Corrigendum: Frontier and hotspot evolution in cerebrotendinous xanthomatosis: a bibliometric analysis from 1993 to 2023.

[This corrects the article DOI: 10.3389/fneur.2024.1371375.].

Frontier and hotspot evolution in cerebrotendinous xanthomatosis: a bibliometric analysis from 1993 to 2023.

Background: Cerebrotendinous xanthomatosis (CTX) is an autosomal recessive disease associated with lipid metabolic disorders. Because of its clinical diversity and rarity, the diagnosis is often unclear. However, there is still a lack of reports on bibliometric analysis of CTX. The aim of this study was to assess the progress and research developments of CTX over the past three decades, identify emerging trends, and establish novel directions for future research. Methods: The eligible literature were screened from the Web of Science Core Collection (WoSCC) database. The annual publication, countries, institutions, authors, journals, keywords and references were visually analyzed by Microsoft Excel 2019, CiteSpace 6.2.R4, VOSviewer 1.6.18 and online bibliometrics website (https://bibliometric.com/). Results: A total of 561 publications from WoSCC were included in this study. The United States is the country with the largest number of publications, and Karolinska Institutet is the institution with the largest number of publications. Björkhem I. ranks as the most published and cited author in the last three decades. Journal of Lipid Research is the most widely published and cited journal. The strongest burst of keywords is "diagnosis." Conclusion: Unraveling the pathogenesis of CTX and improving its diagnosis and treatment continue to be critical challenges that require urgent attention. Future research endeavors will be centered on enhancing the efficiency and accuracy of early diagnosis and intervention.

Bibliometric analysis of worldwide research on Polycythemia Vera in the 21st century.

Polycythemia vera (PV) is a myeloproliferative tumor with low incidence and complex symptoms, affecting patients' quality of life and shortening their life span. Since the beginning of the 21st century, there has been an update but a need for uniform consensus regarding diagnosing and treating PV. With the continued interest of researchers in this field, a bibliometric study of PV is necessary. This paper aims to analyze articles on PV through bibliometric software to provide collaborative information and new ideas for researchers in this field. We collected PV-related publications in the Web of Science Core Collection database from 2000 to 2023. The included literature was analyzed using Citespace (6.2.R2), VOSviewer (1.6.19), and Bibliometrix. The study included country/region, institution, authors, journals, keywords, and references, and a visual knowledge network diagram was constructed. Microsoft Excel 2013 was also used for statistical analysis. A total of 1,093 articles were eventually included. The number of PV-related publications has steadily increased from 2000 to the present, with great potential for future growth. The US and US institutions have contributed more to this field, with the US ranking first in the number of publications, total citations, and centrality. Alessandro M. Vannucchi is the most published author. Tefferi, Ayalew is the most cited author. And BLOOD has the most publications, topping the list of the eleven high-productivity core source journals. The most cited article was "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders" (Baxter, EJ, 2005). By examining the keywords, we found that the diagnosis and typing of true erythrocytosis, the use of ruxolitinib, and the tyrosine kinase JAK2 are the research hotspots in the field; genetic and molecular research in the field of true erythrocytosis is a cutting-edge topic in the field; and risk factors for true erythrocytosis is a cutting-edge hotspot issue in the field. The fruitful research in this century has laid the foundation for developing the field of PV. The information in this article will provide researchers with current hotspots and future potential in the discipline, helping the field achieve more extraordinary breakthroughs. Currently, research should focus on increasing global multicenter collaborative research in diagnosis and treatment to develop scientifically recognized diagnostic and treatment protocols and new clinical drug research. Our proposed model of global innovation collaboration will provide strong support for future research.

Bibliometric analysis of lupus nephritis in children from 1999 to 2022: A review.

