bluuebunny/crossref_metadata_2025_split huggingface dataset:
Response and resistance to cladribine in patients with advanced systemic mastocytosis: a registry-based analysis
Authors: Lübke Johannes , Naumann Nicole , Metzgeroth Georgia , Kreil Sebastian , Brand Timo , Horny Hans-Peter , Sotlar Karl , Cross Nicholas C. P. , Fabarius Alice , Valent Peter , Hofmann Wolf-Karsten , Reiter Andreas , Schwaab Juliana 
Published: April 2023

Abstract
We sought to evaluate the efficacy of the purine analogue cladribine in 79 patients with advanced systemic mastocytosis (AdvSM) using data from the ‘German Registry on Disorders of Eosinophils and Mast Cells (GREM)’. The overall response rate according to modified Valent criteria (46 evaluable patients) for first- (1L) and second-line (2L) cladribine treatment was 41% (12/29) and 35% (6/17, P = 0.690), respectively, and the median overall survival (OS, all patients evaluable) was 1.9 years (n = 48) and 1.2 years (n = 31; P = 0.311). Univariate and multivariable analyses of baseline and on-treatment parameters identified diagnosis of mast cell leukemia (hazard ratio [HR] 3.5, 95% confidence interval [CI, 1.3–9.1], P = 0.012), eosinophilia ≥ 1.5 × 109/L (HR 2.9 [CI 1.4–6.2], P = 0.006) and < 3 cycles of cladribine (HR 0.4 [CI 0.2–0.8], P = 0.008) as independent adverse prognostic parameters for OS. There was no impact of other laboratory (anemia, thrombocytopenia, serum tryptase) or genetic markers (mutations in SRSF2, ASXL1 or RUNX1) on OS. In consequence, none of the recently established prognostic scoring systems (MARS, IPSM, MAPS or GPSM) was predictive for OS. Modified Valent criteria were superior to a single factor-based response assessment (HR 2.9 [CI 1.3–6.6], P = 0.026). In conclusion, cladribine is effective in 1L and 2L treatment of AdvSM. Mast cell leukemia, eosinophilia, application of < 3 cycles and a lack of response are adverse prognostic markers.
German specialists treating testicular cancer follow different guidelines with resulting inconsistency in assessment of retroperitoneal lymph-node metastasis: clinical implications and possible corrective measures
Authors: Schoch Justine , Haunschild Kathrin , Strauch Angelina , Nestler Kai , Schmelz Hans , Paffenholz Pia , Pfister David , Persigehl Thorsten , Heidenreich Axel , Nestler Tim 
Published: April 2023

Abstract
Background
Testicular germ cell tumors (GCTs) are aggressive but highly curable tumors. To avoid over/undertreatment, reliable clinical staging of retroperitoneal lymph-node metastasis is necessary. Current clinical guidelines, in their different versions, lack specific recommendations on how to measure lymph-node metastasis.

Objective
We aimed to assess the practice patterns of German institutions frequently treating testicular cancer for measuring retroperitoneal lymph-node size.

Methods
An 8‐item survey was distributed among German university hospitals and members of the German Testicular Cancer Study Group.

Results
In the group of urologists, 54.7% assessed retroperitoneal lymph nodes depending on their short-axis diameter (SAD) (33.3% in any plane, 21.4% in the axial plane), while 45.3% used long-axis diameter (LAD) for the assessment (42.9% in any plane, 2.4% in the axial plane). Moreover, the oncologists mainly assessed lymph-node size based on the SAD (71.4%). Specifically, 42.9% of oncologists assessed the SAD in any plane, while 28.5% measured this dimension in the axial plane. Only 28.6% of oncologists considered the LAD (14.3% in any plane, 14.3% in the axial plane). None of the oncologists and 11.9% of the urologists (n = 5) always performed an MRI for the initial assessment, while for follow-up imaging, the use increased to 36.5% of oncologists and 31% of urologists. Furthermore, only 17% of the urologists, and no oncologists, calculated lymph-node volume in their assessment (p = 0.224).

