The Case Report on Dentatorubral-Pallidoluysian Atrophy / 罕见病研究

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenera-tive disease rarely reported in China. Its symptoms include: cerebellar ataxia, epilepsy, dementia, myoclonus and choreoathetosis. We reported one case of adult-onset DRPLA family in the article. The female proband developed ataxia at the age of 45, followed by cognitive impairment and possible seizure. Five people in her family had similar symptoms. In addition, cranial MRI showed atrophy of the brainstem and cerebellar, as well as diffuse white matter lesions. Analysis of the ATN1 gene showed CAG repeat sizes to be 14/54 in the proband. Besides, we reviewed relevant literature published in recent years to improve the understanding of the disease, including its clinical manifestations, genetic characteristics, diagnosis and treatments.

La enfermedad como signo y las formas simbólicas del cuerpo enfermo en el horizonte de comprensión de la dermatología decimonónica / The disease as sign and the symbolic forms of the sick body on the horizon of comprehension d the dermatology nineteenth

Resumen La dermatología clínica del siglo XIX fundamenta y despliega un pensamiento por similitud en una estética de la representación de lo que puede ser comprendido como enfermedad. Así, las superficies patológicas suministran un material de reflexión y apropiación del cuerpo enfermo donde confluyen cuerpo, lenguaje y acontecimiento para darle sentido a las alteraciones que confluyen en el estado patológico. Este texto reflexiona sobre esas formas simbólicas del nombrar la enfermedad que tienen lugar en descripciones, observaciones clínicas, tratados de dermatología, atlas de anatomía patológica o álbumes iconográficos del siglo XIX, donde es posible ver un régimen de visualización de lo patológico que se revela en la percepción médica de la lesión y su localización. El análisis de las enfermedades de la piel que desfiguran al rostro a partir de algunos registros clínicos e iconográficos de finales del siglo XIX pone en juego un vínculo entre imagen y palabra en términos de una eficacia dermatológica en las maneras de capturar la experiencia de la enfermedad, que tiene al cuerpo enfermo como teatro de sensibilidad performativa.

Abstract The clinical dermatology of the nineteenth century bases and displays a thought by similarity in an aesthetic representation of what can be understood as disease. Thus, the pathological surfaces provide a material of reflection and appropriation of the sick body where body, language and event converge to give meaning to the alterations that converge in the pathological state. This text reflects on these symbolic forms of naming the disease that occur in descriptions, clinical observations, treaties of dermatology, Atlas of pathological anatomy or iconographic albums of the nineteenth century, where it is possible to see a regime of visualization of the pathological that reveals itself in the medical perception of the lesion and its location. The analysis of skin diseases that disfigure the face from some clinical and iconographic records from the end of the 19th century puts into play a link between image and word in terms of dermatological efficacy in ways of capturing the experience of the disease, which has the sick body as a theater of performative sensitivity.

Clinical manifestations and imaging analysis of cervicogenic headache / 中国骨伤

OBJECTIVE@#To investigate the clinical characteristics and mechanism of cervicogenic headache.@*METHODS@#Fifty-seven patients with cervicogenic headache who were treated from May 2013 to December 2017 and had complete imaging data were selected, including 18 males and 39 females with an average age of(43.26±10.39) years old ranging from 20 to 63 years old. The duration of the disease was 4 months to 35 years with a mean of (11.74±9.47) years. The pain situation, iconography and Tinel sign were analyzed.@*RESULTS@#The patients with cervicogenic headache often had bilateral pain. The regions mainly concentrated in the temporal region, with occipital, head or orbit pains. The VAS scores decreased with the duration of the disease. There were many cases of disc herniation(91.30%), vertebral instability(73.91%), atlantoaxial displacement(56.52%), curvature change of cervicogenic vertebra(54.35%). The number of positive Tinel sign points was between 3 and 24 (13.58±5.8) per patient. The number and extent of Tinel sign were significantly different between the affected side and healthy side(<0.05). C₂,₃ facet joints(92.98%), post mastoid(89.47%), occipital concavity(89.47%), C₃,₄ facet joints(84.21%), third occipital nerve(80.70%) were the positive Tinel sign points in patients with cervicogenic headache.@*CONCLUSIONS@#The iconography changes of cervicogenic headache and Tinel sign may contribute to the clinical diagnosis and mechanism of the disease.

