简介：AbstractBackground:The Tubridge™ flow diverter (TFD) was recently developed in China; however, its safety and efficacy in treating large cavernous carotid artery aneurysms (LCCAs) are unclear. Our objective was to evaluate the safety and efficacy of the TFD in patients receiving TFDs to treat LCCAs (10-25 mm).Methods:Between June 2013 and May 2014, seven patients with LCCAs were enrolled in our study, and all seven patients underwent TFD implantation combined with coils.Results:Angiographic follow-up images were available for all seven patients at a median of 57.5 ± 16.7 (range, 6-69) months. Seven patients obtained favorable angiographic results defined as O'Kelly-Marotta Scale C and D. Clinical follow-up data were available for all seven patients at a median of 73.32 ± 3.6 (range, 66-78) months. No patients developed new neurological deficits. Six patients achieved a modified Rankin scale score of 0, and diplopia improved in the remaining patient.Conclusions:The results were excellent for the aneurysms treated with TFDs in our patients with LCCAs. TFDs are feasible for the treatment of LCCAs, but a multicenter, controlled clinical trial is needed to evaluate the long-term safety and efficacy of the TFD to treat LCCAs.

简介：AbstractBackground:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder associated with loss of motor neurons. Our objective was to explore the epidemiology, clinical features, and survival factors of 1809 patients with ALS.Methods:We analyzed 1809 ALS patients, who were recruited from the Peking University Third Hospital from January 2005 to December 2015. Demographic data and disease-related parameters were collected. Kaplan-Meier curves were used to compare survival time. Cox proportional hazards function and the hazard ratio were used to identify adjusted prognostic predictors.Results:The results showed that the average annual incidence in Beijing alone was 0.38 cases/100,000 person-years and the mean age of onset was 48.88 ± 11.35 (95% confidence interval [CI]: 48.17-49.85) years. The median survival time from onset to death/tracheostomy was 58.89 ± 33.03 (95% CI: 51.46-63.84) months. In the adjusted Cox proportional hazard model, age of onset, diagnosis delay, rate of disease progression (Amyotrophic Lateral Sclerosis Functional Rating Scale Revised decline [points/month]), and body mass index all had an independent effect on survival in ALS.Conclusions:Our study provides information on epidemiology, clinical features, and survival factors of patients with ALS in China. These results can be helpful in clinical practice, clinical trial design, and validation of new tools to predict disease progression.

简介：AbstractObjective:Well-defined germ-line mutations in the PTCH1 gene are associated with syndromic multiple basal cell carcinomas (BCCs). Here, we used whole exome sequencing (WES) to identify the role of patched-1 in patients with multiple, unusually large BCCs.Methods:A 72-year old patient presenting with numerous BCCs progressing to large ulcerating lesions was enrolled. WES was used to identify the pathogenic gene locus.Results:Genetic work-up by WES identified a homozygous PTCH1 nonsense mutation in the tumor tissue but not present in her blood cells or in non-lesional skin. In addition, heterozygous missense mutations were identified in three cancer-associated genes (EPHB2, RET, and GALNT12) in blood cells as well as in lesional and non-lesional skin. We also tested systemic immune therapy as a potentially beneficial approach to treat patients with numerous large BCCs on scatted areas of involvement. A rapid and sustained response to nivolumab was noted, suggesting that it is an efficacious drug for long-term therapeutic outcome.Conclusion:PTCH1, EPHB2, RET, and GALNT12 may potentially contribute to the synergistic oncogene driven malignant transformation manifesting as multiple, unusually large BCCs.

简介：AbstractIntracranial atherosclerosis (ICAS)-related large vascular occlusion (LVO) was an intractable subtype of acute ischemic stroke (AIS), which always needed rescue angioplasty and stenting and complicated the procedure of endovascular recanalization. Diagnosing ICAS-LVO accurately and early was helpful for both clinical treatment and trials. Digital subtraction angiography (DSA) was unable to provide an early and rapid diagnosis of ICAS-LVO based on current studies. A variety of pre-DSA methods had been used to distinguish ICAS-LVO with other subtypes of ischemic stroke, such as medical histories, clinical presentations, computed tomography or angiography (CT/CTA), and magnetic resonance imaging (MRI/MRA). This article briefly reviewed the status quo of the diagnosis and treatment of ICAS-LVO and summarized early diagnostic methods of ICAS-LVO from different aspects.

简介：AbstractAlthough the first-line rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone regimen (R-CHOP) substantially improved outcomes for patients with diffuse large B-cell lymphoma (DLBCL), 40% of the patients suffered from relapsed/refractory disease and had poor survival outcomes. The detailed mechanism underlying R-CHOP resistance has not been well defined. For this review, we conducted a thorough search for literature and clinical trials involving DLBCL resistance. We discussed DLBCL biology, epigenetics, and aberrant signaling of the B-cell receptor (BCR), phosphatidylinositol 3-kinase (PI3K)/Akt, nuclear factor kappa light chain enhancer of activated B-cells (NF-κB), and the Janus kinase (JAK)/signal transducer and activator of transcription 3 (STAT3) pathways as defining mechanisms of DLBCL heterogeneity and R-CHOP resistance. The cell of origin, double- or triple-hit lymphoma and double-protein-expression, clonal evolution, tumor microenvironment, and multi-drug resistance help to contextualize DLBCL resistance in an (epi)genetically and biologically comparative manner. With better understanding of the biological and molecular landscape of DLBCL, a more detailed classification system and tailored treatments will ideally become available to further improve the prognosis of DLBCL patients.

