bluuebunny/crossref_metadata_2025_split huggingface dataset:
Symptomatic care of late-onset Alexander disease presenting with area postrema-like syndrome with prednisolone; a case report
Authors: Zardadi Safoura , Razmara Ehsan , Rasoulinezhad Maryam , Babaei Meisam , Ashrafi Mohammad Reza , Pak Neda , Garshasbi Masoud , Tavasoli Ali Reza 
Published: July 2022

Abstract
Background
Alexander disease (AxD) is classified into AxD type I (infantile) and AxD type II (juvenile and adult form). We aimed to determine the potential genetic cause(s) contributing to the AxD type II manifestations in a 9-year-old male who presented area postrema-like syndrome and his vomiting and weight loss improved after taking prednisolone.

Case presentation
A normal cognitive 9-year-old boy with persistent nausea, vomiting, and a significant weight loss at the age of 6 years was noticed. He also experienced an episode of status epilepticus with generalized atonic seizures. He showed non-febrile infrequent multifocal motor seizures at the age of 40 days which were treated with phenobarbital. He exhibited normal physical growth and neurologic developmental milestones by the age of six. Occasionally vomiting unrelated to feeding was reported. Upon examination at 9 years, a weak gag reflex, prominent drooling, exaggerated knee-deep tendon reflexes (3+), and nasal tone speech was detected. All gastroenterological, biochemical, and metabolic assessments were normal. Brain magnetic resonance imaging (MRI) revealed bifrontal confluent deep and periventricular white matter signal changes, fine symmetric frontal white matter and bilateral caudate nucleus involvements with garland changes, and a hyperintense tumefactive-like lesion in the brain stem around the floor of the fourth ventricle and area postrema with contrast uptake in post-contrast T1-W images. Latter MRI at the age of 8 years showed enlarged area postrema lesion and bilateral middle cerebellar peduncles and dentate nuclei involvements. Due to clinical and genetic heterogeneities, whole-exome sequencing was performed and the candidate variant was confirmed by Sanger sequencing. A de novo heterozygous mutation, NM_001242376.1:c.262 C > T;R88C in exon 1 of the GFAP (OMIM: 137,780) was verified. Because of persistent vomiting and weight loss of 6.0 kg, prednisolone was prescribed which brought about ceasing vomiting and led to weight gaining of 3.0 kg over the next 3 months after treatment. Occasional attempts to discontinue prednisolone had been resulting in the reappearance of vomiting.

Conclusions
This study broadens the spectrum of symptomatic treatment in leukodystrophies and also shows that R88C mutation may lead to a broad range of phenotypes in AxD type II patients.
Role of complete blood cell count parameters in the diagnosis of neonatal sepsis
Authors: Worku Minichil , Aynalem Melak , Biset Sirak , Woldu Berhanu , Adane Tiruneh , Tigabu Abiye 
Published: July 2022

Abstract
Background
Neonatal sepsis is one of the leading causes of neonatal morbidity and mortality in developing countries like Ethiopia. The investigation of neonatal sepsis needs the application of inclusive diagnostic tools. Therefore, this study aimed to assess the role of CBC parameters in diagnosing neonatal sepsis.

Methods
A comparative cross-sectional study was conducted from September 2020 to November 2021 at the University of Gondar Comprehensive Specialized Hospital. A total of 250 neonates were included using a convenient sampling technique. A structured questionnaire and a data collection sheet were used to obtain the socio-demographic and clinical characteristics of the study participants. A venous blood sample was collected for CBC and blood culture tests. Epi-Info Version 7 and SPSS Version 25 were used for data entry and analysis, respectively. The data distribution was checked by the Shapiro-Wilk test. Then, an independent t-test was conducted to compare CBC parameters, and the significant parameters were recruited for the ROC curves analysis. The Younden index test was used to determine the cutoff point for the sensitivity and specificity. A p-value of <0.05 was considered statistically significant.

Results
Out of 250 study participants, 144 (57.6%) were males, with a median age of 6 days (IQR = 4 days). Early-onset and late-onset sepsis were developed in about 29.6% (37/250) and 70.4% (88/250) of the neonates, respectively. The TLC and ANC parameters were significantly lower in cases than in control groups. The TLC, Hgb, lymphocyte count, and ANC parameters have a sensitivity of 64.8, 68, 33.6, and 49.6%, respectively. Their specificity in the diagnosis of neonatal sepsis was 64.8, 53.6, 83.2, and 90.4%, respectively.

