We carefully read the recent study by Liu et al on the effectiveness of tofacitinib for refractory Behcet’s syndrome (BS).1 In total, 13 patients with BS were enrolled, including 5 cases with vascular involvement, 7 with gastrointestinal (GI) ulcers and one suffering polyarthritis. As an add-on approach, tofacitinib improved the outcomes in all patients with vascular lesions, but unchanged or worsened in 5/7 GI involvement cases. Their observation give support to the hypothesis that BS is not an independent disease, but a heterogeneous and multisystemic complex syndrome.2 Different major organ involvements, vascular and GI involvement could link to its unique underlying immunopathological mechanisms, which led to radical treatment responses.3 In their study, the only manifestation of ocular involvement was scleritis in one patient with polyarthritis, which is not a specific ophthalmological feature of BS. Notably, uveitis is one of the specific features of ocular involvement in patients with BS,4 which may lead to visual loss in up to 25% of patients in spite of biological agents.5 Here, we reported the efficacy and safety profile of …
Quality of life (QoL) was worse in Parkinson's disease patients with mild cognitive impairment (PD-MCI) or dementia (PDD) than PD patients with normal cognition (PD-NC). The aim of this study was to investigate and compare the potential heterogeneous determinants of QoL in PD patients with different cognitive statuses.We recruited 600 PD patients, including 185 PD-NC patients, 336 PD-MCI patients and 79 PDD patients, in this cross-sectional study. All patients completed the QoL assessment by the 39-item Parkinson's Disease Questionnaire (PDQ-39), as well as clinical evaluations and neuropsychological tests. The determinants of the QoL were analyzed by multiple stepwise regression analysis.QoL was more impaired across the three groups (PD-NC < PD-MCI < PDD). The Unified Parkinson's Disease Rating Scale part III (UPDRS-III) score, Geriatric Depression Rating Scale (GDS) score and daily levodopa equivalent dose (LED) were independent variables of PDQ-39 in PD-NC patients. The GDS score, disease duration, UPDRS-III score, Epworth Sleepiness Score (ESS) and sex were independent variables of PDQ-39 in PD-MCI patients. The GDS score and disease duration were independent variables of PDQ-39 in PDD patients.The determinants of QoL in PD-NC, PD-MCI and PDD patients were heterogeneous. Motor function was considered to be the most crucial determinant for QoL in PD-NC, while depression was indicated to be the most vital determinant for PD-MCI and PDD. For QoL improvement, clinicians might need to focus more on motor function in PD-NC patients and on depression in PD-MCI and PDD patients.
Abstract Background T lymphocytes are one of the major components of the adaptive immunity in Behçet’s syndrome (BS) pathology. To further understand of the role of T cells in Behçet’s syndrome (BS), we explore disease related T cell receptor (TCR) repertoires. Methods We performed a bulk sequencing of the TCR beta chain (TRB) of peripheral T-cells collected from 45 BS patients with panuveitis, intestinal involvement, and cardiovascular lesions and 10 cases of healthy controls (HC). Data analysis included peptide sequences, diversity analysis, variable (V)-joining (J) gene usage and K-nearest neighbor algorithm. Results We found significant differences in V, J and V-J combinations between the BS patients and HC. The decrease in TCR clone diversity indicates clonal expansion in BS. Although no significant difference in TCR clone diversity was observed among patient subgroups, the patients with panuveitis displayed the highest heterogeneous TCR distribution. In addition, a set of V-J genes could effectively discriminate between active and inactive BS patients with an area under the receiver operating characteristics (ROC) curve of 0.88 (95% CI: 0.77-1.00). Conclusions Clonal T cell expansion has been observed in BS patients. TCR profiles could help in the discrimination between active and inactive BS.
Objective To evaluate the feasibility and efficiency of transthoracic echocardiography(TTE) combined with real-time three dimensional echocardiography(RT-3DE)in guiding transcatheter closure of atrial septal defect(TCASD)without X-ray.Methods Eleven patients with atrial septal defects(ASD)underwent the procedure of TCASD guided by TTE combined with RT-3DE.The position of the catheter and transporting sheath,the location and deploying of ASD occluder(ASO)were monitored by muhisection echoscan.especially in RT-3DE.The results were compared with those guided by conventional method(TTE and X-ray).Results All the operations were held successfully.The complications of cardiac tamponade,shedding of occluder,residual shunt,atrioventricular valve regurgitation,embolism and arrhythmia were not observed.Compared with the conventional procedure of the control group,the operation time was significantly prolonged[(65.76±14.15)min versus(45.50±20.88)min,P＜0.000)].Conclusions TCASD was tested to be safe and efficient guided by TTE combined RT-3DE without X-ray,especially for children and pregnant women.It may be expected used widely in the future independently from X-ray.