Lupus nephritis (LN) is a complication of systemic lupus erythematosus and a damaging disease of the kidney. The injury of LN in children is more serious than that in adults. However, the literature in this field is numerous and complex, which brings great challenges for researchers to extract information. The purpose of this study is to carry out bibliometric analysis and visualization of published literatures, and identify current research hotspots and future research trends in this field. Literature was retrieved from the Web Of Science database from 1999 to 2022. The literature was analyzed and visualized using Citespace 6.1.R6, VOSviewer 1.6.18, and Microsoft Excel 2019. A total of 1059 articles were included in this study. In the past 13 years, an increase in the number of publications every year. Brunner HI is the author with the highest number of published and cited papers in this field, followed by Wenderfer SE. The United States and China are the countries with the highest number of published papers. University Toronto is the most productive institution, followed by University Cincinnati. The most prolific journal was Pediatric nephrology (IF 2.67), followed by lupus (IF 2.21). Lupus was cited the most, followed by Pediatric nephrology. The keyword burst showed the earliest and longest burst was antiphospholipid antibody, validation/risk/rituximab/safety is the current research hotspot. The article with the highest number of citations was Hochberg MC 1997 published in Arthritis Rheum. This study provides valuable information summary for the field of LN in children, which is helpful to strengthen the cooperation among countries, institutions and authors, and promote the research in the field of LN in children.

Loke zupa decoction attenuates bronchial EMT-mediated airway remodelling in chronic asthma through the PI3K-Akt/HIF-1α signaling pathway.

CONTEXT: Loke zupa decoction (Lok) is a well-established classic Chinese folk remedy for asthma. OBJECTIVE: We sought to investigate the effect and mechanism of Lok on asthma airway remodelling and provide novel insights for the prevention and treatment of asthma. MATERIALS AND METHODS: For in vitro experiments, BEAS-2B cells were assigned into six groups: Control, TGF-ß1 (10 µM), TGF-ß1 + Lok-20, TGF-ß1 + Lok-40, TGF-ß1 + Lok-80 µg/mL and TGF-ß1 + SB431542 (5 µM). CCK8 and wound healing assays were performed. For in vivo experiments, 60 female BALB/c mice were randomly divided into 5 groups: Control, model, Lok-4.55, Lok-9.1, and DEX group. Lok was administrated by gavage during the challenge stage for 8 consecutive weeks (4.55 and 9.1 g/kg/day). We investigated airway inflammation and airway remodelling in the lungs and verified the activation status of EMT-related markers and the PI3K-Akt/HIF-1α signalling pathway. RESULTS: In vitro, Lok efficiently inhibited TGF-ß1-induced BEAS-2B cell proliferation ability (cell viability 165% vs. 105%) and migration (migration areas 85% vs. 35%) without affecting their normal growth (IC50 274.2 µg/mL at 48 h). In vivo, Lok effectively protected mice from asthma, as evidenced by decreased histological damage and level of cytokines in BALF (IL-4, IL-13 and TGF-ß1) by 17%-77%. Mechanistic research revealed that Lok reduced the levels of EMT-related molecules and significantly downregulated the PI3K-Akt/HIF-1α signalling pathway. DISCUSSION AND CONCLUSIONS: Our findings provide novel insights into the protective effect of Lok on asthma and the underlying mechanisms, providing a theoretical basis and potential treatment possibilities for this patient population.

Bibliometric and visual analysis of Kawasaki disease in children from 2012 to 2022.

Background: In recent years, the incidence of Kawasaki disease among the pediatric population has experienced a significant increase. With complications mainly affecting the cardiovascular system, Kawasaki disease has received widespread attention from scholars worldwide. Numerous articles on Kawasaki disease in children have been published far. However, there is a lack of studies that use visualization methods to perform a bibliometric analysis of the relevant literature. This study aims to obtain overall information on the output characteristics of publications on childhood Kawasaki disease between 2012 and 2022 through bibliometric analysis, identify research hotspots and frontiers, and provide new ideas and references for future clinical and scientific research. Methods: Literature meeting the inclusion criteria was screened from the Web of Science Core Collection, PubMed, and Scopus databases. Visual analysis of the literature by country, institution, journal, author, keywords, and references was performed using Citespace (6.1.R6), VOSviewer (1.6.18), and the online bibliometric website (https://bibliometric.com/). Results: A total of 4,867 eligible publications were included. The number of annual publications is generally rising, rapidly increasing since 2019. Among countries and institutions, China and KAOHSIUNG CHANG GUNG MEMORIAL HOSPITAL have the highest output of articles. With 104 publications, Ho-Chang Kuo has a high impact in the field of KD. The most cited author is Jane W. Newburger. The most prolific journal is FRONTIERS IN PEDIATRICS. CIRCULATION is the most frequently co-cited journal. The most popular keyword in frequency and centrality is "immunoglobulin". The reference with the highest burst intensity was Verdoni L, LANCET, 2020. Conclusion: Kawasaki disease in children remains a hot topic among pediatricians worldwide and is receiving increasing attention. We innovated the "national-institutional-journal" model, which promotes further international cooperation in this field. The hot topics in the field of pediatric KD are "KD pathogenesis", "immunoglobulin resistance and complementary therapy", and "cardiovascular complications". Frontiers include disease-related ("multisystem inflammatory syndrome", "coronavirus disease 2019", "hypotension"), treatment-related ("procalcitonin", " anakinra"), and pathogenesis ("polymerase chain reaction").