Conclusion
Total leucocyte count, ANC, and platelet count all showed significant associations with neonatal sepsis. Besides, the TLC, ANC, and platelet counts had good sensitivity and specificity in diagnosing neonatal sepsis. Therefore, these parameters can be used as a diagnostic tool for neonatal sepsis in resource-limited areas.
An investigation into religious awareness as a crucial factor in adherence to COVID-19 medical directives in Palestine
Authors: Saeedi Munther , Al-Othman Nihad , Rabayaa Maha , Dwaikat Saeed 
Published: July 2022

Abstract
Background
Coping with the pandemic caused by the SARS-COV- 2 has been a global challenge. To this end, several measures have been adopted to control the transmission of the disease and to ensure public safety. One factor that has greatly affected the community’s behaviors, attitudes, and practices in Palestine has been religious beliefs.

Aim
This study aims to investigate the role of religion as a factor in adherence to the COVID-19 medical directives in Palestine.

Methods
A descriptive cross-sectional study was performed from August to October 2021. In this study, 1,353 participants were asked to complete a questionnaire that consisted of 20 items that measured the impact of religious beliefs and the role played by religious scholars in the promotion and application of medically-approved health directives and the rectification of COVID-19 related information. The data were analyzed by using SPSS version 22 software.

Results
More than 50% of the participants agreed that religion has a positive impact on community adherence to the health instructions in the majority of studied items. The responses were significantly variable based on the age and place of residence in most of the questions (p-value < 0.05). However, gender and to a lesser extent, the level of education affected the responses to many research aspects less significantly.

Conclusions
Religion could be an effective tool in dealing with challenging health issues such as COVID-19. Intervention programs can be developed based on the community’s religious beliefs, attitudes, and practices, to dispel myths regarding the disease and to encourage community commitment and adherence to health directives.
Stent patency rates and prognostic factors of endovascular intervention for iliofemoral vein occlusion in post-thrombotic syndrome
Authors: Chen Rencong , Feng Ruijia , Jiang Suiting , Chang Guangqi , Hu Zuojun , Yao Chen , Jia Benyuan , Wang Shenming , Wang Siwen 
Published: July 2022

Abstract
Objective
Post-thrombotic syndrome (PTS), an important complication of deep venous thrombosis (DVT), adversely affects patients’ quality of life. Endovascular intervention in PTS can relieve symptoms rapidly with high therapeutic value. This study mainly focuses on how to improve postoperative stent patency rates and aims to find prognostic factors impacting patency.

Methods
According to the specific inclusion and exclusion criteria, PTS patients who underwent endovascular intervention at the First Affiliated Hospital of Sun Yat-sen University from December 1, 2014, to December 31, 2019, were included in this single-center prospective study. Follow-up data were collected and analyzed regularly over 2 years.

Results
Overall, 31 PTS patients were enrolled in the study. The mean age of these patients was 55.39 ± 11.81, including 19 male patients. Stent implantation was successful in 22 PTS patients, with a technical success rate of 70.97%. The average Villalta scores of the stent-implanted group and the non-stent-implanted group were 5.95 ± 2.57 and 5.78 ± 2.95, respectively, with no significant difference observed. In the stent-implanted group, the perioperative patency rate was 81.81% (18/22), and the follow-up patency rates were 68.18% (15/22) within 3 months, 59.09% (13/22) within 6 months, 45.45% (10/22) within 1 year, and 36.36% (8/22) within 2 years. Based on the stent placement segments, the 22 PTS patients were divided into two subgroups: the iliofemoral vein balloon dilation + iliofemoral vein stent implantation (FV-S) subgroup and the iliofemoral vein balloon dilation + iliac vein stent implantation (FV-B) subgroup. In the FV-S subgroup, the perioperative patency rate was 100.00% (14/14), and the follow-up patency rates were 85.71% (12/14), 71.43% (10/14), 57.14% (8/14) and 50.00% (7/14), which were higher than those for overall stent patency of all patients. The postoperative patency rates in the FV-B subgroup were 50.00% (4/8), 37.50% (3/8), 37.50% (3/8), 25.00% (2/8), and 12.50% (1/8). The secondary postoperative patency rates in the FV-B subgroup were 100.00% (8/8), 87.50% (7/8), 75.00% (6/8), 62.50% (5/8) and 50.00% (4/8).

