bluuebunny/crossref_metadata_2025_split huggingface dataset:
Laboratory monitoring of nusinersen safety
Authors: Goedeker Natalie L. , Gibbons Jennifer L. , Varadhachary Arun S. , Connolly Anne M. , Zaidman Craig M. 
Published: March 2021

AbstractIntroductionThis retrospective study reports our tertiary care center's experience with intrathecal nusinersen administration in children and adults with spinal muscular atrophy (SMA).MethodsWe reviewed safety monitoring laboratory results and need for procedural sedation and fluoroscopy‐guidance in all SMA patients receiving nusinersen between February 2017 and March 2020.ResultsFifty‐eight patients ages 1 mo‐ 56 y received 494 nusinersen doses. There were 166 laboratory abnormalities in 45 patients. Most were either mild (145 [87.3%]) or were transient proteinuria (18 [10.8%]). None altered nusinersen treatment. Twenty‐eight patients required either general anesthesia (75 doses) or anxiolysis with oral midazolam (133 doses, including 6 patients [23 doses] with SMA type I). Eight patients with complicated spines (45 doses) required fluoroscopic guidance. One treatment‐related serious adverse event (emesis leading to intubation) occurred during general anesthesia. Two children had asymptomatic increased intracranial pressure. No patients discontinued treatment due to adverse events.DiscussionIntrathecal nusinersen is generally safe and well‐tolerated, including in patients requiring oral anxiolysis, general sedation, and fluoroscopic guidance. Frequent serial laboratory monitoring did not identify any persistent significantly abnormal findings or alter treatment.
Thymoma pathology and myasthenia gravis outcomes
Authors: Menon Deepak , Katzberg Hans , Barnett Carolina , Pal Prodipto , Bezjak Andrea , Keshavjee Shaf , Bril Vera 
Published: March 2021

AbstractIntroductionThere is limited evidence regarding the impact of World Health Organization (WHO) subtype of thymoma on post‐thymectomy outcome of thymoma‐associated myasthenia gravis (TAMG). The objective was to determine if the pathological subtypes of thymoma were associated with post‐thymectomy outcomes of myasthenia gravis (MG), in patients with TAMG.MethodsWe performed a retrospective study of consecutive patients with TAMG who attended the neuromuscular clinic between January 2018 and December 2019 with a minimum follow‐up of 1 y after thymectomy. Outcome measures were MG Impairment Index (MGII), single‐simple question (SSQ), Myasthenia Gravis Foundation of America post‐intervention status (MGFA PIS) and non‐responder MG status at last assessment.ResultsNinety‐five patients were included; mean age at onset was 48.1 ± 12.1 y; 54(56.8%) were females. Thirteen patients developed MG post‐thymectomy. The most common thymoma was WHO type B2 in 39 (41.1%). Most patients (40, 42.1%) had Masaoka stage II thymoma. There was no association of thymoma subtypes or Masaoka stage of disease with age, gender, MG phenotype, serology, post‐thymectomy onset, interval from onset to thymectomy, MGII, SSQ, MGFA PIS, or non‐responder status. Associations were found between positive serology and lower MGII (11.1 ± 14.2 vs 23 ± 12.9, P = .050), thymic follicular hyperplasia (TFH) and higher SSQ (89.3 ± 11.7 vs 80.1 ± 20.2, P‐.043), and lack of recurrence and higher SSQ (84.1 ± 18 vs 72.5 ± 20, P = .037).DiscussionThe WHO pathological subtype of thymoma did not correlate with MG outcomes. However, positive acetylcholine antibody serology, presence of TFH, and non‐recurrence of thymoma predict a favorable outcome.
How accurately do mechanophores report on bond scission in soft polymer materials?
Authors: Dubach Friso F. C. , Ellenbroek Wouter G. , Storm Cornelis 
Published: March 2021