Conclusion
Clear and consistent measurement instructions are urgently needed to be present in all guidelines across different specialistic fields involved in testicular cancer management.
C-terminal frameshift variant of TDP-43 with pronounced aggregation-propensity causes rimmed vacuole myopathy but not ALS/FTD
Authors: Ervilha Pereira Pedro , Schuermans Nika , Meylemans Antoon , LeBlanc Pontus , Versluys Lauren , Copley Katie E. , Rubien Jack D. , Altheimer Christopher , Peetermans Myra , Debackere Elke , Vanakker Olivier , Janssens Sandra , Baets Jonathan , Verhoeven Kristof , Lammens Martin , Symoens Sofie , De Paepe Boel , Barmada Sami J. , Shorter James , De Bleecker Jan L. , Bogaert Elke , Dermaut Bart 
Published: April 2023

AbstractNeuronal TDP-43-positive inclusions are neuropathological hallmark lesions in frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Pathogenic missense variants in TARDBP, the gene encoding TDP-43, can cause ALS and cluster in the C-terminal prion-like domain (PrLD), where they modulate the liquid condensation and aggregation properties of the protein. TDP-43-positive inclusions are also found in rimmed vacuole myopathies, including sporadic inclusion body myositis, but myopathy-causing TDP-43 variants have not been reported. Using genome-wide linkage analysis and whole exome sequencing in an extended five-generation family with an autosomal dominant rimmed vacuole myopathy, we identified a conclusively linked frameshift mutation in TDP-43 producing a C-terminally altered PrLD (TDP-43p.Trp385IlefsTer10) (maximum multipoint LOD-score 3.61). Patient-derived muscle biopsies showed TDP-43-positive sarcoplasmic inclusions, accumulation of autophagosomes and transcriptomes with abnormally spliced sarcomeric genes (including TTN and NEB) and increased expression of muscle regeneration genes. In vitro phase separation assays demonstrated that TDP-43Trp385IlefsTer10 does not form liquid-like condensates and readily forms solid-like fibrils indicating increased aggregation propensity compared to wild-type TDP-43. In Drosophila TDP-43p.Trp385IlefsTer10 behaved as a partial loss-of-function allele as it was able to rescue the TBPH (fly ortholog of TARDBP) neurodevelopmental lethal null phenotype while showing strongly reduced toxic gain-of-function properties upon overexpression. Accordingly, TDP-43p.Trp385IlefsTer10 showed reduced toxicity in a primary rat neuron disease model. Together, these genetic, pathological, in vitro and in vivo results demonstrate that TDP-43p.Trp385IlefsTer10 is an aggregation-prone partial loss-of-function variant that causes autosomal dominant vacuolar myopathy but not ALS/FTD. Our study genetically links TDP-43 proteinopathy to myodegeneration, and reveals a tissue-specific role of the PrLD in directing pathology.
Quality-of-life outcomes with endoscopic and microscopic type I tympanoplasty—a prospective cohort study
Authors: Pap István , Kovács Márton , Bölcsföldi Barbara , Szakács Zsolt , Gerlinger Imre , Imreh Bence , Csongor Alexandra , Warta Vilmos , Szanyi István 
Published: April 2023

Abstract
Purpose
Endoscopic type I tympanoplasty was originally introduced in the 1990s and the extensive spread of this practice can be easily observed. The conventional technique performed involves the repair of a tympanic membrane perforation and is defined as microscopic type I tympanoplasty. The aim of this study is the comparison of quality-of-life (QoL) outcomes with endoscopic to that with microscopic type I tympanoplasty.

Methods
All patients, or in the case of children with the aid of a parent, were asked to complete a novel QoL questionnaire drafted by our study group. The analysis was performed with descriptive statistics—mean, SD and relative frequency—and with a mixed model (generalized least squares fit). A two-sided p value of < 0.05 was regarded as statistically significant.

Results
A total of 83 patients completed the questionnaire, 38 in the endoscopic group and 45 in the microscopic group. Every question represented a different. A statistically significant result was found in favor of the endoscopic approach regarding average hospitalization rate (p = 0.003) and cosmetic outcomes (p = 0.015). No statistically significant difference was otherwise observed between the groups.

Conclusions
Based on our prospective cohort study, the QoL outcomes of endoscopic type I tympanoplasty in terms of postoperative pain, headache, nausea, vomiting, dizziness, taste disorder and hearing were comparable to the microscopic type I tympanoplasty. In regard to cosmetics, an increase in desirable results was achieved in the endoscopic group, particularly the average hospitalization rate proved to be statistically significantly lower than in the microscopic group.
Favorable effect of ripasudil use on surgical outcomes of microhook ab interno trabeculotomy
Authors: Okuda Mina , Mori Sotaro , Ueda Kaori , Sakamoto Mari , Kusuhara Sentaro , Yamada-Nakanishi Yuko , Nakamura Makoto 
Published: April 2023

Abstract
Purpose
We have previously demonstrated that prolonged use of glaucoma medications was associated with a poor surgical outcome of ab interno trabeculotomy (µTLO). Given that almost all types of glaucoma eye drop either enhance the drainage through the uveoscleral pathway or reduce aqueous humor production, we hypothesized that prolonged use of these medications might cause disuse atrophy of the conventional pathway. In contrast, ripasudil increases the conventional outflow and eventually shows a favorable outcome of µTLO. This study aimed to evaluate the effect of ripasudil use on µTLO outcomes.