Iconografia na investigação e intervenção de processos psicossociais / Iconography in research and intervention of psychosocial processes

Discute a iconografia como instrumento metodológico de investigação e intervenção psicossocial, a partir dos conceitos vigotskianos de imaginação e criação. Descreve a construção de um acervo iconográfico (desenhos, fotografias e vídeos) produzido em pesquisas com crianças e jovens de comunidades litorâneas. O método utilizado identificou, catalogou, descreveu e interpretou imagens produzidas em investigações e intervenções psicossociais do Grupo de Pesquisa entre 2007 e 2012. O inventário resultou em 72 desenhos, 127 fotografias e 42 vídeos. Os resultados indicam o uso do método como autoimagens(desenhos e fotografias), registro de ações(vídeos e fotografias) e acervo pessoal (fotografias). A análise de conteúdo a partir dos conceitos de imaginação e criação identificou desenhos infantis como expressão da realidade da vida cotidiana; as fotografias demonstram a apropriação de elementos espaciais, históricos e culturais das comunidades e o protagonismo de crianças e jovens no contexto comunitário; os vídeos adicionam movimento e áudio aos processos identificados nas produções de desenhos e fotografias. Conclui-se que instrumentos iconográficos potencializam pesquisas e intervenções com crianças e jovens; a criação imagética permite a objetivação da imaginação e a expressão de subjetividades.

Discusses the iconography as a methodological tool for research and psychosocial intervention from Vygotsky's concepts of imagination and creation. Describes the construction of an iconographic collection (drawings, photographs and videos), produced in research with children and young people from coastal communities. The method identified, cataloged, described and interpreted images produced in psychosocial research and interventions of the Research Group between 2007 and 2012. The inventory resulted in 72 drawings, 127 photos and 42 videos. The results indicate the use of method as self-images(drawings and photographs), actions recording(videos and photographs) and private collection(photographs). The content analysis based on the concepts of imagination and creation identified children's drawings as expression of the reality of everyday life; the photographs demonstrate the spatial, historical and cultural appropriation of elements within the community and the protagonism of children and youth in the communitarian context; the videos adds motion and audio to the identified processes at the productions of drawings and photographs. In conclusion, the iconographic instruments potentiate research and interventions with children and youth; imagetic creation enables the objectification of imagination and the expression of subjectivities.

O uso das fontes na condução da pesquisa histórica / El uso de las fuentes en la conducción de la investigación histórica / The use of sources in historical research

RESUMO Objetivo: instrumentalizar pesquisadores, estudantes e interessados em história da enfermagem e saúde na condução de estudos com a metodologia da história oral, documental e estudos iconográficos. Método: reflexão teórico-metodológica realizada à luz da experiência e conhecimento dos autores que orienta a compreensão da utilização da história oral, documental e iconográfica. Resultados: explicita os tipos de abordagem e seus detalhamentos desde a etapa de coleta, organização, tratamento, análise e interpretação dos dados. A condução da Pesquisa Histórica tem relação direta com a preservação e a qualidade dos documentos, situando-se no campo das pesquisas qualitativas que utilizam estratégias metodológicas diferenciadas e validadas para a construção do conhecimento histórico. Conclusão: o conhecimento e a instrumentalização sobre o desenvolvimento de pesquisas históricas em Enfermagem e saúde, utilizando adequadamente os métodos, técnicas ou disciplinas para sua implementação, propicia a melhoria na qualidade dos estudos sóciohistóricos e sua utilização.

RESUMEN Objetivo: Instrumentalizar a los investigadores, estudiantes e interesados ​​en historia de la enfermería y salud en la conducción de estudios con la metodología de la historia oral, documental y estudios iconográficos. Método: reflexión teórica metodológica realizada a la luz de la experiencia y conocimiento de los autores que orienta la comprensión de la utilización de la historia oral, documental e iconográfica. Resultados: explicita los tipos de enfoque y sus detallados desde la etapa de recolección, organización, tratamiento, análisis e interpretación de los datos. La conducción de la Investigación Histórica tiene una relación directa con la preservación y la calidad de los documentos, situándose en el campo de las investigaciones cualitativas que utilizan estrategias metodológicas diferenciadas y validadas para la construcción del conocimiento histórico. Conclusión: el conocimiento y la instrumentalización sobre el desarrollo de investigaciones históricas en Enfermería y salud, utilizando adecuadamente los métodos, técnicas o disciplinas para su implementación, propicia la mejora en la calidad de los estudios socioeconómicos y su utilización.

