bluuebunny/crossref_metadata_2025_split huggingface dataset:
Childhood arterial ischemic stroke due to mineralizing angiopathy: an 18‐year single‐center experience
Authors: Gorodetsky Carolina , Pulcine Elizabeth , Krishnan Pradeep , Singh Jaspal , Moharir Mahendranath , MacGregor Daune , Bhathal Ishvinder , deVeber Gabrielle , Dlamini Nomazulu 
Published: May 2021

Mineralizing angiopathy is a unique, age‐specific stroke syndrome characterized by basal ganglia infarction and lenticulostriate calcification after minor head injury in early childhood. There is limited understanding of the pathophysiology, course, and clinical outcome of this syndrome. We describe the clinical and radiographical phenotype of a single‐center, consecutively enrolled cohort of children with mineralizing angiopathy from January 2002 to January 2020 and provide a comparative analysis to previously published literature. Fourteen children were identified. Previously unreported findings include: stroke onset in eight children older than 18 months; presence of basal ganglia hemorrhage in four; multifocal basal ganglia infarcts in three; presence of additional non‐basal ganglia calcifications in three; and presence of thrombophilia in one. Seven children had moderate‐to‐severe neurological deficits. There was no symptomatic stroke recurrence (mean follow‐up 3y 7mo, SD 1y 7mo). Our expanded phenotype highlights distinct characteristics of mineralizing angiopathy in children and has the potential to inform future research.
What this paper adds

Children with mineralizing angiopathy are often misdiagnosed as having a limb fracture despite normal x‐rays.

A magnetic resonance imaging‐only approach may miss this entity.

Non‐contrast computed tomography, in addition to MRI is recommended to identify calcifications in idiopathic arterial ischemic stroke.

Most children have moderate‐to‐severe neurological sequela.
High and dry in days gone by: Life‐history theory predicts Appalachian mountain stream fish assemblage transformation during historical drought
Authors: Malone Eric W. , Perkin Joshuah S. , Keith Gibbs William , Padgett Matthew , Kulp Matthew , Moore Stephen E. 
Published: May 2021

AbstractUnderstanding the ecological dimensions of drought is critical for predicting how humans and nature will be affected by the expected increased prevalence of drought in the future. We tested life‐history‐based predictions for fish assemblage responses to drought using retrospective analysis of long‐term (1986–2003) fish surveys from two streams in the Appalachian Mountains of North America. We hypothesised that (1) fish assemblage composition would correlate with wet and dry hydrologic conditions as assemblages fluctuated within a loose equilibrium and (2) life‐history traits of fishes would correlate with dry versus wet periods such that opportunistic life‐history strategists would dominate during drought. Results showed fish assemblage changes in Little River and Cataloochee Creek correlated with drought severity measured one year prior to fish surveys. Fish assemblages at all three sampling sites in Little River and two sites in Cataloochee Creek fluctuated within a loose equilibrium, while the remaining two sites in Cataloochee Creek indicated directional change. Life‐history traits for fishes in Cataloochee Creek correlated with one‐year time lag fluctuations in drought caused by opportunistic species being dominant during drought and periodic/equilibrium species dominant during wet periods. Time series plots of fish abundances aggregated by life‐history strategy revealed dominance of opportunistic species emerged at the onset of a multi‐year drought spanning 1998–2004, particularly for the two sites undergoing directional change. Our work provides empirical evidence for theoretical linkages between life history and environmental fluctuations and can ultimately be used to predict stream fish community response to future drought regimes.
Treatment patterns and disease course of previously untreated Primary Central Nervous System Lymphoma: Feasibility of MTX‐based regimens in clinical routine
Authors: Sieg Noëlle , Naendrup Jan‐Hendrik , Gödel Philipp , Balke‐Want Hyatt , Simon Florian , Deckert Martina , Gillessen Sarah , Kreissl Stefanie , Bröckelmann Paul J. , Borchmann Peter , von Tresckow Bastian , Heger Jan‐Michel 
Published: May 2021

