Conocimiento en estudiantes de enfermería sobre sepsis quirúrgico / Knowledge in nursing students about surgical sepsis

Objetivo: Determinar el nivel de conocimiento de los estudiantes de enfermería de la Universidad Técnica de Ambato sobre sepsis quirúrgica. Material y método: La presente investigación tiene un diseño de desarrollo observacional, de tipo descriptivo, cohorte transversal, con un enfoque cuantitativo, ya que el nivel de cono-cimiento se verá representado mediante tablas y gráficos para des-cribir la problemática del periodo octubre 2023 febrero 2024. Re-sultados: Se evidencia un alto porcentaje de respuestas incorrectas por cada ítem por parte de los estudiantes. La categoría Nivel de Conocimiento sobre Definición de Sepsis, fue respondida de ma-nera incorrecta con un porcentaje del 83,9%, la categoría Nivel de Conocimiento sobre Diagnóstico de Sepsis obtuvo 51,7% y, por úl-timo, la Nivel de Conocimiento sobre Tratamiento de Sepsis con el 29,2%. Conclusiones: El nivel de conocimiento de los estudiantes sobre Sepsis Quirúrgica es malo, debido a que existe una subesti-mación de la gravedad de la sepsis como afección potencialmente mortal, lo que puede traer un impacto negativo en los pacientes[AU]

Objective: Determine the level of knowledge of nursing students at the Technical University of Ambato about surgical sepsis. Mate-rials and methods: This research has an observational, descriptive, transversal development design, with a quantitative approach since the level of knowledge will be represented through tables and gra-phs to describe the problems of the period October 2023-February 2024. Results: A high percentage of incorrect answers for each item by the students is evident. The category Level of Knowledge about Definition of Sepsis was answered incorrectly with a percentage of 83.9%, the category Level of Knowledge about Diagnosis of Sepsis obtained 51.7% and, finally, the category Level of Knowledge about Treatment of Sepsis. Sepsis with 29.2%. Conclusions: The level of knowledge of students about Surgical Sepsis is poor because there is an underestimation of the severity of sepsis as a potentially fatal condition, which can have a negative impact on patients[AU]

Objetivo: Determinar o nível de conhecimento dos estudantes de enfermagem da Universidade Técnica de Ambato sobre sepse ci-rúrgica. Material e método: Esta pesquisa possui desenho de coor-te observacional, descritivo, transversal, com abordagem quantita-tiva, uma vez que o nível de conhecimento será representado por meio de tabelas e gráficos para descrever o problema no período de outubro de 2023 a fevereiro de 2024. Resultados: Uma parada. É evidente o percentual de respostas incorretas para cada item por parte dos alunos. A categoria Nível de Conhecimento sobre Defi-nição de Sepse foi respondida incorretamente com percentual de 83,9%, a categoria Nível de Conhecimento sobre Diagnóstico de Sepse obteve 51,7% e por fim, a categoria Nível de Conhecimen-to sobre Tratamento de Sepse com 29,2%. Conclusões: O nível de conhecimento dos estudantes sobre a Sepse Cirúrgica é baixo, pois há uma subestimação da gravidade da sepse como uma condição potencialmente fatal, que pode ter um impacto negativo nos pa-cientes[AU]

Autopsy findings of fatal retroperitoneal hemorrhage after traumatic rupture of bilateral renal angiomyolipoma

ABSTRACT The present work reports the autopsy findings of a unique case characterized by fatal retroperitoneal hemorrhage following the traumatic rupture of bilateral renal angiomyolipomas. Renal angiomyolipomas are generally benign tumors with an unpredictable clinical course, ranging from asymptomatic to sudden rupture and hemorrhagic shock. They may be associated with genetic disorders such as tuberous sclerosis complex. The case under investigation is unprecedented in the medical literature due to its bilateral nature and fatal outcome. Autopsy analysis revealed an extensive retroperitoneal hemorrhage originating from bilateral ruptured tumors. Microscopic examination found features consistent with bilateral renal angiomyolipoma. Circumstantial information identified a traffic accident before the death, considering it as the cause of the tumors' traumatic rupture. In this case, due to the severity of the situation, immediate medical measures—such as fluid resuscitation, coagulopathy correction, and surgical treatment, which are usually lifesaving—could not be performed. This led to the patient being declared dead at the scene of the crash.

A cross sectional study on the determinants of railway fatalities in different age groups

Background: Over the last few decades many railway accidents and fatalities have occurred in India as well as worldwide. Cities having a high economic activity and are complex spatial structures, are supported by transport systems. The evolution of mass rapid transport system in the form of sub-urban railway network has increased railway casualties in the cities as the railroads pass through residential and commercial zones. Methods: This cross-sectional observational study was conducted at R. G. Kar medical college and hospital police Morgue attached to the department of forensic medicine and toxicology (FMT), Kolkata on the randomly selected medico legal postmortems over last 4 years. Results: Railway accidents were common in males with mean age group of 38.98 years and was almost identical with the female mean age. The accidents mostly occurred in the office hours during the day time. Chi square test of association gave significant results between the different age groups with sex and grievous injuries sustained the people. Injuries sustained by the train showed that they were found mostly over the head followed by spine and thorax. Major cause of death was due to head injuries. Conclusions: The article gives an idea about the relevant demographic profiles and association with the age-related factors that can be taken up as means to prevent the accidents.

