bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ketogenic Diet Therapy for Drug-Resistant Epilepsy and Cognitive Impairment in Children With Tuberous Sclerosis Complex
Authors: Fang Yu , Li Dan , Wang Man , Zhao Xia , Duan Jing , Gu Qiang , Li Baomin , Zha Jian , Mei Daoqi , Bian Guangbo , Zhang Man , Zhang Huiting , Hu Junjie , Yang Liu , Yu Lifei , Li Hua , Liao Jianxiang 
Published: May 2022

ObjectiveTuberous sclerosis complex (TSC) is a rare disease with a high risk of epilepsy and cognitive impairment in children. Ketogenic diet (KD) therapy has been consistently reported to be beneficial to TSC patients. In this study, we aimed to investigate the efficacy and safety of KD in the treatment of drug-resistant epilepsy and cognitive impairment in children with TSC.MethodsIn this multicenter study, 53 children (33 males and 20 females) with drug-resistant epilepsy or cognitive impairment caused by TSC were retrospectively recruited from 10 hospitals from January 1, 2010, to December 31, 2020. Intention-to-treat analysis was used to evaluate seizure reduction and cognition improvement as outcomes after KD therapy.ResultsOf the 53 TSC patients included, 51 failed to be seizure-free with an average of 5.0 (range, 4–6) different anti-seizure medications (ASMs), before KD therapy. Although the other two patients achieved seizure freedom before KD, they still showed psychomotor development delay and electroencephalogram (EEG) abnormalities. At 1, 3, 6, and 12 months after the KD therapy, 51 (100%), 46 (90.2%), 35 (68.6%), and 16 patients (31.4%) remained on the diet therapy, respectively. At these time points, there were 26 (51.0%), 24 (47.1%), 22 (43.1%) and 13 patients (25.5%) having ≥50% reductions in seizure, including 11 (21.6%), 12 (23.5%), 9 (17.6%) and 3 patients (5.9%) achieving seizure freedom. In addition, of 51 patients with psychomotor retardation, 36 (36 of 51, 70.6%) showed cognitive and behavioral improvements. During the KD therapy, no serious side effects occurred in any patient. The most common side effects were gastrointestinal disturbance (20 of 53, 37.7%) and hyperlipidemia (6 of 53, 11.3%). The side effects were gradually relieved after adjustment of the ketogenic ratio and symptomatic treatment.ConclusionKD is an effective and safe treatment for TSC-related drug-resistant epilepsy and cognitive impairment in children. KD can reduce seizure frequency and may potentially improve cognition and behavior.
Effects of Vascular Comorbidity on Cognition in Multiple Sclerosis Are Partially Mediated by Changes in Brain Structure
Authors: Marrie Ruth Ann , Patel Ronak , Figley Chase R. , Kornelsen Jennifer , Bolton James M. , Graff Lesley A. , Mazerolle Erin L. , Helmick Carl , Uddin Md Nasir , Figley Teresa D. , Marriott James J. , Bernstein Charles N. , Fisk John D. 
Published: May 2022

ObjectiveVascular comorbidities are associated with reduced cognitive performance and with changes in brain structure in people with multiple sclerosis (MS). Understanding causal pathways is necessary to support the design of interventions to mitigate the impacts of comorbidities, and to monitor their effectiveness. We assessed the inter-relationships among vascular comorbidity, cognition and brain structure in people with MS.MethodsAdults with neurologist-confirmed MS reported comorbidities, and underwent assessment of their blood pressure, HbA1c, and cognitive functioning (i.e., Symbol Digit Modalities Test, California Verbal Learning Test, Brief Visuospatial Memory Test-Revised, and verbal fluency). Test scores were converted to age-, sex-, and education-adjusted z-scores. Whole brain magnetic resonance imaging (MRI) was completed, from which measures of thalamic and hippocampal volumes, and mean diffusivity of gray matter and normal-appearing white matter were converted to age and sex-adjusted z-scores. Canonical correlation analysis was used to identify linear combinations of cognitive measures (cognitive variate) and MRI measures (MRI variate) that accounted for the most correlation between the cognitive and MRI measures. Regression analyses were used to test whether MRI measures mediated the relationships between the number of vascular comorbidities and cognition measures.ResultsOf 105 participants, most were women (84.8%) with a mean (SD) age of 51.8 (12.8) years and age of symptom onset of 29.4 (10.5) years. Vascular comorbidity was common, with 35.2% of participants reporting one, 15.2% reporting two, and 8.6% reporting three or more. Canonical correlation analysis of the cognitive and MRI variables identified one pair of variates (Pillai's trace = 0.45, p = 0.0035). The biggest contributors to the cognitive variate were the SDMT and CVLT-II, and to the MRI variate were gray matter MD and thalamic volume. The correlation between cognitive and MRI variates was 0.50; these variates were used in regression analyses. On regression analysis, vascular comorbidity was associated with the MRI variate, and with the cognitive variate. After adjusting for the MRI variate, vascular comorbidity was not associated with the cognitive variate.ConclusionVascular comorbidity is associated with lower cognitive function in people with MS and this association is partially mediated via changes in brain macrostructure and microstructure.
Association Between Healthy Eating Index-2015 and Kidney Stones in American Adults: A Cross-Sectional Analysis of NHANES 2007–2018
Authors: Yin Shan , Wang Jiahao , Bai Yunjin , Yang Zhenzhen , Cui Jianwei , Xiao Yunfei , Wang Jia 
Published: May 2022