AbstractMechanophores are molecules that are incorporated into a host material and react to the local mechanical condition—the state of stress or strain—of that host material. Among their many purposes is that of a reporter: Mechanophores whose optical activity changes in response to mechanical cues can reveal bulk material processes that are ordinarily hidden, such as fatigue and fracture. Moreover, they may do so well before a material is fully fractured. To extract quantitative information from the optical signals from embedded mechanophores it is important that the mechanophores, which are generally a minority component of the material, report proportionally and unambiguously on the mechanical condition of the bulk. This is particularly important for early reporting of damage and wear, for which the optical signal from the mechanophore should accurately reflect bulk bond scission. In this article, we develop and analyze a general theory for the quality of optical mechanoreporting by mechanophores in soft materials, based on the Bell‐Evans theory of bond breaking. We find, that at the typical low fractions in which mechanophores are incorporated the overall change in strength is limited, but that the proportionality of the reporting can be off by significant amounts, particularly at short times after loading but, for non‐scissile bonds, at long times as well.
Assessment of treatment burden and complexity in cystic fibrosis: A quality improvement project
Authors: Hente Elizabeth , Weiland Jeanne , Mullen Lisa , Hawke Jesse , Schuler Christine L. , Filigno Stephanie S. , Siracusa Christopher 
Published: March 2021

AbstractBackgroundTreatment regimens for cystic fibrosis (CF) continue to evolve and grow in complexity. Treatment regimen burden, and associated sequelae, are incompletely understood.ObjectiveQuality improvement (QI) methods were used to investigate treatment burden of CF care, family and care team partnerships, and potential interventions to reduce burden.MethodsPatients 6–24 years with CF and caregivers of patients 6–13 years were surveyed. Portions of validated tools and existing surveys measured burden and family‐care team partnership. An automated report calculated treatment complexity. Plan‐do‐study‐act cycles tested survey administration during CF visits and run charts tracked progress. Interventions to reduce burden were tracked, and bidirectional assessments explored partnerships among patients, families and clinicians.ResultsOver 6 months, 110 patients and 62 caregivers completed assessments. Caregivers reported lower burden/higher quality of life (74.0, range 22.2–100) than patients (66.5, range 16.7–100). The mean treatment complexity score was 17.2 (range 6–34). Treatment complexity and burden increased with patient age (p < .05 and p < .01 respectively). Lower lung function correlated with higher patient‐reported burden (p < .01) and higher treatment complexity (p < .0001). As burden increased, providers more often performed select interventions (discussed combining treatments, simplified regimens, or involved other team members (p < .05 for each)). Families reported high partnership (mean scores 4.7–4.8, 5 = high), and providers reported high utilization of partnership tools (tool used in 77% of encounters).ConclusionWe assessed, quantified, and responded to treatment burden and complexity in real‐time during outpatient CF visits. Systematic and individualized assessments of treatment complexity and burden may enhance treatment adherence while preserving quality of life.
Management, outcome, and novel classification system of periprosthetic fractures in patients with transcutaneous osseointegrated prosthetic systems (TOPS)—a retrospective cohort analysis
Authors: Örgel Marcus , Petri Maximilian , Ranker Alexander , Wirries Nils , Graulich Tilman , Krettek Christian , Winkelmann Marcel , Aschoff Horst-Heinrich 
Published: March 2021

Abstract
Introduction
Transcutaneous osseointegrated prosthetic systems (TOPS) are anchored prosthetic systems for major limb loss. Sometimes TOPS patients suffer from periprosthetic fractures. The aim of this study was to analyze the management and outcomes of periprosthetic fractures in patients with TOPS and to introduce a novel classification system for this entity.

Material/methods
Since 2010, 140 patients were treated with TOPS after transfemoral amputation in two centers in Germany. Fifteen patients sustained periprosthetic fractures, with five intra- and ten postoperative fractures. The outcome was analyzed by Prosthesis Mobility Questionnaire (PMQ), K-level and prosthesis wear time per day. A subgroup analysis for the body mass index (BMI) was performed.

Results
All postoperative fractures were treated with implant-retaining osteosynthesis. Fourteen fractures healed without complications after a mean of 3 months. One postoperative fracture developed a clinically asymptomatic firm non-union. No Endo-Fixstem had to be removed. For the fracture and control group, a significant increase of the PMQ (p < 0.001) and K-level (p < 0.001) was observed after TOPS treatment compared to the preoperative baseline. Furthermore, the subgroup analysis showed a significant increase of the PMQ and K-level for both normal weight (p = 0.002) and overweight patients (p < 0.001). Of interest, overweight patients even showed a significantly higher increase in scores compared to normal weight patients, regardless of periprosthetic fracture.