AbstractBackgroundPrimary central nervous system lymphoma (PCNSL) is a rare type of aggressive lymphoma of the central nervous system. Treatment strategies improved significantly over the past decades differ regionally but mainly consist of rituximab and high‐dosed methotrexate (MTX)‐based therapies.MethodsWe assessed clinical outcomes of 100 patients with newly diagnosed PCNSL between 2010‐2020 at the University Hospital of Cologne, Germany.ResultsPatients were 23‐88 years of age and either treated with MTX‐based regimens (PRIMAIN, MARTA, MATRix), individual regimens, or best supportive care, respectively. Overall response rates were generally high (66,7‐83,8%), but different organ toxicities required dose adjustments in most groups. Two‐year overall survival rates were 57,9% (PRIMAIN), 63,6% (MARTA), 65,4% (MATRix), and 37,5% (Other), respectively. Out of 9 patients suffering from relapse >12 months from primary diagnosis, 7 patients (77,8%) received methotrexate‐based salvage therapy with 2‐year overall survival of 4/6 patients (66,7%).ConclusionAlthough a relevant proportion of patients are not eligible for clinical trials due to age, performance status, or comorbidities, these results prove feasibility of different MTX‐based treatment strategies in clinical routine. Even elderly patients displayed surprisingly favorable outcomes. However, with compromising organ toxicities, reduction of intensity should be part of strategies in future clinical trials.
Very late onset neuromyelitis optica spectrum disorders
Authors: Nakahara Keiichi , Nakane Shunya , Nagaishi Akiko , Narita Tomoko , Matsuo Hidenori , Ando Yukio 
Published: May 2021

AbstractBackground and purposeNeuromyelitis optica spectrum disorder (NMOSD) often presents in the elderly with an insidious onset of symptoms and aggressive progression. There have been anecdotal cases of very late onset (VLO)‐NMOSD, but case series reports are rare. The aim of this retrospective study was to clarify the clinical features of VLO‐NMOSD.MethodsAccording to the age at onset, we classified patients with NMOSD into three subgroups: ≤49 years, early onset NMOSD (EO‐NMOSD); 50–69 years, late onset NMOSD (LO‐NMOSD); and ≥70 years, VLO‐NMOSD. We evaluated the clinical characteristics, magnetic resonance imaging (MRI) findings, laboratory data, and immunotherapies of the groups.ResultsOverall, 12 men and 64 women with a median (interquartile range) age at onset and duration of disease of 42.0 (29.0–55.8) years and 70.0 (16.3–143.0) months, respectively, were included. Eight (11%) patients had VLO‐NMOSD, 22 (29%) had LO‐NMOSD, and 46 (61%) had EO‐NMOSD. Patients with EO‐NMOSD had a significantly longer interval between episodes as well as time between the first symptom and diagnosis of NMOSD than did those with VLO‐NMOSD and LO‐NMOSD (p = 0.046). Optic neuritis and nerve lesions on MRI were significantly less frequent in patients with VLO‐NMOSD than in those with LO‐NMOSD and EO‐NMOSD (p = 0.002 and p = 0.028, respectively). In contrast, patients with VLO‐NMOSD had higher nadir Expanded Disability Status Scale and Nurick scale scores and a significantly longer spinal lesion length than did those with LO‐NMOSD and EO‐NMOSD (p = 0.029, p = 0.049, and p = 0.032, respectively).ConclusionsPatients with VLO‐NMOSD tend to develop severe myelitis with long cord lesions but not optic neuritis.
Treatment expectations and perception of therapy in adult patients with spinal muscular atrophy receiving nusinersen
Authors: Meyer Thomas , Maier André , Uzelac Zeljko , Hagenacker Tim , Günther René , Schreiber‐Katz Olivia , Weiler Markus , Steinbach Robert , Weyen Ute , Koch Jan Christoph , Kettemann Dagmar , Norden Jenny , Dorst Johannes , Wurster Claudia , Ludolph Albert C. , Stolte Benjamin , Freigang Maren , Osmanovic Alma , Petri Susanne , Grosskreutz Julian , Rödiger Annekathrin , Griep Ramona , Gaudlitz Marcel , Walter Bertram , Münch Christoph , Spittel Susanne 
Published: May 2021

