’s disease (BD), 40 periodic fever, aphthous stomatitis, pharyngitis, adenitis, 35 familial Mediterranean fever, 26 hyper-IgD syndrome, 22 TNF-receptor associated periodic fever syndrome, 17 undefined recurrent fevers] were randomly selected from the Eurofever Registry. A set of 11 experienced clinicians/researchers blinded to the original diagnosis evaluated the patients. Using the table consensus as gold standard (agreement ≥ 80%), the PEDBD, ISG and ICBD criteria were applied to BD patients and to confounding diseases with other autoinflammatory conditions in order to define their sensitivity, specificity and accuracy.

Results
At the end of the third round, a consensus was reached in 139/210 patients (66.2%). The patients with a consensus ≥80% were classified as confirmed BD (n = 24), and those with an agreement of 60–79% as probable BD (n = 10). When comparing these patients with the confounding diseases group, an older age at disease onset, the presence of oral and genital ulcers, skin papulo-pustular lesions, a positive pathergy test and posterior uveitis were BD distinctive elements. The ISG, ICBD and PEDBD criteria were applied to confirmed BD and to the confounding disease group, showing a sensitivity of 0.50, 0.79 and 0.58, a specificity of 1.00, 0.97 and 0.99, and an accuracy of 0.91, 0.94 and 0.92, respectively.

Conclusions
The PEDBD criteria were very specific, while the ICBD were more sensitive. The complexity of childhood BD suggests larger prospective international cohorts to further evaluate the performance of the criteria.
Effectiveness of biological targeted therapies may discriminate seronegative from seropositive rheumatoid arthritis
Authors: Iannone Florenzo , Lopalco Giuseppe , Cacciapaglia Fabio , Venerito Vincenzo , Perniola Simone , Fornaro Marco 
Published: November 2023

Abstract

Objective
To assess the real-world effectiveness of targeting biologic DMARDs (bDMARDs) in rheumatoid arthritis (RA) patients negative for rheumatoid factor (RF) and anti-citrullinated protein antibodies (ACPA).

Methods
We retrospectively selected 81 seronegative and 404 seropositive RA patients receiving treatment with abatacept, anti-TNF alpha or tocilizumab. Effectiveness was evaluated by analysing drug survival using Kaplan–Meyer analysis over 10-year follow-up. Survival rates were compared by log rank test, and hazard ratios (HRs) of therapy discontinuation were estimated through multivariate Cox regression.

Results
Clinical characteristics were similar between the two groups, except for a significantly higher percentage of inadequate responders to prior bDMARDs in the seronegative RA patients (P = 0.02). Among seronegative RA, tocilizumab demonstrated a survival rate of 73.9% with a mean survival time (MST) of 76.8 months (95% CI 61–92), which was significantly higher than abatacept [37.5%, MST 37.1 months (95% CI 22–51; P = 0.01)]. Anti-TNF alpha therapy fell in the middle [50.0%, MST 63.5 months (95% CI 47–79)] but the difference was not significant. Nevertheless, seropositive RA patients did not show significantly different drug survival rates. Negative predictors of drug discontinuation were RF/ACPA positivity (HR 0.56) and sex male (HR 0.58), but treatment with abatacept (HR 1.88) or anti-TNF alpha (HR 1.79), no co-therapy with conventional DMARDs (HR 1.74), absence of bone erosions (HR 1.41) and higher HAQ (HR 1.58) were positive predictors.

Conclusions
To confirm these preliminary findings and to explore the hypothesis of a distinctive therapeutic algorithm in seronegative RA, prospective studies on larger cohorts are needed.
Antenatal corticosteroid therapy, delivery intervals and perinatal mortality in low-resource settings
Authors: Mwita Stanley , Kamala Benjamin , Konje Eveline , Katabalo Deogratias , Msanga Delfina R , Marwa Karol J , Basinda Namanya , Kongola Gilbert , Jande Mary , Dewey Deborah 
Published: November 2023

Background
Uncertainty exists regarding the ideal interval between the administration of antenatal corticosteroids (ACS) and delivery. The study’s objective was to assess the risks of perinatal mortality and respiratory distress syndrome (RDS) among preterm neonates whose mothers gave birth within 48 h of the administration of ACS and those whose mothers gave birth between 48 h and 7 days.

Methods
The study design was a secondary analysis of data from an observational prospective chart review study that was carried out in Tanzania in 2020. Preterm infants born to mothers who got at least one dose of ACS between 28 and 34 weeks of pregnancy were included.

