关键词： pediatric inflammatory multisystem syndrome  

摘要： Multisystem inflammatory syndrome in children (MIS-C) is a severe inflammatory response described in children after infection with severe acute respiratory syndrome coronavirus 2. We present a case of a 9-year-old African American boy with 2 distinct illnesses that were both consistent with MIS-C. He first presented in the early stages of our understanding of MIS-C with predominantly neurologic and gastrointestinal symptoms and demonstrated elevated inflammatory markers consistent with MIS-C. He was treated with intravenous immunoglobulin with complete resolution of signs and symptoms. After 7 months of good health, he returned with a second, distinct illness characterized by fever, rash, gastrointestinal symptoms, and elevated inflammatory markers that met the criteria for MIS-C. In addition, we identified new dilatation of the left anterior descending coronary artery. He improved rapidly after treatment with intravenous immunoglobulin, aspirin, and steroids. Our report highlights the need to achieve a better understanding of this entity's pathogenesis and clinical course and to improve anticipatory guidance for children with MIS-C.

关键词： electroretinography   paediatric   retinal disease   retinopathy   GOSH-ERG   ISCEV-ERG  

摘要： Purpose To assess the diagnostic accuracy and agreement between a paediatric electroretinography protocol used at Great Ormond Street Hospital (GOSH-ERG) and the 'gold standard' international protocol (ISCEV-ERG) in health and disease. Methods Patient databases between 2010 and 2020 were screened to identify children with an ISCEV-ERG recorded within four years of a GOSH-ERG. Electroretinogram (ERG) component peak times and amplitudes were re-measured, and data were analysed in terms of absolute abnormality and proportional deviation from respective reference ranges. Abnormality was defined by the retinal system affected and by individual ERG a- and b-wave component analysis. Results A total of 59 patients were included: 38 patients had retinal disease defined by an abnormal ISCEV-ERG and 21 had normal ISCEV-ERGs. When absolute abnormality was defined by combined retinal systems, the GOSH-ERG showed an excellent overall sensitivity of 95% (accuracy 86%). Individual retinal systems showed good-excellent sensitivity (67%-100%) and specificity (68%-97%). Electroretinogram (ERG) component sensitivities ranged between 60% and 97% and specificities between 79% and 97% dependent upon the protocol step. The proportional relationship appeared mostly linear between protocols. Electroretinogram (ERG) morphology was comparable for both protocols in a range of retinal diseases including those with pathognomonic ERGs. Conclusion We demonstrate the high diagnostic accuracy of a paediatric ERG protocol (GOSH-ERG) relative to ISCEV standard ERGs. The close proportional deviation and similar waveform morphology indicate ERGs from each protocol are similarly affected in disease. This encourages the use of the GOSH-ERG protocol in the screening, diagnosis and monitoring of retinal disease in children who are unable to comply with the rigorous ISCEV-ERG protocol.

关键词： C-RFA   Hip   Arthritis   Pain  

摘要： Objective To establish the effectiveness of cooled radiofrequency ablation in managing hip pain from osteoarthritis at 6 months after receiving treatment in patients who failed conservative treatments and are not surgical candidates due to comorbidities or unwillingness to undergo arthroplasty surgery by targeting the femoral and obturator branches and assessing the degree of hip pain relief and change of function. Materials and methods This prospective pilot study includes a total of 11 consecutive patients experiencing persistent chronic hip pain in the setting of advanced osteoarthritis. Patients initially underwent anesthetic blocks of the obturator and femoral nerve branches to determine cooled radiofrequency ablation candidacy. After adequate response to the anesthetic blocks (> 50% immediate pain relief), patients were subjected to the procedures 2-3 weeks later. Treatment response was evaluated utilizing clinically validated questionnaires and visual analog score in order to assess impact on pain severity, stiffness, and functional activities of daily living. Follow-up outcome scores were collected up to 6 months after cooled radiofrequency ablation procedure. Results A total of 11 hips were treated consecutively between August 2019 and March 2020 (mean patient age 61.4 years;8 M:3F). The mean total HOOS score improved significantly from baseline at 17.0 +/- 6.0 to 52.9 +/- 5.4 at a mean of 6.2 months after treatment (p < 0.0001), with significant improvement in mean pain score from 16.1 +/- 6.6 to 53.4 +/- 7.4 (p < 0.0001) and mean stiffness score from 15.0 +/- 8.1 to 53.6 +/- 11.0 (p < 0.0001). No major complications were encountered. No patients went on to re-treatment, surgery, or other intervention. Conclusion Image-guided obturator and femoral nerve cooled radiofrequency ablation is effective and safe in treating chronic hip pain/stiffness in the setting of advanced osteoarthritis.

