bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effect of 3 NR3C1 Mutations in the Pathogenesis of Pituitary ACTH Adenoma
Authors: Miao Hui , Liu Yang , Lu Lin , Gong Fengying , Wang Linjie , Duan Lian , Yao Yong , Wang Renzhi , Chen Shi , Mao Xinxin , Zhang Dongyun , Heaney Anthony P , Zhu Huijuan 
Published: August 2021

Abstract

Context
Glucocorticoids act through the glucocorticoid receptor (GR) encoded by the nuclear receptor subfamily 3 group C member 1 (NR3C1) gene.

Objective
This study aimed to examine the function of NR3C1 variants and their possible pathogenic role in Cushing disease (CD).

Methods
Next-generation sequencing was conducted in 49 CD patients. Corticotroph tumor GR protein expression was examined by immunohistochemistry (IHC). Constructs harboring the 3 NR3C1-mutant and wild-type (WT) GR were transfected into the murine corticotropic adenoma cell line (AtT-20), and GR protein expression was quantified by Western blot. Translocation activity was assessed by immunofluorescence and effects of the GR mutants on corticotroph tumor proliferation, pro-opiomelanocortin (POMC) transcription, and ACTH secretion were tested.

Results
Clinical features were similar in patients harboring the NR3C1 mutations and WT GR. Recurrent adenomas showed higher GR IHC scores than nonrecurrent tumors. In vitro studies demonstrated that the p.R469X mutant generated a truncated GR protein, and the p.D590G and p.Y693D GR mutants resulted in lower GR expression. Dexamethasone (DEX) treatment of AtT-20 cells demonstrated decreased DEX-induced nuclear translocation, increased cell proliferation, and attenuated suppression of POMC transcription of 3 GR mutants. Interestingly, the p.R469X GR mutant resulted in increased murine corticotroph tumor ACTH secretion compared to WT GR.

Conclusion
Our findings identify 3/49 (6.1%) consecutive human corticotroph tumors harboring GR mutations. Further findings demonstrate the role NR3C1 plays in CD pathogenesis and offer insights into a novel treatment approach in this patient subset.
Molecular “Hozo”: Thermally Stable Yet Conformationally Flexible Self-Assemblies Driven by Tight Molecular Meshing
Authors: Zhan Yi-Yang , Hiraoka Shuichi 
Published: August 2021

Abstract
Various noncovalent molecular interactions have been employed as driving forces to construct well-defined discrete self-assemblies. Among them, coordination and hydrogen bonds are widely used due to their high directionality and appropriate bond strength. However, the utilization of nondirectional, week molecular interactions for this purpose still presents a key challenge in supramolecular self-assembly. To tackle this critical issue, we presented a novel design concept, molecular “Hozo”, that the components with large, indented complementary hydrophobic surfaces tightly mesh with each other driven by the hydrophobic effect in water. Based on this concept, we developed a series of water-soluble cube-shaped molecular assemblies, i.e., nanocubes, composed of six molecules of identical gear-shaped amphiphiles (GSAs) with the aid of van der Waals (vdW) and cation-π interactions as well as the hydrophobic effect. The nanocubes exhibit unique properties derived from molecular meshing of the building blocks, such as high thermal stability yet as high conformational flexibility as biological molecules and emission whose intensity is affected by the structural change of the nanocube.
Chlorpyrifos and other pesticide exposure and suspected developmental delay in children aged under 5 years: a case-control study in Phitsanulok, Thailand
Authors: Juntarawijit Yuwayong , Chaichanawirote Uraiwan , Rakmeesri Paphada , Chairattanasakda Punaphop , Pumyim Varintorn , Juntarawijit Chudchawal 
Published: August 2021

