bluuebunny/crossref_metadata_2025_split huggingface dataset:
Real‐world evidence of the safety and survival with CD19 CAR‐T cell therapy for relapsed/refractory solid organ transplant‐related PTLD
Authors: McKenna Marshall , Epperla Narendranath , Ghobadi Armin , Liu Jieqi , Lazaryan Aleksandr , Ibrahim Uroosa , Jacobson Caron A. , Naik Seema G. , Nastoupil Loretta , Chowdhury Sayan Mullick , Voorhees Timothy J. , Jacobs Miriam T. , Farooq Umar , Osman Keren , Olszewski Adam J. , Ahmed Sairah , Evens Andrew M. 
Published: May 2023

SummaryThe use of CD19 chimeric antigen receptor T‐cell (CAR‐T) therapy for relapsed/refractory solid organ transplantation (SOT)‐related post‐transplant lymphoproliferative disorder (PTLD) is not well studied. We conducted a multicentre, retrospective analysis of adults with relapsed/refractory SOT‐associated PTLD. Among 22 relapsed/refractory SOT‐PTLD patients, the pathology was monomorphic B cell. Prior SOTs included 14 kidney (64%), three liver (14%), two heart (9%), one intestinal (5%), one lung (5%), and one pancreas after kidney transplant (5%). The median time from SOT to PTLD diagnosis was 107 months. Pre‐CAR‐T bridging therapy was used in 55% of patients, and immunosuppression was stopped completely before CAR‐T infusion in 64%. Eighteen (82%) patients experienced cytokine release syndrome: one (5%) each grade (G) 3 and G4. The immune effector cell‐associated neurotoxicity syndrome was observed in 16 (73%) patients: six (27%) G3 and two (9%) G4. The overall response rate was 64% (55% complete response). Three patients (14%) experienced allograft rejection after CAR‐T. The two‐year progression‐free survival and overall survival rates were 35% and 58%, respectively. Additionally, the achievement of CR post‐CAR‐T was strongly associated with survival. Collectively, the safety and efficacy of CD19 CAR‐T therapy in relapsed/refractory SOT‐related PTLD appeared similar to pivotal CAR‐T data, including approximately one‐third of patients achieving sustained remission.
Sustained response off treatment in eltrombopag for children with persistent/chronic primary immune thrombocytopenia: A multicentre observational retrospective study in China
Authors: Wang Zhifa , Wang Lijuan , Liu Yan , Meng Jinxi , Dong Shuyue , Ma Jingyao , Hu Yu , Chen Zhenping , Cheng Xiaoling , Wu Runhui 
Published: May 2023

SummaryEltrombopag (ELT) is effective and safe in adult persistent/chronic immune thrombocytopenia (p/cITP); a proportion could achieve a sustained response off treatment (SRoT); however, data on children are lacking. We attempted to analyse SRoT of ELT in children with p/cITP in this study. A multicentre retrospective observational study was performed in November 2022 for children with p/cITP who used ELT alone for >2 months between January 2017 and November 2021. Clinical data of pre‐, during and post‐ELT were collected. SRoT was defined as maintaining a platelet count of ≥30 × 109/L without rescue therapy for at least 6 months off ELT. There were 143 patients enrolled; 69.2% (99/143) achieved an overall response of 43.3% and 25.9% achieved complete response (CR) and response (R). Among the 35 patients analysed from whom ELT was withdrawn, 71.4% (25/35) showed SRoT after discontinuing ELT without additional ITP therapy, with a median follow‐up of 0.94 (range, 0.53–3.8) years, equal to 17.5% (25/143) in all patients treated with ELT. Compared with the patients with relapse (n = 10), the SRoT patients (n = 25) had a higher rate of CR (80% [20/25] vs. 40% [4/10]), shorter interval time from initiation to taper (6.4 months vs. 9.4 months), longer time from taper to withdrawal (1.1 years vs. 0.3 years) and a longer duration of ELT treatment (1.6 years vs. 0.5 years) with p < 0.05. Patients who achieved CR could attain SRoT more easily (p = 0.02). ELT had a response in 69.2% of children with p/cITP and 17.5% of them attained SRoT with good tolerance. The patients who achieved CR and began ELT treatment as early as possible, with a longer treatment duration and slower tapering, had a higher probability of SRoT.
Validation of a prediction model for post‐chemotherapy fibrosis in nonseminoma patients
Authors: Gerdtsson Axel , Torisson Gustav , Thor Anna , Grenabo Bergdahl Anna , Almås Bjarte , Håkansson Ulf , Törnblom Magnus , Negaard Helene F. S. , Glimelius Ingrid , Halvorsen Dag , Karlsdóttir Ása , Haugnes Hege Sagstuen , Larsen Signe Melsen , Holmberg Göran , Wahlqvist Rolf , Tandstad Torgrim , Cohn‐Cedermark Gabriella , Ståhl Olof , Kjellman Anders 
Published: May 2023

