bluuebunny/crossref_metadata_2025_split huggingface dataset:
W246G Mutant ELOVL4 Impairs Synaptic Plasticity in Parallel and Climbing Fibers and Causes Motor Defects in a Rat Model of SCA34
Authors: Nagaraja Raghavendra Y , Sherry David M , Fessler Jennifer L. , Stiles Megan A. , Li Feng , Multani Karanpreet , Orock Albert , Ahmad Mohiuddin , Brush Richard , Anderson Robert E , Agbaga Martin-Paul , Deak Ferenc 
Published: January 2021

Abstract
Background: Spinocerebellar ataxias (SCA) are a group of neurodegenerative disorders characterized by neuronal degeneration leading to loss of motor coordination. A number of different mutations gives rise to different types of SCA with characteristic ages of onset, symptomatology, and rates of progression. SCA type 34 (SCA34) is an age-related cerebellar neurodegenerative disorder caused by mutations in the fatty acid elongase-4 (ELOVL4). The ELOVL4 is an essential enzyme that mediates biosynthesis of Very Long Chain Saturated and Polyunsaturated Fatty Acids (VLC-SFA and VLC-PUFA, resp., ≥28 carbons) that are critical for the normal function of brain, skin, retina, Meibomian glands, and testes in which ELOVL4 is expressed. Global deletion or homozygous expression of truncated mutant ELOVL4 that lack VLC-SFA and VLC-PUFA biosynthesis cause severe skin disorders, seizures and neonatal mortality in rodents and humans. Methods: To understand role of ELOVL4 and its products in neuronal function and to evaluate the consequences of ELOVL4 mutations in pathogenesis of age-related SCA34, we generated a rat model of SCA34 by knock-in of the SCA34-causing 736T>G (p.W246G) ELOVL4 mutation. We performed biochemical, neuroanatomical and behavioral analyses by rotorod to measure motor function. We used electrophysiological recordings from cerebellar slices to determine the impact of the W246G mutation on neuronal function. Results were analyzed using GraphPad Prism Statistical software. Results: Heterozygous and homozygous rats carrying the W246G mutation developed impaired motor deficits by two months of age. To understand the mechanism of these motor deficits, we performed electrophysiological studies using cerebellar slices from rats homozygous for W246G mutant ELOVL4 and found marked reduction of long-term potentiation at parallel fiber synapses and long-term depression at climbing fiber synapses onto Purkinje cells. Neuroanatomical analysis of the cerebellum up 6 months of age showed normal cytoarchitectural organization despite the early-onset motor deficits and defects in synaptic plasticity. Conclusions: Our results point to ELOVL4 and its products being essential for motor function and cerebellar synaptic plasticity. The results further suggest that in SCA34 patients, ataxia arises from primary impairment of synaptic plasticity and cerebellar network desynchronization that precedes cerebellar degeneration and loss of motor coordination with aging.
Relationship between enlarged perivascular space in hippocampus and P300 event-related potential in patients with acute lacunar stroke
Authors: Huang Shan , Zhang Zhixiang , Mei Yuqing , Huang Kuankuan , Feng Yiping , Gu Xia , Zhang Qiaoyang , Zhang Min , Yun Wenwei 
Published: January 2021

Abstract
Background

This study aimed to investigate the relationship between enlarged perivascular space in hippocampus (H-EPVS) and P300 event-related potential (ERP) in patients with acute lacunar stroke.
Methods

Patients with acute lacunar stroke hospitalized in Changzhou No.2 people's Hospital from February 2019 to July 2020 were collected. All the patients performed the P300 auditory oddball task and a full set of cognitive function tests. We recorded P300 latency and amplitude by EMG evoked potential instrument. H-EPVS showed round, oval and linear structures on MRI T2-weighted images of hippocampus, with clear boundaries and consistency in the direction of the perforating arterioles. Dividing the total number of H-EPVS ≥ 7 into extensive H-EPVS group (n = 61) and non-extensive H-EPVS group (n = 53). ROC curve was used to analyze the relationship between P300 ERP and cognitive function of extensive H-EPVS in patients with acute lacunar stroke.
Results