PurposeTo explore the association between Healthy Eating Index (HEI)-2015 and kidney stones in an American adult population.Materials and MethodsNational Health and Nutrition Examination Survey (NHANES) datasets from 2007 to 2018 were used. Participants aged ≥ 20 years who reported kidney stone history and dietary recall were included. Weighted proportions, multivariable analysis and spline smoothing were used to evaluate the associations between HEI-2015 and nephrolithiasis by adjusting gender, age, race, poverty income ratio, body mass index, education level, marital status, smoking, alcohol intake, energy level, vigorous activity, moderate activity, and some comorbidities.ResultsTotally 30 368 American adults were included, with weighted mean age [standard deviation (SD)] of 47.69 (16.85) years. The overall mean HEI-2015 score (SD) was 50.82 (13.80). In the fully-adjusted multivariable model, HEI-2015 was negatively correlated with urolithiasis [odds ratio (OR) = 0.991; 95% confidence interval (CI) 0.988 to 0.994]. Compared with the first quartile of HEI-2015, the population in the fourth quartile of HEI-2015 had a lower prevalence of kidney stones (OR = 0.716; 95% CI 0.635 to 0.807). The association was modified by education and vigorous activity.ConclusionsHEI-2015 is inversely associated with the prevalence of kidney stones, which means better diet quality is associated with a lower risk of nephrolithiasis.
Sphingomyelin Phodiesterase Acid-Like 3A Promotes Hepatocellular Carcinoma Growth Through the Enhancer of Rudimentary Homolog
Authors: Zhang Yu , Chen Weipeng , Cheng Xin , Wang Feiran , Gao Cheng , Song Fei , Song Fengliang , Liang Xiaoliang , Fang Wanzhi , Chen Zhong 
Published: May 2022

BackgroundHepatocellular carcinoma (HCC) is one of the most common malignant tumors worldwide, with unclear pathogenesis. Sphingomyelin phodiesterase acid-like 3A (SMPDL3A) affects cell differentiation and participates in immune regulation. However, its molecular biological function in HCC has not yet been elucidated.MethodsData from 180 HCC patients were analyzed the relationship between the expression of SMPDL3A in liver cancer tissues and the prognosis of liver cancer patients. Crispr-Cas9 dual vector lentivirus was used to knock out SMPDL3A in HCC cell lines. The effects of SMPDL3A on cell viability were determined by CCK8 assay, clone formation experiment, cell cycle assay, cell scratch, TUNEL experiment and flow cytometry. Xenograft tumor assays in BALB/c nude mice confirmed that SMPDL3A promoted tumor growth and in vivo. Preliminary exploration of SMPDL3A interacting protein by mass spectrometry analysis and co-immunoprecipitation.ResultsThis study showed that the expression of SMPDL3A in HCC tissue differed from that in tumor-adjacent tissues. Moreover, the overall survival rate and tumor-free survival rate of patients with high-SMPDL3A expression were significantly lower than those with low-SMPDL3A expression. SMPDL3A expression was closely related to the level of protein induced by PIVKA-II, liver cirrhosis, tumor diameter, microvascular invasion, and Barcelona clinic liver cancer staging. Thus, SMPDL3A is an independent risk factor that affects the tumor-free survival rate and overall survival rate of HCC patients. In vitro study using Crispr-Cas9 genome editing technology revealed the knockout effect of SMPDL3A on cell proliferation, apoptosis, and migration. Cell counting kit-8 assay and clone formation experiment showed that sgSMPDL3A inhibited tumor cell proliferation and migration. Flow cytometry and TUNEL assay showed that sgSMPDL3A promoted apoptosis in tumors. Moreover, sgSMPDL3A inhibited tumor growth during subcutaneous tumor formation in nude mice. Immunohistochemistry of Ki67 and PNCA also indicated that sgSMPDL3A inhibited subcutaneous tumor proliferation in tumor-bearing nude mice. Further experiments showed that SMPDL3A interacts with the enhancer of rudimentary homolog (ERH).ConclusionsHigh-SMPDL3A expression was related to poor prognosis of patients with HCC. Knockout of SMPDL3A inhibited the proliferation and migration and accelerated the migration of HCC cells. SMPDL3A interacted with ERH to affect the tumorigenesis and progression of HCC.
What Is the Most Suitable Agent Combined With Apatinib for Transarterial Chemoembolization Treatment in Advanced Hepatocellular Carcinoma Patients? A Systematic Review and Network Meta-analysis
Authors: Hui Fuhai , Xu Chang , Xu Xiangbo , Chen Jiangxia , Geng Hefeng , Yang Chao , Zhang Yingshi 
Published: May 2022