Background: Developmental delay among children under 5 years of age is a serious global public health problem and much research has been carried out to find potential causes. Pesticides - especially organophosphates - are suspected to be one of the main causes of the problem. This study aimed to investigate the association between pesticide use by the mother during pregnancy and preschool children development using a case-control study.Methods: Data on prenatal and postnatal pesticide exposure of 442 children with suspected developmental delay, and 413 controls with normal development were included for analysis. The children were matched for gender, age, and residency. Data on pesticide exposure were collected via interview with the mother, and data on pregnancy outcomes abstracted from hospital records. Results: Chlorpyrifos exposure significantly increased the risk of developmental delay with an odds ratio (OR) of 3.71 (95% CI 1.03-13.36) for ever use of the pesticide, and an OR of 5.92 (95% CI 1.01-34.68) for postnatal exposure (p <0.05). Some other pesticides also had a positive association with developmental delay but none were statistically significant (p <0.05). Those pesticides were insecticide, fungicide, herbicide, and molluscicide. Individual pesticides with a positive association were glyphosate, paraquat, butachlor, methyl parathion (pholidon), savin, methomyl, endosulfan, carbosulfan, methamidophos, monochrotofos, mancozeb, and bordeaumixture.Conclusions: This case-control study found that chlorpyrifos and some other pesticides exposure during pregnancy were positively associated with developmental delay in children aged under 5 years. Further research should be conducted to better understand this potential effects of pesticides on child neurodevelopment, and the public - especially those who plan to have families - should be informed.
Knowledge Accumulation, Privacy, and Growth in a Data Economy
Authors: Cong Lin William , Xie Danxia , Zhang Longtian 
Published: August 2021

We build an endogenous growth model with consumer-generated data as a new key factor for knowledge accumulation. Consumers balance between providing data for profit and potential privacy infringement. Intermediate good producers use data to innovate and contribute to the final good production, which fuels economic growth. Data are dynamically nonrival with flexible ownership while their production is endogenous and policy-dependent. Although a decentralized economy can grow at the same rate (but are at different levels) as the social optimum on the Balanced Growth Path, the R&D sector underemploys labor and overuses data—an inefficiency mitigated by subsidizing innovators instead of direct data regulation. As a data economy emerges and matures, consumers’ data provision endogenously declines after a transitional acceleration, allaying long-run privacy concerns but portending initial growth traps that call for interventions. This paper was accepted by Kay Giesecke, finance.
A Technology-Enabled Adaptation of Face-to-Face Caregiver-Mediated JASPER Intervention: Preliminary Examination of Video Conferenced Caregiver Coaching
Authors: Shire Stephanie Y. , Worthman Lisa Baker , Arbuckle Stacy 
Published: August 2021

Abstract
Family-centered practices that involve direct participation of caregivers as part of intervention is critical to effective early intervention. However, regularly scheduled, in person service delivery is not always possible in remote communities, prompting a need for adaptations to the delivery of services, such as the use of live video conferencing to coach caregivers in strategies to promote their children's development. In this study, caregivers and their children ages 2–9 with autism who were living in rural and remote Canadian communities were included. A concurrent multiple baseline design across participants was applied to examine the effects of live video conference caregiver coaching on children's time jointly engaged with caregivers and caregivers' intervention strategy implementation. Results indicated that all children demonstrated greater time jointly engaged and caregivers demonstrated greater use of strategies in comparison to baseline. The results of this study offer preliminary evidence of the effectiveness of real time video conference coaching for caregivers engaging their children with ASD in play.
Embedded nanometer position tracking based on enhanced phasor analysis: erratum
Authors: Ma Hongqiang , Liu Yang 
Published: August 2021

We present an erratum to our Letter [Opt. Lett. 46, 3825 (2021)OPLEDP0146-959210.1364/OL.433740]. This erratum corrects an error in Eq. (2). All the simulations and experiments in the original Letter were performed using the correct equation, and therefore, this correction does not affect the results and conclusions of the original Letter.
Ein IT-gestützter Innovationsprozess in der öffentlichen Verwaltung: Rahmenkonzept, Ideenmanagementsysteme und Online-Whiteboards
Authors: Leible Stephan , Ludzay Max , Nüttgens Markus 
Published: August 2021

ZusammenfassungDieser Beitrag befasst sich mit der Innovationsfähigkeit der öffentlichen Verwaltung in Deutschland und den damit verbundenen Herausforderungen und Treibern. Auf der Basis semistrukturierter Interviews mit Politiker:innen und Führungskräften der Hamburger Verwaltung und der Sichtung und Einordnung wissenschaftlicher Literatur wurde ein Rahmenkonzept zur Gestaltung des Innovationsprozesses in der Verwaltungsdomäne entwickelt. Die Phasen des Rahmenkonzepts werden durch verschiedene IT-Lösungen, wie unter anderem einem übergreifenden Ideenmanagementsystem, gestützt. Zur vereinfachten Auswahl passgenauer Anwendungen, wurden in einer Marktanalyse verfügbare Ideenmanagementsysteme und Online-Whiteboards gesammelt und anhand vorab formulierter Bewertungskriterien ausgewertet. Das Rahmenkonzept und die ausgewählten IT-Lösungen wurden durch zwei halbtägige Workshops mit Bürger:innen und Mitarbeiter:innen der Hamburger Verwaltung erprobt und evaluiert. Die Ergebnisse bestätigen die Eignung der Anwendungen und im Ansatz die Praktikabilität des entwickelten Rahmenkonzepts für den Innovationsprozess in der öffentlichen Verwaltung.
Direct anterior approach (DAA) vs. conventional approaches in total hip arthroplasty: A RCT meta-analysis with an overview of related meta-analyses
Authors: Lazaru Philip , Bueschges Simon , Ramadanov Nikolai 
Published: August 2021

