bluuebunny/crossref_metadata_2025_split huggingface dataset:
A phase II, multicenter, open-label trial to evaluate the safety and efficacy of ISU303 (Agalsidase beta) in patients with Fabry disease
Authors: Hwang Soojin , Lee Beom Hee , Kim Woo-Shik , Kim Dae-Seong , Cheon Chong Kun , Lee Chang Hwa , Choi Yunha , Choi Jin-Ho , Kim Ja Hye , Yoo Han-Wook 
Published: September 2022

Background:
Fabry disease (FD) is caused by a deficiency in the activity of the lysosomal enzyme, α-galactosidase A (α-Gal A), which leads to globotriaosylceramide (Gb3) deposition in multiple tissues. The current management of FD is enzyme replacement therapy (ERT). We report on the efficacy and safety of a new agalsidase beta, ISU303, in FD.

Methods:
Ten patients (7 males, 3 females) were enrolled and administered a 1 mg/kg dose of ISU303, every other week for 6 months. The primary endpoint was the normalization of plasma Gb3 level. The secondary endpoints were the changes from baseline in urine Gb3 and the plasma and urine lyso-globotriaosylsphingosine (lyso-Gb3) level. Echocardiography, renal function test, and pain-related quality of life were also assessed before and after administration. Safety evaluation was performed including vital signs, laboratory tests, electrocardiograms, antibody screening tests, and adverse events at each visit.

Results:
At 22 weeks of treatment, plasma and urine Gb3 level decreased by a mean of 4.01 ± 1.29 μg/mL (range 2.50–5.70) (P = .005) and 1.12 ± 1.98 μg/mg Cr. (range 0.04–5.65) (P = .017), respectively. However, no significant difference was observed in plasma and urine lyso-Gb3 levels. Echocardiography also was not changed. Renal function and pain-related quality of life showed improvements, but there was no clinical significance. No severe adverse events were observed. Only 1 patient developed an anti-drug antibody without neutralizing activity during the trial.

Conclusion:
This study showed the efficacy and safety of ISU303. Treatment with ISU303 significantly resulted in plasma and urine Gb3 decrease in patients with FD. These results suggest that ISU303 is safe and effective and can alternative ERT for FD.
Association between the LINC00673 rs11655237 C> T polymorphisms with cancer risk in the Chinese population: A meta-analysis
Authors: Zhang Hongyu , Wu Baixiu , Liang Ka , Ke Liuhua , Ma Xingxuan , Luo Changliu , He You 
Published: September 2022

Objectives:
The present study aimed to conduct a meta-analysis of previously published studies in order to clarify the association of long noncoding RNA (lncRNAs) LINC00673 rs11655237 C> T polymorphism with cancer risk.

Design:
Systematic review and meta-analysis.

Setting:
Electronic databases of PubMed, EMBASE, Web of Science, Cochrane Library, Chinese National Knowledge Infrastructure, and Wanfang Database were used to search relevant studies. Studies published up to October 20, 2019 were included. The included studies were assessed in the following genetic model: allelic model, homozygote model, Heterozygote model, dominant model, recessive model. Data syntheses were conducted using STATA 12.0.

Participants:
Participants with various types cancers were included.

Primary and secondary outcome measures:
Odds ratio (ORs) and 95% confidence interval (CIs) were calculated to assess the risk of tumor.

Results:
Seven articles including 7 case-control studies, 7423 cases and 11,049 controls were adopted for meta-analysis. Our result demonstrated that LINC00673 rs11655237 C> T was related to the cancer among all model including allelic model (T vs C: pooled OR = 1.24, 95% CI = 1.16–1.41, P < .001), homozygous model (TT vs CC: pooled OR=1.54, 95% CI = 1.36–1.76, P < .001), heterozygous model (CT vs CC: pooled OR=1.24, 95% CI = 1.16–1.32, P < .001), dominant model (CT + TT vs CC: pooled OR=1.28, 95% CI = 1.20–1.36, P < .001) and recessive model (TT vs CT+ CC: pooled OR=1.42, 95% CI = 1.25–1.61, P < .001). Subgroup analysis also demonstrated that polymorphisms at this site also increased the risk of neuroblastoma.