ObjectiveTo validate Vergouwe's prediction model using the Swedish and Norwegian Testicular Cancer Group (SWENOTECA) RETROP database and to define its clinical utility.Materials and methodsVergouwe's prediction model for benign histopathology in post‐chemotherapy retroperitoneal lymph node dissection (PC‐RPLND) uses the following variables: presence of teratoma in orchiectomy specimen; pre‐chemotherapy level of alpha‐fetoprotein; β‐Human chorionic gonadotropin and lactate dehydrogenase; and lymph node size pre‐ and post‐chemotherapy. Our validation cohort consisted of patients included in RETROP, a prospective population‐based database of patients in Sweden and Norway with metastatic nonseminoma, who underwent PC‐RPLND in the period 2007–2014. Discrimination and calibration analyses were used to validate Vergouwe's prediction model results. Calibration plots were created and a Hosmer–Lemeshow test was calculated. Clinical utility, expressed as opt‐out net benefit (NBopt‐out), was analysed using decision curve analysis.ResultsOverall, 284 patients were included in the analysis, of whom 130 (46%) had benign histology after PC‐RPLND. Discrimination analysis showed good reproducibility, with an area under the receiver‐operating characteristic curve (AUC) of 0.82 (95% confidence interval 0.77–0.87) compared to Vergouwe's prediction model (AUC between 0.77 and 0.84). Calibration was acceptable with no recalibration. Using a prediction threshold of 70% for benign histopathology, NBopt‐out was 0.098. Using the model and this threshold, 61 patients would have been spared surgery. However, only 51 of 61 were correctly classified as benign.ConclusionsThe model was externally validated with good reproducibility. In a clinical setting, the model may identify patients with a high chance of benign histopathology, thereby sparing patients of surgery. However, meticulous follow‐up is required.
Respecting entitlements in legislative bargaining: A matter of preference or necessity?
Authors: Gantner Anita , Oexl Regine 
Published: May 2023

AbstractWe investigate whether fairness concerns overrule strategic behaviour in legislative bargaining with entitlements. In a lab experiment, we vary bargaining power by using either majority rule or dictator rule to implement a division. We apply coarse measures in order to assess whether entitlements are respected. Our results show that with experience over one third of proposers make “extreme” offers, assigning at most 10% of the surplus to partners whose consent is not needed under the respective rule. Having observed extreme outcomes in the past increases the likelihood of own extreme proposals. Overall, we find significant limitations in the acknowledgment for others' entitlements, in particular for groups in which individual contributions differ.
Autoimmune cerebellar ataxia
Authors: Yaguchi Hiroaki , Kudo Akihiko , Yabe Ichiro 
Published: May 2023

AbstractAmong the different forms of cerebellar ataxia, autoimmune cerebellar ataxia (ACA) or immune‐mediated cerebellar ataxia (IMCA), which appears to be based on an autoimmune mechanism, has long been considered important from the viewpoint of paraneoplastic syndrome. With the expansion of the concept of immune‐mediated neurological diseases and the identification of many novel autoantibodies, ACA has recently become an important neurological syndrome. Although no definitive diagnostic criteria of ACA exist, Hadjivassilou et al suggested diagnostic criteria for primary ACA in 2020 and Dalmau and Graus showed proposed diagnostic criteria for ACA in 2022. The proposed diagnostic criteria for ACA by Dalmau and Graus emphasize the importance of antibody reliability. In the future, additional studies should be conducted to identify new antibodies associated with ACA and to clarify the importance of each antibody. Moreover, ACA is a disease for which immunological therapeutic intervention is feasible and requires early treatment to maintain cerebellar function. Many cases with ACA have reported the efficacy of immunotherapy. The concept of ACA may have the potential for further expansion and becomes increasingly important in the diagnosis of cerebellar ataxia.
Predictors of pouch failure: A tertiary care inflammatory bowel disease centre experience
Authors: Ricardo Alison P. , Kayal Maia , Plietz Michael C. , Khaitov Sergey , Sylla Patricia , Dubinsky Marla C. , Greenstein Alexander 
Published: May 2023

