bluuebunny/crossref_metadata_2025_split huggingface dataset:
Adult‐onset Still's disease in Western Australia: Epidemiology, comorbidity and long‐term outcome
Authors: Nossent Johannes , Raymond Warren , Keen Helen , Preen David B. , Inderjeeth Charles A. 
Published: August 2022

AbstractAimAdult‐onset Still’s disease (ASD) is a rare, potentially life‐threatening autoinflammatory condition. As reported prevalence shows regional variation and long‐term outcome data are scarce, we investigated epidemiology and long‐term health outcomes of ASD in Western Australia (WA).MethodsPopulation‐based cohort study using longitudinally linked administrative health data from all WA hospitals between 1999 and 2013 for ASD patients (ICD‐10‐AM M06.1) and controls matched for age, gender, and index year. Rate ratios and odds ratios (RR/OR) with 95% confidence intervals (CI) compared ASD patients with controls.ResultsThe average ASD incidence (n = 52) was 0.22/100 000 with 2.4/100 000 point‐prevalence as of December 31, 2013. ASD patients (median age 41.5 years, 59.6% female) had higher odds of previous liver disease (OR 2.67, 95% CI 1.31‐5.45), fever (OR 54.10, 95% CI 6.60‐433.0), rash (OR 15.70, 95% CI 4.08‐60.80), and serious infections (OR 4.36, 95% CI 2.11‐22.80) than controls. Despite biological disease‐modifying antirheumatic drugs in 27% of patients, ASD patients had higher odds for joint replacement (n = 7, 13.5%) (OR 45.5, 95% CI 4.57‐93.70), osteoporosis (OR 31.3, 95% CI 3.43‐97), and serious infections (RR 5.68; 95% CI 6.61‐8.74) during follow up. However, crude mortality (11.5% vs 7.5%; P = 0.34), survival at 1 and 5 years (P= 0.78), and last modified Charlson Comorbidity score (median 2 vs 2) were similar between groups.ConclusionThe epidemiology and demographics of ASD in Western Australia fall within the internationally reported range. ASD patients present increased rates of liver disease, rash, and serious infections before disease onset. Mortality following ASD was not increased for 5 years despite high rates of chronic arthritis requiring joint replacement, serious infections, and osteoporosis.
Canakinumab treatment in a young girl with refractory chronic recurrent multifocal osteomyelitis associated with pyoderma gangrenosum
Authors: Acierno Sabrina , Angrisani Francesca , Marino Achille , Caporali Roberto Felice , Cimaz Rolando , Giani Teresa 
Published: August 2022

AbstractBackgroundChronic recurrent multifocal osteomyelitis (CRMO) is a bone inflammatory disorder characterized by osteolytic, usually multiple, symmetric lesions. Diagnosis is one of exclusion, and no standardized therapies are available. Presumed deregulation of the interleukin (IL)‐1β axis, as observed in 2 monogenic autoinflammatory conditions such as Majeed syndrome (LPIN2 mutations) and deficiency of IL‐1 receptor antagonist (IL1RN mutations) with CRMO‐like bone involvement, suggests the blockade of IL‐1 as potentially useful also in this condition, even if scarce data are available.Case presentationWe report the case of a 13‐year‐old girl affected by a multidrug‐resistant and pyoderma gangrenosum‐complicated CRMO treated with canakinumab, a human monoclonal antibody targeting IL‐1β.ConclusionIn this young patient pyoderma gangrenosum and CRMO showed a rapid and satisfactory response to canakinumab, although over time a decreased efficacy in controlling bone disease was observed.
Unique and common TCR repertoire features of Ni2+‐, Co2+‐, and Pd2+‐specific human CD154 + CD4+ T cells
Authors: Riedel Franziska , Aparicio‐Soto Marina , Curato Caterina , Münch Lucas , Abbas Amro , Thierse Hermann‐Josef , Peitsch Wiebke K. , Luch Andreas , Siewert Katherina 
Published: August 2022

