Late events after anti-CD19 CAR T-cell therapy for relapsed/refractory B-cell non-Hodgkin lymphoma.

Background: The short-term complications from chimeric antigen receptor T-cell therapy (CART) are well characterized, but the long-term complications still need to be further investigated. Therefore, herein, we will review the currently available literature published on the late adverse events following CART. Methods: We reviewed published data available from pivotal trials and real-world experiences with anti-CD19 CART (CART19) for adults with lymphoma. We defined late events as occurring or persisting beyond 1 month after CART infusion. We focused our literature review on the following late-event outcomes post-CART19: cytopenia, immune reconstitution, infections, and subsequent malignancies. Results: Grade 3-4 cytopenia beyond 30 days occurs in 30%-40% of patients and beyond 90 days in 3%-22% of patients and is usually managed with growth-factor and transfusion support, along with neutropenic prophylaxis. B-cell aplasia and hypogammaglobulinemia are expected on-target off-tumor effects of CART19, 44%-53% of patients have IgG < 400 mg/dL, and approximately 27%-38% of patients receive intravenous immunoglobulin (IVIG) replacement. Infections beyond the initial month from CART19 are not frequent and rarely severe, but they are more prevalent and severe when patients receive subsequent therapies post-CART19 for their underlying disease. Late neurotoxicity and neurocognitive impairment are uncommon, and other causes should be considered. T-cell lymphoma (TCL) after CART is an extremely rare event and not necessarily related to CAR transgene. Myeloid neoplasm is not rare post-CART, but unclear causality given heavily pretreated patient population is already at risk for therapy-related myeloid neoplasm. Conclusion: CART19 is associated with clinically significant long-term effects such as prolonged cytopenia, hypogammaglobulinemia, and infections that warrant clinical surveillance, but they are mostly manageable with a low risk of non-relapse mortality. The risk of subsequent malignancies post-CART19 seems low, and the relationship with CART19 and/or prior therapies is unclear; but regardless of the possible causality, this should not impact the current benefit-risk ratio of CART19 for relapsed/refractory B-cell non-Hodgkin lymphoma (NHL).

A Case Report of Primary Aldosteronism and Extensive Hypertension-Mediated Organ Damage.

Primary aldosteronism, the most common curable form of secondary hypertension, is associated with greater hypertension-related organ damage and cardiovascular complications compared to primary essential hypertension. The authors present a case involving a 41-year-old Black male admitted to the emergency department with left hemiparesis and blurred vision persisting for one hour, accompanied by markedly elevated blood pressure (220/140 mmHg). The patient was asymptomatic by then, and, aside from a history of tobacco smoking and occasional cannabis use, lacked significant medical comorbidities. Further investigations revealed a right acute hemorrhagic stroke, bilateral grade 4 hypertensive retinopathy, chronic kidney disease with end-stage renal disease, hypokalemia, and an elevated aldosterone/renin ratio. An abdominal CT scan showed bilateral adrenal hyperplasia. The patient was diagnosed with primary aldosteronism with extensive hypertension-mediated organ damage. This case highlights the significant harm caused by undiagnosed primary aldosteronism-induced secondary hypertension, emphasizing the importance of timely diagnosis and intervention to prevent organ damage.

Multidisciplinary Outpatient Clinic of Neurocutaneous Diseases: Five-year Experience of a Pediatric Tertiary Hospital in Portugal.

INTRODUCTION: Neurocutaneous syndromes (NCS) are a heterogeneous group of conditions with multiorgan involvement and diverse manifestations, evolving throughout life with significant morbidity. A multidisciplinary approach to NCS patients has been advocated, although a specific model is not yet established. The aim of this study was 1) to describe the organization of the recently created Multidisciplinary Outpatient Clinic of Neurocutaneous Diseases (MOCND) at a Portuguese pediatric tertiary hospital; 2) to share our institutional experience focusing on the most common conditions, neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC); 3) to analyze the advantages of a multidisciplinary center and approach in NCS. METHODS: Retrospective analysis of 281 patients enrolled in the MOCND over the first five years of activity (October 2016 to December 2021), reviewing genetics, family history, clinical features, complications, and therapeutic strategies for NF1 and TSC. RESULTS: The clinic works weekly with a core team of pediatricians and pediatric neurologists supported by other specialties as needed. Of the 281 patients enrolled, 224 (79.7%) had identifiable syndromes such as NF1 (n = 105), TSC (n = 35), hypomelanosis of Ito (n = 11), Sturge-Weber syndrome (n = 5), and others. In NF1 patients, 41.0% had a positive family history, all manifested café-au-lait macules, 38.1% neurofibromas with 45.0% being large plexiform neurofibromas. Sixteen were under treatment with selumetinib. Genetic testing was performed in 82.9% of TSC patients with pathogenic variants found in TSC2 gene in 72.4% patients (82.7% if considered contiguous gene syndrome). Family history was positive in 31.4%. All TSC patients presented hypomelanotic macules and fulfilled diagnostic criteria. Fourteen patients were being treated with mTOR inhibitors. CONCLUSION: Offering a systematic and multidisciplinary approach to NCS patients enables timely diagnosis, promotes a structured follow-up, and encourages discussion to outline management plans for optimal care to every patient, with significant impact on the quality of life of patients and families.

