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V876E) in CACNA1S. see more CONCLUSION Our data indicate that the p.V876E mutation in CACNA1S contributes to the early onset of neuromuscular symptoms and unusual clinical phenotypes of HypoPP. © 2020 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.Electrochemistry belongs to an important branch of chemistry that deals with the chemical changes produced by electricity and the production of electricity by chemical changes. Therefore, it can not only act a powerful tool for materials synthesis, but also offer an effective platform for sensing and catalysis. As extraordinary zero-dimensional materials, carbon-based quantum dots (CQDs) have been attracting tremendous attention due to their excellent properties such as good chemical stability, environmental friendliness, nontoxicity and abundant resources. Compared with the traditional methods for the preparation of CQDs, electrochemical (EC) methods offer advantages of simple instrumentation, mild reaction conditions, low cost and mass production. In return, CQDs could provide cost-effective, environmentally friendly, biocompatible, stable and easily-functionalizable probes, modifiers and catalysts for EC sensing. However, no specific review has been presented to systematically summarize both aspects until now. In this review, the EC preparation methods of CQDs are critically discussed focusing on CQDs. We further emphasize the applications of CQDs in EC sensors, electrocatalysis, biofuel cells and EC flexible devices. This review will further the experimental and theoretical understanding of the challenges and future prospective in this field, open new directions on exploring new advanced CQDs in EC to meet the high demands in diverse applications. © 2020 Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim.BACKGROUND Patients undergoing endoscopic resection of neoplasms with both sinonasal and skull base involvement can develop chronic rhinosinusitis (CRS) after treatment and may occasionally benefit from additional endoscopic sinus surgery (ESS). We investigate risk factors and outcomes associated with revision ESS (rESS) after endoscopic skull-base surgery (SBS) for neoplasms with combined sinonasal and skull base involvement. METHODS A retrospective review of patients with neoplasms with both sinonasal and skull base involvement who underwent endoscopic resection at a single tertiary care academic institution from 2004 through 2017 was performed. Eighty-three patients were included. Main outcome measures included incidence and timing of revision surgery, Lund-Mackay (LM) scores, and 22-item Sino-Nasal Outcome Test (SNOT-22) scores. RESULTS rESS was performed in 21 (25%) cases, 15 (18%) of which were due to CRS. Time between initial resection and rESS was an average of 42 months (range, 6 to 142 months). Pre-SBS and post-SBS LM scores were not significantly different (5.0 vs 4.7, p = 0.640), although pre-SBS and post-SBS SNOT-22 scores showed significant improvement (32.6 vs 24.5, p = 0.030). Malignant pathology correlated with need for rESS (odds ratio [OR] 5.07, p = 0.04), as well as treatment including chemotherapy (OR 5.10, p = 0.003) and radiation (OR 4.15, p = 0.013). CONCLUSION A significant proportion of patients develop clinically significant sinusitis after endoscopic SBS for neoplasms with combined sinonasal and skull base involvement and may benefit from rESS. Intervention occurred, on average, 3.5 years after initial tumor resection. Malignant pathology, radiation therapy, and chemotherapy correlate with need for rESS. © 2020 ARS-AAOA, LLC.BACKGROUND Aspirin-exacerbated respiratory disease (AERD) refers to the combination of asthma, chronic rhinosinusitis with nasal polyposis (CRSwNP), and acute respiratory tract reactions to ingestion of aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs). There have been no in the literature investigating diagnostic delay in AERD. We aimed to investigate whether delay of diagnosis of AERD is associated with poorer clinical outcomes as well as to characterize the role of specialty evaluation in diagnosis. METHODS We conducted a retrospective observational study of 254 subjects with incident AERD diagnoses between 2009 and 2016 among Kaiser Permanente Northern California (KPNC) members. Descriptive and bivariate statistics were employed to analyze clinical characteristics and outcomes of AERD subjects with and without delay in diagnosis (defined as 1 year or greater from symptom onset to diagnosis). RESULTS Of the 254 patients in the AERD cohort, 24.4% had a delayed diagnosis. Patients with allergies were significantly less likely to have a delay in diagnosis (p less then 0.01). Patients with a delay in diagnosis were more likely to have 2 or more courses of systemic steroids (p = 0.04). Allergists, otolaryngologists, and primary care physicians diagnosed 56%, 36%, and 8% of patients, respectively. There was no association between provider specialty at time of diagnosis and delay in diagnosis (p = 0.22). CONCLUSION A substantial proportion of AERD patients have a diagnostic delay. Patients with allergies have a lower risk for this delay. This study is the first to describe diagnostic delay in AERD patients. © 2020 ARS-AAOA, LLC.OBJECTIVES To investigate the predictors and clinical impact of left ventricular reverse remodeling (LVRR) after MitraClip (MC) therapy for degenerative (DMR) and functional mitral regurgitation (FMR). BACKGROUND MC therapy induces LVRR in patients with mitral regurgitation (MR) at high-risk for surgery. However, specific data on predictors of LVRR therapy are limited. METHODS This study included 164 patients treated by MC implantation with complete clinical and echocardiographic evaluation at baseline, 6 months, and 12 months. LVRR was defined as a decrease of ≥10% of the left ventricular end-diastolic diameter after 12 months and was found in 49% of the patients. RESULTS LVRR was associated with significantly reduced event rate 2 years after MC procedure. In the total cohort, multivariate regression analysis determined severe recurrent/residual MR after 12 months (p = .010, odds ratio [OR] = 0.26), male gender (p = .050, OR = 0.49) and left ventricular ejection fraction (LVEF) less then 20% (p = .046, OR = 0.
My Website: https://www.selleckchem.com/products/stc-15.html
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