A total of 114 patients with acute lacunar stroke were included, and 61 (53.5%) patients were found to have extensive H-EPVS. The P300 latency in patients with extensive H-EPVS( mean and SD, 393.61 ± 30.36) was significantly higher than in non-extensive H-EPVS (mean and SD, 356.4 ± 19.59 ms, t = -7.870, P < 0.01). There was no significant difference in P300 amplitude between the two groups (P > 0.05). In addition, The cognitive function (the field of verbal fluency) in patients with extensive H-EPVS (mean and SD, -0.41 ± 0.50) was significantly lower than in non-extensive H-EPVS (mean and SD, 0.47 ± 0.54, t = 9.017, P < 0.01). Further analysis shows that in patients with acute lacunar stroke, P300 latency was positively associated with H-EPVS counts (r = 0.726, P = 0.000). H-EPVS counts was negatively associated with cognitive function score (the field of verbal fluency) (r=-0.705, P = 0.000). ROC curve analysis showed that the index of cognitive impairment diagnosed by P300 in patients with acute lacunar stoke was 0.796.
Conclusion

Extensive H-EPVS has a high incidence in patients with acute lacunar stroke. The P300 latency may be used as an early indicator to evaluate the verbal fluency function of extensive H-EPVS patients with acute lacunar stroke.
Association Between Community Environment and Dependency Among the Elderly From a Service Provision Perspective
Authors: Pan YiYang , Chen Yuan , Cui PingYu , WaiLi Nuremaguli , Li Ying 
Published: January 2021

Abstract
Background: The prevalence of dependency is high among the elderly worldwide and increases with increasing life expectancy. The objective of this study was to explore from the perspective of resource demand the association between community environmental resources and dependency among the elderly. Methods: The study was designed as a population-based cross-sectional study. A total of 950 participants aged ≥ 60 years from 22 locations in China were selected using a complex multistage sampling design. All data were collected using questionnaires and face-to-face interviews. The dependency was assessed using the Chinese version of the Minnesota Multiphasic Personality Inventory-II. The community environment was assessed using 43 items. Logistic regression analysis was used to evaluate the association between the community environment and dependency. Cluster analysis was used and demonstrated that dependency was mainly associated with community primary preventive care service resources. Results: In the analysis result, both age-specific and social support levels-stratified results showed that the dependency associated with community environmental resources differed among the elderly by age group and level of social support. Conclusions: The levels of dependency were significantly associated with community environment resources. Our results suggest that the dependent on local environment resources may consider as the resource needs among elderly.
Perioperative Use of Serum Creatinine and Postoperative Acute Kidney Injury: a Single Centre, Observational Retrospective Study to Explore Physicians’ Perception and Practice.
Authors: Villa Gianluca , De Rosa Silvia , Calabrisotto Caterina Scirè , Nerini Alessandro , Saitta Thomas , Innocenti Dario Degl , Paparella Laura , Bocciero Vittorio , Allinovi Marco , De Gaudio Angelo R. , Ostermann Marlies , Romagnoli Stefano 
Published: January 2021

Abstract
Background. Postoperative acute kidney injury (PO-AKI) is a leading cause of short- and long-term morbidity and mortality, as well as progression to chronic kidney disease (CKD). The aim of this study was to explore the physicians’ attitude toward use of serum creatinine (sCr) for identification of patients at risk for PO-AKI and patients who should be reassessed in the long-term for progression toward CKD. We also evaluated the incidence and risk factors associated with PO-AKI and renal function deterioration in patients undergoing major surgery for malignant disease.Methods. Adult oncological patients who underwent major abdominal surgery from November 2016 to February 2017 were considered for this single centre, observational retrospective study. Routinely available sCr values were used to define AKI in the first three postoperative days. Long-term kidney dysfunction (LT-KDys) was defined as a reduction in the estimated glomerular filtration rate by more than 10 ml/min/m2 at 12 months postoperatively. A questionnaire was administered to 125 physicians caring for the enrolled patients to collect information on local attitudes regarding use of sCr perioperatively and its relationship with PO-AKI.Results. A total of 423 patients were observed. sCr was not available in 59 patients (13.9%); the remaining 364 (86.1%) had at least one sCr value measured to allow for detection of postoperative kidney impairment. Among these, PO-AKI was diagnosed in 8.2% of cases. Of the 334 patients who had a sCr result available at 12-month follow up, 56 (16.8%) developed LT-KDys. Data on long-term kidney function were not available for 21% of patients. Interestingly, 33 of 423 patients (7.8%) did not have a sCr result available in the immediate postoperative period or long-term. All the physicians who participated in the survey (83 out of 125) recognised that postoperative assessment of sCr is required after major oncological abdominal surgery, particularly in those patients at high-risk for PO-AKI and LT-KDys.Conclusion. PO-AKI after major surgery for malignant disease is common but clinical practice of measuring sCr is variable. As a result, the exact incidence of PO-AKI and long-term renal prognosis are unclear, including in high-risk patients.Trial registration. Retrospectively registered in Clinicaltrialgov NCT04341974
PROLIFERATE: An Adaptable Framework to Evaluate Participatory Research Products
Authors: Plaza Maria Alejandra Pinero De , Archibald Mandy , Lawless Michael , Ambagtsheer Rachel , Mudd Alexandra , McMillan Penelope , Kitson Alison 
Published: January 2021