Conclusions:
Our results find that rs11655237 contributed to occurrence of cancer in all models in Chinese population.
Analysis of risk factors for bile duct injury in laparoscopic cholecystectomy in China: A systematic review and meta-analysis
Authors: Yang Shaohua , Hu Sheng , Gu Xiaohui , Zhang Xiaowen 
Published: September 2022

Background:
To explore the risk factors of bile duct injury in laparoscopic cholecystectomy (LC) in China through meta-analysis.

Methods:
The study commenced with a search and selection of case–control studies on the risk factors for bile duct injury in LC in China using the following databases: PubMed, EMBASE, ScienceNet.cn, CNKI, Wanfang Data, and VIP. Data were extracted from the collected documents independently by 2 researchers, following which a meta-analysis of these data was performed using Revman 5.3.

Results:
The compilation of all data from a total of 19 case–control studies revealed that among 41,044 patients, 458 patients experienced bile duct injury in LC, accounting for the incidence rate of 1.12% for bile duct injury. The revealed risk factors for bile duct injury were age (≥40 years) (odds ratio [OR] = 6.23, 95% CI [95% confidence interval]: 3.42–11.33, P < .001), abnormal preoperative liver function (OR = 2.01, 95% CI: 1.50–2.70, P < .001), acute and subacute inflammation of gallbladder (OR = 8.35, 95% CI: 5.32–13.10, P < .001; OR = 4.26, 95% CI: 2.73–6.65, P < .001), thickening of gallbladder wall (≥4 mm) (OR = 3.18, 95% CI: 2.34–4.34, P < .001), cholecystolithiasis complicated with effusion (OR = 3.05, 95% CI: 1.39–6.71, P = .006), and the anatomic variations of the gallbladder triangle (OR = 11.82, 95% CI: 6.32–22.09, P < .001). However, the factors of gender and overweight (body mass index ≥ 25 kg/m2) were not significantly correlated with bile duct injury in LC.

Conclusions:
In the present study, age (≥40 years), abnormal preoperative liver function, gallbladder wall thickening, acute and subacute inflammation of the gallbladder, cholecystolithiasis complicated with effusion, and anatomic variations of the gallbladder triangle were found to be closely associated with bile duct injury in LC.
Effect of real-time feedback on patient’s outcomes and survival after cardiac arrest: A systematic review and meta-analysis
Authors: Lv Guang Wei , Hu Qing Chang , Zhang Meng , Feng Shun Yi , Li Yong , Zhang Yi , Zhang Yuan Yuan , Wang Wen Jie 
Published: September 2022

Purpose:
This study investigated the effect of real-time feedback on the restoration of spontaneous circulation, survival to hospital discharge, and favorable functional outcomes after hospital discharge.

Methods:
PubMed, ScienceDirect, and China National Knowledge Infrastructure databases were searched to screen the relevant studies up to June 2020. Fixed-effects or random-effects model were used to calculate the pooled estimates of relative ratios (RRs) with 95% confidence intervals (CIs).

Results:
Ten relevant articles on 4281 cardiac arrest cases were identified. The pooled analyses indicated that real-time feedback did not improve restoration of spontaneous circulation (RR: 1.13, 95% CI: 0.92–1.37, and P = .24; I
2 = 81%; P < .001), survival to hospital discharge (RR: 1.27, 95% CI: 0.90–1.79, and P = .18; I
2 = 74%; P < .001), and favorable neurological outcomes after hospital discharge (RR: 1.09, 95% CI: 0.87–1.38; P = .45; I
2 = 16%; P = .31). The predefined subgroup analysis showed that the sample size and arrest location may be the origin of heterogeneity. Begg’s and Egger’s tests showed no publication bias, and sensitivity analysis indicated that the results were stable.