关键词： neuroendocrine tumors   pancreatic endocrine tumour   pancreatic islet cell   pancreatic pathology   pancreatic surgery  

摘要： Objective Recent studies have found aristaless-related homeobox gene (ARX)/pancreatic and duodenal homeobox 1 (PDX1), alpha-thalassemia/mental retardation X-linked (ATRX)/death domain-associated protein (DAXX) and alternative lengthening of telomeres (ALT) to be promising prognostic biomarkers for non-functional pancreatic neuroendocrine tumours (NF-PanNETs). However, they have not been comprehensively evaluated, especially among small NF-PanNETs (<= 2.0 cm). Moreover, their status in neuroendocrine tumours (NETs) from other sites remains unknown. Design An international cohort of 1322 NETs was evaluated by immunolabelling for ARX/PDX1 and ATRX/DAXX, and telomere-specific fluorescence in situ hybridisation for ALT. This cohort included 561 primary NF-PanNETs, 107 NF-PanNET metastases and 654 primary, non-pancreatic non-functional NETs and NET metastases. The results were correlated with numerous clinicopathological features including relapse-free survival (RFS). Results ATRX/DAXX loss and ALT were associated with several adverse prognostic findings and distant metastasis/recurrence (p<0.001). The 5-year RFS rates for patients with ATRX/DAXX-negative and ALT-positive NF-PanNETs were 40% and 42% as compared with 85% and 86% for wild-type NF-PanNETs (p<0.001 and p<0.001). Shorter 5-year RFS rates for <= 2.0 cm NF-PanNETs patients were also seen with ATRX/DAXX loss (65% vs 92%, p=0.003) and ALT (60% vs 93%, p<0.001). By multivariate analysis, ATRX/DAXX and ALT status were independent prognostic factors for RFS. Conversely, classifying NF-PanNETs by ARX/PDX1 expression did not independently correlate with RFS. Except for 4% of pulmonary carcinoids, ATRX/DAXX loss and ALT were only identified in primary (25% and 29%) and NF-PanNET metastases (62% and 71%). Conclusions ATRX/DAXX and ALT should be considered in the prognostic evaluation of NF-PanNETs including <= 2.0 cm tumours, and are highly specific for pancreatic origin among NET metastases of unknown primary.

关键词： Drug interactions   Complementary and alternative medicine   Cancer treatment   Chemotherapy   Cancer outpatients  

摘要： Purpose The aim of our study was to analyze the use of complementary and alternative medicine (CAM) supplements, identify possible predictors, and analyze and compile potential interactions of CAM supplements with conventional cancer therapy. Methods We included outpatient cancer patients treated at a German university hospital in March or April 2020. Information was obtained from questionnaires and patient records. CAM-drug interactions were identified based on literature research for each active ingredient of the supplements consumed by the patients. Results 37.4% of a total of 115 patients consumed CAM supplements. Potential interactions with conventional cancer treatment were identified in 51.2% of these patients. All types of CAM supplements were revealed to be a potential source for interactions: vitamins, minerals, food and plant extracts, and other processed CAM substances. Younger age (< 62 years) (p = 0.020, phi c = 0.229) and duration of individual cancer history of more than 1 year (p = 0.006, phi c = 0.264) were associated with increased likelihood of CAM supplement use. A wide range of different CAM supplement interactions were reviewed: effects of antioxidants, cytochrome (CYP) interactions, and specific agonistic or antagonistic effects with cancer treatment. Conclusion The interaction risks of conventional cancer therapy with over-the-counter CAM supplements seem to be underestimated. Supplements without medical indication, as well as overdoses, should be avoided, especially in cancer patients. To increase patient safety, physicians should address the risks of interactions in physician-patient communication, document the use of CAM supplements in patient records, and check for interactions.