AbstractBackground and purposeThis was an investigation of treatment expectations and of the perception of therapy in adult patients with 5q‐associated spinal muscular atrophy (5q‐SMA) receiving nusinersen.MethodsA prospective, non‐interventional observational study of nusinersen treatment in adult 5q‐SMA patients was conducted at nine SMA centers in Germany. The functional status, treatment expectations and perceived outcomes were assessed using the Amyotrophic Lateral Sclerosis Functional Rating Scale—extended (ALS‐FRS‐ex), the Measure Yourself Medical Outcome Profile (MYMOP2), the Treatment Satisfaction Questionnaire for Medication (TSQM‐9) and the Net Promoter Score (NPS).ResultsIn all, 151 patients were included with a median age of 36 years (15–69 years). SMA type 3 (n = 90, 59.6%) prevailed, followed by type 2 (33.8%) and type 1 (6.6%). In SMA types 1–3, median ALS‐FRS‐ex scores were 25, 33 and 46 (of 60 scale points), respectively. MYMOP2 identified distinct treatment expectations: head verticalization (n = 13), bulbar function (n = 16), arm function (n = 65), respiration (n = 15), trunk function (n = 34), leg function (n = 76) and generalized symptoms (n = 77). Median symptom severity decreased during nusinersen treatment (median observational period 6.1 months, 0.5–16 months) from 3.7 to 3.3 MYMOP2 score points (p < 0.001). The convenience of drug administration was critical (49.7 of 100 TSQM‐9 points, SD 22); however, the overall treatment satisfaction was high (74.3, SD 18) and the recommendation rating very positive (NPS +66).ConclusionsNusinersen was administered across a broad range of ages, disease durations and motor function deficits. Treatment expectations were highly differentiated and related to SMA type and functional status. Patient‐reported outcomes demonstrated a positive perception of nusinersen therapy in adult patients with 5q‐SMA.
Differentiation or federalisation: Which democracy for the future of Europe?
Authors: Fabbrini Sergio 
Published: May 2021

AbstractDifferentiation has become a central topic of debate in the EU. Generally, it is considered a positive device for advancing integration in crucial policies, letting the unwilling states opt out from the new regimes. However, the debate has not sufficiently acknowledged that policy differentiation has been made possible by governance differentiation. It was the 1992 Maastricht Treaty's decision to inaugurate an intergovernmental regime for core state power policies, distinct from the supranational regime regulating single market policies, that allowed differentiation to flourish. Differentiation and intergovernmentalism are thus inter‐connected. During multiple crises of the last decade, intergovernmental governance has shown its undemocratic effects, thus soliciting a critical reappraisal of the differentiation logic. The federalisation of the EU appears a more promising alternative strategy for advancing integration and, at the same time, meeting the democratic expectations of the EU.
Genomic data support management of anadromous Arctic Char fisheries in Nunavik by highlighting neutral and putatively adaptive genetic variation
Authors: Dallaire Xavier , Normandeau Éric , Mainguy Julien , Tremblay Jean‐Éric , Bernatchez Louis , Moore Jean‐Sébastien 
Published: May 2021

AbstractDistinguishing neutral and adaptive genetic variation is one of the main challenges in investigating processes shaping population structure in the wild, and landscape genomics can help identify signatures of adaptation to contrasting environments. Arctic Char (Salvelinus alpinus) is an anadromous salmonid and the most harvested fish species by Inuit people, including in Nunavik (Québec, Canada), one of the most recently deglaciated regions in the world. Unlike many other anadromous salmonids, Arctic Char occupy coastal habitats near their natal rivers during their short marine phase restricted to the summer ice‐free period. Our main objective was to document putatively neutral and adaptive genomic variation in anadromous Arctic Char populations from Nunavik and bordering regions to inform local fisheries management. We used genotyping by sequencing (GBS) to genotype 18,112 filtered single nucleotide polymorphisms (SNP) in 650 individuals from 23 sampling locations along >2000 km of coastline. Our results reveal a hierarchical genetic structure, whereby neighboring hydrographic systems harbor distinct populations grouped by major oceanographic basins: Hudson Bay, Hudson Strait, Ungava Bay, and Labrador Sea. We found genetic diversity and differentiation to be consistent both with the expected postglacial recolonization history and with patterns of isolation‐by‐distance reflecting contemporary gene flow. Results from three gene–environment association methods supported the hypothesis of local adaptation to both freshwater and marine environments (strongest associations with sea surface and air temperatures during summer and salinity). Our results support a fisheries management strategy at a regional scale, and other implications for hatchery projects and adaptation to climate change are discussed.
Effect of data spatial scale on the performance of fish habitat models
Authors: Núñez‐Riboni Ismael , Akimova Anna , Sell Anne F. 
Published: May 2021