Results
A total of 346 preterm neonates (294 singletons and 52 twins) were exposed to ACS. Compared to infants born 48 h following the first dose of ACS, those exposed to the drug between 48 h and 7 days had significantly decreased rates of perinatal mortality and RDS. Multivariable analysis revealed that infants exposed ACS between 48 h and 7 days prior to delivery had lower risk of perinatal mortality (aRR 0.30, 95% CI 0.14–0.66) and RDS (aRR 0.27, 95% CI 0.14–0.52).

Conclusion
The first dose of ACS given between 48 h and 7 days before delivery was associated with a lower risk of perinatal mortality and RDS than when the first dose was given &lt;48 h before delivery. To improve neonatal outcomes, healthcare providers should consider administering ACS to mothers at the appropriate time.
EU financial market regulation a decade from the financial-crisis-era reforms: crisis, uncertainty, and capacity
Authors: Moloney Niamh 
Published: November 2023

Abstract
This article reflects on the effectiveness of European Union (EU) financial market regulation, a decade or so after the closure of the EU’s massive financial-crisis-era reform programme. To do so, it considers the first significant test of the financial-crisis-era reforms: the March 2020 period of acutely elevated financial stability risk as the COVID-19 pandemic intensified, global markets roiled, and the investment fund sector experienced large-scale disruption. It relates the EU’s broadly successful management of this period to, first, the resilience of the legislative choices made and refined over the financial crisis era as regards investment fund regulation and, secondly, to technocratic action, by the European Securities and Markets Authority (ESMA) and the European Systemic Risk Board (ESRB), that facilitated rule amplification, risk monitoring, supervisory coordination, and supervisory convergence and thereby supported the earlier legislative choices. Drawing on the March 2020 experience, the article goes on to consider the capacity of EU financial market regulation more generally to manage what are increasingly dynamic risks to financial market stability. It identifies a strengthening of the EU’s capacity in this regard, but also persistent and intractable risks to this capacity, including as regards legitimation.
Molecular phylogeny of the fungus gnat subfamilies Sciophilinae and Leiinae (Mycetophilidae), with notes on Sciaroidea incertae sedis (Diptera: Bibionomorpha)
Authors: Burdíková Nikola , Kaspřák David , Kjærandsen Jostein , Tóthová Andrea Špalek , Ševčík Jan 
Published: November 2023

Abstract
The phylogeny of the subfamilies Sciophilinae and Leiinae (Diptera: Mycetophilidae) is reconstructed, based on three nuclear (18S, 28S, CAD) and three mitochondrial (16S, COI, Cytb) gene fragments. The analysis supports the monophyly of Sciophilinae in a strict sense, without the Paratinia Mik, 1874 genus group and genera Aneura Marshall, 1896 and Taxicnemis Tonnoir and Edwards, 1927, the latter two revealed as being the most plesiomorphic within Mycetophilidae. The subfamily Leiinae is found monophyletic, but only in much narrower sense than it was defined based on recent morphological analyses. The genus TaiwanosciaroidesSaigusa, 2018 synon. nov. is considered to be a junior synonym of DeimyiaKallweit, 2002. A new molecular phylogeny of Sciaroidea is presented, based on longer sequences of nuclear genes (18S, 28S, CAD) and three mitochondrial gene fragments (12S, 16S, COI), including 10 genera of the Sciaroidea incertae sedis group. Eight of them grouped together (ufboot2 = 93), including RangomaramaJaschhof and Didham, 2002 (family Rangomaramidae). Two outlier genera, Ohakunea Edwards, 1927 and HeterotrichaChandler, 2002, grouped within Sciophilinae and as the sister-taxon to Sciaridae, respectively. Our results spark a debate over how to interpret and combine molecular and morphological data, and we refrain from reclassifications at this point.
Increased Diversity of Citrus Tristeza Virus in Europe
Authors: Zindović Jelena , Čizmović Miroslav , Vučurović Ana , Margaria Paolo , Škorić Dijana 
Published: November 2023

