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This review provides an in depth assessment of the cumulative knowledge of ABCA4-associated retinopathy - clinical manifestations, genetic complexity, pathophysiology as well as current and proposed therapeutic approaches. Secretory proteostasis integrates protein synthesis, processing, folding and trafficking pathways that are essential for efficient cellular secretion. For the retinal pigment epithelium (RPE), secretory proteostasis is of vital importance for the maintenance of the structural and functional integrity of apical (photoreceptors) and basal (Bruch's membrane/choroidal blood supply) sides of the environment it resides in. This integrity is achieved through functions governed by RPE secreted proteins, which include extracellular matrix modelling/remodelling, angiogenesis and immune response modulation. Impaired RPE secretory proteostasis affects not only the extracellular environment, but leads to intracellular protein aggregation and ER-stress with subsequent cell death. Ample recent evidence implicates dysregulated proteostasis as a key factor in the development of age-related macular degeneration (AMD), the leading cause of blindness in the developed world, and research aiming to characterise the roles of various proteins implicated in AMD-associated dysregulated proteostasis unveiled unexpected facets of the mechanisms involved in degenerative pathogenesis. This review analyses cellular processes unveiled by the study of the top 200 transcripts most abundantly expressed by the RPE/choroid in the light of the specialised secretory nature of the RPE. Functional roles of these proteins and the mechanisms of their impaired secretion, due to age and genetic-related causes, are analysed in relation to AMD development. Understanding the importance of RPE secretory proteostasis in relation to maintaining retinal health and how it becomes impaired in disease is of paramount importance for the development and assessment of future therapeutic advancements involving gene and cell therapies. OBJECTIVES The objectives of this series are to evaluate the results of vaginal myomectomy and to assess the postoperative adhesion risk. METHODS It was a prospective observational study of fifty patients. All patients underwent a vaginal myomectomy, followed six months after laparoscopy to assess and treat postoperative adhesions. RESULTS Myomectomies were performed via an anterior, posterior and mixed colpotomy in 42 %, 54 % and 4 % of the cases, respectively. Laparoconversion was necessary in three cases (6 %). Laparoscopic evaluation was performed in 24 patients (48 %). CP21 adhesions were present in 14 cases (58.33 %); the adhesions did not affect the Fallopian tubes and ovaries. CONCLUSION Vaginal myomectomy was feasible, not limited to single myomectomy this which is in agreement with previously published series. Post adhesion risk seems weaker and does not concern the appendices, but this requires to be confirmed. BACKGROUND AND AIMS We previously reported safety and effectiveness of EUS-guided hepaticogastrostomy (EUS-HGS) using a long, partially covered metal stent (LP-CMS) for malignant biliary obstruction (MBO). In this study, we aimed to evaluate long-term outcomes of EUS-HGS in the expanded cohort. METHODS A total of 110 patients undergoing EUS-HGS using a LP-CMS in 2 centers were retrospectively studied. Technical and functional success, adverse events, recurrent biliary obstruction (RBO), and reinterventions were evaluated. RESULTS The cause of MBO was pancreatic cancer in 50% and the location of MBO was distal in 68%. The stent length was 8 cm in 2%, 10 cm in 84%, and 12 cm in 15%, with a median intragastric stent length of 54 mm. Technical and functional success rate was 100% and 94%. Adverse event rate was 25% (mild 15%, moderate 7%, severe 3%), but about one-half of adverse events were mild transient fever and abdominal pain. RBO developed in 33%, with a median cumulative time to RBO of 6.3 months. The major cause of RBO was hyperplasia at an uncovered portion. The presence of prior biliary drainage and short intragastric stent length were significantly associated with RBO. Reintervention for RBO was successfully achieved through the EUS-HGS route in 92%. The remaining reintervention procedures were 1 EUS-HGS and 2 percutaneous transhepatic biliary drainage all in cases with hilar MBO. CONCLUSIONS EUS-HGS using a LP-CMS for unresectable MBO was safe and effective. RBO was not uncommon, but reintervention through the EUS-HGS route was technically possible in most cases. The study analyzed health care workers' (HCWs) knowledge, practices, and attitudes regarding COVID-19. A cross-sectional survey was conducted from 4th February to 8th February 2020 involving a total of 1357 HCWs across 10 hospitals in Henan, China. Of those surveyed, 89% of HCWs had sufficient knowledge of COVID-19, more than 85% feared self-infection with the virus, and 89.7% followed correct practices regarding COVID-19. In addition to knowledge level, some risk factors including work experience and job category influenced HCWs' attitudes and practice concerning COVID-19. Measures must be taken to protect HCWs from risks linked to job category, work experience, working hours, educational attainment, and frontline HCWs. OBJECTIVE Several parameters are known to predict the survival of glioblastoma (GB), including extent of resection and MGMT promotor methylation. Staining for glial fibrillary acidic protein (GFAP) is a common component of routine histological work-up, but its clinical utility in GB is unclear. The aim of the present study was to analyze the predictive value of quantitative GFAP measurements for survival of patients with GB. METHODS All subjects in our institutional database of patients with primary GB who underwent surgery between 2011 and 2014 with examination of immunohistochemical staining of GFAP were included. Percentage GFAP staining was measured in 5% increments (5-100%). Univariate and multivariate analyses were performed between GFAP values and survival data. Clinically relevant cut-offs for GFAP staining were identified by receiver operating characteristic (ROC) curves. RESULTS The final cohort consisted of 272 GB patients with available quantitative GFAP measurements (mean age, 62 (±11.1) years, 117 females [43%]).
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