Worldwide Bronchiolitis obliterans research: A bibliometric analysis of the published literature between 2002 and 2022.

Bronchiolitis obliterans (BO) is a rare and irreversible chronic respiratory disease. The diagnosis of BO is challenging, and there still needs to be specific therapies and uniform treatment guidelines available. Research on BO has grown steadily over the past 20 years, and with the continued interest of researchers in this area, a bibliometric study of BO becomes necessary. This topic aims to assess the current state of research in BO over the last 2 decades and to identify research hotspots and emerging directions. Information on BO-related articles were obtained from the Science Citation Index Expand of the Web of Science Core Collection (WOSCC [SCI-E]) database. Citespace (6.1.R6), VOSviewer (1.6.18), and the online bibliometrics website (https://bibliometric.com/) were used for bibliometric analysis mainly to include country/region, institution, author, journal, keywords, and references and to construct visual knowledge network diagrams. A total of 4153 publications from the WOSCC [SCI-E] database were included in this study. Most publications come from the United States, Japan, and Germany, which collaborate relatively more frequently. Research institutions in the United States, especially the University of Washington, published the largest number of BO-related articles. Regarding authors, Vos, R is the most productive author, while Verleden, GM is the most influential in BO. In addition, JOURNAL OF HEART AND LUNG TRANSPLANTATION is the journal with the most published articles. The most cited article is Estenne M, 2002. Based on the clustering analysis of keywords and references, the diagnosis of bronchiolitis obliterans syndrome (BOS), treatment of BOS, and risk factors of BO are the current research hotspots and future research trends. We analyzed the publication trends in BO by bibliometrics and mapped the knowledge network of major contributing countries/regions, institutions, authors, and journals. Current research hotspots were found based on the main keywords and references. The outcome may help researchers identify potential collaborators, collaborating institutions, and hot fronts in BO to enhance collaboration on critical issues and improve the diagnosis and treatment of BO.

Network pharmacology and molecular docking to explore Polygoni Cuspidati Rhizoma et Radix treatment for acute lung injury.

BACKGROUND: Polygoni Cuspidati Rhizoma et Radix (PCRR), a well-known traditional Chinese medicine (TCM), inhibits inflammation associated with various human diseases. However, the anti-inflammatory effects of PCRR in acute lung injury (ALI) and the underlying mechanisms of action remain unclear. AIM: To determine the ingredients related to PCRR for treatment of ALI using multiple databases to obtain potential targets for fishing. METHODS: Recognized and candidate active compounds for PCRR were obtained from Traditional Chinese Medicine Systems Pharmacology, STITCH, and PubMed databases. Target ALI databases were built using the Therapeutic Target, DrugBank, DisGeNET, Online Mendelian Inheritance in Man, and Genetic Association databases. Network pharmacology includes network construction, target prediction, topological feature analysis, and enrichment analysis. Bioinformatics resources from the Database for Annotation, Visualization and Integrated Discovery were utilized for gene ontology biological process and Kyoto Encyclopedia of Genes and Genomes network pathway enrichment analysis, and molecular docking techniques were adopted to verify the combination of major active ingredients and core targets. RESULTS: Thirteen bioactive compounds corresponding to the 433 PCRR targets were identified. In addition, 128 genes were closely associated with ALI, 60 of which overlapped with PCRR targets and were considered therapeutically relevant. Functional enrichment analysis suggested that PCRR exerted its pharmacological effects in ALI by modulating multiple pathways, including the cell cycle, cell apoptosis, drug metabolism, inflammation, and immune modulation. Molecular docking results revealed a strong associative relationship between the active ingredient and core target. CONCLUSION: PCRR alleviates ALI symptoms via molecular mechanisms predicted by network pharmacology. This study proposes a strategy to elucidate the mechanisms of TCM at the network pharmacology level.