AbstractBackgroundApart from Ni2+, Co2+, and Pd2+ ions commonly trigger T cell‐mediated allergic contact dermatitis. However, in vitro frequencies of metal‐specific T cells and the mechanisms of antigen recognition remain unclear.MethodsHere, we utilized a CD154 upregulation assay to quantify Ni2+‐, Co2+‐, and Pd2+‐specific CD4+ T cells in peripheral blood mononuclear cells (PBMC). Involved αβ T cell receptor (TCR) repertoires were analyzed by high‐throughput sequencing.ResultsPeripheral blood mononuclear cells incubation with NiSO4, CoCl2, and PdCl2 increased frequencies of CD154 + CD4+ memory T cells that peaked at ~400 μM. Activation was TCR‐mediated as shown by the metal‐specific restimulation of T cell clones. Most abundant were Pd2+‐specific T cells (mean 3.5%, n = 19), followed by Co2+‐ and Ni2+‐specific cells (0.6%, n = 18 and 0.3%, n = 20) in both allergic and non‐allergic individuals. A strong overrepresentation of the gene segment TRAV9‐2 was unique for Ni2+‐specific TCR (28% of TCR) while Co2+ and Pd2+‐specific TCR favorably expressed TRAV2 (8%) and the TRBV4 gene segment family (21%), respectively. As a second, independent mechanism of metal ion recognition, all analyzed metal‐specific TCR showed a common overrepresentation of a histidine in the complementarity determining region 3 (CDR3; 15% of α‐chains, 34% of β‐chains). The positions of the CDR3 histidine among metal‐specific TCR mirrored those in random repertoires and were conserved among cross‐reactive clonotypes.ConclusionsInduced CD154 expression allows a fast and comprehensive detection of Ni2+‐, Co2+‐, and Pd2+‐specific CD4+ T cells. Distinct TCR repertoire features underlie the frequent activation and cross‐reactivity of human metal‐specific T cells.
Helsinki in Zion: Hospital ethics committees and political gatekeeping in Israel/Palestine
Authors: Shalev Guy 
Published: August 2022

AbstractThis article looks at six months of the author's repeated attempts to obtain the approval of three Helsinki Committees (HCs, Israeli hospitals’ research ethics committees) to conduct ethnographic research with Palestinian physicians in Israeli hospitals. While the research was eventually approved and carried out in two of these institutions, correspondence with HC representatives, as well as evidence of their informal moves with institutions’ management, reflect their perceptions of the risks the study posed. In the Israeli hospital, acknowledging Palestinian political subjectivity challenges the definition of Israeli nationhood as exclusively Jewish and contaminates the allegedly politically neutral medical sphere. These committees exerted their power to serve their institutions and state ideology. This, I argue, should not be understood as anomalous instances of negligence. I show how the committees’ censorship was attuned to the Declaration of Helsinki as their guiding text and Zionism as their underlying ideology. Embedded in the powerful regimes of ethics, bureaucracy, science, and health, ethics committees employ “unarmed power” that is beyond critique. They are well‐oiled “anti‐politics machines,” rearticulating political concerns into a depoliticized moral discourse. As such, they not only limit academic inquiry but also redefine, in political terms, the realm of the moral.
Management of prolonged meconium evacuation in preterm infants: A survey‐based analysis in German Neonatal Intensive Care Units
Authors: Lange Matthias , Figura Yannick , Böhne Carolin , Beske Florian , Bohnhorst Bettina , Heep Axel 
Published: August 2022

AbstractAimDue to the functional immaturity of bowel motility, a delayed passage frequently requires evacuation of meconium in preterm infants. Often rectal enemas and oral laxatives are used to manage these bowel evacuation disorders.MethodsAn online survey was sent to all 163 high‐level Neonatal Intensive Care Units (NICUs) in Germany. The participants were queried on rectal enemas, laxative therapy and outcome incidences.ResultsA total of 110/163 (67.5%) hospitals participated in the study. 103/110 (93.6%) participating sites applied rectal enemas in cases of delayed meconium evacuation and 63/110 (57.3%) additionally used oral laxatives. In total, 15 different solutions and 7 different application systems were used for rectal instillation. Preterm infants receiving enemas within the first 48 hours after birth were found to have a significantly lower incidence of FIP (p = 0.006). Altogether 8 different oral laxatives were utilised.ConclusionTherapeutic approaches to the management of prolongated meconium evacuation differ widely among German NICUs. Our survey highlights the diversity of applied substances, means of application and differences in duration and frequency of interventions. Macrogol was commonly used in neonates as an oral laxative despite the lack of approval from the manufacturer.
A risk calculator to predict suicide attempts among individuals withearly‐onsetbipolar disorder
Authors: Goldstein Tina R. , Merranko John , Hafeman Danella , Gill Mary Kay , Liao Fangzi , Sewall Craig , Hower Heather , Weinstock Lauren , Yen Shirley , Goldstein Benjamin , Keller Martin , Strober Michael , Ryan Neal , Birmaher Boris 
Published: August 2022