ABSTRACT Objective: to equip, researchers, students and anyone interested in the history of nursing and health to develop research using oral and documentary history and iconographic studies. Method: theoretical methodological reflection in the light of the authors' experience and knowledge, guiding the understanding about the use of oral, documentary and iconographic history. Results: expresses the types of approaches and their details, ranging from the collection, organization and treatment to the analysis and interpretation of the data. The development of Historical Research is directly related with the preservation and quality of the documents, as part of qualitative research that uses different and validated methodological strategies to construct historical knowledge. Conclusion: the knowledge and tools to develop historical research in Nursing and health, appropriately using the methods, techniques or disciplines for its implementation, favor improvements in the quality and use of sociohistorical studies.

Application of ultrasonography in diagnosis and differential diagnosis of Takayasu arteritis / 吉林大学学报(医学版)

Objective:To investigate the clinical application value of ultrasound in the diagnosis and differential diagnosis of Takayasu arteritis (TA ). Methods: A total of 39 suspected TA patients were examined by ultrasonography,erythrocyte sedimentation rate (ESR),C reactive protein (CRP)and CT angiography (CTA), and the ultrasound examination results were compared and analyzed with the final chinical diagnosis results. Results:Of the 39 cases of suspected TA patients which had been examined by ultrasound,33 cases were diagnosed as TA,2 cases were diagnosed as atherosclerosis (AS),2 cases were diagnosed as fibromuscular dysplasia (FMD) and 2 cases were diagnosed as thromboangiitis obliterans (TAO).Compared with final chinical diagnosis results, 1 FMD patient was misdiagnosed as TA,and the rest of the diagnostic results were consistent with the final chinical diagnosis results.Conclusion:Ultrasound examination can provide objective and accurate basis for the diagnosis and differential diagnosis of TA,and it is an effective method of imaging examination.

Application of OBI system in intensity-modulated radiotherapy for nasopharyngeal carcinoma / 中华放射肿瘤学杂志

Objective To examine the application of On-Board Imaging (OBI) system-based image-guided radiotherapy (IGRT) in the improvement of the precision of intensity-modulated radiotherapy (IMRT) for nasopharyngeal carcinoma.Methods Ten patients with nasopharyngeal carcinoma were treated with IMRT using the OBI system. The IGRT images after positioning, position adjustment, and treatment were observed and recorded to investigate the image difference between CT simulation and IGRT. Results The deviations in the x (lateral), y (cranial-caudal), and z (ventral-dorsal) directions between CT simulation and IGRT images were 0.22±1.00 mm,-0.37±1.28 mm, and 0.04±1.36 mm, respectively, after positioning, 0.29±0.76 mm,-0.04±0.78 mm, and -0.01±0.92 mm, respectively, after position adjustment, and 0.20±0.78 mm, 0.16±0.80 mm, and 0.05±0.92 mm, respectively, after treatment. The probabilities of a ≤1 mm deviation in the x, y, and z directions were 81.0%, 77.6%, and 88.2%, respectively, after positioning, 92.5%, 96.4%, and 96.4%, respectively, after position adjustment, and 91.7%, 94.9%, and 96.8%, respectively, after treatment. Conclusions The application of OBI system-based IGRT is very important in the improvement of the precision of fractionated IMRT for patients with nasopharyngeal carcinoma. The position of the patient should be adjusted based on the IGRT image after positioning in order to correct set-up error and effectively increase the precision of fractionated IMRT.

Imaging features and prognostic analysis of high-grade renal clear cell carcinoma / 中华泌尿外科杂志

Objective Investigating the CT features and related prognostic factors of high-grade renal clear cell carcinoma.Methods The data of 141 patients with renal clear cell carcinoma treated by radical nephrectomy in our hospital from November 2012 to April 2017 were analyzed retrospectively.There were 102 males and 39 females.Age from 30 to 86 years old.The tumors were located in the left side in 73 cases and 68 in the right.The tumor size ranged from 1.6 cm to 12.7 cm.50 cases of clinical stage T1a,stage T1b 67 cases,24 cases above T2.All patients had CT examination before operation.According to the postoperative pathological nuclear grade,patients were divided into high grade group (Ⅲ-Ⅳ) and low grade group (grade Ⅰ-Ⅱ),clinical data and CT findings were compared between the two groups (tumor size,capsule,CT,with or without leafs,with or without hemorrhagic necrosis),survival situation after the operation.Result The results of CT examination of 141 cases of this study indicated there were 109 cases of complete capsule,85 cases of tumor showed lobulation,102 cases of tumor showed hemorrhagic necrosis,the average CT value is 10-72 HU,35.4 HU in average;enhanced CT value is 32-308 HU,102.1 HU in average.After pathological examination,the nuclear classification was 66 cases in high grade group and 75 cases in low grade group.The CT examination in thc high grade group and the low grade group showed the number of cases with complete capsule [44 cases (33.3％) vs.65 cases (13.3％)] and plain CT scan value [(38.9 ± 1.1) HU) vs.(32.3 ± 1.1) HU],the difference was statistically of significance (P ＜0.01).The high level of patients in group T1 and group above T2 stage were 46 cases and 20 cases.And the cases of low grade group were 71 cases and 4 cases,there was significant difference between two groups (P ＜ 0.01).The 141 cases were followed up for 2-58 months,with an average of 26.4 months.There was statistical significance difference between the two groups of T1 patients and patients above T2 (P ＜ 0.01).The results of CT examination were compared.There were significant differences in the case of intact capsule and the value of CT scan (P ＜ 0.01).The overall survival rate between the two groups was statistically significant (P ＜ 0.05).Conclusions The CT examination of high-grade renal cell carcinoma shows that most of the capsule is not complete and the CT value of the scan is much higher.The pathological grading of renal cell careinoma indicates the malignancy of tumor cells,which is closely related to prognosis.