ObjectivesSeveral systematic reviews and meta-analyses on short-term outcomes between total hip arthroplasty (THA) through direct anterior approach (DAA) compared to THA through conventional (including anterior, anterolateral, lateral transgluteal, lateral transtrochanteric, posterior, and posterolateral) approaches (CAs) in treatment of hip diseases and fractures showed contradicting conclusions. Our aim was to draw definitive conclusions by conducting both a fixed and random model meta-analysis of quality randomized controlled trials (RCTs) and by comparison with related meta-analyses.DesignWe performed a systematic literature search up to May 2020 to identify RCTs, comparing THA through DAA with THA through CAs and related meta-analyses. We conducted risk of bias and level of evidence assessment in accordance with the Cochrane’s Risk of Bias 2 tool and with the guidelines of the Centre for Evidence-Based Medicine. We estimated mean differences (MD) with 95% confidence intervals (CI) through fixed and random effects models, using the DerSimonian and Laird method. Heterogeneity was assessed using tau-square (τ2). Our conclusions take into account the overall results from related meta-analyses.ResultsNine studies on THA through DAA met the criteria for final meta-analysis, involving 998 patients. Three studies were blinded RCTs with a level I evidence, the other 6 studies were non-blinded RCTs with a level II evidence. We came to the following results for THA through DAA compared to THA through CAs: operation time (I2= 92%, p<0.01; fixed: MD = 15.1, 95% CI 13.1 to 17.1; random: MD = 18.1, 95% CI 8.6 to 27.5); incision length (I2= 100%, p<0.01; fixed: MD = -2.9, 95% CI -3.0 to -2.8; MD = -1.1, 95% CI -4.3 to 2.0); intraoperative blood loss (I2= 87%, p<0.01; fixed: MD = 51.5, 95% CI 34.1 to 68.8; random: MD = 51.9, 95% CI -89.8 to 193.5); VAS 1 day postoperatively (I2= 79%, p = 0.03; fixed: MD = -0.8, 95% CI -1.2 to -0.4; random: MD = -0.9, 95% CI -2.0 to 0.15); HHS 3 months postoperatively (I2= 52%, p = 0.08; fixed: MD = 2.8, 95% CI 1.1 to 4.6; random: MD = 3.0, 95% CI -0.5 to 6.5); HHS 6 months postoperatively (I2= 0%, p = 0.67; fixed: MD = 0.9, 95% CI -1.1 to 2.9; random: MD = 0.9, 95% CI -1.1 to 2.9); HHS 12 months postoperatively (I2= 0%, p = 0.79; fixed: MD = 0.7, 95% CI -0.9 to 2.4; random: MD = 0.7, 95% CI -0.9 to 2.4). We compared our findings with 7 related meta-analyses.ConclusionsConsidering the results of our meta-analysis and the review of related meta-analyses, we can conclude that short-term outcomes of THA through DAA were overall better than THA through CAs. THA through DAA had a shorter incision length, a tendency towards a lower pain VAS 1 day postoperatively and better early postoperative functional outcome than THA through CAs. The intraoperative blood loss showed indifferent results. THA through DAA had a longer operation time than THA through CAs.
Toward prediction of abscopal effect in radioimmunotherapy: Pre-clinical investigation
Authors: Mihaylov Ivaylo B. , Totiger Tulasigeri M. , Giret Teresa M. , Wang Dazhi , Spieler Benjamin , Welford Scott 
Published: August 2021

Purpose
Immunotherapy (IT) and radiotherapy (RT) can act synergistically, enhancing antitumor response beyond what either treatment can achieve separately. Anecdotal reports suggest that these results are in part due to the induction of an abscopal effect on non-irradiated lesions. Systematic data on incidence of the abscopal effect are scarce, while the existence and the identification of predictive signatures or this phenomenon are lacking. The purpose of this pre-clinical investigational work is to shed more light on the subject by identifying several imaging features and blood counts, which can be utilized to build a predictive binary logistic model.