Key words:
Echocardiography; Heart septal defects,atrial
A novel self-powered biosensor has been developed for the detection of chloramphenicol (CAP) based on difunctional triple helix molecular switch (THMS)-mediated DNA walkers. The biosensor utilizes the CAP aptamer as the recognition element, a DNA walker and capacitor as dual signal amplification strategies, and a digital multimeter (DMM) as the data readout equipment. In the presence of the target, the CAP aptamer in THMS specifically binds with CAP to release a signal transduction probe (STP) and opens the H1 hairpin structure in the biocathode to trigger the DNA walker and form a double-stranded DNA structure. Then, [Ru(NH
Wheat-dependent exercise-induced anaphylaxis (WDEIA) is a severe allergic condition in which wheat ingestion together followed by physical exercise induces anaphylaxis. For patients with WDEIA, omega-5 gliadin is considered to be one of the major allergens.To analyse the clinical features and allergen spectrum of WDEIA and to investigate the relationship between WDEIA and serum levels of platelet-activating factor (PAF), interleukin (IL)-9 and IL-33.Medical histories and conditions of WDEIA cases were collected and summarized, with allergen tests of wheat proteins measured at the same visit. Of the 33 patients enrolled, 13 also had serum levels of PAF, IL-9 and IL-33 measured. The healthy control (HC) group consisted of 13 healthy individuals, who also underwent both the wheat-protein allergen tests and the inflammatory-mediator tests.All patients experienced severe allergic reaction during exercise after wheat ingestion. Manifestations of WDEIA included facial oedema, generalized urticaria and respiratory symptoms. Unconsciousness was also observed in 21 cases. In the patient group, 57.6% were confirmed as hypersensitive to glyceraldehyde-3-phosphate dehydrogenase (GAPDH), while 54.5% were allergic to omega-5 gliadin. PAF concentration was significantly higher in patients with WDEIA compared with HCs, whereas there was no significant difference in IL-9 or IL-33 between the two groups.WDEIA is a rare type of anaphylaxis. GAPDH and omega-5 gliadin may be the most common allergy-causing wheat proteins for Chinese people. PAF may be associated with the onset and development of WDEIA.
// Rong-Si Wu 1, 3, * , Jing-Jing Hong 1, 3, * , Jia-Fa Wu 1, 4, * , Shen Yan 1, 3, * , Di Wu 1, 3 , Na Liu 2 , Qing-Feng Liu 1, 3 , Qiu-Wan Wu 2 , Yuan-Yuan Xie 1, 3 , Yun-Jia Liu 1, 3 , Zhong-Zheng Zheng 1, 3 , Err-Cheng Chan 5 , Zhi-Ming Zhang 2 and Bo-An Li 1, 3 1 State Key Laboratory of Cellular Stress Biology, Innovation Center for Cell Signaling Network, School of Life Sciences, Xiamen University, Xiamen, Fujian, China 2 The First Affiliated Hospital, Xiamen University, Xiamen, Fujian, China 3 Engineering Research Center of Molecular Diagnostics, Ministry of Education, School of Life Sciences, Xiamen University, Xiamen, Fujian, China 4 College of Food and Bioengineering, Henan University of Science and Technology, Luoyang, China 5 Department of Medical Biotechnology and Laboratory Science, Chang Gung University, Taoyuan, Taiwan * These authors have contributed equally to this work Correspondence to: Bo-An Li, email: bali@xmu.edu.cn Zhi-Ming Zhang, email: zhangzhiming164@yahoo.cn Err-Cheng Chan, email: chanec@mail.cgu.edu.tw Keywords: mammary tumor, EMT, OVOL2, TGF-β signaling, Smad4 Received: August 23, 2016 Accepted: March 09, 2017 Published: April 11, 2017 ABSTRACT Great progress has been achieved in the study of the role of TGF-β signaling in triggering epithelial-mesenchymal transition (EMT) in a variety of cancers; however, the regulation of TGF-β signaling during EMT in mammary tumor metastasis has not been completely defined. In the present study, we demonstrated that OVOL2, a zinc finger transcription factor, inhibits TGF-β signaling-induced EMT in mouse and human mammary tumor cells, as well as in mouse tumor models. Data from the Oncomine databases indicated a strong negative relationship between OVOL2 expression and breast cancer progression. Moreover, our experiments revealed that OVOL2 inhibits TGF-β signaling at multiple levels, including inhibiting Smad4 mRNA expression and inducing Smad7 mRNA expression, blocking the binding between Smad4 and target DNA, and interfering with complex formation between Smad4 and Smad2/3. These findings reveal a novel mechanism that controls the TGF-β signaling output level in vitro and in vivo . The modulation of these molecular processes may represent a strategy for inhibiting breast cancer invasion by restoring OVOL2 expression.