AbstractObjectivesTo build a one‐year risk calculator (RC) to predict individualized risk for suicide attempt in early‐onset bipolar disorder.MethodsYouth numbering 394 with bipolar disorder who completed ≥2 follow‐up assessments (median follow‐up length = 13.1 years) in the longitudinal Course and Outcome of Bipolar Youth (COBY) study were included. Suicide attempt over follow‐up was assessed via the A‐LIFE Self‐Injurious/Suicidal Behavior scale. Predictors from the literature on suicidal behavior in bipolar disorder that are readily assessed in clinical practice were selected and trichotomized as appropriate (presence past 6 months/lifetime history only/no lifetime history). The RC was trained via boosted multinomial classification trees; predictions were calibrated via Platt scaling. Half of the sample was used to train, and the other half to independently test the RC.ResultsThere were 249 suicide attempts among 106 individuals. Ten predictors accounted for >90% of the cross‐validated relative influence in the model (AUC = 0.82; in order of relative influence): (1) age of mood disorder onset; (2) non‐suicidal self‐injurious behavior (trichotomized); (3) current age; (4) psychosis (trichotomized); (5) socioeconomic status; (6) most severe depressive symptoms in past 6 months (trichotomized none/subthreshold/threshold); (7) history of suicide attempt (trichotomized); (8) family history of suicidal behavior; (9) substance use disorder (trichotomized); (10) lifetime history of physical/sexual abuse. For all trichotomized variables, presence in the past 6 months reliably predicted higher risk than lifetime history.ConclusionsThis RC holds promise as a clinical and research tool for prospective identification of individualized high‐risk periods for suicide attempt in early‐onset bipolar disorder.
Factors associated with person‐centered care during pregnancy and birth for Black women and birthing people in California
Authors: Altman Molly R. , Afulani Patience A. , Melbourne Daphina , Kuppermann Miriam 
Published: August 2022

AbstractIntroductionIn the United States, adverse birth outcomes are most prevalent among Black women and birthing people, who are known to experience increased mistreatment and disrespect in care. The purpose of this paper is to describe findings from a study that used two validated scales to examine factors associated with experiences of person‐centered care during pregnancy and birth among Black birthing people in California.MethodsWe used data from 234 Black women and birthing people who were recruited between January and September 2020 as part of a validation study for the Person‐Centered Prenatal Care (PCPC) and the Person‐Centered Maternity Care (PCMC‐US) scales. Bivariate and multivariate analyses were conducted to examine the associations between PCPC and PCMC‐US scores and sociodemographic, health‐related, and care‐related factors.ResultsThe factors associated with lower PCPC scores were having foreign‐born parents, having public or no insurance, part‐time employment, unstable housing, late start of prenatal care (in the second or third trimester), poorer self‐rated health (less than very good), and lack of continuity of care with prenatal providers. Factors associated with lower PCMC‐US scores were having public insurance, late start of prenatal care, longer length of stay in facility following birth, poorer self‐rated health, lack of continuity of care between prenatal care and birth providers, and racial discordance with birth provider.ConclusionsOur analysis highlights the contributions of intersecting identities as well as health‐related and care‐related factors to Black birthing people's experiences of care in California. Continuity of care and provider racial concordance, in particular, were shown to improve pregnancy and birth experiences.
Real‐life efficacy and safety of idelalisib in 55 double‐refractory follicular lymphoma patients
Authors: Isidori Alessandro , Loscocco Federica , Visani Giuseppe , Paolasini Sara , Scalzulli Potito , Musto Pellegrino , Perrone Tommasina , Guarini Attilio , Pastore Domenico , Mazza Patrizio , Tonialini Lorenzo , Pavone Vincenzo , De Santis Gaetano , Tarantini Giuseppe 
Published: August 2022