AbstractAimRates of pouch failure after total proctocolectomy with ileal pouch−anal anastomosis (IPAA) range from 5% to 18%. There is little consistency across studies regarding the factors associated with failure, and most include patients who underwent IPAA in the pre‐biologic era. Our aim was to analyse a cohort of patients who underwent IPAA in the biologic era at a large‐volume inflammatory bowel disease institution to better determine preoperative, perioperative and postoperative factors associated with pouch failure.MethodsA retrospective cohort analysis was performed with data from an institutional review board approved prospective database with ulcerative colitis or unclassified inflammatory bowel disease patients who underwent total proctocolectomy with IPAA at Mount Sinai Hospital between 2008 and 2017. Preoperative, perioperative and postoperative data were collected and univariate and multivariate analyses were performed to identify factors associated with increased risk of pouch failure.ResultsOut of 664 patients included in the study, pouch failure occurred in 41 (6.2%) patients, a median of 23.3 months after final surgical stage. Of these, 17 (41.4%) underwent pouch excision and 24 (58.5%) had diverting ileostomies. The most common indications for pouch failure were Crohn's disease like pouch inflammation (CDLPI) (n = 17, 41.5%), chronic pouchitis (n = 6, 14.6%), chronic cuffitis (n = 5, 12.2%) and anastomotic stricture (n = 4, 9.8%). On multivariate analysis, pre‐colectomy biologic use (hazard ratio [HR] 2.25, 95% CI 1.09–4.67), CDLPI (HR 3.18, 95% CI 1.49–6.76) and pouch revision (HR 2.59, 95% CI 1.26–5.32) were significantly associated with pouch failure.ConclusionsPouch failure was significantly associated with CDLPI, preoperative biologic use and pouch revision; however, reassuringly it was not associated with postoperative complications.
Patient and multidisciplinary team perspectives on watch and wait in rectal cancer
Authors: Mohan Helen , Rabie Mohammed , Walsh Ciaran , Harji Deena , Sutton Paul , Geh Ian , Jackson Ian , Helbren Emma , Evans Martyn , Jenkins John T. 
Published: May 2023

AbstractThis article adopts a multidisciplinary approach, including surgery, oncology, radiology and patient perspectives, to discuss the key points of debate surrounding a watch and wait approach. In an era of shared decision‐making, discussion of watch and wait as an option in the context of complete clinical response is appropriate, although it is not the gold standard treatment. Key challenges are the difficulty in assessing for a complete clinical response, prediction of recurrence and access to timely diagnostics for surveillance. Salvage surgery has good results if regrowth is detected early but does have imperfect outcomes, with only a 90% salvage rate. Good communication with patients about the risks and alternatives is essential. Patients undergoing watch and wait should ideally be enrolled in prospective registries or clinical trials.
Assessment of the physical dependence potential of difelikefalin: Randomized placebo‐controlled study in patients receiving hemodialysis
Authors: Spencer Robert H. , Munera Catherine , Shram Megan J. , Menzaghi Frédérique 
Published: May 2023

AbstractDifelikefalin is a selective kappa opioid receptor agonist approved for treating moderate‐to‐severe pruritus in adults undergoing hemodialysis (HD). Difelikefalin is not a controlled substance under the Controlled Substances Act. This study assessed the potential for developing physical dependence on difelikefalin in patients undergoing HD. Eligible patients received open‐label difelikefalin after each dialysis session for 3 weeks before entering a 2‐week double‐blind phase, when they were randomized to either continue difelikefalin or to switch to receiving placebo. Signs of physical withdrawal were assessed using the Clinical Opiate Withdrawal Scale (COWS), several patient‐reported scales, and physiological measures. The primary end point was the between‐group difference in mean maximum COWS total scores during the double‐blind phase; the mean difference (placebo − difelikefalin) was compared against a predefined noninferiority limit (+4). Thirty‐five patients (57.1% male; 91.4% Black or African American; median [range] age 58 [28–77] years) were included, of which 30 were randomized (placebo, n = 14; difelikefalin, n = 16). The least squares mean difference in maximum COWS total scores was 0.52 (95% confidence interval [CI]: −0.56, 1.59). The upper CI limit (1.59) was below +4, indicating that patients who discontinued difelikefalin (placebo group) had similar withdrawal scores to patients who continued difelikefalin. Additional assessments supported the COWS results, showing no meaningful differences between groups in physiological measures or in patient‐reported measures of sleep or physical withdrawal. These results demonstrate that abruptly discontinuing chronic difelikefalin treatment in patients undergoing HD does not produce signs or symptoms of physical withdrawal.
Adnexotropic and granulomatous mycosis fungoides following TNF‐α inhibitor treatment
Authors: Doughty Hayden , Scripture Andrew , Carter Joi B. , Sriharan Aravindhan , Yan Shaofeng , Lansigan Frederick , Momtahen Shabnam 
Published: May 2023