Pediatric inflammatory multisystemic syndrome in Brazil: sociodemographic characteristics and risk factors to death

Abstract Objective: To determine the incidence of MIS-C in Brazil, describe the clinical and sociodemographic characteristics of the pediatric population affected by MIS-C and compare mortality and lethality outcomes with isolated Covid-19 and MIS-C cases. Methods: Observational and retrospective cohort study of cases of MIS-C associated with Covid-19 in the Brazilian population between 04/01/2020 and 04/17/2021. Data from the Ministry of Health's epidemiological bulletin up to the 15th epidemiological week of 2021, were used. The analyzes were descriptive through absolute and relative frequencies. The significance level is 5% in Stata 16.0 package. Results: Between 04/01/2020 and 04/07/2021, 903 cases of MIS-C associated with Covid-19 were notified in Brazil, of which, the largest part (55.26%) were male, between 0 and 4 years old (45.29%), from the Southeast region (38.76%). The deaths (61; 6.7%) were higher in the female gender, between 0 and 4 years old (47.54%) and in the Southeast region (34.43%). It was identified that the risk of death by MIS-C related to Covid-19 is 5.29 (CI = 2.83; 9.87 and P-value = <0.001) times higher in adolescents from 15-19 years old than in other age groups when compared to 0-4 years old children. Also, the residency in North region was as risk factor to death (RR = 3.72, IC = 1.29; 10.74 e P-value = 0.008). Conclusion: In this study, despite the numbers showing more deaths from zero to 4 years old, the risk for teenagers is notably higher. In addition, Brazil's Northern region is a risk factor that reaffirms social inequality and poor access to health.

Applicability of feature selection combined with Boosting algorithm in severity prediction of non-fatal occupational injuries in miners / 环境与职业医学

Background Identification and analysis of influencing factors of occupational injury is an important research content of feature selection. In recent years, with the rise of machine learning algorithms, feature selection combined with Boosting algorithm provides a new analysis idea to construct occupational injury prediction models. Objective To evaluate applicability of Boosting algorithm-based model in predicting severity of miners' non-fatal occupational injuries, and provide a basis for rationally predicting the severity level of miners' non-fatal occupational injuries. Methods The publicly available data of the US Mine Safety and Health Administration (MSHA) from 2001 to 2021 on metal miners' non-fatal occupational injuries were used, and the outcome variables were lost working days < 105 d (minor injury) and ≥ 105 d (serious injury). Four different feature sets were screened out by four feature selection methods including least absolute shrinkage and selection operator (Lasso) regression, stepwise regression, single factor + Lasso regression, and single factor + stepwise regression. Logistic regression, gradient boosting decision tree (GBDT), and extreme gradient boosting (XGBoost) were selected to construct prediction models by training with the four feature sets. A total of 12 prediction models of severity of miners' non-fatal occupational injuries were built and their area under the curve (AUC), sensitivity, specificity, and Youden index were calculated for model evaluation. Results According to the results of four feature selection methods, age, time of accident occurrence, total length of service, cause of injury, activities that triggered injury occurrence, body part of injury, nature of injury, and outcome of injury were identified as influencing factors of non-fatal occupational injury severity in miners. Feature set 4 was the optimal set screened out by single factor+stepwise regression and the GBDT model presented the best predictive performance in predicting the severity of non-fatal occupational injuries. The associated specificity, sensitivity, and Youden index were 0.7530, 0.9490, and 0.7020, respectively. The AUC values of logistic regression, GBDT, and XGBoost models trained by feature set 4 were 0.8526 (95%CI: 0.8387, 0.8750), 0.8640 (95%CI: 0.8474, 0.8806), and 0.8603 (95%CI: 0.8439, 0.8773), respectively, higher than the AUC values trained by feature set 2 [0.8487 (95%CI: 0.8203, 0.8669), 0.8110 (95%CI: 0.8012, 0.8344), and 0.8439 (95%CI: 0.8245, 0.8561), respectively] . The AUC values of GBDT and XGBoost models trained by feature set 4 were higher than that of logistic regression model. Conclusion The performance of the prediction models constructed by predictors screened out by two feature selection methods is better than those by single feature selection methods. At the same time, under the condition of optimal feature set, the performance of model prediction based on Boosting is better than that of traditional logistic regression model.