SummaryIdelalisib, a reversible inhibitor of PI3Kδ (phosphoinositide‐3 kinase delta), showed remarkable activity in the phase II DELTA trial, leading to its approval by the European Medicines Agency (EMA) in patients with relapsed/refractory (R/R) follicular lymphoma (FL). However, real‐life data on idelalisib are scarce. We treated 55 double‐refractory FL patients with idelalisib in a real‐life setting. With a median exposure to idelalisib of 10 months (range 1–43), overall response rate was 73%, the highest ever reported. Non‐haematological toxicities were mild and manageable. At 12 months, 80% of patients were alive, and 72% disease‐free. The efficacy and safety of idelalisib was confirmed in a real‐life setting.
A review on computer‐aided chemogenomics and drug repositioning for rationalCOVID‐19 drug discovery
Authors: Maghsoudi Saeid , Taghavi Shahraki Bahareh , Rameh Fatemeh , Nazarabi Masoomeh , Fatahi Yousef , Akhavan Omid , Rabiee Mohammad , Mostafavi Ebrahim , Lima Eder C. , Saeb Mohammad Reza , Rabiee Navid 
Published: August 2022

AbstractApplication of materials capable of energy harvesting to increase the efficiency and environmental adaptability is sometimes reflected in the ability of discovery of some traces in an environment―either experimentally or computationally―to enlarge practical application window. The emergence of computational methods, particularly computer‐aided drug discovery (CADD), provides ample opportunities for the rapid discovery and development of unprecedented drugs. The expensive and time‐consuming process of traditional drug discovery is no longer feasible, for nowadays the identification of potential drug candidates is much easier for therapeutic targets through elaborate in silico approaches, allowing the prediction of the toxicity of drugs, such as drug repositioning (DR) and chemical genomics (chemogenomics). Coronaviruses (CoVs) are cross‐species viruses that are able to spread expeditiously from the into new host species, which in turn cause epidemic diseases. In this sense, this review furnishes an outline of computational strategies and their applications in drug discovery. A special focus is placed on chemogenomics and DR as unique and emerging system‐based disciplines on CoV drug and target discovery to model protein networks against a library of compounds. Furthermore, to demonstrate the special advantages of CADD methods in rapidly finding a drug for this deadly virus, numerous examples of the recent achievements grounded on molecular docking, chemogenomics, and DR are reported, analyzed, and interpreted in detail. It is believed that the outcome of this review assists developers of energy harvesting materials and systems for detection of future unexpected kinds of CoVs or other variants.
MOG‐antibody‐associated disorder with hypothalamic lesions associated with hypersomnia and decrease of orexin in CSF: A case report
Authors: Menjo Kanako , Ashida Shinji , Murata Shohei , Tanaka Eijirou , Fujii Chihiro , Tanaka Masami , Tanaka Keiko , Kanbayashi Takashi , Mizuno Toshiki 
Published: August 2022

AbstractBackgroundMyelin oligodendrocyte glycoprotein antibody‐associated disease (MOGAD) is an inflammatory disorder of the central nervous system. Here, we are the first to report a MOGAD patient with hypersomnolence caused by bilateral hypothalamic lesions.Case presentationThe case involved a 51‐year‐old female patient with MOGAD who showed hypersomnolence. She had been diagnosed with MOGAD at 50 years old and admitted due to the development of optic neuritis and encephalitis while under corticosteroid and immunosuppressant treatment. These were improved after the introduction of intravenous methylprednisolone and plasma exchange. The orexin level in the cerebrospinal fluid (CSF) was decreased to 142.2 pg/mL (<200 pg/mL) upon admission and improved to a normal level (298.3 pg/mL) after immunotherapy. Bilateral hypothalamic lesions and reduction of the orexin level in the CSF were considered associated with hypersomnia.ConclusionThe orexin level in CSF was useful to monitor hypothalamic dysfunction in a patient with MOGAD.
Population pharmacokinetics of everolimus in adult liver transplant patients: Comparison to tacrolimus disposition and extrapolation to pediatrics
Authors: Itohara Kotaro , Yano Ikuko , Nakagawa Shunsaku , Sugimoto Mitsuhiro , Hirai Machiko , Yonezawa Atsushi , Imai Satoshi , Nakagawa Takayuki , Hira Daiki , Ito Takashi , Hata Koichiro , Hatano Etsuro , Terada Tomohiro , Matsubara Kazuo 
Published: August 2022