AbstractRecent publications have documented an increased prevalence of cutaneous T‐cell lymphoma (CTCL) in patients undergoing tumor necrosis factor alpha (TNF‐α) inhibitor therapy. Herein, we present an uncommon manifestation of mycosis fungoides (MF) with unique pathological findings after the initiation of adalimumab therapy for the treatment of psoriasis. One year after starting treatment, the patient noticed a slowly growing, eroded plaque on the left cheek, the biopsy of which demonstrated mixed granulomatous and adnexotropic lymphocytic infiltrate with features characteristics of MF. In the following months, the patient developed pink‐ and violet‐colored scaly plaques on the right posterior upper arm and right medial upper arm. Biopsy of these plaques also revealed findings compatible with MF. T‐cell receptor (TCR) clonality studies by PCR revealed identical T‐cell clones in the samples obtained from the cheek, right posterior upper arm, and right medial upper arm. TCR clonality studies of a long‐standing psoriatic plaque on the right thigh failed to reveal similar T‐cell clones. Blurring of histopathologic presentation by TNF‐α inhibitors could greatly complicate the identification of MF subtypes. Providers treating patients with TNF‐α inhibitors must be aware of the risk of cutaneous lymphoma development and the potential deviations from their expected presentations. In patients without an initial biopsy, the possibility of pre‐existing CTCL with psoriasiform presentation should be considered.
Neuropsychiatric phenotypes in children with Noonan syndrome
Authors: Naylor Paige E. , Bruno Jennifer L. , Shrestha Sharon Bade , Friedman Marcelle , Jo Booil , Reiss Allan L. , Green Tamar 
Published: May 2023

AbstractAimWe investigated neuropsychiatric outcomes in children with Noonan syndrome and addressed limitations in previous research with a focus on prepubertal children, comparison to typically developing children, comprehensive neuropsychiatric evaluation, and controlling for overall cognitive abilities.MethodForty‐five children with Noonan syndrome (mean = 8 years 6 months, SD = 2 years 2 months; 29 females) and 40 typically developing children (mean = 8 years 9 months, SD = 2 years; 22 females) were evaluated with objective, parent‐report, and psychiatric interview measures.ResultsChildren with Noonan syndrome demonstrated elevated symptoms across attention‐deficit/hyperactivity disorder (ADHD) (attention, hyperactivity, and inhibition), autism spectrum disorder (ASD) (maintaining social relationships, behavioral rigidity, and sensory sensitivity), and oppositional defiant disorder (ODD) (aggression) symptom clusters relative to typically developing children (all p < 0.05). Group differences in nearly all parent‐report measures were significant after accounting for variations in intellectual functioning, suggesting that increased neurodevelopmental symptoms are not simply driven by overall intelligence. Twenty out of 42 children with Noonan syndrome met criteria for ADHD, eight out of 42 for ODD, and 11 out of 43 demonstrated clinically significant symptoms seen in children with ASD.InterpretationChildren with Noonan syndrome are at increased risk for a range of ADHD, ASD, and ODD associated symptoms. A dimensional approach reveals significant ASD symptoms in Noonan syndrome that do not emerge when using the currently accepted categorical diagnostic approach.What this paper adds

Neuropsychiatric disorders occur in more than half of children with Noonan syndrome.
Children with Noonan syndrome demonstrate highly variable neurodevelopmental symptom profiles.
Children with Noonan syndrome display variable impairments in attention, hyperactivity, and inhibition.
Specific social concerns include behavioral rigidity, transitions, and difficulties maintaining social relationships.
Children with Noonan syndrome display variably elevated levels of aggression and emotional dysregulation.
Assessment of myocardial function in infants conceived by assisted reproductive technologies using deformation imaging over the first year of age: A cohort study
Authors: Smith Aisling , Franklin Orla , Mocanu Edgar , McCallion Naomi , EL‐Khuffash Afif 
Published: May 2023