Conclusion:
The meta-analysis had shown that the implementation of real-time audiovisual feedback was not associated with improved restoration of spontaneous circulation, increased survival, and favorable functional outcomes after hospital discharge.
Clinicopathological and prognostic significance of SOX9 expression in gastric cancer patients: A meta-analysis
Authors: Wang Qian , Chen Hao , Yang Congying , Liu Yi , Li Feng , Zhang Chunfang 
Published: September 2022

Background:
SOX9 is a potential prognostic marker in gastric cancer (GC) patients. This meta-analysis aimed to highlight the clinicopathological and prognostic implications of SOX9 expression in GC patients.

Methods:
A systematic literature search was conducted to identify relevant studies by the electronic literature databases (PubMed, Web of Science, EMBASE and Chinese databases). Review Manager version 5.4 was employed to evaluate the pooled odds ratio (OR) or hazard ratio (HR) with 95% confidence intervals (CIs).

Results:
Seventeen studies with a total of 2893 GC patients were enrolled in this meta-analysis. The analysis with ten articles clarified that higher expression of SOX9 was observed in GC cancers than that of normal gastric samples (OR = 16.26; 95% CI: 8.16 to 32.42; P < .00001). Consequently, the results also showed that SOX9 expression was closely associated with age (OR = 1.34; 95% CI: 1.04–1.72; P = .03), tumor size (OR = 0.67; 95% CI: 0.49–0.91; P = .01), histological differentiation (OR = 0.62; 95% CI: 0.36–1.06; P = .002), tumor stage (OR = 0.48; 95% CI: 0.20–1.12; P = .04), lymph node metastasis (OR = 0.36; 95% CI: 0.19–0.67; P = .0010) and advanced TNM stage (OR = 0.46; 95% CI: 0.30–0.70; P = .0003), but not significantly related to gender, distant metastasis and vascular invasion. Furthermore, high SOX9 expression could significantly indicate poorer overall survival (OS) (HR = 1.40; 95% CI: 1.14–1.72; P = .001).

Conclusion:
SOX9 overexpression might be related to poor prognosis and could serve as a potential predictive marker of poor clinicopathological prognosis factor in GC patients.
Using the alluvial plot to visualize the network characteristics of 100 top-cited articles on attention-deficit/hyperactivity disorder (ADHD) since 2011: Bibliometric analysis
Authors: Tsai Ya-Ching , Chien Tsair-Wei , Wu Jian-Wei , Lin Chien-Ho 
Published: September 2022

Background:
Attention-deficit/hyperactivity disorder (ADHD) is a common neuro developmental disorder that affects children and adolescents. It is estimated that the prevalence of ADHD is 7.2% throughout the world. There have been a number of articles published in the literature related to ADHD. However, it remains unclear which countries, journals, subject categories, and articles have the greatest influence. The purpose of this study was to display influential entities in 100 top-cited ADHD-related articles (T100ADHD) on an alluvial plot and apply alluvial to better understand the network characteristics of T100ADHD across entities.

Methods:
Using the PubMed and Web of Science (WoS) databases, T100ADHD data since 2011 were downloaded. The dominant entities were compared using alluvial plots based on citation analysis. Based on medical subject headings (MeSH terms) and research areas extracted from PubMed and WoS, social network analysis (SNA) was performed to classify subject categories. To examine the difference in article citations among subject categories and the predictive power of MeSH terms on article citations in T100ADHD, one-way analysis of variance and regression analysis were used.

Results:
The top 3 countries (the United States, the United Kingdom, and the Netherlands) accounted for 75% of T100ADHD. The most citations per article were earned by Brazil (=415.33). The overall impact factor (IF = citations per 100) of the T100ADHD series is 188.24. The most cited article was written by Polanczyk et al from Brazil, with 772 citations since 2014. The majority of the articles were published and cited in Biol Psychiatry (13%; IF = 174.15). The SNA was used to categorize 6 subject areas. On the alluvial plots, T100ADHD’s network characteristics were successfully displayed. There was no difference in article citations among subject categories (F = 1.19, P = .320). The most frequently occurring MeSH terms were physiopathology, diagnosis, and epidemiology. A significant correlation was observed between MeSH terms and the number of article citations (F = 25.36; P < .001).