A Clinical Research of Self-made Jian-pi-hua-zhuo-xiao-yu Tang in the Treatment of Non-alcoholic Fatty Liver Disease / 浙江中医药大学学报

Objection]To study the clinical effect of self-prescribed jianpihuazhuoxiaoyu decoction in treating the patients with non-alcoholic liver of fat. [Methods]45 patients were treated with self-prescribed Jianpihuazhuoxiaoyu decoction in treatment group and other 45 patients with Polyene Phosphatidylcholine Capsules and Ursodeoxycholic Acid. Observe the patients'body mass index(BMI),radiological changes,liver function(AST/ALT) and lipids(TG/TC/HDL/LDL). [Results] The total effective rates were 91.3%,82.6% respectively, compared with the control group,the treatment group significantly improved liver function,lipid content decreased,obesity weight was effectively controlled and reduced,sonogram also showed improvement in the texture of the liver,compared with control group there were significant differences(P<0.05),difference was statistically significant.

Aloysio de Castro, o precursor da neurossemiologia no Brasil / Aloysio de Castro, the pioneer of neuro-semiology in Brazil

Este artigo trata de um pouco da vida e obra de um excepcional professor,médico, crítico literário e musical, compositor e poeta. Aloysio de Castro perfez um curso médico excepcional e o concluiu de modo brilhante e inovador com tese a respeito das desordens da marcha, posteriormente considerada uma "verdadeira obra literária". Em 1907, de volta ao Rio de Janeiro, após ter passado por treinamento em Paris com Pierre Marie, instituiu o primeiro ambulatório didático da cidade do Rio de Janeiro, e, alguns anos depois (1914), publicou o primeiro livro brasileiro totalmente voltado para a neurossemiologia. Nesse livro, Aloysio de Castro mais uma vez inova, apresentando fotografias - e não desenhos - e trechos de filmes dos seus pacientes, tornando-se também um dos pioneiros na cinematografia como método de investigação e de ensino médico no Brasil

This article deals with some aspects of the life and work of a greatteacher and physician, who was also musical and literary critic, composerand poet. Aloysio de Castro fulfilled an exceptional medicalschool course and concluded it in a brilliant and innovative way presentinga thesis on gait disorders, which came to be defined as a "genuine literary work". In 1907, back to Rio de Janeiro, after havingundergone training in Paris with Pierre Marie, he installs the first instructionalclinic in the city of Rio de Janeiro, and some years later (1914), publishes the first Brazilian book devoted entirely to neurosemiology. In this book Aloysio de Castro once again innovates bypresenting photographs - and not drawings - and film clips of his patients,and thus becomes one of the pioneers in the cinematographyas a method of research and medical education in Brazil

Representações iconográficas e literárias da produção de alimentos: espaços, atores e ideologias entre Antiguidade e Idade Média / The iconographic and literary representations of food production: spaces, actors and ideologies between Antiquity and Middle Ages

Propõe-se um estudo iconográfico e literário do trabalho sazonal como ciclo produtivo, entre Antiguidade e Idade Média Tardia. Procurou-se estabelecer laços entre iconografias e economia e sociedade que as produziram. A representação auspiciosa prevalece, no panorama histórico das iconografias ligadas à previsão e à esperança de abundância. No âmbito da sociedade medieval elas estão fortemente atreladas ao tema da mudança das estações, da passagem cíclica temporal, do percurso de nascimento, morte e ressurreição. As representações dos trabalhos e das ocupações dos meses se transformam, e essa transformação é, muito mais que um reflexo, uma reflexão da sociedade sobre espaço e sobre atores do ciclo de produção alimentar. A Antiguidade legou à Idade Média uma variedade ampla de imagens fundamentadas, tanto na noção de personificação como de referências para as atividades ligadas aos ciclos sazonais, legado que será avaliado e modificado com base na nova experiência religiosa e social que permeia o período de maior presença dos ciclos dos meses ilustrados pelas atividades de produção do campo.