AbstractBackground and AimThere is emerging evidence of cardiovascular remodeling and functional impairment in individuals conceived via Assisted Reproductive Technologies (ART). The aim of this study was to serially assess myocardial function and pulmonary hemodynamic measurements in infants conceived via ART over the first year of age and to compare them to a cohort of spontaneously conceived controls.MethodsThis was a prospective, observational study. Echocardiography was performed at Day 2, 6 months and 1 year of age. Biventricular function was assessed by deformation analysis. Pulmonary artery acceleration time (PAAT) and left ventricular (LV) eccentricity index (LVEI) provided surrogate measures of pulmonary vascular resistance (PVR).ResultsFifty infants conceived via ART were compared to 50 spontaneously conceived controls. There were no differences in baseline infant demographics between the two groups. At 1 year of age right ventricular (RV) basal and RV mid cavity diameters were higher in the ART group. PAATi was lower and LVEI higher in the ART group at 6 months and 1 year. In the ART group, LV global longitudinal strain, LV systolic strain rate, LV early diastolic strain rate and RV free wall strain were lower on Day 2, 6 months, and 1 year of age in comparison to the control group (all p < .05). Within the ART group, on linear regression, maternal age, the type of ART treatment or egg characteristics did not influence PAAT or deformation measurements.ConclusionOur findings suggest that greater cardiovascular surveillance of ART conceived infants may be warranted.
Quantification of invertebrates on fungal fruit bodies by the use of time‐lapse cameras
Authors: Lunde Lisa F. , Ferkingstad Bendik , Wegger Hermann , Høye Toke T. , Mann Hjalte M. R. , Birkemoe Tone 
Published: May 2023

Abstract
Fungi and invertebrates comprise a major part of biodiversity in dead wood ecosystems and invertebrates depend on fungi to utilise the dead wood resource. Many invertebrates also visit the long‐lived fruit bodies of wood‐decay fungi to feed on spores, the hymenium or other invertebrates. However, as traditional sampling methods are labour‐intensive, we know little of these interactions.
In this study, we use time‐lapse cameras to monitor invertebrates visiting the hymenium of a common wood‐decay fungus, Fomitopsis pinicola, and explain their activity in terms of temporal variation, temperature and presence of Gyrophaena boleti, a highly abundant fungivorous beetle living primarily in fruit bodies of F. pinicola.
The most common invertebrates on F. pinicola fruit bodies were Coleoptera, Araneae, Diptera, Gastropoda and Chilopoda. The invertebrate activity exhibited strong temporal variation with higher abundance during night and, for Coleoptera, earlier in the season. We discuss how this might correlate with the sporulation period of F. pinicola. The presence of G. boleti had a positive impact on the predatory Lordithon lunulatus and Ipidia binotata, and a negative impact on the fungivorous Thymalus limbatus and Peltis ferruginea. Chilopoda and L. lunulatus were ephemeral visitors, while the fungivorous Coleoptera and Araneae stayed the longest.
We estimated the invertebrates' visitation frequency and duration, which would be time‐consuming to obtain with traditional methods. We offer improvements to our method and urge future research on invertebrate–fungus interactions to quantify invertebrate visits to fungal fruit bodies.
Vitality characterization of stressed trees based on non‐destructive and real‐time monitoring of stem water content
Authors: Liu Lei , Song Runze , Han Qiaoling , Zhao Yue , Zhao Yandong 
Published: May 2023

AbstractThe detection of stem water content is necessary as it is an important indicator for measuring woody plant vitality. However, the relationship between stem water content, determined by non‐destructive, real‐time, and long‐term monitoring, and woody plant vitality remains undefined. In this study, the response of woody plant vitality to stem water content under different stress (freeze–thaw, pest, or drought) was analysed by mining the dynamic characteristics of the stem water content in different woody plants at the temporal scales of year, month, and day. Compared with unstressed trees, stressed trees had contrasting diurnal patterns. The stem water content in Populus koreana Rehd. during the freeze period was much lower than that during the thaw period, and opposite diurnal variation trends were observed during the freeze and thaw periods. The stem water content in infected Lagerstroemia indica was lower than that in uninfected L. indica, and the amplitude of the diurnal variation curve was lower in infected than in uninfected L. indica. Under drought stress, the more severe the water shortage, the lower the stem water content in Malus micromalus. When it was below a certain threshold, the diurnal variation trend was opposite to that without water shortage. In conclusion, stem water content dynamics can be used to evaluate the cold, pest, and drought response of trees, which could monitor tree health and guide forest assessment.