Method
The medical charts of 218 patients who underwent µTLO were analyzed retrospectively. We compared the 1-year outcome between ripasudil users versus nonusers by using propensity score matching. We set the covariates as age, sex, glaucoma types, preoperative intraocular pressure (IOP), the mean deviation values of visual field tests, the presence or absence of concomitant cataract surgery, trabecular meshwork incision range, the presence or absence of any glaucoma medication except ripasudil and duration of glaucoma medical therapy. Success was defined as a postoperative IOP between 5 and 21 mmHg, a ≥ 20% IOP reduction from baseline, and no additional glaucoma surgery at postoperative 1 year.

Result
Fifty-seven patients each were allocated to the ripasudil users or nonusers. The 1-year success rates were 74% in ripasudil users and 51% in nonusers (p = 0.01). Kaplan‒Meier survival curves also showed that the ripasudil users had a higher survival distribution (p = 0.01).

Conclusion
The patients who took ripasudil showed a favorable 1-year outcome of µTLO.
Two decades of gastric and gastroesophageal junction cancer surgery
Authors: Plum Patrick S. , Pamuk Aylin , Barutcu Atakan G. , Mallmann Christoph , Niesen Emanuel , Berlth Felix , Zander Thomas , Chon Seung-Hun , Moenig Stefan P. , Quaas Alexander , Bruns Christiane J. , Hoelscher Arnulf H. , Alakus Hakan 
Published: April 2023

Abstract
Purpose
Diagnosis and treatment of gastric and gastroesophageal junction cancer have undergone many critical changes during the last two decades. We addressed the question of how clinical reality outside of clinical trials has changed for gastric and gastroesophageal junction cancer patients in a European center for upper gastrointestinal surgery.

Methods
In this retrospective cohort study, patients undergoing (sub)total gastrectomy for gastric or gastroesophageal junction adenocarcinoma between 1996 and 2017 in a tertiary upper gastrointestinal center were included. The time was divided into a) before (1996–2006) (pre-CTx) and b) after (2006–2017) (CTx) the MAGIC trial. Data were comprehensively analyzed for demographics, tumor stage, perioperative treatment, surgery, histopathology, and survival rates (SR).

Results
737 patients (32% female) underwent gastrectomy, 255 patients in the pre-CTx era and 482 patients in the CTx era. The median age was 65 years and the median follow-up was 27.5 months for surviving patients. Around 16.9% of patients received neoadjuvant treatment in the pre-CTx era versus 46.3% in the CTx era. The 3-year survival rate (3-YSR) was 46.4% in the pre-CTx and 60.9% in the CTx era (p < 0.001). For pretreated patients, 3-YSR was 39.0% (pre-CTx) versus 55.3% (CTx) (p = 0.168). Survival rate (SR) for locally advanced tumor stages (cT3/cT4) was higher when neoadjuvant therapy was administered (3-YSR: 56.7% vs 40.6%; p = 0.022). There were no significant differences according to sex (p = 0.357), age (p = 0.379), pT category (p = 0.817), pN stage (p = 0.074), cM stage (p = 0.112), Laurén classification (p = 0.158), and SRs (3-YSR: 60.3% vs 59.4%; p = 0.898) between the MAGIC and FLOT regimens.

Conclusions
Survival rates have dramatically improved for gastric cancer patients during the last two decades. MAGIC and FLOT regimens showed similar results in the postsurgical follow-up.
The exposed Mule Creek vent deposits record the structure of a volcanic conduit during a hybrid explosive–effusive eruption
Authors: Unwin Holly E. , Tuffen Hugh , Wadsworth Fabian B. , Phillips Emrys R. , James Mike R. , Foster Annabelle , Kolzenburg Stephan , Castro Jonathan M. , Porritt Lucy A. 
Published: April 2023