Conclusions
For PTS patients with iliofemoral vein occlusion but patent inflow, iliofemoral vein stent implantation is a more efficient therapeutic option than iliofemoral vein balloon dilation with iliac vein stent implantation for PTS patients.
Quantitative proteomic analysis of Bi Zhong Xiao decoction against collagen-induced arthritis rats in the early and late stages
Authors: He Cailin , Wang Yang , Wen Yuqi , Li Teng , Hu En , Zeng Siqing , Xiong Xingui 
Published: July 2022

AbstractBackgroundRheumatoid arthritis (RA) is a chronic, progressive, systemic autoimmune inflammatory disease. Bi Zhong Xiao decoction (BZXD) performs multiple functions for rheumatoid arthritis (RA) treatment for decades. In this study, we aimed to study the protein alterations of BZXD in the early and late stages of RA.MethodsSprague–Dawley rats were randomly divided into the Control, collagen-induced arthritis (CIA) and BZXD groups. Clinical assessment, paw thickness, weight changes and serum inflammatory cytokine levels were used to evaluate anti-inflammatory effects. Histopathological tests were performed to assess the improvement of inflammation and synovial hyperplasia. Moreover, we analyzed the proteins profiling of synovial tissue samples with different time intervals after BZXD treatment by Isobaric Tag for Relative Absolute (ITRAQ) quantitative proteomics technology. To further explore the interrelationships among differentially expressed proteins (DEPs), we used DAVID Bioinformatics Resources v6.8 and STRING 11.0 for bioinformatics analysis. Besides, the western blot and immunohistochemistry were exerted to verify related proteins.ResultsIn our study, BZXD ameliorated joint inflammation, and suppressed the pathological changes in arthrosis of CIA rats. The proteomic analysis demonstrated that CIA rats were mainly involved in two significant pathways (the focal adhesion and the ECM-receptor interaction) in the early stage. BZXD down-regulated the expression of proteins involved in these pathways, such as CAV1, CHAD, COL3A1, COL5A2, COL6A1, and COL6A5. Additionally, BZXD exerts anti-inflammatory effects in the late stage mainly by increasing the expression of FASN and affecting fatty acid metabolism.ConclusionBZXD exerts therapeutic effects on RA through multi-pathways in the early and late stages. This work may provide proteomic clues for treating RA by BZXD.
Access to healthcare for people experiencing homelessness in the UK and Ireland: a scoping review
Authors: McNeill Sarah , O’Donovan Diarmuid , Hart Nigel 
Published: July 2022

Abstract
Background
People experiencing homelessness (PEH) have poorer physical and mental health than the general population. They are also more likely to have less access to healthcare. These processes of access can be better understood using Levesque’s access framework which addresses both supply (service provision) and demand (user abilities).

Methods
Following the Joanna Briggs Institute (JBI) guidelines, electronic peer-reviewed databases were searched in February 2022 for studies published since 2000 related to access to healthcare for PEH ages 16 and older in the United Kingdom (UK) and Ireland. Retrieved articles were screened and those eligible were selected for data extraction. Qualitative and quantitative studies were included.

Results
Fifty-six papers out of 538 identified were selected and aliased. Six main themes were identified: staff education, flexibility of systems, service coordination, patient preparedness, complex health needs and holistic care. These relate to the Levesque access framework.

Conclusions
Improving access to healthcare for PEH requires changes to how services are provided and how service-user abilities are supported.
Harm reduction implementation among HIV service organizations (HSOs) in the U.S. south: a policy context analysis and results from a survey of HSOs
Authors: Stanton Megan C. , Ali Samira B. , McCormick Katie 
Published: July 2022

Abstract
Background
HIV service organizations are integral to serving communities disproportionately impacted by the HIV and opioid epidemics in the U.S. South. Addressing these intersecting epidemics requires implementation of evidence-based approaches, such as harm reduction. However, little is known about the extent to which Southern HIV service organizations implement harm reduction. This manuscript examines: 1) the implementation context of harm reduction in the South, 2) Southern HIV service organization implementation of harm reduction, and 3) the impact of different contexts within the South on HIV service organization implementation of harm reduction.