Conclusion
Periprosthetic fractures do not necessarily worsen outcomes of TOPS treatment. Proper classification and standardized appropriate treatment strategies according to fracture morphology are paramount for reliably good outcomes. We recommend to not remove or exchange the implant (Endo-Fixstem) even if it is assembly. Higher BMI did not have an impact onto rehabilitation success after TOPS to major limb loss of the lower extremity.
Distance from the magnification device contributes to differences in lower leg length measured in patients with TSF correction
Authors: Ahrend Marc-Daniel , Rühle Michael , Springer Fabian , Baumgartner Heiko 
Published: March 2021

Abstract
Introduction
In absence of deformity or injury of the contralateral leg, the contralateral leg length is used to plan limb lengthening. Length variability on long-leg weight-bearing radiographs (LLR) can lead to inaccurate deformity correction. The aim of the study was to (1) examine the variability of the measured limb length on LLR and (2) to examine the influence of the position of the magnification device.

Materials and methods
The limb lengths of 38 patients during deformity correction with a taylor-spatial-frame were measured retrospectively on 7.3 ± 2.6 (4–13) LLR per patient. The measured length of the untreated limb between LLR were used to determine length variability between LLR in each patient. To answer the secondary aim, we took LLR from a 90 cm validation distance. A magnification device was placed in different positions: at the middle of the 90 cm distance (z-position), 5 cm anterior and 5 cm posterior from the z-position, at the bottom and top of the validation distance as well as 5 cm medial and 15 cm lateral from the z-position.

Results
The measured length variability ranged within a patient from 10 to 50 mm. 76% of patients had a measured limb length difference of ≥ 2 cm between taken LLR. Compared to length measurement of the 90 cm test object with the magnification device in the z-position (90.1 cm), positioning the device 5 cm anterior led to smaller (88.6 cm) and 5 cm posterior led to larger measurements (91.7 cm). The measured length with the magnification device at the bottom, top, medial or lateral (90.4; 89.9; 90.2; 89.8 cm) to the object differed not relevantly.

Conclusions
High variability of limb length between different LLR within one patient was observed. This can result from different positions of the magnification device in the sagittal plane. These small changes in positioning the device should be avoided to achieve accurate deformity correction and bone lengthening. This should be considered for all length and size measurements on radiographs.
Application of multi-objective Bayesian shape optimisation to a sharp-heeled Kaplan draft tube
Authors: Daniels S. J. , Rahat A. A. M. , Tabor G. R. , Fieldsend J. E. , Everson R. M. 
Published: March 2021

AbstractThe draft tube of a hydraulic turbine plays an important role for the efficiency and power characteristics of the overall system. The shape of the draft tube affects its performance, resulting in an increasing need for data-driven optimisation for its design. In this paper, shape optimisation of an elbow-type draft tube is undertaken, combining Computational Fluid Dynamics and a multi-objective Bayesian methodology. The chosen design objectives were to maximise pressure recovery, and minimise wall-frictional losses along the geometry. The design variables were chosen to explore potential new designs, using a series of subdivision-curves and splines on the inflow cone, outer-heel, and diffuser. The optimisation run was performed under part-load for the Kaplan turbine. The design with the lowest energy-loss identified on the Pareto-front was found to have a straight tapered diffuser, chamfered heel, and a convex inflow cone. Analysis of the performance quantities showed the typically used energy-loss factor and pressure recovery were highly correlated in cases of constant outflow cross-sections, and therefore unsuitable for use of multi-objective optimisation. Finally, a number of designs were tested over a range of discharges. From this it was found that reducing the heel size increased the efficiency over a wider operating range.
Enzymatic reactions in teleocidin B biosynthesis
Authors: Awakawa Takayoshi 
Published: March 2021