Perspectivas sobre as necessidades essenciais das crianças na primeira infância no contexto da pandemia da COVID-19: uma revisão integrativa / Perspectivas sobre las necesidades esenciales de los niños en la primera infancia en el contexto de la pandemia de COVID-19: Una revisión integradora / Perspectives on the essential needs of children in early childhood in the context of the COVID-19 pandemic: an integrative review

RESUMO Objetivo O presente estudo objetiva identificar as perspectivas científicas sobre as implicações da pandemia da COVID-19 na primeira infância, focando nas necessidades essenciais da criança. Método Foi realizada uma revisão integrativa da literatura, que incluiu cinco bases de dados. O processo envolveu a identificação do problema, o desenvolvimento de uma pergunta orientadora, pesquisa na literatura, análise dos dados e síntese dos resultados. Foram incluídos artigos originais que envolviam crianças com menos de seis anos, publicados entre 2020 e 2022. A análise foi conduzida tendo como referencial teórico as necessidades essenciais da criança. Resultados Foram examinados 22 artigos. Os resultados indicaram um aumento na sobrecarga e no estresse parental. As crianças apresentaram alterações comportamentais, perturbações no padrão do sono, uso mais frequente de mídias digitais e diminuição de atividades realizadas ao ar livre. Fatores protetores, relacionados ao vínculo familiar, cuidado e acolhimento da criança, foram identificados. O fechamento de creches e escolas distanciou os cuidadores dos profissionais da educação e de outros pais, mas sua reabertura trouxe maior apoio às famílias. Necessidades de relacionamentos contínuos de apoio, experiências adequadas ao desenvolvimento e comunidades solidárias foram mais evidenciadas. No entanto, foram encontrados menos aspectos relacionados à proteção física e ao estabelecimento de limites. Experiências sobre diferenças individuais não foram identificadas. Conclusão A pesquisa científica revelou vulnerabilidades substanciais e potencialidades no cuidado à saúde da criança durante a pandemia de COVID-19. Esses resultados sugerem impactos significativos no bem-estar e no desenvolvimento infantil, implicando na necessidade de ações específicas por parte da enfermagem no contexto da Atenção Primária à Saúde.

RESUMEN Objetivo Identificar el conocimiento científico acerca de las implicaciones de la pandemia de COVID-19 en la primera infancia desde la perspectiva de las necesidades esenciales de los niños. Método Se realizó una revisión integrativa de la literatura en cinco bases de datos, que incluye la identificación del problema, la formulación de una pregunta orientadora, la búsqueda de la literatura, el análisis de los datos y una síntesis de los resultados. Se incluyeron artículos originales que trataban sobre niños menores de seis años y que fueron publicados entre los años 2020-2022. A. El análisis se llevó a cabo utilizando el marco teórico de las necesidades esenciales de los niños. Resultados Se analizaron 22 artículos. Se observó una sobrecarga y un incremento del estrés en los padres. Los niños mostraron cambios en su comportamiento y patrones de sueño, un mayor uso de medios digitales y una menor participación en actividades al aire libre. Se identificaron factores de protección relacionados con el vínculo familiar, el cuidado y recibimiento del niño. El cierre de guarderías y escuelas aisló a los cuidadores de los profesionales de la educación y de otros padres, aunque la reapertura proporcionó un mayor apoyo a las familias. Las necesidades más evidentes fueron las de relaciones de apoyo contínuas, experiencias apropiadas para el desarrollo y comunidades de apoyo. Hubo menos énfasis en aspectos de protección física y establecimiento de límites. No se identificaron experiencias respecto a diferencias individuales. Conclusión El conocimiento científico destaca vulnerabilidades y aspectos positivos significativos en la atención de la salud infantil que sugieren un impacto en el bienestar y desarrollo de los niños, con repercusiones para el desempeño de los profesionales de enfermería en el ámbito de la atención primaria de salud.