PurposeCombined therapy with transarterial chemoembolization (TACE) and apatinib is superior in therapeutic effect compared with TACE alone in patients with hepatocellular carcinoma (HCC). To determine the most suitable agent combined with apatinib for TACE treatment, we did a systematic review and network meta-analysis.MethodsFour electronic databases were searched from inception until November 2021. Randomized controlled trials (RCTs) and retrospective studies that combined therapy of TACE and apatinib (TACE+A) compared with TACE alone were included. We performed random-effect pairwise and network meta-analyses to summarize the outcomes about efficacy and safety.ResultsForty-five original studies including 3,876 patients were included. In terms of efficacy, we evaluated treatment response, 6 months overall survival (OS), 1 year OS, 6 months progression-free survival (PFS), 1 year PFS, alphafetoprotein (AFP), matrix metalloproteinase 9 (MMP9), and vascular endothelial growth factor (VEGF). Significant differences always appear in TACE agent subgroups of adriamycin, platinum, and fluorouracil from both pairwise and network meta-analysis, while significant differences could also be found in apatinib dosage of 500 and &gt;500 mg/day subgroups and in both RCT and retrospective study subgroups. From second time network analysis, compared with TACE alone, subgroups with TACE agents of oxaliplatin, cisplatin, pirarubicin, epirubicin, and 5-fluorouracil ranked front. In addition, the safety of adriamycin, platinum, and fluorouracil subgroups is acceptable.ConclusionsIn conclusion, the most suitable agents in TACE combined with apatinib were adriamycin+platinum ± fluorouracil combination therapy.Systematic Review RegistrationThe study was registered with https://www.crd.york.ac.uk/PROSPERO/display_record.php?RecordID=311650, PROSPERO, CRD4202022311650
Case Report: Male Lobular Breast Cancer in Hereditary Cancer Syndromes
Authors: Carnevali Ileana , Tedaldi Gianluca , Pensotti Valeria , Sahnane Nora , Micello Donata , Rovera Francesca , Sessa Fausto , Tibiletti Maria Grazia 
Published: May 2022

BackgroundLobular breast carcinoma (LBC) is considered an exceptionally rare disease in men, including only 1% of all male breast malignancies. The majority of LBCs have negative immunohistochemical staining for E-cadherin (CDH1) expression, and the loss of CDH1 function was traditionally implicated in the tumorigenesis of diffuse gastric cancer as well as LBC. It is well recognized that LBC in women could be involved in both hereditary breast and ovarian cancer (HBOC) and hereditary diffuse gastric cancer (HDGC) syndromes; however, there are no data present in literature about the involvement of male LBC in these inherited conditions.MethodsBRCA1, BRCA2, and CDH1 genes were performed on DNA from peripheral blood using next-generation sequencing (NGS), Sanger sequencing, and multiplex ligation-dependent probe amplification analyses. BRCA2 and CDH1 somatic gene analyses were performed on breast tumoral DNA using the NGS sequencing approach.Results and conclusionsHere, we describe two men affected by LBC, the carriers of a pathogenic variant of BRCA2 and CDH1 genes, respectively. Our data, including somatic and germline results, demonstrate a strong relationship between male LBC and HBOC/HDGC syndromes, excluding a sporadic origin of LBC in these two patients. Male LBC could represent a sentinel cancer for inherited syndrome identification, and early identification of cancer susceptibility could improve cancer prevention both for men and women in these families. The history of the LBC patient carrier of the CDH1 variant suggests to include male LBC genetic testing criteria and male breast surveillance in HDGC guidelines.
E-Cigarette Usage and Arthritis in the United States, a Nationwide Cross-Sectional Survey
Authors: Tian Yi , Jiao Zhihua , Mao Yingying , Zhang Zhenyu 
Published: May 2022