Abstract
BackgroundParticipatory research (PR) – the practice of involving stakeholders in research processes – is growing in prominence in health research because it can enhance the impact of research and the translation of research-based knowledge. Yet, the literature indicates that the products of PR studies are rarely evaluated empirically to track, demonstrate, and expand their claimed effectiveness and impact. This lack of measurement tools and frameworks can limit the effectiveness of knowledge translation (KT). MethodsWe introduce a framework for evaluating the products of PR called PROLIFERATE. We use an inductive combination of formative and summative evaluation methods to pilot test the framework on a Frailty PR communication product (a video) to determine the methods’ functionality.ResultsPROLIFERATE demonstrates adeptness for evaluating barriers and enablers of PR product uptake, effectiveness, and impact. It can identify ways to address barriers by assessing knowledge user perspectives on the comprehensibility of the product, emotional resonance, motivation to change, and future accessibility. ConclusionsPROLIFERATE can enable longitudinal and cross-sectional measurement of PR products in implementation and integrated KT efforts. It can evaluate and track the effectiveness and impact of different types of PR products in a situational responsive manner which compares users, platforms, and other factors in a replicable way.
Significantly Decreased Islet β Cell Function and Increased Fasting Plasma Glucose in Patients With Chronic Hepatitis B: a Cross-sectional Study
Authors: Liu Dafeng , Zhou Lingyun , Zhang Xinyi , Zeng Yilan , Bai Lang , Wu Dongbo , Tang Hong 
Published: January 2021

Abstract
Background: The contributing factors of abnormal glucose metabolism and the characteristics of the homeostasis model assessment of β cell function (HOMA-β) value in chronic hepatitis B (CHB) patients are unclear and worth studying.Method: This cross-sectional study recruited 110 CHB patients (CHB group) and 110 patients without hepatitis B virus (non-HBV group); the groups were matched according to sex, age, and body mass index. The contributing factors of abnormal glucose metabolism and the characteristics and differences in glucose metabolism parameters between the two groups were analyzed.Results: The abnormal glucose metabolism rate was higher in CHB patients with liver cirrhosis (LC) and patients with hepatitis B envelope antigen (HBeAg) (-) status. In addition, under the same glucose metabolism conditions, the fasting plasma glucose (FPG) levels of the CHB group was higher than that of the non-HBV group, especially in those with LC that had higher FPG levels (all p=0.000), while the HOMA-β values was significantly lower in the CHB group than in the non-HBV group, especially under normal glucose tolerance conditions (all p=0.000). Further analyses revealed that the main contributing factors of abnormal glucose metabolism were HBeAg (-) status and hepatitis B envelope antibody levels, but HBV serological and virological indicators had no direct effect on the HOMA-β value.Conclusion: These findings provide a reference that will allow clinicians to monitor abnormal glucose metabolism in CHB patients, especially those with LC or HBeAg (-) status, focus on the protection of islet β-cell function, and avoid the application of insulin secretagogues in CHB patients with abnormal glucose metabolism.
Se Alleviated Oxidative Stress-Mediated Complex Poisoning Mechanism in Pb-Treated Chicken Kidneys: Inflammation, Heat Shock Response, and Autophagy
Authors: Miao Zhiying , Yu Weikang , Wang Yueyang , Gu Xianhong , Teng Xiaohua 
Published: January 2021