简介：AbstractBackground:Early detection of gastric cancer (GC) has been the topic of major efforts in China. This study aimed to explore the risk factors associated with GC and to provide evidence for the selection of a high-risk population of GC.Methods:Based on the cancer screening cohort of the National Cancer Screening Program in Urban China, GC patients diagnosed by endoscopy and pathological examinations constituted the case group, and controls were 1:3 matched by sex and age (±5 years) individually. The variables were selected by univariable analysis of factors such as body mass index (BMI), dietary habits, lifestyle, stomach disease history, and family history of GC; and multivariable logistic regression was used to analyze the influencing factors of GC and to calculate the odds ratio (OR) of related factors and its 95% confidence interval (CI).Results:A total of 215 GC cases and 645 matched healthy controls were included in the final analysis, with a median age of 61 years for the case and control groups. Overall analysis showed that high educational level (above primary school) (OR = 0.362, 95% CI = 0.219-0.599, P < 0.001), overweight/obesity (BMI ≥24 kg/m2; OR= 0.489, 95% CI = 0.329-0.726, P < 0.001), cigarette smoking (OR = 3.069, 95% CI = 1.700-5.540, P < 0.001), alcohol consumption (OR = 1.661, 95% CI = 1.028-2.683, P = 0.038), history of stomach disease (OR = 6.917, 95% CI = 4.594-10.416, P < 0.001), and family history of GC in first-degree relatives (OR = 4.291, 95% CI = 1.661-11.084, P = 0.003) were significantly correlated with the occurrence of GC. Subgroup analyses by age and gender indicated that GC risk was still increased in the presence of a history of stomach disease. A history of chronic gastritis, gastric ulcer, or gastric polyposis was positively associated with GC, with adjusted ORs of 4.155 (95% CI = 2.711-6.368), 1.839 (95% CI = 1.028-3.288), and 2.752 (95% CI = 1.197-6.326).Conclusions:Subjects who smoke, drink, with history of stomach disease and family history of GC in first-degree relatives are the high-risk populations for GC. Therefore, attention should be paid to these subjects for GC screening.

简介：AbstractBackground:Mastectomy techniques have been extended to nipple-sparing mastectomy (NSM). This study aimed to assess the actual application of NSM in China and identify the factors influencing postoperative complications.Methods:The clinical data of 615 patients (641 surgeries) undergoing NSM from January 1st, 2018 to December 31st, 2018 at 28 centers nationwide were retrospectively analyzed to obtain the rate of NSM and investigate factors related to NSM surgery.Results:The proportion of NSM surgery performed in this study was 2.67% (17/641). Malignant breast tumors accounted for the majority of NSM surgery (559/641, 87.2%). A total of 475 (77.3%) patients underwent NSM combined with reconstructive surgery. The rate of reconstruction decreased with age in our study, and implants were the most common option (344/641, 53.7%) in reconstruction. Radial incision was the most selected method regardless of reconstruction. However, for those who underwent reconstruction surgery, 18.4% (85/462) of cases also chose curvilinear incision, while in the simple NSM surgery group, more patients chose circumareolar incision (26/136, 19.1%). The tumor-to-nipple distance (TND) influenced postoperative complications (P = 0.004). There were no relationships between postoperative complications and tumor size, tumor location, histologic grade, molecular subtype, nipple discharge, and axillary lymph nodes.Conclusions:NSM surgery is feasible and only TND influenced postoperative complications of NSM surgery. But the proportion of NSM surgery performed is still low in nationwide centers of China. The selection criteria for appropriate surgical methods are important for NSM in clinical practice. To optimize clinical applications of NSM, further multicenter prospective randomized controlled studies are needed.Trial Registration:ChiCTR.org.cn, ChiCTR1900027423; http://www.chictr.org.cn/showprojen.aspx?proj=38739

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简介：摘 要：滑模在水电站竖井衬砌施工中应用普遍，具有成本低、效率高的特点。常规滑模施工只有一个井口，滑模受力情况较简单。乌东德出线竖井由电梯井、电缆竖井、通风竖井、母线竖井，以及过道、前厅等组成，局部结构空间狭小，单层预埋件设施及门窗孔洞多，结构极其复杂，其中第一段高程987.00m～854.08m，竖井高度132.92m；第二段高程1144.70m～987.00m，竖井高度157.7m。滑模施工具有高差大、小井多且不均匀布置、井间作业空间狭小、竖井型体精度要求高等特点。本文针对以上特点，重点研究在复杂断面不均匀受力条件下的滑模设计和纠偏措施，并成功应用于乌东德地下电站出线竖井，进度、质量均满足要求，可为类似项目提供借鉴和参考。