This study investigated the genetic diversity of Citrus tristeza virus (CTV) isolates from Montenegro and Croatia, European countries with the northernmost citrus-growing regions situated on the Eastern Adriatic coast. Fifteen complete or nearly complete CTV genomes were reconstructed by high-throughput sequencing of samples collected in distinct municipalities in Montenegro and Opuzen municipality in Croatia. Phylogenetic analyses assigned some of the sequences to VT and T30 strains, previously recorded in Europe, while remarkably other isolates were placed in S1 and RB groups, which have not been reported in Europe so far. In addition, a new phylogenetic lineage comprising only isolates from Montenegro was delineated and tentatively proposed as the MNE cluster. Recombination analysis revealed evidence of 11 recombination events in the sequences obtained in this study, between isolates of related strains, within isolates of the same strain, and between distant strains. These findings show that CTV diversity in Europe is higher than reported before and calls for the reevaluation of management strategies. [Formula: see text] Copyright © 2024 The Author(s). This is an open access article distributed under the CC BY-NC-ND 4.0 International license .
Long COVID in Icelandic Children: A Matched Cohort Study of Nonspecific Symptoms Following SARS-CoV-2 Infection
Authors: Thors Valtyr , Bjornsdottir Kristin Lilja , Love Thorvardur Jon , Haraldsson Asgeir 
Published: November 2023

Purpose:
Pediatric severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infections are usually mild and the mortality rates are low, but concerns have been raised about long-term symptoms that may resemble other postinfectious syndromes. Studies with robust control groups and high response rates have been few.

Methods:
We obtained identifiers for all 837 Icelandic children diagnosed with SARS-CoV-2 by PCR between March 2020 and June 2021 and contacted them by telephone. We asked about 10 physical and mental symptoms being present at least twice weekly for at least 2 months. Participants who reported symptoms were contacted again a year later. For each subject who completed the questionnaire, an age- and sex-matched comparator without SARS-CoV-2 infection was asked to complete the same questionnaire, and the risk difference was calculated.

Results:
Responses from 643 cases and 602 comparators were analyzed. Children who had been infected with SARS-CoV-2 were more likely to report one or more symptoms, except for anxiety/depression and sleep disturbances. Fatigue and loss of concentration were evidently more common in cases among teenagers (risk difference: 15%; 95% CI: 7–22% and 15%; 95% CI: 7–23%, respectively). At the second follow-up, close to a third of Long COVID cases had resolved but some participants had developed new persistent symptoms.

Conclusion:
Symptoms of Long COVID in children are common and impact their quality of life. The importance of further unraveling the pathophysiology of acute and long-term symptoms following SARS-CoV-2 infection in children is vital as well as potential preventive measures.
Maternal HIV Status and Risk of Infant Mycobacterium tuberculosis Infection as Measured by Tuberculin Skin Test
Authors: Mecha Jerphason , Escudero Jaclyn N. , Richardson Barbra A. , Maleche-Obimbo Elizabeth , Matemo Daniel , Kinuthia John , John-Stewart Grace , LaCourse Sylvia M. 
Published: November 2023

Background:
The effect of maternal HIV on infant Mycobacterium tuberculosis (Mtb) infection risk is not well-characterized.

Methods:
Pregnant women with/without HIV and their infants were enrolled in a longitudinal cohort in Kenya. Mothers had interferon gamma-release assays (QFT-Plus) and tuberculin skin tests (TST) at enrollment in pregnancy; children underwent TST at 12 and 24 months of age. We estimated the incidence and correlates of infant TST-positivity using Cox proportional hazards regression.

Results:
Among 322 infants, 170 (53%) were HIV-exposed and 152 (47%) were HIV-unexposed. Median enrollment age was 6.6 weeks [interquartile range (IQR): 6.1–10.0]; most received Bacillus Calmette-Guerin (320, 99%). Thirty-nine (12%) mothers were TST-positive; 102 (32%) were QFT-Plus-positive. Among HIV-exposed infants, 154 (95%) received antiretrovirals for HIV prevention and 141 (83%) of their mothers ever received isoniazid preventive therapy (IPT). Cumulative 24-month infant Mtb infection incidence was 3.6/100 person-years (PY) [95% confidence interval (CI): 2.4–5.5/100 PY]; 5.4/100 PY in HIV-exposed infants (10%, 17/170) versus 1.7/100 PY in HIV-unexposed infants (3.3%, 5/152) [hazard ratio (HR): 3.1 (95% CI: 1.2–8.5)]. More TST conversions occurred in the first versus second year of life [5.8 vs. 2.0/100 PY; HR: 2.9 (95% CI: 1.0–10.1)]. Infant TST-positivity was associated with maternal TST-positivity [HR: 2.9 (95% CI: 1.1–7.4)], but not QFT-Plus-positivity. Among HIV-exposed children, Mtb infection incidence was similar regardless of maternal IPT.