ABSTRACT Objective Identify scientific knowledge regarding the implications of the COVID-19 pandemic on early childhood, specifically from the perspective of children's essential needs. Method An integrative literature review was conducted on five databases. The review process involved identifying a research problem, creating a guiding question, performing a literature search, analyzing data, and synthesizing the results. The review included original articles about children under six, published between 2020 and 2022. The theoretical framework of the child's essential needs was used for analysis. Results A total of 22 articles were analyzed. Findings indicated an increase in parental overload and stress levels. The children exhibited behavioral changes, sleep pattern disorders, increased digital media use, and reduced outdoor participation. Protective factors related to the familial bond, child care, and child reception were identified. Closure of daycare centers and schools resulted in a distance between the caregiver, the education professionals, and the fellow parents. In contrast, the reopening of those places brought added support to families. The needs, such as ongoing supportive relationships, developmentally appropriate experiences, and supportive communities, were more highlighted. Physical protection and setting boundaries were less prominent aspects. No experiences examining individual differences were identified. Conclusion The scientific knowledge points to significant vulnerabilities and certain potentialities in child health care during the COVID-19 pandemic. Those results suggest considerable impacts on child well-being and development and imply significant implications for nursing practices in Primary Health Care.

Josicélia Dumêt Fernandes' professional trajectory: contributions to psychiatric and mental health nursing / Trayectoria profesional de Josicélia Dumêt Fernandes: aportes a la enfermería psiquiátrica y de salud mental / Trajetória profissional de Josicélia Dumêt Fernandes: contribuições para a enfermagem psiquiátrica e de saúde mental

ABSTRACT Objectives: to analyze nurse Josicélia Dumêt Fernandes' life story, with emphasis on her work in the psychiatry and mental health fields. Methods: historical, qualitative research. Semi-structured interviews and documentary research were used as data collection techniques, collected from September to October 2021. For data analysis, we opted for the content analysis method and comparison with the Foucauldian philosophical framework. Results: four categories emerged: Transforming herself and mental health practices; (Re)framing professional practice; Nursing practice and power relations; and The paths and implications in the psychiatry and mental health fields. Final Considerations: the study of the biographer demonstrates a search for transformation of herself and mental health practices, with a rupture in paradigms and reframing of her practice in psychiatry and mental health.

RESUMEN Objetivos: analizar la historia de vida de la enfermera Josicélia Dumêt Fernandes, con énfasis en su trabajo en el área de psiquiatría y salud mental. Métodos: investigación histórica, cualitativa. Se utilizaron como técnicas de recolección de datos entrevistas semiestructuradas e investigación documental, recolectados de septiembre a octubre de 2021. Para el análisis de los datos, se optó por el método de análisis de contenido y comparación con el marco filosófico foucaultiano. Resultados: surgieron cuatro categorías: Transformación del yo y de las prácticas de salud mental; El (re)significado de la práctica profesional; Práctica de enfermería y relaciones de poder; y Los caminos e implicaciones en el campo de la psiquiatría y la salud mental. Consideraciones Finales: el estudio de la biógrafa demuestra una búsqueda de transformación de ella misma y de las prácticas de salud mental, rompiendo paradigmas y resignificando su práctica en psiquiatría y salud mental.

RESUMO Objetivos: analisar a história de vida da enfermeira Josicélia Dumêt Fernandes, com ênfase em sua atuação na área da psiquiatria e saúde mental. Métodos: pesquisa histórica, do tipo qualitativa. Foram utilizadas como técnicas de coleta de dados a entrevista semiestruturada e a pesquisa documental, coletados de setembro a outubro de 2021. Para a análise dos dados, optou-se pelo método de análise de conteúdo e a confrontação com o referencial filosófico foucaultiano. Resultados: emergiram quatro categorias: A transformação de si e das práticas em saúde mental; A (re)significação da prática profissional; A prática de enfermagem e as relações de poder; e Os caminhos e implicações no campo da psiquiatria e da saúde mental. Considerações Finais: o estudo da biografada demonstra uma busca por transformação de si e das práticas de saúde mental, com ruptura de paradigmas e ressignificações da sua prática em psiquiatria e saúde mental.