AbstractSilicic volcanic eruptions commonly begin with the explosive ejection of pyroclastic material, before transitioning to gentler effusion-dominated activity. Well-exposed dissected silicic systems are scarce and poorly studied, hindering the advances in our understanding of the explosive–effusive transition needed to improve interpretations of volcanic unrest and hazard forecasting. The Mule Creek vent (New Mexico, USA) is a dissected silicic conduit that records the processes controlling conduit formation and evolution, and the role tuffisites (fractures filled with variably welded pyroclasts) play in conduit dynamics. Here, we use decimeter-scale photo-mapping of lithostratigraphic units and thin section analysis to differentiate and interpret three dominant emplacement styles during vent evolution. First, there was repeated deposition and erosion of pyroclastic material at the conduit walls, recorded by erosive surfaces in pyroclastic breccia and agglomerates at the conduit margins. Second, sub-vertical domains of dense melt-dominated magma were emplaced and preserved as glass-dominated vitrophyre and brecciated vitrophyre, with the textural hallmarks of assembly from welding of pyroclasts. Finally, the sub-horizontal fracturing of previously deposited lithologies produced laterally cross-cutting tuffisites. The vent deposits track the widening and then narrowing of the conduit through time and reflect progressive insulation and generally higher temperatures towards the conduit center as pyroclasts accumulate. Welding of pyroclastic fill and the formation of dense vitrophyres towards the conduit center lowers deposit porosity and effective wall permeability. This drives localized gas pressure increases and results in gas-driven fracturing, generating tuffisites, which act as transient outgassing pathways. The structure of the Mule Creek vent records an explosive–effusive transition, constraining the processes controlling conduit evolution and aiding our interpretation of volcanic unrest.
Quantification of diffuse CO2 flux emission from the crater lagoon of Vila Franca do Campo Islet (São Miguel, Azores)
Authors: Andrade César , Zanon Vittorio , Viveiros Fátima , Cruz J Virgílio , Pimentel Adriano , Coutinho Rui 
Published: April 2023

AbstractTo investigate unexplored areas and apply methodologies suitable for monitoring volcanic activity, a pioneer study was undertaken in the crater lagoon of Vila Franca do Campo Islet to identify possible CO2degassing zones in the marine environment. This study was carried out using a floating accumulation chamber, which to the best of our knowledge, has only been applied to lacustrine environments.A survey was carried out in the crater lagoon in August 2017, resulting in a total of 143 CO2flux measurements. Other parameters, such as water temperature, pH, electrical conductivity, and depth, were also determined at each measuring point, covering a study area of 0.02 km2. At one of the deepest sites with higher CO2flux values, water samples were collected at depth for determination of stable isotopic composition.Water temperatures ranged between 21.0 and 27.0 °C, pH from 8.24 to 8.89, and electrical conductivity from 52.5 to 53.9 mS/cm. As expected, due to the marine water composition, sampled waters are of the Na-Cl type. CO2flux varied between 0.581 and 1.0 g m-2d-1(average = 2.967 g m-2d-1). A single CO2population, characterized by low CO2fluxes, points to a biogenic CO2origin (δ13C = −9.62‰). The estimated value for the total CO2emitted from the water surface is 0.05 t d-1. CO2flux measurements were also compared with the structural features that cross the tuff cone, but no clear relation was observed with fractures/faults.This methodology should be applied to other coastal marine areas, especially where anomalous gas emissions have been reported.
New perspectives in pediatric dialysis technologies: the case for neonates and infants with acute kidney injury
Authors: Parolin Mattia , Ceschia Giovanni , Vidal Enrico 
Published: April 2023

AbstractAdvancements in pediatric dialysis generally rely on adaptation of technology originally developed for adults. However, in the last decade, particular attention has been paid to neonatal extracorporeal therapies for acute kidney care, an area in which technology has made giant strides in recent years. Peritoneal dialysis (PD) is the kidney replacement therapy (KRT) of choice in the youngest age group because of its simplicity and effectiveness. However, extracorporeal blood purification provides more rapid clearance of solutes and faster fluid removal. Hemodialysis (HD) and continuous KRT (CKRT) are thus the most used dialysis modalities for pediatric acute kidney injury (AKI) in developed countries. The utilization of extracorporeal dialysis for small children is associated with a series of clinical and technical challenges which have discouraged the use of CKRT in this population. The revolution in the management of AKI in newborns has started recently with the development of new CKRT machines for small infants. These new devices have a small extracorporeal volume that potentially prevents the use of blood to prime lines and dialyzer, allow a better volume control and the use of small-sized catheter without compromising the blood flow amount. Thanks to the development of new dedicated devices, we are currently dealing with a true “scientific revolution” in the management of neonates and infants who require an acute kidney support.
Repurposing drugs against Alzheimer’s disease: can the anti-multiple sclerosis drug fingolimod (FTY720) effectively tackle inflammation processes in AD?
Authors: Leßmann Volkmar , Kartalou Georgia-Ioanna , Endres Thomas , Pawlitzki Marc , Gottmann Kurt 
Published: April 2023