AbstractThe teleocidin B family members are terpene indole compounds isolated from Streptomyces bacteria, and they strongly activate protein kinase C (PKC). Their unique structures have attracted many researchers in the natural product chemistry and pharmacology fields, and numerous isolation and bioactivity studies have been conducted. The accumulated information has facilitated the identification of the enzymatic reactions in teleocidin biosynthesis, and new developments in structural biology have strongly aided efforts to clarify the finer points of these reactions. This review describes the recent biochemical and structural biological studies to reveal their reaction mechanisms, with a primary focus on the terpene cyclization triggered by the C-N bond formation by P450 oxygenase (TleB), the prenyltransferase (TleC), and the methyltransferase (TleD). This new knowledge will benefit future engineering studies to create unnatural PKC activators.
Attachment characteristics and emotion regulation difficulties among maladaptive and normal daydreamers
Authors: Sándor Alexandra , Bugán Antal , Nagy Attila , Bogdán Lilla Stella , Molnár Judit 
Published: March 2021

AbstractMaladaptive daydreaming is an excessive form of daydreaming which causes significant distress and functional impairment to the affected individuals. Research on maladaptive daydreaming has intensified in recent years, but its pathogenesis has not yet been clearly elucidated. The aim of the study was to examine the attachment characteristics and the difficulties in emotion regulation among maladaptive and normal daydreamers. 717 individuals were recruited online, 106 of whom were screened as maladaptive daydreamers. The results of the Attachment Style Questionnaire revealed a specific attachment style among maladaptive daydreamers, who were characterized by ‘Ambivalent-fearful’ attachment characteristics, while normal daydreamers showed ‘Secure-independent’ attachment style. Regarding emotion regulation difficulties, maladaptive daydreamers showed a significantly higher level of deficit on each subscale of the Difficulties in Emotion Regulation Scale compared to normal daydreamers. The findings highlighted the potential role of early attachment experiences in the etiology and pathogenesis of maladaptive daydreaming, as well as the presence of severe emotion regulation deficits among problematic daydreamers. The results revealed by the present study might give contributions to the development of psychotherapeutical treatment of maladaptive daydreaming.
Examining the effectiveness of cognitive bias modification for perfectionism in exploration of the mediating and moderating effects of body dissatisfaction and self-efficacy
Authors: Wang Yun-Han , Wang Yun-Lin , Misener Kaylee , Libben Maya 
Published: March 2021

AbstractThe relationship between perfectionism, body dissatisfaction, and self-efficacy is unclear. This study attempted to distinguish the relationship between different dimensions of perfectionism and to examine how they relate to body dissatisfaction and self-efficacy. Experiment 1 examined the effectiveness of two types of Cognitive Bias Modification for Interpretation (CBM-I) techniques in the induction of perfectionism. Experiment 2 explored the mediation and moderation effects of perfectionism facets, body dissatisfaction, and self-efficacy in the induction of perfectionism. Participants were randomly assigned to one of the four CBM-I conditions and completed self-report measures of trait and state perfectionism, body dissatisfaction, self-efficacy, as well as a behavioural task that assessed perfectionistic behaviours before and after the CBM-I induction. The results indicated no significant differences in perfectionism between the experimental groups and the control groups following the perfectionism induction. Using baseline participant characteristics, body dissatisfaction was found to mediate socially-prescribed perfectionism and self-efficacy. Self-oriented perfectionism moderated the association between body dissatisfaction and self-efficacy. State perfectionism may not be influenced by a single session (30 trials) of CBM-I training. Treatment targeting body dissatisfaction may enhance self-efficacy in socially-prescribed perfectionists. Further, interventions that decrease self-oriented perfectionism may reduce body dissatisfaction while increasing self-efficacy.
Informal Workplace Learning in Swedish Police Education– A Teacher Perspective
Authors: Sjöberg David , Holmgren Robert 
Published: March 2021

AbstractIn the literature, informal learning is considered to be of great importance to employees’ development of workplace learning. The aim of this study, which involves respondents from a Swedish police education unit, was to contribute knowledge about Swedish police education teachers’ informal learning as regards conditions for learning, learning activities and learning experiences resulting from their engagement in these activities. The results of the study are based on daily digital logbook notes made by 25 police and university teachers during one month and subsequent interviews with these teachers. The main conclusions can be summarised as follows: 1) The culture, structure and materials of the workplace are important parts of teachers’ conditions for informal learning. 2) The teachers’ informal learning activities are characterised by intentional learning, where supporting interactions with colleagues in their own teams and other, more knowledgeable, colleagues are highly valued. Furthermore, the most common triggers for teachers’ informal learning are issues relating to pedagogy and digital technology, and face-to-face contacts with colleagues are preferred. 3) The police teachers describe their learning experiences made in the course of informal learning activities as a transition from an instructor-based to a teaching-oriented approach, while the learning of the university teachers is focused on contextualisation of their teaching by integrating academic knowledge into the police practice. The article concludes with a brief discussion about the possibilities and limitations of informal learning.
Joint Modeling of Distances and Times in Point-Count Surveys
Authors: Martin-Schwarze Adam , Niemi Jarad , Dixon Philip 
Published: March 2021