Conclusions:
Mtb infection incidence (by TST) by 24 months of age was ~3-fold higher among HIV-exposed children, despite high maternal IPT uptake. Overall, more TST conversions occurred in the first 12 months compared to 12–24 months of age, similar in both HIV-exposed and HIV-unexposed children.
Physician Assistant Student Attitudes About People With Serious Mental Illness
Authors: McQuillan Matthew A. , Wright Matthew , Zechner Michelle R. , Di Prospero Elizabeth , Karyczak Sean , Cimmino Meredith L. , Murphy Ann A. 
Published: November 2023

Introduction
This study explored the attitudes of physician assistant (PA) students toward the future care of individuals with serious mental illness (SMI). Physician assistant student education offers a unique opportunity to confront and reduce bias toward individuals with SMI. However, no previous literature has documented PA student attitudes toward SMI.

Methods
Preclinical students (n = 7) from a single PA program were invited to participate in 2 focus groups exploring attitudes toward the future care of patients with SMI. The focus groups lasted approximately 60 minutes and were conducted by non-PA faculty using a question guide.

Results
Four themes emerged from interviews: perceptions about the population, knowledge of mental health conditions, approach to care, and program curriculum input.

Discussion
Several themes aligned with the participant's level of training. Participants demonstrated didactic knowledge yet lacked clinical exposure to provide context. Future studies should focus on developing interactive learning techniques to prepare students for patients with SMI.
Prediction of an impaired myocardial work using infarct size in acute myocardial infarction
Authors: He Xiao-Ting , Zhang Jie , Pan Yang , Yu Fan , Tang Ge , Zhu Li-Zhou , Qin Yi-Nan , Zheng Xiao-Zhi 
Published: November 2023

Background
The relationship between myocardial infarct size (MIS) on late gadolinium-enhanced cardiac MRI (LGE MRI) and myocardial work (MW) indices assessed with MW echocardiography (MWE) has not been well characterized. This study aimed to determine an impaired MW using MIS in patients with acute myocardial infarction.

Methods
Left ventricular (LV) two-dimensional speckle-tracking echocardiography, MWE, and LGE MRI were performed in 33 patients with ST-segment elevation myocardial infarction and in 30 age- and sex-comparable controls. LV global longitudinal strain (GLS), global work index (GWI), global constructive work (GCW), global wasted work (GWW), and global work efficiency (GWE) and MIS were acquired, respectively.

Results
MIS was negatively correlated with GWI (r = −0.60, P = 0.005), GCW (r =- 0.66, P = 0.002) and GWE (r = −0.71, P = 0.0004), but positively correlated with GLS (r = 0.68, P = 0.001). With the receiver operating characteristic curve, the cutoff value of MIS for the prediction of an impaired GLS was 16.5% [area under the curve (AUC) = 0.867)], an impaired GWI was 19.2% (AUC = 0.727), an impaired GCW was 19.2% (AUC = 0.725), an increased GWW was 15.8% (AUC = 0.656), an impaired GWE was 15.8% (AUC = 0.880).

Conclusion
MIS is a strong predictor of impaired MW. Timely reduction of infarct size is essential to improve myocardial function.
Early-onset scoliosis
Authors: Moreno Mateo Fernando , Bovonratwet Patawut , Peiró García Alejandro 
Published: November 2023

Purpose of reviewThe aim of this review is to provide the reader with the most updated available information so that it can be helpful in the approach of patients with early-onset scoliosis (EOS).Recent findingsWhile confirming the efficacy and safety of classic techniques for the treatment of EOS such as traditional growing rods or Mehta casting, recent research suggests that there is room for improvement with less invasive techniques.SummaryThe most important goal when treating patients with EOS should be to promote rib cage expansion and lung development. Different techniques have been described and may be used depending on the specific patient's characteristics.
Optimizing cardiac status in the preliver transplant candidate
Authors: Stachel Maxine W. , DePasquale Eugene C. 
Published: November 2023

Purpose of review
Liver transplant is a widely accepted therapy for end-stage liver disease. With advances in our understanding of transplant, candidates are increasingly older with more cardiac comorbidities. Cardiovascular disease also represents a leading cause of morbidity and mortality posttransplant.

Recent findings
Preoperative cardiac risk stratification and treatment may improve short-term and long-term outcomes after liver transplant. Importantly, the appropriate frequency of surveillance has not been defined. Optimal timing of cardiac intervention in end-stage liver disease is likewise uncertain.