Methods
To examine implementation context, authors analyzed nation-wide harm reduction policy and drug-related mortality data. To examine HIV service organization implementation of harm reduction, authors performed frequency distributions on survey data (n = 207 organizations). Authors then constructed logistic regressions, using state mortality data and policy context as predictors, to determine what contextual factors predicted HIV service organization implementation of harm reduction.

Results
Drug-related mortality data revealed an increased need for harm reduction, and harm reduction policy data revealed an increased political openness to harm reduction. Frequency distributions revealed that approximately half of the HIV service organizations surveyed reported that their organizations reflect a harm reduction orientation, and only 26% reported providing harm reduction services. Despite low utilization rates, HIV service organizations indicated a strong interest in harm reduction. Logistic regressions revealed that while increased mortality rates do not predict HIV service organization implementation of harm reduction, a harm reduction-friendly policy context does.

Discussion
This study highlights how regions within a high-income country can face unique barriers to healthcare and therefore require a unique understanding of implementation context. Study findings indicate a rapidly changing implementation context where increased need meets increased political opportunity to implement harm reduction, however there is a lag in HIV service organization adoption of harm reduction. Financial resources, capacity building, and continued policy advocacy are required for increased HIV service organization adoption of harm reduction.
Exploring the role of leaders in enabling adaptive capacity in hospital teams – a multiple case study
Authors: Fagerdal Birte , Lyng Hilda Bø , Guise Veslemøy , Anderson Janet E. , Thornam Petter Lave , Wiig Siri 
Published: July 2022

Abstract
Background
Resilient healthcare research studies how healthcare systems and stakeholders adapt and cope with challenges and changes to enable high quality care. Team leaders are seen as central in coordinating clinical care, but research detailing their contributions in supporting adaptive capacity has been limited. This study aims to explore and describe how leaders enable adaptive capacity in hospital teams.

Methods
This article reports from a multiple embedded case study in two Norwegian hospitals. A case was defined as one hospital containing four different types of teams in a hospital setting. Data collection used triangulation of observation and interviews with leaders, followed by a qualitative content analysis.

Results
Leaders contribute in several ways to enhance their teams’ adaptive capacity. This study identified four key enablers; (1) building sufficient competence in the teams; (2) balancing workload, risk, and staff needs; (3) relational leadership; and (4) emphasising situational understanding and awareness through timely and relevant information.

Conclusion
Team leaders are key actors in everyday healthcare systems and facilitate organisational resilience by supporting adaptive capacity in hospital teams. We have developed a new framework of key leadership enablers that need to be integrated into leadership activities and approaches along with a strong relational and contextual understanding.
Persistence of Staphylococcus spp. in milk from cows undergoing homeopathy to control subclinical mastitis
Authors: Ferreira Elka Machado , Romero Letícia Castilho , Cunha Maria de Lourdes Ribeiro de Souza d , Malagó Junior Wilson , Camargo Carlos Henrique , Barioni Júnior Waldomiro , Zafalon Luiz Francisco 
Published: July 2022

Abstract
Background
Mastitis is one of the major diseases in dairy cattle, as it causes great economic losses to producers due to the reduction of milk production and changes in the quality of the product. The disease is mainly caused by bacteria of the genus Staphylococcus spp., these microorganisms can express various virulence factors, such as biofilms for example. In herds with organic management, producers and technicians use unconventional ways to treat and control the disease, such as homeopathy. However, it is not known if this type of treatment is able to control pathogenic bacteria such as those of the genus Staphylococcus, of relevance to animal and human health. Thus, the objective of this study was to investigate the production of biofilm in vitro and its genes by Staphylococcus spp. isolated in the milk of cows treated with homeopathy, as well as the persistence of microorganisms in animals.

Methods
Ninety-nine isolates of Staphylococcus spp. from cows treated and not treated with homeopathy were identified by internal transcribed space-polymerase chain reaction and investigated for the presence of the icaABCD, bap, aap, atlE, and bhp genes and in vitro biofilm production using the adhesion method on polystyrene plates. The enzyme restriction profile was determined by Pulsed-Field Gel Electrophoresis. Clusters of S. aureus and S. epidermidis with three or more isolates had an isolate selected for Multilocus Sequence Typing.