AbstractEverolimus has recently been used to prevent graft rejection in liver transplantation and reduces the incidence of kidney dysfunction caused by calcineurin inhibitors. In this study, a population pharmacokinetic analysis was conducted to improve the individualization of everolimus therapy. Japanese post‐liver transplant patients whose blood everolimus concentrations were measured between March 2018 and December 2020 were included in this study. A nonlinear mixed‐effect modeling program was used to explore covariates that affect everolimus pharmacokinetics. Individual everolimus pharmacokinetic parameters estimated by the post‐hoc Bayesian analysis using the final model were compared with the tacrolimus dose per trough concentration (D/C) ratio in each patient. The final model was extrapolated to pediatric liver transplant patients for external evaluation. A total of 937 concentrations from 87 adult patients were used in the model‐building process. Everolimus clearance was significantly affected by the estimated glomerular filtration rate, concomitant use of fluconazole, sex, as well as total daily dose of everolimus (TDM effect). The estimated individual apparent clearance of everolimus by the post‐hoc Bayesian analysis was moderately correlated with the D/C ratio of tacrolimus in each patient (R2 = 0.330, p < 0.0001). The estimation accuracy in pediatric patients was considerably high, except for one infant out of 13 patients. In conclusion, population pharmacokinetic analysis clarified several significant covariates for everolimus pharmacokinetics in liver transplant patients. Everolimus pharmacokinetics moderately correlated with tacrolimus pharmacokinetics and could be extrapolated from adult to pediatric patients by body size correction, except for infants.
Repertoires of citizen action in hybrid settings
Authors: Gaventa John 
Published: August 2022

AbstractMotivationIn recent years, a growing literature has emerged analysing how organized citizen action can achieve more accountable and inclusive governance. While this literature is mostly focused on relatively open, democratic, or more stable contexts, often in western democracies, only a small proportion of the world's population lives in such settings. Rather, authoritarianism is growing and democratic spaces are closing globally.PurposeThis article explores how hybrid political settings—those which have both democratic and non‐democratic features, fragmented authority, and closing civic space—affect strategies of citizen action.Methods and approachDrawing from a large number of single and comparative case studies of citizen action, mainly from Mozambique, Myanmar, Nigeria, and Pakistan, the article builds on work by Tilly and Tarrow on repertoires of contention. Based on these, the article develops a typology of eight repertoires of citizen action through which citizens may express their grievances and attempt to hold authorities to account.FindingsThe literature suggests that there may be little space for contentious social and political action in closed or authoritarian settings. This article finds, however, that a rich and diverse tapestry of citizen action may be seen in the “hybrid” settings (those which have both democratic and non‐democratic features). Shaped by political opportunities as well as by key trigger events, these repertoires will vary according to their visibility and their level of “ruliness” and may shift or re‐enforce one another over time, contributing to building blocks for broader accountability and democratic governance.Policy implicationsPolicy and donor interventions aimed at supporting citizen‐led action for accountability have often focused on the most visible and ruly of these strategies. However, we find that the more hidden or more unruly forms of action are important in and of themselves and help to create building blocks for broader changes. For those who are interested in supporting citizen‐led accountability, mapping these broader repertoires will be important to understand the full range of accountability strategies available, going beyond more commonly understood approaches.
Pathogenicity of three Botryosphaeriaceae fungi, Diplodia scrobiculata, Diplodia mutila, and Dothiorella californica, isolated from coast redwood (Sequoia sempervirens) in California
Authors: Lee Christopher A. , Rooney‐Latham Suzanne , Brown Albre A. , McCormick Megan , Baston David 
Published: August 2022

AbstractThree Botryosphaeriaceae species, not previously recorded on coast redwood, were detected from native redwood stands in Humboldt Co., CA during general surveys between 2014 and 2017. Pathogenicity experiments with three of these fungi, Diplodia scrobiculata, Diplodia mutila, and Dothiorella californica, were performed on 2‐year‐old redwood seedlings to fulfil Koch's postulates and prove pathogenicity. Only D. scrobiculata produced lesions significantly longer than control lesions, although they were generally smaller than those of more virulent Botryosphaeriaceae pathogens in previous studies. In a separate pathogenicity study of D. scrobiculata on younger seedlings without secondary growth, inoculations on wounded stems resulted in black lesions that were much more externally obvious than the wound inoculations on lignified stems after 90 days. However, no lesions developed on non‐wounded stems of younger seedlings. Lesion lengths caused by D. scrobiculata were consistent between the two studies. On one wound‐inoculated, non‐woody seedling, D. scrobiculata was recovered from asymptomatic stem tissue 2 cm below the inoculation site, indicating possible pathogen latency. These experiments indicate that there are more Botryosphaeriaceae fungi involved in coast redwood dieback than previously known and confirm the pathogenicity of D. scrobiculata on primary and secondary tissue of coast redwood.