Materials and methods
This proof-of-principle study was performed on Lewis Lung Carcinoma in a syngeneic, subcutaneous murine model. Nineteen mice were used: four as control and the rest were subjected to combined RT plus IT regimen. Tumors were implanted on both flanks and after reaching volume of ~200 mm3 the animals were CT and MRI imaged and blood was collected. Quantitative imaging features (radiomics) were extracted for both flanks. Subsequently, the treated animals received radiation (only to the right flank) in three 8 Gy fractions followed by PD-1 inhibitor administrations. Tumor volumes were followed and animals exhibiting identical of better tumor growth delay on the non-irradiated (left) flank as compared to the irradiated flank were identified as experiencing an abscopal effect. Binary logistic regression analysis was performed to create models for CT and MRI radiomics and blood counts, which are predictive of the abscopal effect.

Results
Four of the treated animals experienced an abscopal effect. Three CT and two MRI radiomics features together with the pre-treatment neutrophil-to-lymphocyte (NLR) ratio correlated with the abscopal effect. Predictive models were created by combining the radiomics with NLR. ROC analyses indicated that the CT model had AUC of 0.846, while the MRI model had AUC of 0.946.

Conclusions
The combination of CT and MRI radiomics with blood counts resulted in models with AUCs of 1 on the modeling dataset. Application of the models to the validation dataset exhibited AUCs above 0.84, indicating very good predictive power of the combination between quantitative imaging and blood counts.
Prevalence and incidence of physical health conditions in people with intellectual disability – a systematic review
Authors: Liao Peiwen , Vajdic Claire , Trollor Julian , Reppermund Simone 
Published: August 2021

ObjectiveTo synthesize evidence on the prevalence and incidence of physical health conditions in people with intellectual disability (ID).MethodsWe searched Medline, PsycInfo, and Embase for eligible studies and extracted the prevalence, incidence, and risk of physical health conditions in people with ID.ResultsOf 131 eligible studies, we synthesized results from 77 moderate- to high-quality studies, which was mainly limited to high-income countries. The highest prevalence estimates were observed for epilepsy, ear and eye disorders, cerebral palsy, obesity, osteoporosis, congenital heart defects, and thyroid disorders. Some conditions were more common in people with a genetic syndrome. Compared with the general population, many health conditions occur more frequently among people with ID, including asthma and diabetes, while some conditions such as non-congenital circulatory diseases and solid cancers occur at the same or lower rate. The latter associations may reflect under-detection.ConclusionsPeople with ID have a health profile more complex than previously known. There is a pressing need for targeted, evidence-informed population health initiatives including preventative programs for this population.
Association of dialysis-related amyloidosis with lower quality of life in patients undergoing hemodialysis for more than 10 years: The Kyushu Dialysis-Related Amyloidosis Study
Authors: Tsuruya Kazuhiko , Arima Hisatomi , Iseki Kunitoshi , Hirakata Hideki ,   The Kyushu Dialysis-Related Amyloidosis Study Group
Published: August 2021

Background
Dialysis-related amyloidosis (DRA) commonly develops in patients undergoing long-term dialysis and can lead to a decline in activities of daily living and quality of life (QOL), mainly owing to orthopedic complications.

Methods
First, we determined utility scores of the EuroQol 5-Dimensions 3-Levels (EQ-5D-3L) questionnaire in 1,323 patients with DRA who had undergone dialysis for more than 10 years and compared the score between those with and without DRA. Second, a 2-year follow-up was also performed, in which patients were divided into three groups: those complicated by DRA from the beginning, those with newly developed DRA within the 2-year period, and those not complicated by DRA throughout the survey period; changes in the EQ-5D-3L utility score were compared. In the group already complicated by DRA at the survey baseline, changes in the EQ-5D-3L utility score were compared according to the dialysis treatment method.