Aim: The prevalence of the use of electronic cigarettes (e-cigarettes) has grown rapidly in the past decade in the United States. While numerous studies have demonstrated combustible cigarette is closely associated with an increased risk of arthritis diseases, little is known about the effect of e-cigarette usage on inflammatory arthritis diseases. We aimed to determinate if e-cigarette usage is associated with an increased risk of inflammatory arthritis.Methods: Data were obtained from the Behavioral Risk Factor Surveillance System, which is the largest national telephone-based survey of randomly sampled adults in the United States. A total of 924,882 participants with information on e-cigarette usage and inflammatory arthritis were included. We used multivariable logistic regression to estimate the risk of arthritis associated with e-cigarette usage.Results: Of the 924,882 participants, there were 30,569 (3.3%) current e-cigarette users, and 314,190 (25.9%) reported to have inflammatory arthritis diseases. In the fully adjusted model, we observed that the odds ratio (OR) (95% confidence interval) of inflammatory arthritis diseases was 1.81 (95% CI, 1.70-1.93) for current e-cigarette users compared with never e-cigarette users. The ORs of inflammatory arthritis diseases were 1.31 (95% CI, 1.18-1.47), and 1.55 (95% CI, 1.42-1.69) among sole e-cigarette and dual users compared with never e-cigarette users, respectively.Conclusions: This is the first study to observe a cross-sectional association between e-cigarette usage and inflammatory arthritis diseases, and the findings were consistent in both sole-e-cigarette users and dual users. Our findings provide evidence that e-cigarette usage might be an important risk factor for arthritis diseases, which may have regulatory implications for e-cigarette control.
Prediction of Aortic Stenosis Progression by 18F-FDG and 18F-NaF PET/CT in Different Aortic Valve Phenotypes
Authors: Murtazalieva Patimat , Ryzhkova Darya , Malev Eduard , Zhiduleva Ekaterina , Moiseeva Olga 
Published: May 2022

Background: Different imaging techniques, such as echocardiography (ECHO) and CT, allow to assess aortic stenosis (AS) severity and could be used to study its progression. But only PET/CT open opportunities to assess activity of valvular inflammation and calcification in vivo. The aim of this study was to assess prognostic value of valvular inflammation and calcification measured by 18F-FDG and 18F-NaF PET/CT in patients with tricuspid (TAV) and bicuspid aortic valve (BAV).Methods: The study included 71 patients aged 40–70 years with mild, moderate and severe asymptomatic calcific AS. Patients were divided into two groups according to valve morphology: with BAV and TAV. All patients underwent standard ECHO, CT calcium scoring PET/CT with 18F-NaF and 18F-FDG. All patients were evaluated during a follow-up visit with evaluation of ECHO parameters. (16.8 ± 4.2 months).Results: TAV and BAV groups were comparable in AS severity by ECHO (peak aortic jet velocity (Vmax): 2.90 [2.60; 3.50] vs. 2.96 [2.55; 3.31] m/s, p = 0.83). TBR max 18F-FDG did not vary in TAV and BAV patients (1.15 [1.06; 1.23] vs. 1.11 [1.03; 1.20], p = 0.39). Both groups did not differ in valvular calcification degree (Agatston score 1,058 [440; 1798] vs. 1,128 [533; 2,360], p = 0.55) and calcification activity assessed by 18F-NaF uptake level (TBR max 1.50 [1.30; 1.78] vs. 1.48 [1.27; 1.83], p = 0.97). 18F-NaF TBR max was associated with AS severity measured by Vmax in men and women with TAV (r = 0.54; p = 0.04 vs. r = 0.53; p = 0.03). In BAV group this relationship was true only in female patients (r = 0.1; p = 0.67 vs. r = 0.7; p = 0.0004). There was no association between Vmax and TBR max 18F-FDG was revealed in TAV and BAV groups. During follow-up period, the most important positive predictors of AS progression in TAV obtained by multinomial logistic regression analysis were Vmax, and 18F-NaF TBR. Whereas in BAV the highest predictive value showed model included age and Vmax.Conclusion:18F-NaF PET/CT may be considered as the valuable predictor for hemodynamic progression of calcific AS in case of TAV. 18F-FDG PET/CT does not play a significant role to predict the AS progression.
High-Precision Ion Beam Figuring of X-Ray Plane Mirrors for the Bendable KB Focusing System
Authors: Zhang Yunyang , Huang Qiushi , Yan Shuai , Yu Jun , Huang Handan , He Yumei , Luo Hongxin , Liu Zhi , Zhang Zhong , Wang Zhanshan 
Published: May 2022