Evidências de validade de critério concorrente e preditiva do Instrumento de Rastreio da Comunicação de Crianças de 0 a 36 meses (IRC-36) / Evidence of concurrent and predictive criterion validity of the Child Communication Screening Instrument from 0 to 36 months (IRC-36)

RESUMO Objetivo Determinar evidências de validade de critério concorrente e preditiva do Instrumento de Rastreio da Comunicação de crianças de 0 a 36 meses (IRC-36). Método Participaram da pesquisa 78 pais/responsáveis de crianças que frequentam o serviço de puericultura das Unidades de Saúde da Família, além de 33 crianças com idades entre 0 e 36 meses, convidadas para segunda etapa do estudo. Na primeira etapa do estudo, 13 profissionais de saúde foram treinados para realizar a aplicação do IRC-36 nos pais/responsáveis das crianças. No segundo momento, os pais responderam a uma nova aplicação do IRC-36 e as crianças foram avaliadas com o Denver II. Resultados O IRC-36 apresentou correlação com o Denver II em mais da metade dos casos, confirmando a validade de critério concorrente do instrumento. Os resultados do IRC-36 da primeira etapa quando correlacionados com o Denver II, não apresentaram valores significativos. O valor de ponto de corte do instrumento foi 12, sendo este o valor de referência entre crianças em risco e sem risco para alteração da comunicação. O instrumento apresentou valor de acurácia dentro dos níveis preconizados e alta sensibilidade. A ocorrência de risco para alteração da comunicação apresentou-se maior na segunda aplicação do IRC-36. Conclusão O estudo apresentou evidências de validade de critério concorrente, indicando que o instrumento possui evidências de medidas de acurácia e de validade para o rastreio da comunicação de crianças de 0 a 36 meses sendo capaz de identificar risco para as alterações da comunicação.

ABSTRACT Purpose To determine evidence of concurrent and predictive criterion validity of the Communication Screening Instrument for children aged 0 to 36 months (IRC-36). Methods 78 parents/guardians of children who attend the childcare service of the Family Health Centers participated in the research, in addition to 33 children aged between 0 and 36 months, invited to the second stage of the study. In its first stage, 13 health professionals were trained to apply the IRC-36 to the children's parents/guardians. In the second moment, the parents responded to a new IRC-36 application, and the children were evaluated with Denver II. Results IRC-36 correlated with Denver II in more than half of the cases, confirming the instrument's concurrent criterion validity. IRC-36 results in the first stage did not significantly correlate with Denver II. The instrument's cutoff value was 12, which is the reference value between children at risk and not at risk of communication disorders. The instrument had high sensitivity and an accuracy value within the recommended levels. The occurrence of risk of communication changes was higher in the second IRC-36 application. Conclusion The study presented evidence of concurrent criterion validity, indicating that the instrument has evidence of accuracy and validity measures to screen communication in children aged 0 to 36 months, being able to identify the risk for communication disorders.

Case Report: Patient with deficiency of ADA2 presenting leukocytoclastic vasculitis and pericarditis during infliximab treatment.

Deficiency of adenosine deaminase 2 (DADA2), first reported in 2014, is a disease with great phenotypic variability, which has been increasingly reported. Therapeutic response depends on the phenotype. We present a case of an adolescent with recurrent fever, oral aphthous ulcers, and lymphadenopathy from 8 to 12 years of age and subsequently presented with symptomatic neutropenia. After the diagnosis of DADA2, therapy with infliximab was started, but after the second dose, she developed leukocytoclastic vasculitis and showed symptoms of myopericarditis. Infliximab was switched to etanercept, with no relapses. Despite the safety of tumor necrosis factor alpha inhibitors (TNFi), paradoxical adverse effects have been increasingly reported. The differential diagnosis between disease new-onset manifestations of DADA2 and side effects of TNFi can be challenging and warrants further clarification.

Assessment of calcinosis in Portuguese patients with systemic sclerosis - a multicenter study.