Application of lost workdays in surveillance and assessment of non-fatal occupational injuries: Based on European Statistics on Accidents at Work / 环境与职业医学

Background The severity of occupational injury in countries such as the United Kingdom, the United States, and Germany is usually analyzed using lost workdays, but in existing occupational injury surveillance research in China, the application of this index is rare. Objective To evaluate the application value of lost workdays in non-fatal occupational injury surveillance, and provide a reference for the construction of occupational injury surveillance index system. Methods The public data of European Statistics on Accidents at Work (ESAW) from 2010 to 2019 on non-fatal injury accidents in 27 member states of the European Union were used. Non-fatal occupational injury is defined as an injury event during occupational activities or at work resulting a victim's absence from work for ≥4 d. According to the European Statistics on Accidents at Work-Summary methodology, the lost workdays were divided into 8 categories (4-6 d, 7-13 d, 14-20 d, 21-30 d, 31-91 d, 92-182 d, 183 d and above, and unknown). Annual percentage change (APC) and the average annual percentage change (AAPC) were used to evaluate the overall trend changes in the incidence rate of non-fatal occupational injury accidents in different lost workdays from 2010 to 2019, and the non-fatal occupational injury accidents in key industries. The characteristics of the occurrence of non-fatal occupational injuries were analyzed in conjunction with the changes in non-fatal occupational injuries in different lost workdays in the industry. Results From 2010 to 2019, the overall incidence of non-fatal occupational injury accidents in the European Union showed a downward trend, and the AAPC was −1.0% (P<0.05). The accident rates of lost workdays of 4-6 d and 92-182 d showed an upward trend, and the AAPC were 7.9% and 5.8% respectively (P<0.05). The average annual accident rates of non-fatal occupational injuries (≥4 d) in Categories C (manufacturing industry), E (water supply, sewage treatment, waste management and remediation), and F (construction industry) showed a linear downward trend, and the AAPC were −3.0%, −2.5%, and −1.5%, respectively (P<0.05). However, among them, the rate of non-fatal occupational injury accidents with 92-182 d of lost workdays in the manufacturing industry showed a significant upward trend, with an AAPC of 3.7% (P<0.001). Conclusion Using lost workdays combined with APC and AAPC by Join-point linear regression analysis can measure the severity and trend changes of non-fatal occupational injury accidents in different industries and different lost workdays. This indicator has an important practical significance in evaluating the effectiveness of occupational injury prevention and control strategies adopted by countries and enterprises.

Overview of work-related diseases and occupational injury surveillance system in the UK / 环境与职业医学

The UK's work-related diseases and occupational injury surveillance system consists of Reporting of Injuries, Diseases and Dangerous Occurrences Regulations 2013 (RIDDOR), Labour Force Survey (LFS), The Health and Occupation Research network in General Practice (THOR-GP), and Reporting to The Health and Occupation Research network by specialist physicians. This article briefly described the scope, content, and methods of each surveillance programme in the UK work-related diseases and occupational injury surveillance system, and summarized their advantages and disadvantages. Among them, employers are required to report to relevant law enforcement authorities by RIDDOR, data are highly accessible but with a concern of serious underreport, and it is the only data source of fatal occupational injuries; LFS, a representative national household sample survey, covering occupational injuries and work-related diseases, is the primary data source of non-fatal occupational injuries and work-related diseases such as stress, anxiety, and depression, but collects non-clinically proven data based on self-perception; general practitioners report clinically confirmed work-related diseases, which is more scientific in attribution and is a good secondary source of work-related diseases; specialist physicians report clinically confirmed cases of higher severity, which is the primary source of data on conditions such as asthma and dermatitis, but may underestimate morbidity. Each surveillance programme of the system has its own characteristics, intersects, and complements each other, which can provide reference for the construction of occupational injury surveillance system in China.

Follow-up and retrospective investigation of pneumoconiosis cases in a county of Ningbo City from 1974 to 2019 / 环境与职业医学

Background Pneumoconiosis is one of the most common and widespread prescribed occupational diseases in China, and there is no cure for it at present. The Healthy China 2030 underlines that the prevention and treatment of pneumoconiosis are the critical element of promoting occupational health. Objective To understand the basic characteristics, social security, and death causes of pneumoconiosis cases in Ninghai County, and to provide a basis for scientific prevention and treatment of pneumoconiosis. Methods Information on pneumoconiosis cases in Ninghai County from 1974 to 2019 was obtained by consulting occupational disease and occupational health information monitoring systems, occupational disease diagnosis institutions, and employers, which may retain original data on occupational pneumoconiosis diagnosis, as well as specialized epidemiological survey projects on pneumoconiosis. Telephone or face-to-face follow-up visits were conducted based on relevant information to complete or verify relevant information such as length of service with dust exposure, type, stage, and diagnosis date of pneumoconiosis. ANOVA, chi-square test, and rank-sum test were used to analyze age of diagnosis and age of death, entitlement to social security and mortality rate of cases/length of service. Results As of the end of 2019, a total of 420 cases of pneumoconiosis were diagnosed among workers exposed to dust in Ninghai County, with 409 cases (97.38%) followed up and 11 cases (2.62%) lost to follow-up. Since the first case of pneumoconiosis was reported in 1974, 39 cases, 278 cases, and 101 cases of pneumoconiosis were reported before 2000, from 2000 to 2009, and from 2010 to 2019, respectively. Two other cases of pneumoconiosis were diagnosed with an unknown year, one as a dead case and one as a lost case. There were 294 surviving pneumoconiosis cases, who mainly lived in Sangzhou Town. The mean age of diagnosis was (58.68±15.37) years old, and the median length of service with exposure to dust was 8.0 (4.0, 15.7) years. The age of diagnosing stage III pneumoconiosis was less than that of stage I and stage II, and the difference between the age of diagnosing stage III and stage II pneumoconiosis was significant (P <0.05). There were 231 cases (78.57%) aged ≥ 60 years, while there were 74 cases aged ≥ 80 years (25.17%). There were 160 cases (54.42%) whose length of service was < 10 years. There were 12.59% and 7.82% of the total cases compensated by work injury insurance and civil compensation by employers, respectively. From the perspective of diagnosis period, the proportion of patients who collected work-related injury insurance and civil compensation from employers in the 2010—2019 year group was higher than that in the <2000 year group or the 2000—2009 year group (P<0.01). There were 115 fatal pneumoconiosis cases, and the fatality rates of stage I, II , and III pneumoconiosis were 23.86%, 18.18%, and 50.75%, respectively. The fatality rate of stage III pneumoconiosis was higher than that of stage I or II (P <0.05). The age of diagnosis and age of death of stage III pneumoconiosis cases were lower than those of stage I and II (P <0.05). The cause of death was definite in 65 patients, including 25 cases (38.46%) of respiratory diseases (except lung cancer) and 28 cases (43.07%) of tumors, and lung cancer accounted for 1/2 of the pneumoconiosis cases that died due to tumors. Conclusion The surviving pneumoconiosis cases in Ninghai are mainly older than 60 years old and largely live in Sangzhou Town. Constructing local rehabilitation stations should be taken as an opportunity to actively prevent and treat pneumoconiosis complications, further improving the quality of life of pneumoconiosis patients.