Abstract
Background: Lead (Pb) is a toxic environmental pollutant and can exerts toxicity in kidneys. It is known that selenium (Se) has an antagonistic effect on Pb poisoning. However, biological events during the process were not well understood in chicken kidneys.Methods: One hundred and eighty male Hyline chickens (7-day-old) were randomly divided into the control group (offering standard diet and potable water), the Se group (offering Na2SeO3-added standard diet and potable water), the Pb group (offering standard diet and (CH3OO)2Pb-added potable water), and the Pb+Se group (offering Na2SeO3-added standard diet and (CH3OO)2Pb-added potable water). On 30th, 60th, and 90th days, kidneys were removed to perform the studies of histological structure, oxidative stress indicators, cytokines, heat shock proteins, and autophagy in the chicken kidneys.Results: The experimental results indicated that Pb poisoning changed renal histological structure; decreased catalase, glutathione-s-transferase, and total antioxidative capacity activities; increased hydrogen peroxide content; induced mRNA and protein expression of heat shock proteins; inhibited interleukin (IL)-2 mRNA expression, and induced IL-4 and IL-12β mRNA expression; inhibited mammalian target of rapamycin mRNA and protein expression, and induced autophagy-related gene mRNA and protein expression in the chicken kidneys. Supplement of Se mitigated the above changes caused by Pb.Conclusion: Our research strengthens the evidence that Pb induced oxidative stress, inflammation, heat shock response, and autophagy and Se administration alleviated Pb poisoning through mitigating oxidative stress in the chicken kidneys.
Longitudinal bidirectional relationships between maternal depressive/anxious symptoms and children’s tics
Authors: Yagi Tomoko , Ando Shuntaro , Usami Satoshi , Yamasaki Syudo , Morita Masaya , Kiyono Tomoki , Hayashi Noriyuki , Endo Kaori , Iijima Yudai , Morimoto Yuko , Kanata Sho , Fujikawa Shinya , Koike Shinsuke , Kano Yukiko , Hiraiwa-Hasegawa Mariko , Nishida Atsushi , Kasai Kiyoto 
Published: January 2021

Abstract
Background: Previous studies have revealed an association between maternal depressive/anxious symptoms and children’s tics. However, the longitudinal relationships between these symptoms remain unclear. We examined the longitudinal relationships between maternal depressive/anxious symptoms and children’s tics across early adolescence in a population-based sample.Methods: The participants consisted of 3,171 children and their mothers from the Tokyo Teen Cohort (TTC) study, a population-representative longitudinal study that was launched in Tokyo in 2012. Maternal depressive/anxious symptoms and children’s tics were examined using self-report questionnaires at the ages of 10 (time 1, T1) and 12 (time 2, T2). A cross-lagged model was used to explore the relationships between maternal depressive/anxious symptoms and children’s tics.Results: Higher levels of maternal depressive/anxious symptoms at T1 were related to an increased likelihood of children’s tics at T2 (β = .06, p = .001). Furthermore, the presence of children’s tics at T1 was positively related to maternal depressive/anxious symptoms at T2 (β = .06, p = .001).Conclusions: These findings suggest a longitudinal bidirectional relationship between maternal depressive/anxious symptoms and children’s tics during early adolescence that may exacerbate each other over time and possibly create a vicious cycle. When an early adolescent has tics, it might be important to identify and treat related maternal depressive/anxious symptoms.
Self-adaptive control system for additive manufacturing using double electrode micro plasma arc welding
Authors: Li Nan , Fan Ding , Huang Jiankang , Yu Shurong , Yuan Wen , Han Miaomiao 
Published: January 2021

Abstract
Wire arc additive manufacturing (WAAM) has been investigated to deposit large-scale metal parts due to its high deposition efficiency and low material cost. However, in the process of automatically manufacturing the high-quality metal parts by WAAM, several problems about the heat build-up, the deposit-path optimization, and the stability of the process parameters need to be well addressed. To overcome these issues, a new WAAM method based on the double electrode micro plasma arc welding (DE-MPAW) was designed. The circuit principles of different metal-transfer models in the DE-MPAW deposition process were analyzed theoretically. The effects between the parameters, wire feed rate and torch stand-off distance, in the process of WAAM were investigated experimentally. In addition, a real-time DE-MPAW control system was developed to optimize and stabilize the deposition process by self-adaptively changing the wire feed rate and torch stand-off distance. Finally, a series of tests were performed to evaluate the control system’s performance. The results show that the capability against interferences in the process of WAAM has been enhanced by this self-adaptive adjustment system. Further, the deposition paths about the metal part’s layer heights in WAAM are simplified. Finally, the appearance of the WAAM-deposited metal layers is also improved with the use of the control system.
Release of pseudosyndactyly in recessive dystrophic epidermolysis bullosa using a dermal regeneration template glove – the Foggia experience.
Authors: Lembo Fedele , Parisi Domenico , Cecchinov Liberato Roberto , Ciancio Francesco , Innocenti Alessandro , Portincasa Aurelio 
Published: January 2021