Results
A total of 1,314 and 931 patients were included in the first and second studies, respectively. EQ-5D-3L utility scores among patients diagnosed with DRA were significantly lower than scores in those not diagnosed with DRA. The reduction in the EQ-5D-3L utility score over the 2-year follow-up was significantly greater in patients newly complicated by DRA during the follow-up period after enrollment but not in those complicated by DRA from the beginning, as compared with patients not complicated by DRA throughout the survey period. The reduction in utility score tended to be lower in patients routinely treated with a β2-microglobulin adsorption column than in untreated patients with DRA.

Conclusion
Complication by DRA in patients undergoing long-term hemodialysis was significantly associated with a decline in QOL.
Detection of moisture and carotenoid content in carrot slices during hot air drying based on multispectral imaging equipment with selected wavelengths
Authors: Long Jiamei , Yang Jia , Peng Jing , Pan Leiqing , Tu Kang 
Published: August 2021

Abstract
Moisture content and carotenoid content are important indicators for evaluating the drying process of carrot slices. There are growing attention to develop non-destructive methods as effectively analytical tools in quality assurance of drying carrot slices. In this study, the characteristic wavelengths of moisture and carotenoid content in carrot slices during hot air drying were extracted based on hyperspectral imaging technology. A multispectral imaging equipment was built after that, and the wavelengths of filters were determined according to the characteristic wavelengths. Based on the successive projection algorithm (SPA), the optimal wavelengths of moisture and carotenoid content were further determined, and prediction models of both were established based on the system. There were 12 filters selected in this study. The results showed that a support vector machine (SVM) prediction model for moisture content was established based on seven optimal wavelengths with 0.991 for the coefficient of determination of prediction set (R
2

p
) and 10.318 for the residual prediction residual (RPD). Based on eight optimal wavelengths, a SVM prediction model for carotenoid content was also established with 0.968 for R
2

p
and 5.337 for RPD. The prediction performance is close to or even better than that based on hyperspectral. The study confirmed the feasibility of using the multispectral imaging equipment to measure the moisture and carotenoid content of carrot slices during drying based on selected wavelengths, laying a foundation for the further preparation of a portable multispectral detector for the quality of dry products.
Modeling and simulations of a Zika virus as a mosquito-borne transmitted disease with environmental fluctuations
Authors: Gokila Chellamuthu , Sambath Muniyagounder 
Published: August 2021

Abstract
This paper deals with the stochastic Zika virus model within the human and mosquito population. Firstly, we prove that there exists a global positive solution. Further, we found the condition for a viral infection to be extinct. Besides that, we discuss the existence of a unique ergodic stationary distribution through a suitable Lyapunov function. The stationary distribution validates the occurrence of infection in the population. From that, we obtain the threshold value for prevail and disappear of disease within the population. Through the numerical simulations, we have verified the reproduction ratio

R

0

S

${R}_{0}^{S}$

as stated in our theoretical findings.
Cross-sectional analysis: clinical presentation of children with persistently low ALP levels
Authors: Semler Oliver , Partsch Carl-Joachim , Das Anibh Martin , Prechtl Andreas , Grasemann Corinna 
Published: August 2021

Abstract

Objectives
Low activity of serum alkaline phosphatase (ALP) is a hallmark of hypophosphatasia (HPP), but low readings of ALP are not always recognized in clinical routine. Understanding the clinical presentations associated with low ALP may contribute to a timelier diagnosis of HPP.

Methods
Data from paediatric patients with low ALP, excluding patients in intensive care and with oncological/haematological disorders, were analysed. Most recent ALP values, previous diagnoses, medication and relevant symptoms were extracted from patient records at nine specialised centres and analysed descriptively. A relationship between body height and ALP values was scrutinised by linear regression.

Results
Of 370 children, 15 (4.1%) had a diagnosis of HPP. In the subgroup without a diagnosis of HPP, 241 (67.9%) out of 355 patients had one or more medical conditions known to be associated with low serum ALP. Of those, hypothyroidism, malnutrition and steroid administration were most frequent. Characteristic symptoms, particularly, short stature, muscle weakness and delay of motor development were more frequent and ALP values were lower in patients with documented HPP diagnosis compared to patients without diagnosis of HPP (Ø z-scores: −2.52) (interquartile range [IQR] = 0.20) vs. −1.96 (IQR = 0.87). A weak positive linear relationship between z-scores of ALP and body height was identified (p<0.001).

Conclusions
This analysis of paediatric patient records elucidates a wide range of disorders associated with low ALP activity. In case of additional specific symptoms, HPP should always be considered as a differential diagnosis.