Fatal sclerosing mesenteritis: a 7-year-old male autopsy case report

ABSTRACT Sclerosing Mesenteritis (SM) is a rare diagnosis, particularly in pediatric patients, and is typically non-fatal when appropriately treated. Although molecular and immunohistochemical alterations have been described, no pathognomonic signature has been identified for this entity. This report presents a case of a seven-year-old boy who suffered sudden cardiorespiratory arrest. Upon autopsy, he was found to have multicentric SM on the upper mesentery, which led to bowel wall thinning and abdominal bleeding with bacterial translocation. We performed comprehensive morphological, immunohistochemical, and molecular analyses. SM is an atypical disorder with diverse clinical manifestations, including a rare but potentially fatal course. Early diagnosis is critical, given its potential severity. To our knowledge, this is the first case report of pediatric mortality linked to SM. Our findings emphasize the importance of increased awareness and early detection of SM in pediatric patients.

Hallazgos en imágenes de tórax en una intoxicación aguda con paraquat: reporte de un caso / Findings on thorax images in acute paraquat poisoning: case report

Resumen El Paraquat es un herbicida ampliamente utilizado para el control de las malezas en Chile. Su ingesta determina una alta probabilidad de mortalidad dado su inherente toxicidad mediante la producción de radicales libres, que afectan a múltiples órganos, principalmente los pulmones; a esto se suma la falta de un tratamiento efectivo. Se presenta el caso clínico de un hombre de 18 años que en un intento suicida consume 50 mL de paraquat (200 g/L), con desenlace fatal. La presentación clínica depende la cantidad de Paraquat ingerida y los hallazgos radiológicos descritos varían según la temporalidad del cuadro e, inclusive, podrían determinar el pronóstico.

Paraquat is an herbicide widely used for weed control in Chile. Its intake determines a high probability of mortality because of its inherent toxicity through the production of free radicals. Multiple organs are affected, mainly the lungs; to this is added the lack of effective treatment. We present the clinical case of an 18-year-old man who in a suicidal attempt swallows 50 mL of paraquat (200 g/L), with a fatal outcome. The clinical presentation depends on the amount of Paraquat ingested. Radiological findings described vary according to the temporality of the condition and could even determine the prognosis

Educação em saúde e final de vida no hospital / Educación en salud y final de la vida en el hospital / Health education and end-of-life at a hospital

Introdução: ações de educação em saúde são incipientes em alguns hospitais. Quando se trata de realizá-las em situações de final de vida, as equipes apresentam limitações. Objetivo: identificar ações de educação em saúde direcionadas às pessoas em final da vida e seus cuidadores, informais e formais, no hospital, além de avaliar o nível de evidência de tais ações. Síntese do conteúdo: o estudo foi realizado entre dezembro de 2019 e janeiro de 2020, nas bases de dados Web of Science, Scopus e Medline. Identificaram-se 6.762 artigos, dos quais 42 integram a análise por serem artigos originais ou de revisão escritos em português, espanhol, inglês ou francês; a amostra dos estudos foi composta por pacientes maiores de 19 anos, com doença avançada e/ou em final de vida ou cuidadores ou profissionais de saúde. Os dados foram agrupados por similaridade do tema das ações, conforme Polit e Beck, e o nível de evidência avaliado segundo Melnyk e Fineout-Overholt. O vídeo foi a ação com maior força de recomendação, seguida por cartilhas. As unidades temáticas foram "Ações para o controle da dor", "Narrativas sobre o final da vida", "Planejamento de cuidados", "Dialogando sobre os cuidados paliativos" e "Comunicação e final de vida". Conclusões: ações de educação em saúde no final da vida devem considerar as tecnologias da informação e da comunicação, além das condições socioculturais, clínicas e cognitivas dessa etapa do adoecimento