Abstract Background Behçet’s syndrome (BS) is a rare variant vasculitis which can involve the eyes and gastrointestinal systems. However, ocular involvement rarely overlaps with intestinal lesions. This study aimed to compare the clinical characteristics and laboratory parameters of ocular BS and intestinal BS patients in China and analyze the differences between two key phenotypes to verify the heterogeneous conditions in BS patients. Methods A retrospective analysis was used to collect the demographic data, clinical characteristics, endoscopic findings, and laboratory parameters from 135 ocular BS and 174 intestinal BS patients. The Mann-Whitney U test and Pearson chi-square or continuity correction was used to analyze the differences between two groups. Results Among 916 BS patients enrolled in this study, ocular BS and intestinal BS accounted for 14.74% (135 cases) and 19.00% (174 cases), respectively. Ocular and intestinal involvements overlapped in only 7 cases (0.76%). Male gender (74.8% vs. 51.1%, P =0.00), erythema nodosum (45.9% vs. 32.2%, P =0.01), and vascular involvement (6.7% vs. 1.7%, P =0.03) were more frequent in the ocular BS group compared with the intestinal BS group. On the contrary, hematologic involvement (7.5% vs. 0.0%, P =0.00) and fever (17.8% vs. 4.4%, P =0.00) were more frequent in the intestinal BS group compared with the ocular BS group. Additionally, the inflammation markers including ESR [26.5 (16.0–41.5) vs. 9.0 (5.0–15.0) mm/H, P =0.00], CRP [14.8 (4.8–33.0) vs. 4.1 (1.6–8.3) mg/L, P =0.00], serum amyloid A [27.4 (10.8–92.3) vs. 11.3 (6.0–24.0) mg/L, P =0.00], and interleukin 6 [8.4 (1.7–18.7) vs. 1.7 (1.5–3.2) pg/mL, P =0.00] were higher in the intestinal BS group than those in the ocular BS group, respectively. Conclusions Ocular BS was more prevalent in male patients and more likely to manifest with erythema nodosum and vascular involvement, while intestinal BS tends to have fever and hematologic disorders with higher inflammation markers. Ocular BS and intestinal BS are two distinct clinical phenotypes and very rarely overlapped.
Patients undergoing anti-tumor necrosis factor (TNF) treatment are more susceptible to latent tuberculosis infection (LTBI). The aim of the current study was to determine the rate of active tuberculosis (TB) in patients with Behçet's disease (BD) pre- and post-anti-TNF treatment and to evaluate the long-term efficacy of LTBI screening as primary prophylaxis in China.This retrospective study included BD patients eligible for anti-TNF therapy at a single institution in Fudan University, China. On the basis of the results of T-SPOT.TB assay, chest radiograph, and history of exposure to TB, patients were screened and regularly followed up at 3-months interval.Eighty-nine BD patients with mean disease duration of 87.5 ± 86.1 months were included. Their median duration of anti-TNF therapy was 10.6 months; 51 patients were treated with Infliximab, 38 with Etanercept, and four with Adalimumab. While 84 patients received a consecutive single anti-TNF drug therapy, five patients switched to a second drug. Twelve patients demonstrated positive results in LTBI screening: three had history of TB exposure and nine were solely T-SPOT.TB-positive patients. Before anti-TNF treatment, LTBI treatment was initiated in 11 patients, and one patient refused treatment. With a median follow-up period of 27.9 months, we observed only one case (1.1%) of intestinal TB during Infliximab treatment.Regardless of anti-TNF treatment, long-term screening via T-SPOT.TB assay might represent a more sensitive approach to identify BD patients with LTBI. As a secondary prophylaxis, the LTBI treatment is effective in a country with high risk of TB.
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