Results
The frequency of S. aureus isolations was similar in treated and untreated cows, while 71.4% of the coagulase-negative identified were isolated in cows treated with homeopathy. The distribution of the operon ica genes was similar in animals with and without treatment, except for the icaD gene, more frequent in treated cows. Production of biofilm was associated with presence of one or more genes from the icaADBC operon. S. aureus revealed a greater diversity and greater dissemination in cows treated and not treated with homeopathy. Sequence Types ST1, ST5, and ST126 were identified in S. aureus.

Conclusions
The presence of biofilm-associated genes and the in vitro production of biofilms, combined with the persistence of clonal profiles of Staphylococcus spp. demonstrate other forms of control for bovine mastitis should be researched for organic production herds.
Identification of a novel heterozygous missense TP63 variant in a Chinese pedigree with split-hand/foot malformation
Authors: Miao Mingzhu , Lu Shoulian , Sun Xiao , Zhao Meng , Wang Jue , Su Xiaotan , Jin Bai , Sun Lizhou 
Published: July 2022

Abstract
Background
Tumor protein p63 is an important transcription factor regulating epithelial morphogenesis. Variants associated with the TP63 gene are known to cause multiple disorders. In this study, we determined the genetic cause of split-hand/foot malformation in a Chinese pedigree.

Methods
For this study, we have recruited a Chinese family and collected samples from affected and normal individuals of the family (three affected and two normal). Whole exome sequencing was performed to detect the underlying genetic defect in this family. The potential variant was validated using the Sanger sequencing approach.

Results
Using whole-exome and Sanger sequencing, we identified a novel heterozygous pathogenic missense variant in TP63 (NM_003722.5: c.921G > T; p.Met307Ile). This variant resulted in the substitution of methionine with isoleucine. Structural analysis suggested a resulting change in the structure of a key functional domain of the p63 protein.

Conclusion
This novel missense variant expands the TP63 variant spectrum and provides a basis for genetic counseling and prenatal diagnosis of families with split-hand/foot malformation or other TP63-related diseases.
Transient neuroinflammation following surgery contributes to long-lasting cognitive decline in elderly rats via dysfunction of synaptic NMDA receptor
Authors: Chen Bo , Qin Guangcheng , Xiao Jingyu , Deng Xiaoyuan , Lin Aolei , Liu Hongliang 
Published: July 2022

Abstract
Background
Perioperative neurocognitive disorders (PNDs) are considered the most common postoperative complication in geriatric patients. However, its pathogenesis is not fully understood. Surgery-triggered neuroinflammation is a major contributor to the development of PNDs. Neuroinflammation can influence N-methyl-D-aspartate receptor (NMDAR) expression or function which is closely associated with cognition. We, therefore, hypothesized that the persistent changes in NMDAR expression or function induced by transient neuroinflammation after surgery were involved in the development of PNDs.

Methods
Eighteen-month-old male Sprague–Dawley rats were subjected to abdominal surgery with sevoflurane anesthesia to establish the PNDs animal model. Then, we determined the transient neuroinflammation by detecting the protein levels of proinflammatory cytokines and microglia activation using ELISA, western blot, immunohistochemistry, and microglial morphological analysis from postoperative days 1–20. Persistent changes in NMDAR expression were determined by detecting the protein levels of NMDAR subunits from postoperative days 1–59. Subsequently, the dysfunction of synaptic NMDAR was evaluated by detecting the structural plasticity of dendritic spine using Golgi staining. Pull-down assay and western blot were used to detect the protein levels of Rac1-GTP, phosphor-cofilin, and Arp3, which contribute to the regulation of the structural plasticity of dendritic spine. Finally, glycyrrhizin, an anti-inflammatory agent, was administered to further explore the role of synaptic NMDAR dysfunction induced by transient neuroinflammation in the neuropathogenesis of PNDs.

Results
We showed that transient neuroinflammation induced by surgery caused sustained downregulation of synaptic NR2A and NR2B subunits in the dorsal hippocampus and led to a selective long-term spatial memory deficit. Meanwhile, the detrimental effect of neuroinflammation on the function of synaptic NMDARs was shown by the impaired structural plasticity of dendritic spines and decreased activity of the Rac1 signaling pathways during learning. Furthermore, anti-inflammatory treatment reversed the downregulation and hypofunction of synaptic NR2A and NR2B and subsequently rescued the long-term spatial memory deficit.

Conclusions
Our results identify sustained synaptic NR2A and NR2B downregulation and hypofunction induced by transient neuroinflammation following surgery as important contributors to the development of PNDs in elderly rats.