INTRODUCTION/OBJECTIVES: The study aims to define the clinical and subclinical calcinosis prevalence, the sensitivity of radiographed site and clinical method for its diagnosis, and the phenotype of Portuguese systemic sclerosis (SSc) patients with calcinosis. METHOD: A cross-sectional multicenter study was conducted with SSc patients fulfilling Leroy/Medsger 2001 or ACR/EULAR 2013 classification criteria, registered in the Reuma.pt. Calcinosis was assessed through clinical examination and radiographs of hands, elbows, knees, and feet. Independent parametric or non-parametric tests, multivariate logistic regression, and sensitivity calculation of radiographed site and clinical method for calcinosis detection were performed. RESULTS: We included 226 patients. Clinical calcinosis was described in 63 (28.1%) and radiological calcinosis in 91 (40.3%) patients, of which 37 (40.7%) were subclinical. The most sensitive location to detect calcinosis was the hand (74.7%). Sensitivity of the clinical method was 58.2%. Calcinosis patients were more often female (p = 0.008) and older (p < 0.001) and had more frequently longer disease duration (p < 0.001), limited SSc (p = 0.017), telangiectasia (p = 0.039), digital ulcers (p = 0.001), esophageal (p < 0.001) and intestinal (p = 0.003) involvements, osteoporosis (p = 0.028), and late capillaroscopic pattern (p < 0.001). In multivariate analysis, digital ulcers (OR 2.63, 95% CI 1.02-6.78, p = 0.045) predicted overall calcinosis, esophageal involvement (OR 3.52, 95% CI 1.28-9.67, p = 0.015) and osteoporosis (OR 4.1, 95% CI 1.2-14.2, p = 0.027) predicted hand calcinosis, and late capillaroscopic pattern (OR 7.6, 95% CI 1.7-34.9, p = 0.009) predicted knee calcinosis. Anti-nuclear antibody positivity was associated with less knee calcinosis (OR 0.021, 95% CI 0.001-0477, p = 0.015). CONCLUSIONS: Subclinical calcinosis high prevalence suggests that calcinosis is underdiagnosed and radiographic screening might be relevant. Multifactorial pathogenesis may explain calcinosis predictors' variability. Key Points • Prevalence of subclinical calcinosis in SSc patients is substantial. • Hand radiographs are more sensitive to detect calcinosis than other locations or clinical method. • Digital ulcers were associated with overall calcinosis, esophageal involvement and osteoporosis were associated with hand calcinosis, and late sclerodermic pattern in nailfold capillaroscopy was associated with knee calcinosis. • Anti-nuclear antibody positivity may be a protective factor for knee calcinosis.

A colaboração interprofissional no programa de educação pelo trabalho para a saúde / La colaboración interprofesional en el programa de educación por el trabajo para la salud / Interprofessional collaboration in the program for education at work for health1

RESUMO Objetivo: identificar os elementos das dimensões colaborativas interprofissionais presentes nos projetos institucionais a serem desenvolvidos no Programa de Educação pelo Trabalho para a Saúde. Método: estudo documental de abordagem qualitativa. A fonte documental consiste em cinco projetos de intervenção elaborados por cinco Universidades Federais da região Nordeste para serem desenvolvidos no Programa de Educação pelo Trabalho para a Saúde Interprofissionalidade. Os dados foram coletados e analisados entre julho de 2020 a março de 2021, com aplicação de matriz de coleta, de elaboração própria. Com auxílio de um software, o estudo está alicerçado no referencial de D'Amour para fins de estabelecimento de categorias conforme preconizadas nas etapas estabelecidas por Bardin. Foram assegurados os aspectos éticos para a pesquisa. Resultados: Foram identificados elementos das dimensões colaborativas do modelo de D'Amour nos 5 projetos, tais como: metas e orientação centrada no usuário; convivência mútua; confiança; ferramentas de formalização; intercâmbio de informações; centralidade e liderança; suporte à inovação e conectividade. Considerações finais: os projetos de intervenção elaborados pelas universidades possuem elementos indutores da Colaboração Interprofissional. No entanto, precisam ser melhor explicitadas as intervenções voltadas para a estruturação do atendimento colaborativo e exercício para a liderança.