Conclusion:
Drawing the alluvial plot to display network characteristics in T100ADHD was a breakthrough. Article subject categories can be classified using MeSH terms to predict T100ADHD citations. Bibliometric analyses of 100 top-cited articles can be conducted in the future.
Nodular fasciitis arising from the buccal region after segmentectomy with rapid growth mimicking postirradiation myxofibrosarcoma: A case report
Authors: Yoshizawa Kunio , Ishii Hiroki , Sakurai Daiju , Inoue Tomohiro , Ueki Koichiro 
Published: September 2022

Rationale:
Nodular fasciitis (NF) can be misdiagnosed as a sarcoma because of its rapid growth and pathological features, such as nuclear atypia and mitoses.

Patient concerns:
We present a rare case of a 72-year-old Japanese man who developed NF with suspected postirradiation myxofibrosarcoma (MFS) after segmentectomy for left-sided osteoradionecrosis (ORN) of the mandible.

Diagnosis:
A mass appeared in the intraoral postoperative wound 1 year after left-sided mandibular segmentectomy and showed rapid growth, reaching a size of 50 mm within 2 months. Incisional biopsy revealed strongly Ki-67-positive spindle-shaped cells with large irregular nuclei and a diagnosis of postirradiation MFS.

Interventions:
The patient was diagnosed with oropharyngeal cancer (T4aN2bM0) and underwent surgical resection of primary oropharyngeal squamous cell carcinoma with selective neck dissection and reconstruction with a rectus abdominis musculocutaneous flap at the age of 57 years, followed by postoperative 66 Gy of radiotherapy combined with cisplatin administration. No recurrent or metastatic lesions of oropharyngeal squamous cell carcinoma have been detected for > 10 years. However, the ORN of the jaw worsened, and the patient underwent sequestrectomy 3 times on the right side of the mandible, followed by a left-sided segmentectomy at the age of 72 years. One year after segmentectomy, a 10-mm mass with soft-to-firm consistency appeared in the buccal mucosa of the wound and grew rapidly. An incisional biopsy revealed MFS. Complete resection under general anesthesia was immediately performed.

Outcomes:
The histopathological diagnosis of the excised specimen was NF without any malignant findings. Two years after surgery, there was no evidence of recurrence or metastasis.

Lessons:
NF grows rapidly and has pathological features similar to sarcoma, making differential diagnosis difficult at the time of incisional biopsy. Further studies should be conducted to determine the clinical and pathological features of this tumor.
Efficacy of decitabine combined with allogeneic hematopoietic stem cell transplantation in the treatment of recurrent and refractory acute myeloid leukemia (AML): A systematic review and meta-analysis
Authors: Zhang Donghui , Chen Jiahui 
Published: September 2022

Background:
This analysis aimed to assess the effect of decitabine combined with allogeneic hematopoietic stem cell transplantation (allo-HSCT) in treating recurrent and refractory acute myeloid leukemia.

Method:
The present analysis was carried out according to the principles of Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline statement. Web of Science, Embase, PubMed, The Cochrane Library, CNKI, VIP, and WanFang Data databases were searched for trials published from their corresponding inception to September 13, 2021. Retrospective research or published randomized controlled trials in Chinese or English were ruled out. The methodological quality of the included studies was assessed using the Physiotherapy Evidence Database scale. Mean differences with 95% confidence intervals were used to analyze continuous data. The I
2 test was used to determine heterogeneity, and the meta-analysis was conducted using Revman 5.4.