AbstractHabitat models are widely used to explore past and predict future shifts in fish distribution. Our literature review reveals a widespread practice of using in situ data or data with the highest possible resolution to train fish habitat models. Using examples of six fish species at two life stages in the North Sea, we demonstrate that the choice of the data resolution is crucial for a model's performance. We matched fish abundance data from a 51‐year long scientific survey at three spatial scales with environmental parameters at seven spatial scales, obtaining a total of 240 data sets. We varied the resolution used for model training and for model predictions and evaluated model performance with various metrics on training and cross‐validating data. Contrary to the common notion, training the model with low‐resolution data generally improved the performance metrics when compared to models built upon in situ or high‐resolution data. The optimal resolution for fish and environmental data was roughly twice the average distance between observations. Training the model with data of higher resolutions often yielded unrealistic fish multidecadal distributional shifts. In turn, best model predictions were achieved with data of higher resolution than the training data. We explain these results with scale‐dependent ecological responses, subscale noise in the raw data, failure of interpolation to create information and failure to comply with the Nyquist–Shannon sampling theorem. This study shows that the choice of an appropriate spatial scale is crucial to correctly predict shifts in fish distribution under climate change.
Cracking simulation‐based cumulative fatigue damage assessment
Authors: Farag Mahmoud M. , El‐Kady Ramy M. , Hammouda Mohammad M. I. 
Published: May 2021

AbstractThe present work is an extension of a previously developed fracture mechanics cracking damage model and highlights the ability of that model to predict the fatigue lifetime of un‐notched round specimens made of a ferrite–pearlite 0.4C‐70/30 carbon steel in the cases of (a) two‐step fully reversed axial loading with low‐to‐high and high‐to‐low sequences and (b) repeated application of fully reversed two‐step axial loading blocks. This model numerically simulates the collective behavior of growing short fatigue cracks originating from the specimen surface. The surface roughness is assumed to resemble microcracks of different sizes and locations along the minimum specimen circumference. Material grains of different phases, sizes, and strengths are randomly distributed over that circumference. Possible activities of surface cracks are predicted against loading cycles till a fracture occurs. Published experimental data on ferritic‐pearlitic steel specimens in fully reversed variable amplitude loading are utilized. Different specimen tests are randomly configured and simulated. The present predictions are in fair agreement with the corresponding experimental results.
Thunderstorms have species and gear‐specific indirect effects on the catchability of Mongolian salmonids
Authors: Golden Abigail S. , Sivaram Sneha , Batsaikhan Ganzorig , Jensen Olaf P. 
Published: May 2021

AbstractClimate change is predicted to cause increasingly frequent and intense storms. Northern Mongolia is already warming at a rate twice the global average, and thunderstorms, defined as intense, short, patchy rains associated with thunder, lightning and high precipitation rates, are becoming more frequent. Because Mongolia's fish populations are lightly exploited, Mongolia provides a model system in which to study the effects of storms on fish behaviour and fishing vulnerability. The impacts of thunderstorm‐related hydrological changes on fishes’ vulnerability to two fishing gears were evaluated. Two thunderstorm‐related factors, turbidity and river stage, reduced catch rates of the salmonids lenok Brachymystax lenok (Pallas) and Baikal grayling Thymallus baicalensis Dybowski. Fly‐fishing gear was more effective than spinning gear in this fishery and retained higher catch rates in extreme conditions. These gear‐specific effects suggest that turbidity and rising river stage affect fishing vulnerability by influencing feeding behaviour.
To hum or not to hum: Neural transcriptome signature of male courtship vocalization in a teleost fish
Authors: Tripp Joel A. , Feng Ni Y. , Bass Andrew H. 
Published: May 2021