RESUMEN Objetivo: identificar los elementos de las dimensiones colaborativas interprofesionales presentes en los proyectos institucionales que se desarrollarán en el Programa de Educación por el Trabajo para la Salud. Método: estudio documental de enfoque cualitativo. La fuente documental consiste en cinco proyectos de intervención elaborados por cinco Universidades Federales de la región Nordeste de Brasil que se desarrollarán en el Programa de Educación por el Trabajo para la Salud Interprofesionalidad. Los datos fueron recogidos y analizados entre julio de 2020 y marzo de 2021, con aplicación de matriz de recolección y elaboración propia. Con ayuda de un software, el estudio está basado en el referencial de D'Amour para fines de establecimiento de categorías conforme preconizadas en las etapas establecidas por Bardin. Se aseguraron los aspectos éticos para la investigación. Resultados: se identificaron elementos de las dimensiones colaborativas del modelo de D'Amour en los 5 proyectos, tales como metas y orientación centrada en el usuario; convivencia mutua; confianza; herramientas de formalización; intercambio de información; centralidad y liderazgo; apoyo a la innovación y la conectividad. Consideraciones finales: los proyectos de intervención elaborados por las universidades poseen elementos inductores de la Colaboración Interprofesional. Sin embargo, necesitan ser mejor explicitadas las intervenciones dirigidas a la estructuración de la atención colaborativa y el ejercicio para el liderazgo.

ABSTRACT Objective: To identify elements from the interprofessional collaborative dimensions in the institutional projects to be developed in the program for education at work for health. Method: Document study with a quantitative approach. The document source includes five intervention projects elaborated by five federal universities from the Brazilian northeast, to be developed in the interprofessional program for education at work for health. Data was collected and analyzed from July 2020 to March 2021, through the application of a collection tool elaborated by the authors. The study, conducted with the aid of software, is based on D'Amour references to establish categories according to the stages elaborated by Bardin. The ethical aspects of the research were guaranteed. Results: We identified elements of the collaborative dimensions of the model by D'Amour in the five projects, such as: goals and user-focused guidance; mutual socialization; trust; formalization tools; information exchange; centrality and leadership; support to innovation and connectivity. Final considerations: intervention projects elaborated by the universities have elements conducive to Interprofessional Collaboration. However, interventions targeted at structuring collaborative care and exercising leadership must be better elaborated.

Percepção de professores sobre intervenção educativa de base fônica / Teachers' perception of phonic-based educational intervention

Além do domínio nas habilidades de consciência fonológica, o aprendizado da leitura e escrita exige o desenvolvimento de outras competências, como, por exemplo, o processamento visual, auditivo e fonológico. Tais habilidades devem ser conhecidas pelo professor alfabetizador, pois ele precisa reconhecer as crianças com dificuldades e buscar estratégias cognitivo-linguísticas para que elas avancem. OBJETIVO: Desvelar a percepção de professores sobre intervenção educativa de base fônica voltada à facilitação da aprendizagem inicial da leitura e escrita MÉTODO: Trata-se de estudo longitudinal, de intervenção, realizado com duas professoras do 2º ano do Ensino Fundamental I de uma escola pública da cidade do Recife, PE. Ambas participaram de uma intervenção educativa realizada com sua turma, em 12 encontros. Ao final de cada encontro, as professoras responderam três perguntas norteadoras sobre as atividades realizadas RESULTADOS: A análise de conteúdo possibilitou a identificação de quatro categorias temáticas: 1- Desafios da alfabetização; 2- Estratégias facilitadoras da aprendizagem da leitura e escrita; 3- Contribuições da intervenção para o processo de alfabetização; 4- Contribuições da intervenção para a identificação de crianças em risco para os transtornos de aprendizagem CONCLUSÃO: De acordo com as professoras, a intervenção foi positiva, contribuiu para o processo de facilitação da aprendizagem e possibilitou a identificação precoce de escolares em risco para transtornos de aprendizagem, sendo importante o desenvolvimento de estudos de intervenção de base fônica para auxiliar a prática docente no ciclo de alfabetização

In addition to mastering phonological awareness skills, learning to read and write requires the development of other skills, such as visual, auditory and phonological processing. Such skills must be known by the literacy teacher, as he needs to recognize children with difficulties in the literacy process, and seek cognitive-linguistic strategies for them to advance. PURPOSE: To unveil the perception of teachers about phonics-based educational intervention aimed at facilitating the initial learning of reading and writing METHODS: This is a longitudinal intervention study carried out with two teachers from the 2nd year of elementary school I of a public school in the city of Recife, Pernambuco state. Both participated in an educational intervention carried out with their class, in 12 meetings. At the end of each meeting, the teachers answered three guiding questions about the activities carried out RESULTS: Content analysis allowed the identification of four thematic categories: 1- Literacy challenges; 2- Facilitating strategies for learning to read and write; 3- Contributions of the intervention to the literacy process; 4- Contributions of the intervention to identify children at risk for learning disabilities CONCLUSION: According to the teachers, the intervention was positive, contributed to the process of facilitating learning and enabled the early identification of students at risk for learning disorders, being important the development of phonics-based intervention studies to help practice teacher in the literacy cycle