Introducción: las acciones de educación para la salud son incipientes en algunos hospitales. Cuando se trata de desplegar estas acciones al final de la vida, los equipos encargados tienen ciertas limitaciones. Objetivo: identificar acciones de educación en salud dirigidas a pacientes intrahospitalarios en el final de la vida y a sus cuidadores (formales e informales), así como evaluar la evidencia de tales acciones. Síntesis de contenido: el estudio se llevó a cabo entre diciembre de 2019 y enero de 2020 en las bases de datos Web of Science, Scopus y MEDLINE. Se identificaron 6.762 artículos, de los cuales 42 forman parte del análisis por ser artículos originales o de revisión escritos en portugués, español, inglés o francés, cuya muestra estuvo conformada por pacientes mayores de 19 años con enfermedad avanzada a al final de la vida, o sus cuidadores o los profesionales de la salud encargados de su cuidado. Los datos fueron agrupados por similitud de las acciones, siguiendo a Polit y Beck, y el nivel de evidencia evaluado, según Melnyk y Fineout-Overholt. El video fue la acción más recomendada, seguida de los folletos. Las unidades temáticas fueron "Acciones para el control del dolor", "Narrativas sobre el final de la vida", "Planificación anticipada de cuidados", "Diálogo sobre cuidados paliativos" y "Comunicación y final de la vida". Conclusiones: las acciones de educación en salud al final de la vida deben considerar las tecnologías de la información y la comunicación, además de las condiciones socioculturales, clínicas y cognitivas durante esta etapa de la enfermedad

Introduction: Health education actions are a challenge in some hospitals. When it comes to performing such actions in the face of end-of-life situations, health teams have some limitations. Objective: To identify health education actions for in-hospital dying patients and their informal and formal caregivers, and to assess the level of evidence of such actions. Content synthesis: Study conducted between December 2019 and January 2020, in the databases Web of Science, Scopus and MEDLINE. A total of 6.762 articles were identified. Among these, 42 articles were considered as they met the following inclusion criteria: original or review article written in Portuguese, Spanish, English, or French, whose sample consisted of patients over 19 years with a terminal disease or at the end of their lives, or their caregivers or health professionals in charge of their care. The data were grouped by similarity of the actions deployed, following Polit and Beck, and the level of evidence evaluated, according to Melnyk and Fineout-Overholt. The video was the action with greater force of recommendation, followed by booklets. The thematic units were: "Actions for pain control", "Narratives about the end-of-life", "Advance care planning", "Talking about palliative care", and "Communication and end-of-life". Conclusions: Health education actions at the end of life should consider information and communication technologies, in addition to the sociocultural, clinical, and cognitive conditions during this stage.

Association between ambient air pollution and fatal stroke mortality in Songjiang District of Shanghai / 上海预防医学

Objective To investigate the risk of fatal stroke mortality associated with short-term exposure to air pollution， and to determine the susceptible population. Methods In this study， daily stroke mortalities of adults between 2012 and 2014 in Songjiang District， Shanghai were collected. Time-stratified case-crossover approach was used to assess the association between daily concentrations of air pollutants and fatal stroke mortalities. Results This study included 514 patients who died from acute strokes. The average concentrations during the study period were 77.45 μg·m-3 for PM2.5， 21.22 μg·m-3 for SO2， and 57.59 μg·m-3 for NO2. The fatal stroke mortality of adults under the age of 65 was found to be significantly associated with NO2. At the time of a Lag of 2 d and 03 d， a significantly higher risk of fatal stroke mortality in relation to NO2 exposure was observed， and the OR values of ischemic stroke mortality for people were 3.86 （1.53-9.75） and 5.83 （1.40-24.34） respectively. People over the age of 65 were more sensitive to increased PM2.5 concentrations， at the time of a Lag of 03 d， fatal strokes increased by 28% when PM2.5 levels increased. A significantly higher risk of fatal stroke mortality in relation to increase of NO2 concentration was observed among people who were overweight or obese. Conclusion A significantly higher risk of fatal stroke mortality is associated with the increase of PM2.5 and NO2. The results also suggest that the susceptible population should take additional precautions to avoid or reduce the risk of fatal strokes.