Abstract
BACKGROUNDː Epidermolysis Bullosa (EB) comprises a heterogeneous group of rare genetic diseases associated with skin blistering caused by minimal trauma. A major and common EB subtype, recessive dystrophic EB (RDEB), is characterized by altered wound healing, inflammatory dysbalance and fibrotic changes associated with reduced to absent collagen VII. Because of its exposed position and its continued use in daily activities, the hand is constantly at risk of microtrauma and is therefore one of the organs most affected by the disease with highly disabling deformities that represent a challenging field in hand surgery practice. METHODSː The Authors present their experience in the microsurgical treatment of pseudosyndactylies comparing the classic dressing with vaseline gauze with an innovative “glove protocol” using Integra® dermal regeneration template. The endpoints analyzed were: healing times, hospital stay time, discomfort for the patient, free-recurrence interval, follow-up range and major complications. RESULTSː A total of 34 procedures were performed on 24 RDEB patients with hand deformities. Compared with the dressing with vaseline gauze, microsurgery followed by application of dermal regeneration template gloves allowed a significant reduction of hospital stay, healing time, and dressing pain as well as an increased recurrence-free interval. CONCLUSIONSː The microsurgical approach followed by our new protocol described in the study has been beneficial in providing consistent and successful long-term results for these patients. Key words : Epidermolysis Bullosa, Pseudosyndactyly, Hand, Dermal regeneration template.
Nutritional perspectives on sickle cell disease in Africa: A systematic review
Authors: Nartey Eunice Berko , Spector Jonathan , Adu-Afarwuah Seth , Jones Catherine L. , Jackson Alan A. , Ohemeng Agartha , Shah Rajiv , Koryo-Dabrah Alice , Kuma Amma Benneh-Akwasi , Hyacinth Hyacinth I. , Steiner-Asiedu Matilda 
Published: January 2021

Abstract
Background: Sickle cell disease (SCD), an inherited blood disorder, predominantly affects individuals in sub-Saharan Africa. Research linking its pathophysiology and nutritional status in African patients has not been previously described. This systematic review aimed to assess the landscape of studies in sub-Saharan Africa focused on nutritional aspects of SCD and highlight gaps in knowledge that could inform priority-setting for future research.Methods: The study was conducted using the Preferred Reporting items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. All original studies involving individuals with any phenotypic variant of SCD and at least one nutritional status outcome; and were conducted between January 1995 and November 2020 in Africa were included. ‘Nutritional status’ was defined by studies on dietary intake, measurements of growth/anthropometry, or nutritional biomarkers. Studies were included if they involved Databases used were Ovid Embase, Medline, Biosis and Web of Science. Results: The search returned 526 articles with 76 studies included in the final analyses. Most investigations (67%) were conducted in Nigeria. Studies were categorized into one of three main categories: descriptive studies of anthropometric characteristics (49%), descriptive studies of macro- or micronutrient intakes (41%), and interventional studies (11%). Findings consistently found growth impairment, especially among children and adolescents from sub-Saharan Africa. Studies assessing macro- and micronutrients generally had small sample sizes and were exploratory in nature. Only four randomized trials were identified, which measured the impact of lime juice, long-chain fatty acids supplementation, ready-to-use supplementary food, and oral arginine on health outcomes. Conclusions: The findings reveal a moderate number of descriptive studies, most with small sample sizes, that focused on various aspects of nutrition and SCD in African patients. There was a stark lack of interventional studies that could be used to inform evidence-based changes in clinical practice. Findings of investigations were generally consistent with data from other regional settings, describing a significant risk of growth faltering and malnutrition among individuals with SCD. There is an unmet need for clinical research to better understand the potential benefits of nutritional-related interventions for patients with SCD in sub-Saharan Africa in order to promote optimal growth and improve health outcomes.