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OBJECTIVE This prospective study was designed to analyze the incidence and the factors associated with impairment in left ventricular systolic function (LVSF) overtime in patients with rheumatoid arthritis (RA) without overt cardiac disease. In particular, we verified the hypothesis that a relationship between worsening of LVSF and markers of RA disease activity exists. METHODS One hundred forty outpatients with RA without overt heart disease underwent clinical, laboratory, and echocardiographic evaluation at baseline and after 35 (interquartile range [IQR] 23-47) months of follow-up. A clinical Disease Activity Index (CDAI) score greater than 10 indicated the presence of moderate-high RA disease activity; data on anticitrullinated peptide antibody (ACPA) positivity were recorded at baseline. Stress-corrected midwall fractional shortening (sc-MFS) was used as a measure of LVSF and was considered impaired if less than 86.5%. RESULTS At 36 (IQR 23-47) months follow-up, impaired sc-MFS was detected in 60 of 140 (43%) patients, compared with 80 patients with normal sc-MFS. Disease duration and activity, ACPA positivity, inflammatory markers, cardiovascular and antirheumatic therapies, and sc-MFS were similar between the two groups at baseline. A multiple logistic regression analysis showed ACPA positivity, moderate-high disease activity (CDAI greater than 10), and disease duration as independent predictors of impaired sc-MFS at follow-up. Finally, a simple clinical score to predict worsening of LVSF at midterm was built (area under the curve of 0.80, with a sensibility and specificity of 78% and 82%, respectively). CONCLUSION Disease duration, ACPA positivity, and moderate-high disease activity are independent prognosticators of LVSF impairment in RA. Adverse changes in heart function could be prevented by good control of inflammation and modulation of autoimmunity. ATR inhibitor © 2020 The Authors. ACR Open Rheumatology published by Wiley Periodicals, Inc. on behalf of American College of Rheumatology.PCNT encodes a large coiled- protein localizing to pericentriolar material and is associated with microcephalic osteodysplastic primordial dwarfism type II syndrome (MOPD II). We report our experience of nine new patients from seven unrelated consanguineous Egyptian families with the distinctive clinical features of MOPD II in whom a customized NGS panel showed homozygous truncating variants of PCNT. The NGS panel results were validated thereafter using Sanger sequencing revealing three previously reported and three novel PCNT pathogenic variants. The core phenotype appeared homogeneous to what had been reported before although patients differed in the severity showing inter and intra familial variability. The orodental pattern showed atrophic alveolar ridge (five patients), rootless tooth (four patients), tooth agenesis (three patients), and malformed tooth (three patients). In addition, mesiodens was a novel finding found in one patient. The novel c.9394-1G>T variant was found in two sibs who had tooth agenesis. CNS anomalies with possible vascular sequelae were documented in two male patients (22.2%). Simplified gyral pattern with poor development of the frontal horns of lateral ventricles was seen in four patients and mild thinning of the corpus callosum in two patients. Unilateral coronal craniosynstosis was noted in one patient and thick but short corpus callosum was an unusual finding noted in another. The later has not been reported before. Our results refine the clinical, neuroradiological, and orodental features and expand the molecular spectrum of MOPD II. © 2020 Wiley Periodicals, Inc.INTRODUCTION Vaginal dilation is often indicated as an intervention for the management of radiation therapy-induced vaginal adhesions and stenosis (RTVAS). However, limited research exists underpinning this intervention and diversity in patient recommendations internationally are reported. In the absence of New Zealand (NZ) national guidelines regarding the management of RTVAS, a survey of NZ radiation therapy departments was conducted to gain an overview of current practice. METHODS A two-section online survey was developed to capture RTVAS education and management overview across NZ. Section one focused on departmental resourcing and section two on local standard practice regarding vaginal dilator usage. One RTVAS education representative from each department was invited to complete the survey. RESULTS Eight of nine NZ departments completed the survey. Consistent treatment indications were identified for RTVAS patient education with the involvement of diverse staffing groups at various time-points throughout the treatment process. Protocols for RTVAS management existed in each RT department with staff commonly trained by informal peer observation. Dilator usage was recommended regardless of patient sexual activity. Agreement was shown regarding the recommended start time of dilator usage and frequency. The recommended duration of dilator use post-treatment varied from 6 months to greater than 36 months. CONCLUSIONS This work illustrates both concordance and diversity in practice and contributes to the limited body of literature available. Further research is warranted to explore patterns of practice between departmental protocols and individual practitioners in further detail. © 2020 The Authors. Journal of Medical Radiation Sciences published by John Wiley & Sons Australia, Ltd on behalf of Australian Society of Medical Imaging and Radiation Therapy and New Zealand Institute of Medical Radiation Technology.An epidemic of an acute respiratory syndrome caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in Wuhan, China, now known as coronavirus disease 2019 (COVID-19), beginning in December 2019 has attracted an intense amount of attention worldwide. As the natural history and variety of clinical presentations of this disease unfolds, the extrapulmonary symptoms of the disease have emerged, especially the symptoms related to the digestive system. While the respiratory mode of transmission is well-known and likely the principal mode of transmission of this disease, the possibility of the fecal-oral route of transmission has also emerged in various case series and clinical scenario. In this review article, we summarized the published literatures to date concerning four different aspects (a) gastrointestinal manifestations of COVID-19 infection; (b) microbiologic and virological investigations; (c) the role of fecal-oral transmission; and (d) prevention/control infection in the digestive endoscopy room.
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