An iconographic and literary study on seasonal work as productive cycle between antiquity and the late middle ages is proposed, looking for boundaries between the iconographies and economy and society that produced them. The auspicious representation prevails in the historical view of iconographies associated with the prediction and hope of abundance. Within the medieval society they are deeply bounded to main themes such as season changes, cyclic passage of time, journey through birth, death and resurrection. The representations of monthly work and activities change, and this change is much more than a mirror reflection, a reflection of society on the space and actors of the food production cycle. The legacy of antiquity to the middle ages is a great amount of images based on notions of personification as well of reference for the activities associated with the seasonal changes, a legacy that will be assessed and changed based on the new social and religious experience. These permeate the century during which most of the month cycles illustrated by productive works in the field are produced.

Natureza e civilização: os painéis decorativos do Salão Nobre do Teatro Amazonas / Nature and civilization: the decorative panels of the Teatro Amazonas foyer

Os painéis decorativos do salão nobre do Teatro Amazonas singularizam-se em relação ao restante da iconografia que decora o edifício, inaugurado em 1896. Na ocasião, as capitais do Pará e do Amazonas, enriquecidas pela economia gomífera, passaram por significativas transformações no tecido urbano e nas formas de sociabilidade, projetando-se nacional e internacionalmente. O artigo procura identificar paralelos entre as representações paisagísticas da natureza amazônica que decoram o salão nobre do edifício, a construção da cidade moderna e os usos sociais do Teatro Amazonas, símbolo do período e emblema da elite enriquecida que se afirmava no cenário regional e nacional. O Teatro Amazonas ocupou o centro da vida social da época, e em seu salão nobre ocorreram verdadeiros rituais de 'civilização' em que os convidados - brasileiros ou estrangeiros - tinham como cenário privilegiado a natureza amazônica representada nos painéis parietais decorativos.

The decorative panels in the foyer of the Teatro Amazonas, inaugurated in 1896, stand out from the rest of the building's ornamental iconography. The rubber trade brought great wealth to the capitals of Pará and Amazonas and as these cities gained national and international fame, their urban fabric and forms of sociability underwent significant changes. The article looks for parallels between the landscape representations of Amazonian nature adorning the building's foyer, the construction of the modern city, and social uses of the Teatro Amazonas, symbol of the era and emblem of the newly rich elite, who were then taking their place on the regional and national stages. The Teatro Amazonas formed the center of the era's social life and its foyer played host to veritable rituals of 'civilization', in which Brazilian and foreign guests enjoyed the fine setting of Amazon's nature as represented in the decorative wall panels.

Iconografia e enfermagem ­ por que e como? / Iconography and nursing ­ why and how?

O artigo apresenta a iconografia como mais uma fonte de pesquisa para a história da enfermagem, justificando que ao estudar-se uma obra de arte, nela está embutida uma representação mental coletiva e quando esta obra inclui a questão da saúde, ela poderá nos dar pistas de nossa atividade cuidadora, como, onde e quem cuidava. O artigo cita exemplos e pontua os momentos da análise iconográfica que é o método usado para desvelar o significado intrínseco e profundo da obra de arte, considerado depositário das idéias e crenças de uma sociedade ou época. Enfatiza o agradável (re) encontro da enfermagem com a arte na perspectiva de (re) afirmar o valor do cuidar e com ele sedimentar nossa identidade profissional, na busca de um futuro onde as enfermeiras possam ser social, econômica e juridicamente reconhecidas.

This article presents the iconography as a source of research for the nursing history and shows that the act of studying artworks is relevant because they contain collective mental representations. When an art piece pictures healthcare, it will provide us with clues on care activity and also on how, where, and who is practicing it. The author exemplifies and comments on moments of iconographic analysis that is the methodology used to reveal the intrinsic and profound meaning of art works considered, in turn, a society's depository of beliefs and ideas. Finally, the author emphasizes the pleasant meeting of nursing and art, placing into perspective the importance of care as the basis for the nurse's professional identity, and looks forward into a future in which nurses can be social, economically, and juridical recognized.