Two trapezoidal plane mirrors of 240 mm in length were fabricated by ion beam figuring (IBF) technology for application in a bendable KB focusing system. The correction of surface height and slope errors in different spatial frequency ranges of the mirrors was studied systematically. After one to two iterations of IBF, the figure height errors of the vertical focusing mirror (VFM) and horizontal focusing mirror (HFM) were improved from 32.4 and 65.4 nm to 2.7 and 7.2 nm (RMS), respectively. If the best-fit sphere of the surface profile was subtracted, the residual two-dimensional height errors were only 1.1 and 1.2 nm (RMS). The slope errors in the low spatial frequency range were corrected much faster than the middle frequency ones (f = ∼1 mm−1), which make the low-frequency slope error much smaller. After IBF, the two-dimensional slope errors of the two mirrors calculated with a spatial interval of 1 and 10 mm were reduced to approximately 0.29 and 0.08 μrad, respectively. Full spatial frequency characterization of the VFM before and after IBF showed that the low-frequency figure errors (f &lt; 1 mm−1) were significantly reduced while the middle- and high-frequency morphologies (f &gt; 1–2 mm−1) remain almost the same as before figuring. The fabricated plane mirrors were applied in the hard X-ray micro-focusing beamline in the Shanghai Synchrotron Radiation Facility (SSRF), which realized a focal spot of 2.4 μm × 2.8 μm at 10 keV.
Computational Pressure-Fluid Dynamics Applied to Index of Microcirculatory Resistance, Predicting the Prognosis of Drug-Coated Balloons Compared With Drug-Eluting Stents in STEMI Patients
Authors: Duan Yang , Wang Yiwen , Zhang Min , Li Zhi , Chen Lei , Miao Hao , Pei Siyu , Lu Yuan , Wang Zhirong 
Published: May 2022

Background: The impairment of microvascular injury on prognosis has increasingly drawn extensive awareness along with the high morbidity and mortality of ST-segment elevation myocardial infarction (STEMI) over recent years. The prognostic significance of computational pressure-fluid dynamics applied to index of microcirculatory resistance, derived from coronary angiography (CPFD-caIMR) in microvascular injury evaluation of STEMI patients remained inconclusive.Methods: A total of 213 patients who met the inclusion criteria were selected retrospectively from 1003 STEMI patients from February 2018 to February 2020. Propensity score matching (PSM) was thereafter finished. CPFD-caIMR of all patients was obtained off-line using the software (FlashAngio, Rainmed Ltd., Suzhou, China) after PPCI. The primary endpoint was to compare the CPFD-caIMR and the incidence of major adverse cardiovascular events (MACEs) between drug-coated balloons (DCB) and drug-eluting stents (DES) groups. The correlation between CPFD-caIMR and MACEs was analyzed, and the prognosis of patients with STEMI was evaluated by CPFD-caIMR by multivariate regression analysis.Results: Totally 213 STEMI patients with successful primary percutaneous coronary intervention (PPCI) were included, of whom 84 patients accepted DCB and 129 patients accepted DES respectively. Baseline characteristics and CPFD-caIMR were comparable between DCB and DES groups after PSM (62 patients in each group). CPFD-caIMR was not significantly different between two groups (DES vs. DCB: mean difference: 2.26, 95% CI -4.05 to 8.57, p = 0.45), and so was it when re-grouped by whether CPFD-caIMR &gt; 40U or not (DES vs. DCB: 34.17% vs. 27.16%, p = 0.29). After a follow-up of 1 year, more MACEs occurred in DES group than DCB group (relative risk: 2.50, 95% CI 1.04 to 6.02, p = 0.04). The predictors of MACEs by multi-variate analysis found that, only time from symptom to balloon (p = 0.03) and time from door to balloon (p &lt; 0.01) were independent predictors of MACEs, independent of treatment with DCB or DES intervention. Furthermore, CPFD-caIMR &gt; 40U became an independent predictor of the combined events including cardiovascular deaths or heart failure readmission irrespective of PSM (odds ratio: 4.07, 95% CI: 1.06 to 7.66, p = 0.04).Conclusion: CPFD-caIMR was a promising method for prognosis, which can predict CV death or heart failure readmission in STEMI patients. DCB was a possible strategy in PPCI of STEMI patients, not inferior to DES based on microvascular injury evaluated by CPFD-caIMR.