Clinical, skeletal muscle pathological and genetic characteristics of fatal infantile hypertonic myofibrillar myopathy / 中华实用儿科临床杂志

Objective:To investigate the clinical, skeletal muscle pathological, and genetic characteristics of fatal infantile hypertonic myofibrillar myopathy (FIHMM).Methods:The clinical manifestations, laboratory assessments data and gene sequencing results of 10 patients diagnosed with FIHMM in Shenzhen Children′s Hospital from February 2017 to April 2021 were retrospectively analyzed.Magnetic resonance imaging (MRI) of both musculoskeletal system and the brain, and electromyogram (EMG) were performed in 3 cases, while muscle biopsy was performed in 2 cases.Results:Among these 10 cases, 1 case was from Northeast China and 1 case from East China, while the rest 8 cases were from South China.Eight of the 10 patients were male, and the other 2 cases were female.They were all born normal and not related to each other.The age of onset varied from 2 to 12 months.The main clinical manifestations for all the patients were progressive rigidity of the rectus abdominis (8 cases), neck muscles (7 cases), rectus abdominis (2 cases) and intercostal muscles (1 case), resulting in respiratory failure.Mildly to moderately elevated serum creatine kinase level was detected (436-5 804 IU/L) (reference range: 24-229 IU/L). Complex repetitive discharges can be seen in the EMG, without any myotonic potential.Muscle fiber degeneration, necrosis, and vacuolar degeneration were noted in the histopathological examination of the vastus lateralis and rectus abdominis.An abnormal red granular deposit was observed in a portion of the field of the modified Gomory Trichrome staining.Immunohistochemistry showed substantial deposition of desmin.Under the electron microscopy, the sarcomere structure of the muscle fibers was seriously disordered, with the destruction of Z-bands and the presence of granular deposits.The whole-exome sequencing identified the same homozygous variation c. 3G>A, p.Met1? of CRYAB gene in all the patients, but heterozygous variation in their parents. Conclusions:Axial muscles involvement, such as rectus abdominis rigidity, is the main clinical characteristic of FIHMM.c.3G>A, p.Met1? mutation in the CRYAB gene is a hotspot mutation in Chinese children.

Preocupação e medo como preditores de fatalismo por COVID-19 no cotidiano de trabalho dos enfermeiros / Worry and fear as predictors of fatalism by COVID-19 in the daily work of nurses / Preocupación y miedo como predictores de fatalismo por COVID-19 en lo cotidiano laboral de enfermeros

Resumo Objetivo: analisar a relação entre a preocupação e o medo da COVID-19 com o fatalismo no cotidiano de trabalho dos enfermeiros. Método: estudo transversal analítico, realizado com 449 enfermeiros. A coleta de dados foi realizada por meio de instrumentos validados no Peru. Na análise, foram utilizados o teste de Shapiro-Wilk e o coeficiente de correlação de Spearman, sendo estimados dois modelos de regressão múltipla, com seleção de variáveis por etapas. Resultados: os enfermeiros apresentaram nível moderado de fatalismo e baixo nível de medo e preocupação com a COVID-19. O primeiro modelo estatístico, que incluiu variáveis sociodemográficas, explica apenas 3% da variância de fatalismo. No entanto, um segundo modelo que inclui medo e percepção explica 33%. Conclusão: a preocupação, o medo e ter sido diagnosticado com COVID-19 foram fatores preditores de fatalismo. Sugere-se a implementação de intervenções psicoemocionais no cotidiano de trabalho, voltadas para profissionais de Enfermagem que apresentem altos níveis de medo ou preocupação, para reduzir o fatalismo e, assim, prevenir consequências fatais da pandemia e promover a saúde.

Abstract Objective: to analyze the relationship between the concern and fear of COVID-19 with fatalism in the daily work of nurses. Method: analytical cross-sectional study carried out with a total of 449 nurses. Data collection was performed using instruments validated in Peru. In the analysis, the Shapiro-Wilk test and the Spearman correlation coefficient were used, and two multiple regression models were estimated, with variable selection in stages. Results: nurses had a moderate level of fatalism and a low level of fear and concern about COVID-19. The first statistical model, which included sociodemographic variables, explains only 3% of the fatalism variance. However, a second model that includes fear and perception explains 33% of it. Conclusion: Worry, fear and having been diagnosed with COVID-19 were predictors of fatalism. It is suggested the implementation of psycho-emotional interventions in daily work - aimed at Nursing professionals who present high levels of fear or concern - to reduce fatalism and prevent fatal consequences of the pandemic and promote health.

Resumen Objetivo: analizar la relación entre la preocupación y el miedo al COVID-19 con el fatalismo, en lo cotidiano laboral de los enfermeros. Método: estudio transversal tipo analítico, realizado en 449 enfermeros. La recolección de datos se realizó mediante instrumentos validados en Perú. En el análisis se utilizó la prueba de Shapiro-Wilk, el coeficiente de correlación de Spearman y se estimaron dos modelos de regresión múltiple, con selección de variables por pasos. Resultados: los enfermeros presentaron un nivel moderado de fatalismo y un nivel bajo de miedo y preocupación por la COVID-19. El primer modelo estadístico que incluyó las variables sociodemográficas apenas explica un 3% de la varianza de fatalismo; sin embargo, un segundo modelo que incluye el miedo y la percepción explica el 33%. Conclusión: la preocupación, el miedo y el haber sido diagnosticado con COVID-19 fueron factores predictores de fatalismo. Se sugiere implementar intervenciones psicoemocionales en el cotidiano laboral - focalizadas en profesionales de Enfermería que presenten altos niveles de miedo o preocupación - para reducir el fatalismo y de esta manera prevenir consecuencias fatales de la pandemia y promover la salud.