Metabolism and toxicity of usnic acid and barbatic acid based on microsomes, S9 fraction, and 3T3 fibroblasts in vitro combined with a UPLC-Q-TOF-MS method.

Introduction: Usnic acid (UA) and barbatic acid (BA), two typical dibenzofurans and depsides in lichen, have a wide range of pharmacological activities and hepatotoxicity concerns. This study aimed to clarify the metabolic pathway of UA and BA and illuminate the relationship between metabolism and toxicity. Methods: An UPLC-Q-TOF-MS method was developed for metabolite identification of UA and BA in human liver microsomes (HLMs), rat liver microsomes (RLMs), and S9 fraction (RS9). The key metabolic enzymes responsible for UA and BA were identified by enzyme inhibitors combined with recombinant human cytochrome P450 (CYP450) enzymes. The cytotoxicity and metabolic toxicity mechanism of UA and BA were determined by the combination model of human primary hepatocytes and mouse 3T3 fibroblasts. Results: The hydroxylation, methylation, and glucuronidation reactions were involved in the metabolic profiles of UA and BA in RLMs, HLMs, and RS9. CYP2C9, CYP3A4, CYP2C8, and UGT1A1 are key metabolic enzymes responsible for metabolites of UA and CYP2C8, CYP2C9, CYP2C19, CYP1A1, UGT1A1, UGT1A3, UGT1A7, UGT1A8, UGT1A9, and UGT1A10 for metabolites of BA. UA and BA did not display evident cytotoxicity in human primary hepatocytes at concentrations of 0.01-25 and 0.01-100 µM, respectively, but showed potential cytotoxicity to mouse 3T3 fibroblasts with 50% inhibitory concentration values of 7.40 and 60.2 µM. Discussion: In conclusion, the attenuated cytotoxicity of BA is associated with metabolism, and UGTs may be the key metabolic detoxification enzymes. The cytotoxicity of UA may be associated with chronic toxicity. The present results provide important insights into the understanding of the biotransformation behavior and metabolic detoxification of UA and BA.

Bibliometric analysis of IgA vasculitis nephritis in children from 2000 to 2022.

Background: IgA vasculitis Nephritis (IgAVN) is a kidney-damaging disease that occurs during the course of IgA vasculitis (IgAV) and is the most serious complication of IgAV. However, there is a lack of reports of bibliometric analysis of IgAVN in children. The purpose of this study is to conduct a bibliometric analysis of IgAVN in children from 2000 to 2022, to explore the current status and cutting-edge trends in the field of IgAVN in children, and to establish new directions for subsequent research. Methods: Screening the literature in the field of IgAVN in children in the Web of Science Core Collection (WoSCC) from 2000 to 2022. Visual analysis of their annual publications, countries, institutions, authors, journals, keywords, and references were using CiteSpace5.8.R3 and VOSviewer1.6.18. Results: A total of 623 publications were included in the study, since the beginning of 2014, there has been an overall increasing trend in the number of articles issued. The most prolific country and institution were China and Zhejiang University. The most frequently cited author was Coppo R, with 331 citations, who has made great contributions to IgAVN. Mao Jianhua, Lee JS and Wyatt Robert J were the most prolific authors, all with 9 articles. Pediatric Nephrology was the most published and cited journal. The highest burst strength keyword is IgA vasculitis, and the highest burst strength reference is Davin JC, 2014. Conclusion: The research hotspots and trends predicted by the analysis of this study provide a reference for in-depth research in this field with a view to promoting the development of IgAVN research in children.