Results:
Eight studies including 795 participants in total were identified. Decitabine and allo-HSCT showed significant reductions in recurrence after transplantation (odds ratio [OR] = 0.29, 95% confidence interval [CI] (0.17, 0.50), P < .00001), leukemia-free survival (OR = 2.17, 95% CI (1.47, 3.21), P < .0001), graft related death (OR = 0.50, 95% CI (0.25, 0.98), P = .04), and significant improvements in complete remission (OR = 0.39, 95% CI = 0.23–0.68, P = .0007) and partial remission (OR = 0.46, 95%CI = 0.27–0.78, P = .004). The median follow-up time, acute graft-versus-host disease, and no remission had no significant difference between treatment and control groups (the median follow-up time: OR = −1.76, 95% CI (−6.28, 2.76), P = .45; acute graft-versus-host disease: OR = 0.72, 95% CI (0.50, 1.03), P = .08; no remission: OR = 3.19, 95%CI = 2.06–4.94, P = .05). Overall, the magnitude of the effect was found to be in the small to moderate range.

Conclusion:
Decitabine combined with allo-HSCT can obtain lower recurrence risk and longer disease-free survival time, and improve the prognosis of patients. The safety is relatively stable. Due to the varying quality level of the included studies, the validation of multiple high-quality studies still needs improvement.
Great prognosis of concurrent anti-GBM disease and IgA nephropathy in a young woman: A case report
Authors: Shaojie Fu , Sensen Su , Jingda Huang , Luyu Wang , Fei Zhang , Jinyu Yu , Zhonggao Xu , Hao Wu 
Published: September 2022

Rationale:
The causal relationship between anti-glomerular basement membrane (anti-GBM) disease and immunoglobulin A (IgA) nephropathy is still unclear and cases of concurrent anti-GBM disease and IgA nephropathy are very rare, especially with a good prognosis and long-term follow-up. Here, we report a case of concurrent anti-GBM disease and IgA nephropathy. By using corticosteroids and cyclophosphamide in combination with plasmapheresis, the patient achieved a very good prognosis with complete normalization of renal function and complete disappearance of hematuria and proteinuria at the subsequent follow-up. To our knowledge, no previous case with such a long follow-up and such a good prognosis have been reported.

Patient concerns:
This case report describes a 26-year-old Chinese woman who presented with fever as the initial symptom, followed by dysmorphic hematuria, overt proteinuria and rapidly worsening renal function. Before admission, the patient received symptomatic supportive treatment such as intravenous albumin infusion, improvement of circulation, but the symptoms were not significantly improved.

Diagnosis:
Per the results of kidney biopsy, the patient was diagnosed with crescentic glomerulonephritis and anti-GBM disease with IgA nephropathy.

Interventions:
The key to obtain a good prognosis was the early application of corticosteroids and cyclophosphamide in combination with plasmapheresis to make the anti-GBM antibody turn negative quickly.

Outcomes:
After 2 weeks of therapy, the patients’ anti-GBM antibody turned negative and serum creatinine improved to a normal range. After 10 months, the patient’s proteinuria level reached complete remission. After 12 months, the patient’s hematuria had disappeared completely.

Lessons:
This case provides experience in the treatment of concurrent anti-GBM disease and IgA nephropathy and highlights the importance of early application of plasmapheresis and immunosuppressive therapy to obtain a good prognosis.
Danshen decoction in the treatment of heart failure: A systematic review and meta-analysis protocol of randomized controlled trials
Authors: Liu Mengnan , Wang Raoqiong , Li Ziyi , Mazhar Maryam , Luo Gang , Yang Sijin 
Published: September 2022

Background:
Heart failure (HF) is considered the clinical endpoint of all cardiovascular diseases (CVDs). Although a large number of HF drugs and therapies have been developed, it is still need to find therapies with better clinical efficacy and fewer side effects. The purpose of this systematic evaluation is to assess the efficacy and safety of Danshen decoction on HF and the improvement of cardiac function (CF).