AbstractRemoval and distance modeling are two common methods to adjust counts for imperfect detection in point-count surveys. Several recent articles have formulated models to combine them into a distance-removal framework. We observe that these models fall into two groups building from different assumptions about the joint distribution of observed distances and first times to detection. One approach assumes the joint distribution results from a Poisson process (PP). The other assumes an independent joint (IJ) distribution with its joint density being the product of its marginal densities. We compose an IJ+PP model that more flexibly models the joint distribution and accommodates both existing approaches as special cases. The IJ+PP model matches the bias and coverage of the true model for data simulated from either PP or IJ models. In contrast, PP models underestimate abundance from IJ simulations, while IJ models overestimate abundance from PP simulations. We apply all three models to surveys of golden-crowned sparrows in Alaska. Only the IJ+PP model reasonably fits the joint distribution of observed distances and first times to detection. Model choice affects estimates of abundance and detection but has little impact on the magnitude of estimated covariate effects on availability and perceptibility.Supplementary materials accompanying this paper appear online.
Seam Formation in Laser Beam Micro-Welding with Spatial Power Modulation
Authors: Aden Mirko , Heinen Paul , Olowinsky Alexander 
Published: March 2021

AbstractWhen laser sources of high brilliance are used for the micro-welding of metals, small seam dimensions are generated. If the spatial power is modulated by a superposition of the linear feed rate and a circular-oscillatory movement of high frequency, the width of the seam is controlled by the amplitude of the circular movement. In this study, the irradiation pattern of the seam was calculated and reveals that some spots of the seam area are not irradiated, while other have been irradiated several times. The seam shapes were visualized with micro cross-sections for different laser powers, linear feed rates and oscillatory amplitudes. Thermal simulations were made to discuss the different seam shapes. A consequence of the oscillatory movement is the appearance of different solidification fronts, which are visible in the micro cross-sections.
Massive Perivillous Fibrinoid Degeneration of Placenta/ Maternal Floor Infarct: A Case Report
Authors: Panat Veena Mangesh 
Published: March 2021

AbstractPlacental pathology can be a cause of early severe FGR leading to perinatal morbidity and mortality with repercussions in future pregnancies. A diagnosis of a placental lesion on ultrasound should have a detailed clinical and histopathological correlation for better management in next pregnancy. We present a case report of “placental massive perivillous fibrinoid degeneration/maternal floor infarction (MPFD)” with very large avascular placenta. This can lead to fetal growth restriction (FGR) and other complications for the fetus. It is also known to be associated with antiphospholipid syndrome. Management with low dose aspirin and low molecular weight heparin achieves good results.
Sirenomelia: Can it be Missed in the First Trimester? A Case Series of A Rare Condition
Authors: Smitha D. S. , Agrawal Purvi , Suman T. P. , Makam Adinarayana V. 
Published: March 2021

AbstractSirenomelia is a very rare congenital developmental disorder of the lower extremities, and all the other derivatives from the caudal mesoderm, mainly the viscera i.e. kidneys, gonads and hindgut. In the majority of cases, it is incompatible with life in the neonatal period as it is associated with renal agenesis. The importance of detecting this anomaly in the first trimester lies in this lethality in order to offer the option of termination of pregnancy at an earlier gestation. Appropriate counselling at diagnosis is essential to give the couple a clear picture of what they might expect postnatally regarding the renal anomalies or the many surgeries which might be required for the lower limbs and viscera. We present a case series (n = 5) diagnosed by ultrasound at our center between January 2014–2020. Through this brief article, we aim to give a key to the antenatal diagnosis of this rare anomaly, an aid on how not to miss it and its subsequent management.