Summary
The approach to risk stratification of cardiovascular disease in end-stage liver disease is outlined, incorporating the AHA/ACC scientific statement on evaluation of cardiac disease in transplant candidates and more recent expert consensus documents. Further study is needed to clarify the ideal timing and approach for cardiovascular interventions.
Regulatory dendritic cell therapy in organ transplantation
Authors: Hadjiyannis Yannis , Thomson Angus W. 
Published: November 2023

Purpose of review
Regulatory dendritic cells (DCregs; also ‘tolerogenic DCs’), innate immune cells that regulate the alloimmune response, are a novel cellular therapy for organ transplantation. Preliminary results from early-phase clinical trials in live donor kidney and liver transplantation are promising. This follows many years of research elucidating mechanisms of action and utility of DCregs. Herein, we review early-phase clinical trial observations and recent advances in the production, modification, and future-trajectory of DCreg in organ transplantation.

Recent findings
Preclinical work has demonstrated the ability of adoptively transferred DCreg to abrogate ischemia-reperfusion injury and promote long-term allograft survival. Good Manufacturing Practice-grade DCregs have been generated in adequate numbers for early-phase trials of autologous DCregs in kidney transplantation and donor-derived DCreg in liver transplantation. These trials have demonstrated feasibility and safety, with preliminary evidence of an influence on host immune reactivity. In both kidney and liver transplantation, reduced effector CD8+ T-cells have been noted, together with other changes that may be conducive to reduced dependence on immunosuppressive therapy.

Summary
Substantial progress has been made in bringing DCreg to clinical testing in organ transplantation. Additional clinical and mechanistic studies are now needed to further explore and garner the full potential of DCreg in organ transplantation.
Neurological and neuro-ophthalmological manifestations of snake bite: a systematic review
Authors: Pandit Kamal , Rawal Aastha , Maskey Himang Man Singh , Nepal Gaurav 
Published: November 2023

Objective:Snakebites, a major health concern in developing countries, affect rural farming communities. Venom, primarily neurotoxin, injected during a snake bite disrupts the nervous system, causing symptoms like muscle weakness, paralysis, altered sensation, and coordination issues. This review focuses on evaluating neurological and neuro-ophthalmological manifestations associated with snakebites.Methods:A database search was conducted in EMBASE and PubMed for studies published from 2000 to 2023. The investigation centered on examining neurological and neuro-ophthalmological symptoms and signs, treatment approaches, treatment outcomes, and long-term complications of snake bites.Results:Neurological and neuro-ophthalmological symptoms were common in both neurotoxic and hemotoxic snake bites, especially in neurotoxic cases. Ptosis was a prevalent manifestation across various snake bites, along with respiratory paralysis, limb weakness, dysphasia, and visual disturbances in some instances. However, most patients improved without residual neurological symptoms after treatment.Conclusions:Understanding patterns of neurological manifestations contributes valuable insights for the comprehensive management of snakebite.
Increasing the tolerance of mothers with children with autism: the effectiveness of cognitive therapy based on mindfulness – experimental research
Authors: Azizi Mojgan , Imannezhad Shima , Moradpoor Mahboobeh , Alaghbandian Erfan , Saeidi Parisa , Sobhani Mohammadreza , Maleki Mohammad Mehdi , Jahangiri Shima , Shojaei Behnaz , Mohammadi Yasaman 
Published: November 2023

Introduction and importance:Autism spectrum disorder significantly impacts the life and psychosocial health of the family, resulting in high levels of anxiety, stress, isolation, and indecisiveness among parents. This study aimed to determine the effectiveness of cognitive therapy based on mindfulness in increasing the tolerance of mothers of children with autism.Case presentation:The study used a semi-experimental pre-test–post-test design with a control group. The study population comprised mothers referred to autism centers in Tehran. Eighty mothers were randomly divided into two groups, with 40 in each group. The Connor and Davidson Resilience Scale was used to measure the level of tolerance in both groups in the pre-test and post-test stages. The experimental group underwent cognitive therapy group therapy based on mindfulness, comprising eight sessions of 120 min. On the other hand, the control group did not receive any intervention.Clinical discussion:The results of the study showed that the tolerance scores of the experimental group significantly increased after the intervention, in both the post-test and follow-up stages.Conclusion:Therefore, the results of this research emphasize the importance of using this intervention in increasing the tolerance of mothers of children with autism spectrum disorder and creating new horizons in the clinical interventions of these people.