AbstractTherapeutic approaches providing effective medication for Alzheimer’s disease (AD) patients after disease onset are urgently needed. Previous studies in AD mouse models and in humans suggested that physical exercise or changed lifestyle can delay AD-related synaptic and memory dysfunctions when treatment started in juvenile animals or in elderly humans before onset of disease symptoms. However, a pharmacological treatment that can reverse memory deficits in AD patients was thus far not identified. Importantly, AD disease-related dysfunctions have increasingly been associated with neuro-inflammatory mechanisms and searching for anti-inflammatory medication to treat AD seems promising. Like for other diseases, repurposing of FDA-approved drugs for treatment of AD is an ideally suited strategy to reduce the time to bring such medication into clinical practice. Of note, the sphingosine-1-phosphate analogue fingolimod (FTY720) was FDA-approved in 2010 for treatment of multiple sclerosis patients. It binds to the five different isoforms of Sphingosine-1-phosphate receptors (S1PRs) that are widely distributed across human organs. Interestingly, recent studies in five different mouse models of AD suggest that FTY720 treatment, even when starting after onset of AD symptoms, can reverse synaptic deficits and memory dysfunction in these AD mouse models. Furthermore, a very recent multi-omics study identified mutations in the sphingosine/ceramide pathway as a risk factor for sporadic AD, suggesting S1PRs as promising drug target in AD patients. Therefore, progressing with FDA-approved S1PR modulators into human clinical trials might pave the way for these potential disease modifying anti-AD drugs.
Optional projection under equivalent local martingale measures
Authors: Biagini Francesca , Mazzon Andrea , Perkkiö Ari-Pekka 
Published: April 2023

Abstract
We study optional projections of ${\mathbb{G}}$
G
-adapted strict local martingales on a smaller filtration ${\mathbb{F}}$
F
under changes of equivalent martingale measures. General results are provided as well as a detailed analysis of two specific examples given by the inverse Bessel process and a class of stochastic volatility models. This analysis contributes to clarify the absence of arbitrage opportunities of market models under restricted information.
Corrosion inhibition of magnesium alloy AZ31 in chloride-containing solutions by aqueous permanganate
Authors: Osipenko Maria A. , Kasach Aliaksandr A. , Adamiec Janusz , Zimowska Małgorzata , Kurilo Irina I. , Kharytonau Dzmitry S. 
Published: April 2023

AbstractIn this work, corrosion of the AZ31 magnesium alloy was examined in 0.05 M NaCl solutions containing 0.01–0.150 mol/dm3 of potassium permanganate as a corrosion inhibitor. A set of electrochemical impedance spectroscopy, linear sweep voltammetry, and hydrogen evolution measurements revealed high inhibitor effectiveness at relatively high (0.150 mol/dm3) KMnO4 concentrations. Based on data of energy-dispersive X-ray analysis, scanning electron microscopy, and Raman spectroscopy, a mechanism of the corrosion inhibition of AZ31 alloy by potassium permanganate in chloride-containing media was proposed.
Guess who? Evaluating individual acoustic monitoring for males and females of the Tawny Pipit, a migratory passerine bird with a simple song
Authors: Oñate-Casado Javier , Porteš Michal , Beran Václav , Petrusek Adam , Petrusková Tereza 
Published: April 2023

AbstractIndividual acoustic monitoring (IAM), based on the analysis of vocal cues, is particularly suitable for the identification and tracking of birds with temporally stable song or call characteristics. Unlike mark-recapture methods, IAM does not require the physical manipulation of individuals, which can have long-lasting behavioural effects. So far, IAM has usually focused on males, as singing females tend to be overlooked in temperate zones. Here, we evaluated the suitability of IAM for both sexes in an isolated population of the Tawny Pipit (Anthus campestris, Motacillidae), a migratory Palearctic species critically endangered in Central Europe, for which female singing has been occasionally documented. We confirmed that songs of all 101 studied individuals, both males and females, were individually distinct. Most individuals used only a single song type in their repertoires, with only three males using two. Of 45 ringed males (that could be unambiguously recognized visually), only two changed their song structure to some extent, either within or between seasons. Multiple individuals often sang structurally similar song types, which nevertheless consistently differed in minor characteristics; such differences were detectable by visual inspection and also affected quantitative analyses of song similarity. Songs sung by females did not have any apparent sex-specific characteristics. Unlike previously suggested, females did not adapt their vocalization to their breeding partner, and we presume their song is also temporally stable. Our findings support IAM as a reliable approach for studying the behaviour and ecology of this passerine species with a small repertoire and simple songs.