Análise dos desfechos maternos e fetais relacionados à COVID-19 durante a gestação / Analysis of maternal and fetal outcomes related to COVID-19 during pregnancy

O SARS-CoV-2 é um vírus RNA transmitido pelo contato direto ou indireto por gotículas infectadas. No que se refere à COVID-19 e à gestação, referências apontam que nesse período as mulheres possuem maior susceptibilidade a complicações obstétricas e perinatais. O presente estudo objetiva compreender e compilar aspectos da infecção e os principais desfechos negativos maternos e fetais documentados na literatura atual, relacionados à infecção pelo novo coronavírus durante a gestação. Trata-se de uma revisão integrativa de literatura embasada pela análise de 2.441 artigos no total, dos quais 62 foram incluídos na pesquisa, sendo 38 deles da base de dados PubMed e 24 da BVS (Biblioteca Virtual em Saúde), nos idiomas inglês e português. Em conclusão, mulheres grávidas com diagnóstico da patologia podem precisar de assistência de alta complexidade. A associação à doença pode apresentar riscos ou complicações como coagulopatias, pré-eclâmpsia, prematuridade e outros desfechos negativos que serão abordados neste artigo.(AU)

SARS-CoV-2 is an RNA virus, transmitted by direct or indirect contact by infected droplets. Regarding to COVID-19 and pregnancy, references indicate that during this period, women are more susceptible to obstetric and perinatal complications. This study aims to understand and compile aspects of infection and the main negative maternal and fetal outcomes documented in the current literature, related to the infection by the new coronavirus during pregnancy. This is an integrative literature review based on the analysis of 2,441 articles in total, of which 62 were included in the survey, 38 from the PubMed database and 24 from BVS (Biblioteca Virtual em Saúde) in English and Portuguese languages. In conclusion, pregnant women diagnosed with the pathology may need highly complex assistance. The association with the disease may present risks for complications such as coagulopathies, pre-eclampsia, prematurity and other negative outcomes that will be addressed in this article.(AU)

Rickettsia conorii infection with fatal complication

ABSTRACT Rickettsial diseases (RD) are a group of endotheliotropic infectious diseases caused by different species of genera Rickettsia. RD are not an uncommon disease and may be misdiagnosed during the evaluation of acute febrile illness due to a lack of reliable serological marker and diagnostic culture methods. Clinical manifestation of RD varies from febrile illness with rashes and myalgia to fatal complications such as shock and respiratory failure. We describe a case of a young male who presented initially with acute febrile illness, followed by shock and respiratory failure, and unfortunately succumbed to death. A post-mortem examination showed histological features of endotheliotropic infection, such as interstitial / perivascular edema in various organs and noncardiogenic pulmonary edema (suggesting increased vascular permeability) and evidence of vasculitis in the lung, liver, and intestines. Molecular studies performed from lung, liver, and kidney tissue confirm the diagnosis of spotted fever group rickettsial disease due to Rickettsia conorii.

Chronic inflammatory diseases, subclinical atherosclerosis, and cardiovascular diseases: Design, objectives, and baseline characteristics of a prospective case-cohort study ‒ ELSA-Brasil

Abstract Objectives This analysis describes the protocol of a study with a case-cohort to design to prospectively evaluate the incidence of subclinical atherosclerosis and Cardiovascular Disease (CVD) in Chronic Inflammatory Disease (CID) participants compared to non-diseased ones. Methods A high-risk group for CID was defined based on data collected in all visits on self-reported medical diagnosis, use of medicines, and levels of high-sensitivity C-Reactive Protein >10 mg/L. The comparison group is the Aleatory Cohort Sample (ACS): a group with 10% of participants selected at baseline who represent the entire cohort. In both groups, specific biomarkers for DIC, markers of subclinical atherosclerosis, and CVD morbimortality will be tested using weighted Cox. Results The high-risk group (n = 2,949; aged 53.6 ± 9.2; 65.5% women) and the ACS (n=1543; 52.2±8.8; 54.1% women) were identified. Beyond being older and mostly women, participants in the high-risk group present low average income (29.1% vs. 24.8%, p < 0.0001), higher BMI (Kg/m2) (28.1 vs. 26.9, p < 0.0001), higher waist circumference (cm) (93.3 vs. 91, p < 0.0001), higher frequencies of hypertension (40.2% vs. 34.5%, p < 0.0001), diabetes (20.7% vs. 17%, p = 0.003) depression (5.8% vs. 3.9%, p = 0.007) and higher levels of GlycA a new inflammatory marker (p < 0.0001) compared to the ACS. Conclusions The high-risk group selected mostly women, older, lower-income/education, higher BMI, waist circumference, and of hypertension, diabetes, depression, and higher levels of GlycA when compared to the ACS. The strategy chosen to define the high-risk group seems adequate given that multiple sociodemographic and clinical characteristics are compatible with CID.