AbstractFor many animal species, vocal communication is a critical social behavior and often a necessary component of reproductive success. Additionally, vocalizations are often demanding motor acts. Wanting to know whether a specific molecular toolkit might be required for vocalization, we used RNA‐sequencing to investigate neural gene expression underlying the performance of an extreme vocal behavior, the courtship hum of the plainfin midshipman fish (Porichthys notatus). Single hums can last up to 2 h and may be repeated throughout an evening of courtship activity. We asked whether vocal behavioral states are associated with specific gene expression signatures in key brain regions that regulate vocalization by comparing transcript expression levels in humming versus non‐humming males. We find that the circadian‐related genesperiod3andClockare significantly upregulated in the vocal motor nucleus and preoptic area‐anterior hypothalamus, respectively, in humming compared with non‐humming males, indicating that internal circadian clocks may differ between these divergent behavioral states. In addition, we identify suites of differentially expressed genes related to synaptic transmission, ion channels and transport, neuropeptide and hormone signaling, and metabolism and antioxidant activity that together may support the neural and energetic demands of humming behavior. Comparisons of transcript expression across regions stress regional differences in brain gene expression, while also showing coordinated gene regulation in the vocal motor circuit in preparation for courtship behavior. These results underscore the role of differential gene expression in shifts between behavioral states, in this case neuroendocrine, motor and circadian control of courtship vocalization.
Bacterial and archaeal lipids trace chemo(auto)trophy along the redoxcline in Vancouver Island fjords
Authors: Kusch Stephanie , Wakeham Stuart G. , Dildar Nadia , Zhu Chun , Sepúlveda Julio 
Published: May 2021

AbstractMarine oxygen minimum zones play a crucial role in the global oceanic carbon, nitrogen, and sulfur cycles as they harbor microbial communities that are adapted to the water column chemistry and redox zonation, and in turn control the water column chemistry and greenhouse gas release. These micro‐organisms have metabolisms that rely on terminal electron acceptors other than O2 and often benefit from syntrophic relationships (metabolic coupling). Here, we study chemo(auto)trophy along the redoxcline in two stratified fjords on Vancouver Island (Canada) using bacterial bacteriohopanepolyols and archaeal ether lipids. We analyze the distribution of these lipid classes in suspended particulate matter (SPM) to trace ammonia oxidation, anaerobic ammonium oxidation (anammox), sulfate reduction/sulfur oxidation, methanogenesis, and methane oxidation, and investigate ecological niches to evaluate potential links between their respective bacterial and archaeal sources. Our results show an unparalleled BHP and ether lipid structural diversity that allows tracing the major redox‐driven metabolic processes at the time of sampling: Both fjords are dominated by archaeal ammonia oxidation and anammox; sulfate‐reducing bacteria may be present in Deer Bay, but absent from Effingham Inlet; methanogenic Euryarchaeota and archaeal and bacterial methanotrophs are detectable at low abundance. Correlation analysis reveals distinct biomarker clusters that provide constraints on the biogeochemical niches of some orphan BHP and ether lipids such as in situ‐produced adenosyl‐BHPs or unsaturated archaeols.
Alleviation of nutrient co‐limitation induces regime shifts in post‐fire community composition and productivity in Arctic tundra
Authors: Klupar Ian , Rocha Adrian V. , Rastetter Edward B. 
Published: May 2021

AbstractRecent unprecedented fires in the Arctic during the past two decades have indicated a pressing need to understand the long‐term ecological impacts of fire in this biome. Anecdotal evidence suggests that tundra fires can induce regime shifts that change tussock tundra to more shrub‐dominated ecosystems. However, the ecological mechanisms regulating these shifts are poorly understood, but are hypothesized to involve changes to nutrient availability in this nutrient limited system. Here we conducted a 4‐year two‐factorial (control: C, nitrogen along: N+, phosphorus alone: P+, nitrogen and phosphorus combined: NP+) fertilization experiment in both unburned and burned tundra to test this hypothesis after a decade of post‐fire recovery. A decade after fire, the burned site exhibited an increase in soil nitrogen and phosphorus availability and a transition toward taller, more productive, and more deciduous vegetation. This shift in vegetation structure, composition, and function was induced at the unburned site through the addition of both NP+ and the alleviation of their co‐limitation. Both burned and unburned tundra responded similarly to fertilizer treatments by increasing leaf area index, greenness, and canopy height in NP+ treatments, and exhibited no significant response in individual N+ or P+ treatments. These results point to a greater need to understand coupled carbon, nitrogen, and phosphorus cycles in this system, and suggest that post‐fire regime shifts are regulated by the alleviation of nitrogen and phosphorus co‐limitation in Arctic tundra.