Methods:
Four databases will be searched to identify any eligible studies, and this protocol does not require ethics committee review as the research is based on published articles. There are no restrictions set for the language, publication date, or status of the study. The clinical effective rate (CER) of HF treatment is considered to be the main result. CF, various serum inflammatory factors, and adverse events were defined as secondary outcomes. When more than 1 article is used to study the changes and results of the same index, we will conduct a meta-analysis. If the heterogeneity is not statistically significant (P > .10 or I
2 < 50%), a fixed-effect model will be established to estimate the overall intervention effect. Otherwise, random effect models will be used to provide more conservative results.

Results:
The results of this meta-analysis will be submitted to a peer-reviewed journal for publication.

Conclusion:
This study will provide reliable evidence-based basis for the clinical application of Danshen decoction in the treatment of HF.
Global trends of researches on radioactive enteritis: A bibliometric and visualization study
Authors: Chen Daman , Zhao Kaibo , Guo Yongqi , Dong Mingxin , Cai Jiaxi , Zhou Yingjia , Wen Weibo , Shen Hongmei 
Published: September 2022

Background:
Radiation enteritis (RE) caused by radiation therapy, can seriously affect human health. Recently, studies on RE have been growing rapidly, but there are no bibliometric studies on RE. This study aims to explore the development trends and research hotspots of RE.

Methods:
Academic papers on the Web of Science were retrieved on the topic of “radioactive enteritis” from the establishment of the database to December 2020. Countries, institutions, and subjects selected in this field were visualized using Citespace, HistCite, and Vosviewer. The annual trends in publications, distribution, co-authorship status, and research hotspots were analyzed.

Results:
The authors ranked first in terms of publication amount were Delaney, Francois, Milliat, and Vozenin-Brotons. The United States had the highest number of posts, followed by China, France, the United Kingdom, and Spain.

Conclusion:
Future research in the field of RE will focus on double-blind clinical trials of RE, and the related mechanisms, such as oxidative stress and apoptosis.
Comparing the diagnostic accuracy of PET and CMR for the measurement of left ventricular volumes and ejection fraction: a system review and meta-analysis
Authors: Ye Yong , Yang Ying , Gong Jie , Shao Wen-wen , Yu Cheng-xin 
Published: September 2022

Background
Cardiac magnetic resonance (CMR) has been recognized as the gold standard for the evaluation of left ventricular (LV) function. Cardiac gated PET allows the simultaneous assessment of LV function with the evaluation of myocardial perfusion and metabolism. But the correlations between PET and CMR remain controversial.

Methods
We conducted a systematic electronic search of PubMed, Embase and the Cochrane Library. Forest plot, spearman correlation analysis and Bland-Altman analysis were used to evaluate the correlations between PET and CMR.

Results
Pooled analysis of 13 studies showed that PET underestimated left ventricular end-diastolic volumes (LVEDV) [mean difference (MD), −15.30; 95% confidence interval (CI), −23.10 to −7.50; P < 0.001] and left ventricular end-systolic volumes (LVESV) (MD, −6.20; 95% CI, −12.58 to 0.17; P = 0.06) but not left ventricular ejection fraction (LVEF) (MD, −0.35; 95% CI, −1.75 to 1.06; P = 0.63). Overall, there were very good correlations between PET and CMR measurements for LVEDV (r, 0.897), LVESV (r, 0.924) and LVEF (r, 0.898). Subgroup analysis indicated that LVEDV ≥180 ml and LVEF <40% reduced the accuracy of PET, especially the measurement of LVEF (r, LVEDV ≥180 vs. r, LVEDV<180: 0.821 vs. 0.944; r, LVEF<40% vs. r, LVEF ≥40%: 0.784 vs. 0.901).

Conclusions
Correlations between PET and CMR measurements of LVEDV, LVESV and LVEF were excellent, but these two methods could not be used interchangeably for accurate measurements of LV volume and LVEF in patients with significantly increased LV volume and decreased LVEF.