Factors influencing the length of emergency room stay and hospital stay in non-fatal bicycle accidents: A retrospective analysis / 中华创伤杂志(英文版)

PURPOSE@#Lengthy hospitalization places a burden on patients and healthcare resources. However, the factors affecting the length of hospital stay (LHoS) and length of emergency room stay (LERS) in non-fatal bicycle accidents are currently unclear. We investigated these factors to inform efforts to minimize hospitalization.@*METHODS@#We performed a retrospective analysis of data from non-fatal injured bicyclists admitted to the Emergency and Critical Care Center at Kyoto Medical Center between January 2012 and December 2016. We measured LHoS, LERS, mechanism of injury, head injury prevalence, polytrauma, operations performed, injury severity score (ISS), abbreviated injury scale (AIS) score, maximum AIS score, and trauma and injury severity score probability of survival. We conducted multiple regression analysis to determine predictors of LHoS and LERS.@*RESULTS@#Within the study period, 82 victims met the inclusion and exclusion criteria and were included. Mean age was (46.0 ± 24.7) years. Overall mean LHoS was (16.8 ± 25.2) days, mean LERS was (10.6 ± 14.7) days, median ISS was 9 (interquartile range (IQR): 3-16), median maximum AIS was 3 (IQR: 1-4), and median trauma and injury severity score probability of survival was 98.0% (IQR: 95.5%-99.6%). Age, maximum AIS, ISS, and prevalence of surgery were significantly greater in long LHoS and LERS group compared with short LHoS and LERS group (p < 0.05). Performance of surgery independently explained LHoS (p = 0.0003) and ISS independently explained LERS (p = 0.0009).@*CONCLUSION@#Surgery was associated with long hospital stays and ISS was associated with long emergency room stays. To improve the quality life of the bicyclists, preventive measures for reducing injury severity or avoiding injuries needing operation are required.

Genetic Prion Disease: Insight from the Features and Experience of China National Surveillance for Creutzfeldt-Jakob Disease / 神经科学通报·英文版

Human genetic prion diseases (gPrDs) are directly associated with mutations and insertions in the PRNP (Prion Protein) gene. We collected and analyzed the data of 218 Chinese gPrD patients identified between Jan 2006 and June 2020. Nineteen different subtypes were identified and gPrDs accounted for 10.9% of all diagnosed PrDs within the same period. Some subtypes of gPrDs showed a degree of geographic association. The age at onset of Chinese gPrDs peaked in the 50–59 year group. Gerstmann–Sträussler–Scheinker syndrome (GSS) and fatal familial insomnia (FFI) cases usually displayed clinical symptoms earlier than genetic Creutzfeldt–Jakob disease (gCJD) patients with point mutations. A family history was more frequently recalled in P105L GSS and D178N FFI patients than T188K and E200K patients. None of the E196A gCJD patients reported a family history. The gCJD cases with point mutations always developed clinical manifestations typical of sporadic CJD (sCJD). EEG examination was not sensitive for gPrDs. sCJD-associated abnormalities on MRI were found in high proportions of GSS and gCJD patients. CSF 14-3-3 positivity was frequently detected in gCJD patients. Increased CSF tau was found in more than half of FFI and T188K gCJD cases, and an even higher proportion of E196A and E200K gCJD patients. 63.6% of P105L GSS cases showed a positive reaction in cerebrospinal fluid RT-QuIC. GSS and FFI cases had longer durations than most subtypes of gCJD. This is one of the largest studies of gPrDs in East Asians, and the illness profile of Chinese gPrDs is clearly distinct. Extremely high proportions of T188K and E196A occur among Chinese gPrDs; these mutations are rarely reported in Caucasians and Japanese.

Genetic Prion Disease: Insight from the Features and Experience of China National Surveillance for Creutzfeldt-Jakob Disease / 神经科学通报·英文版

Human genetic prion diseases (gPrDs) are directly associated with mutations and insertions in the PRNP (Prion Protein) gene. We collected and analyzed the data of 218 Chinese gPrD patients identified between Jan 2006 and June 2020. Nineteen different subtypes were identified and gPrDs accounted for 10.9% of all diagnosed PrDs within the same period. Some subtypes of gPrDs showed a degree of geographic association. The age at onset of Chinese gPrDs peaked in the 50-59 year group. Gerstmann-Sträussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI) cases usually displayed clinical symptoms earlier than genetic Creutzfeldt-Jakob disease (gCJD) patients with point mutations. A family history was more frequently recalled in P105L GSS and D178N FFI patients than T188K and E200K patients. None of the E196A gCJD patients reported a family history. The gCJD cases with point mutations always developed clinical manifestations typical of sporadic CJD (sCJD). EEG examination was not sensitive for gPrDs. sCJD-associated abnormalities on MRI were found in high proportions of GSS and gCJD patients. CSF 14-3-3 positivity was frequently detected in gCJD patients. Increased CSF tau was found in more than half of FFI and T188K gCJD cases, and an even higher proportion of E196A and E200K gCJD patients. 63.6% of P105L GSS cases showed a positive reaction in cerebrospinal fluid RT-QuIC. GSS and FFI cases had longer durations than most subtypes of gCJD. This is one of the largest studies of gPrDs in East Asians, and the illness profile of Chinese gPrDs is clearly distinct. Extremely high proportions of T188K and E196A occur among Chinese gPrDs; these mutations are rarely reported in Caucasians and Japanese.