Overall survival nomogram and relapse-related factors of clear cell sarcoma of the kidney: A study based on published patients.

Background: Rarity limits the breadth of study on clear cell sarcoma of the kidney (CCSK). There is currently no predictive model that quantifies the overall survival (OS) of CCSK and a few large sample-based analysis of relapse-related factors. Methods: Patients were collected both from the Surveillance, Epidemiology, and End Results (SEER) database and case report articles extracted from the global online document database to form 2 groups. The first was the OS group, which was used to build and verify the nomogram for predicting the OS of CCSK. Independent predictors of OS were screened by Cox regression analysis to develop the nomogram. Nomogram accuracy was assessed by C-index, receiver operating characteristic (ROC), calibration, and decision curve analysis (DCA) curves. In addition, the difference in OS between receiving radiotherapy or not in stage I patients was analyzed by the Chi-square test. The second was the relapse group, which was used to analyze the relapse-related factors by Cox regression analysis and the Kaplan-Meier method with the log-rank test. Result: 256 patients were included in the OS group. The stage, chemotherapy, and radiotherapy were independent OS-related factors of CCSK, and the nomogram for predicting the OS of CCSK was established based on them. The results of the C-index, ROC, calibration, and DCA curves showed that the nomogram has good discrimination, accuracy, and clinical profitability. The Chi-squared test showed no significant difference in OS with receiving radiotherapy or not in stage I patients. The relapse group included 153 patients, of which 60 relapsed. The univariate Cox regression analysis showed no correlation between radiotherapy and relapse. The multivariate Cox regression analysis showed that stage and surgery/chemotherapy sequence were the independent factors for relapse. The log-rank test of seven chemotherapeutic drugs showed that etoposide (E), cyclophosphamide (C), vincristine (V), and doxorubicin (D) (all P < 0.05) had significant differences in preventing relapse, and then drew the relapse-free survival curves of these four drugs. Conclusion: Our nomogram accurately quantified the OS of CCSK. There was no significant difference in OS between receiving radiotherapy or not in stage I patients. Stage, surgery/chemotherapy sequence, and the use of ECVD were relapse-related factors. Radiotherapy had no significant contribution to preventing relapse.

Henoch-Schönlein Purpura With Scrotal Involvement: A Case Report and Literature Review.

Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood and affects the small blood vessels, leading to arthritis, abdominal pain, and renal involvement. However, scrotal involvement is a rare complication of HSP and scrotal pain. Swelling is the most frequent clinical presentation and can be easily confused with testicular torsion. If not treated in time, the scrotal inflammation will result in irreversible testicular necrosis. We report a 6-year-old male with HSP and scrotal involvement, characterized by swelling and pain on the left side of the scrotum, rashes on both lower extremities, and epididymitis. He was treated with conservative care, corticosteroids, and antibiotic therapy. We were able to avoid surgical intervention. On the 10 days of treatment, he recovered sufficiently well and was discharged. We have reviewed the literature related to HSP with scrotal involvement, identified 21 cases, and revealed that steroid therapy and/or antibiotics are the first-line of therapy in children with scrotal involvement. Vasculitis in the scrotum may predispose to testicular torsion, which is a complication that should not be overlooked. Clinicians should be aware of the atypical types of HSP. Timely diagnosis and appropriate treatment are essential for achieving the best results.

Graphene quantum dots as nanoprobes for fluorescent detection of propofol in emulsions.

We report a new fluorescent detection method for propofol based on graphene quantum dots (GQDs). Citric acid (CA) was selected as the carbon precursor, and fluorescent GQDs were prepared by carbonizing CA. The product, which efficiently quenched the fluorescence of GQDs, could be obtained through the oxidation of propofol in the presence of horseradish peroxidase and hydrogen peroxide. The fluorescence intensity ratio of GQDs (F/F 0) was positively correlated with the concentration of propofol, which ranged within 5.34-89.07 mg l-1, the limit of detection was 0.5 mg l-1 and the limit of quantity was 5.34 mg l-1. The developed fluorescence method reported in the present study is simple, sensitive, reproducible, and can serve in determining propofol contents in emulsions.