关键词： environment-friendly electrolyte   Langmuir adsorption isotherm   mixed-type corrosion inhibitors   sugar industry   sulfamic acid  

摘要： Sulfamic acid (H3NSO3), which is also known as amino sulfuric acid, sulfamidic acid, amidosulfonic acid, and aminosulfonic acid, is an industrially useful water-soluble colorless crystalline solid. Sulfamic acid solutions of different concentrations are widely used for the removal of scales and metal oxides deposits from the surface of metals and ceramics. Sulfamic acid solutions (5-10%) serve as alternative electrolytes for the removal of rusts and lime scales to replace relatively more irritating and volatile hydrochloric and sulfuric acid-based electrolytes. Sulfamic acid solutions can be regarded as relatively more environmentally friendly and less toxic electrolytes for the cleaning of metallic surfaces. However, similar to the popular acidic solutions, sulfamic acid based electrolytes are corrosive to metal and metallic structures. Therefore, organic compounds based corrosion inhibitors of natural and synthetic origin are widely used to prevent corrosive damage of metallic surfaces in such electrolytes. These compounds become effective by adsorbing on the metallic surface following through the Langmuir adsorption isotherm model. Using electrochemical analyses it was observed that organic corrosion inhibitors in sulfamic acid based electrolytes behave as mixed- and interface-type corrosion inhibitors and they become effective by adsorbing at the metal/electrolyte interfaces. The adsorption of most of the corrosion inhibitors mostly follows physiochemisorption mechanism.

关键词： bamboo-concrete interfaces   bond initiation   bonding strength   lab experiments   modeling and simulation   treated and untreated bamboos  

摘要： The study investigated the feasibility of replacing the steel bars by bamboo in concrete members as alternative materials in the rural and peri-urban areas. The present study also investigated the effects of bonds between concrete and bamboo on the strength of concrete reinforced with treated and untreated bamboo. The study further examined the effect of split bamboo and whole bamboo when used as concrete reinforcements and compare with the strength of concrete reinforced by steel bars. Laboratory tests such as pull out test, flexural test, bamboo water absorption test, bamboo tensile test, and mechanical crushing test were done for this purpose. Nonlinear finite element model using ABAQUS was used to simulate the problem and validate the laboratory results. The results indicated that the bond at the interface between concrete and bamboo greatly affected the strength of bamboo reinforced concrete. The results also revealed the potential of the treated split bamboos for use as an alternative partial replacement of steel bars in concrete reinforcement and a cost-effective approach that can be useful in most rural and peri-urban regions where the relatively low purchasing power of the steel bars, as well as the simple structures, are dominant.

摘要： 一、研究背景 十四五规划指出"提高高等教育质量,分类建设一流大学和一流学科",随着高校"双一流"建设的持续深入推进,对高校师资队伍提出了更高的要求,人才人事工作的科学性和有效性亟待进一步提升.其他专技人员全称为其他专业技术人员,是指高校除教师以外的其他专业技术人员,主要从事档案管理、图书馆管理、信息技术、财务管理等支持服务工作,是高校行政管理队伍的重要组成部分.

摘要： MALAT1, an abundant lncRNA specifically localized to nuclear speckles, regulates alternative-splicing (AS). The molecular basis of its role in AS remains poorly understood. Here, we report three conserved, thermodynamically stable, parallel RNA-G-quadruplexes (rG4s) present in the 3′ region of MALAT1 which regulates this function. Using rG4 domain-specific RNA-pull-down followed by mass-spectrometry, RNA-immuno-precipitation, and imaging, we demonstrate the rG4 dependent localization of Nucleolin (NCL) and Nucleophosmin (NPM) to nuclear speckles. Specific G-to-A mutations that abolish rG4 structures, result in the localization loss of both the proteins from speckles. Functionally, disruption of rG4 in MALAT1 phenocopies NCL knockdown resulting in altered pre-mRNA splicing of endogenous genes. These results reveal a central role of rG4s within the 3′ region of MALAT1 orchestrating AS.

关键词： P. olseni   DNA metabarcoding   eDNA   Real-time PCR assay   Illumina   Bivalves  

摘要： Perkinsus olseni is a protozoan parasite that infects a wide variety of molluscs worldwide, causing economic losses in the aquaculture sector. Consequently, Perkinsosis has been catalogued by the World Organization for Animal Health (O. the present study, we analysed the spatial and temporal distribution of transmissible stages of Perkinsus olseni in an endemic area of the parasite from 2016 to 2018. The pathogen was detected using high-throughput sequencing of the 18S rRNA gene and a specific real-time PCR assay (qPCR) in samples of water, sediment and several bivalve species. Histopathological assays were also conducted on bivalve samples. P. olseni was not detected in environmental samples by qPCR;however, eDNA sequencing revealed its presence in both the water and sediment at all sampled points, showing a seasonal pathogen prevalence. As expected, the parasite was detected in clams, but a few cases were also found in mussels and cockles. The presence of the parasite was confirmed in Cerastoderma edule by histology and qPCR using RNA to evaluate the presence of proliferative life stages of the parasite. Therefore, this is the first time that P. olseni has been found in C. edule but with low abundance and infection intensity levels.