Ocular findings from otoneurological examinations in children with and without dyslexia: a systematic review with meta-analysis

Abstract Objective: To investigate ocular movements measures of vectoelectro-nystagmography and video-nystagmography in dyslexic children and compare with measures of typical children. Methods: A systematic review of observational studies comparing the ocular movements differences between dyslexic and typical children with no publication date or language restriction. The literature survey included the bibliographic databases MEDLINE, ScienceDirect, Scopus, Web of Science, SciELO, Lilacs and CENTRAL. Gray literature databases were also searched, including: OpenGrey.eu, DissOnline.de, The New York Academy of Medicine and WorldCat. The meta-analysis was performed using software RevMan 5.3 (Cochran Collaboration). Results: A total of 2375 articles were found of which 113 fell within the inclusion criteria. Among these, 52 were duplicates (found in more than one research source), and 45 articles were selected for reading in full. Thirteen (13) articles were included for analysis and discussion. Meta-analysis showed statistical differences between the two groups for the total number of saccades and duration of fixation. Conclusion: The study revealed that children with dyslexia have longer duration of fixation and fewer saccades during ocular movements on vectoelectro-nystagmography and videonystagmography when compared to children without dyslexia.

Case Report: Wide Spectrum of Manifestations of Ligase IV Deficiency: Report of 3 Cases.

DNA ligase IV deficiency is a rare autosomal recessive disorder associated with impaired DNA repair mechanisms. Most patients with DNA repair defects present with neurologic deficits, combined immunodeficiency, bone marrow failure, and/or hematologic neoplasia. We present 3 unrelated cases of ligase IV deficiency with different clinical presentations. Patient 1 presented at the age of 5 with bone marrow failure, dysmorphic features, and T and B lymphopenia. A compound heterozygous variant L19W/K635fs in the LIG4 gene was identified. Patient 2 presented at the age of 16 with recurrent infections. He had agammaglobulinemia and absent B cells. A homozygous R278H in the LIG4 gene was identified. Patient 3 was referred for vitiligo and B-cell lymphopenia (low class-switched B cells) and hypogammaglobulinemia. Homozygous R278H in LIG4 was also identified. In the last few years, the spectrum of clinical manifestations caused by ligase IV deficiency has widened, making it very difficult to establish an accurate clinical diagnosis. The use of NGS allows a proper diagnosis and provides a better prognosis and adequate family counseling.

Gender differences in clinical features and outcomes of a Portuguese systemic sclerosis cohort.

Evidence for the role of sex in the clinical manifestations of systemic sclerosis (SSc) patients is emerging. Some multicenter cohorts have shown that male SSc patients have more severe disease and worse survival. To assess the differences in clinical manifestations and survival in Portuguese SSc patients according to gender. Data from male and female adult SSc patients included in the Rheumatic Diseases Portuguese Register (Reuma.pt) were analysed and compared. Survival was calculated for patients included in Reuma.pt. within the first two years of diagnosis (inception cohort). In total, 1054 adult patients with SSc were included, 12.5% males. No differences in demographic features and comorbidities were found between the sexes, except for a higher rate of cigarette smokers among men. Diffuse cutaneous SSc and anti-topoisomerase antibodies were more prevalent in males than females. Additionally, male patients presented significantly more myositis, interstitial lung disease and gastric involvement. There were no differences in the patterns of drug use between the sexes. During follow-up, more deaths were reported in men than women (12.1% vs 7.3%, p = 0.04). The overall 1-, 3-, and 5-year survivals from diagnosis of the inception cohort (N = 469) for men vs women were 96.4% vs 98.2%, 93% vs 95.9%, and 75.8% vs 93.2%, respectively, with statistically significant differences (p < 0.01). This study confirms the existence of gender differences in clinical and immunological SSc features. Although SSc is less common in men than women, men have a more severe expression of skin and internal organ involvement and worse survival. Key Points • There are differences in SSc disease manifestations between sexes. • Males more commonly have diffuse cutaneous SSc, anti-topoisomerase antibodies, pulmonary and musculoskeletal involvement. • In the inception cohort, men had worse survival rates than women.

Ensino da consulta de enfermagem na formação do enfermeiro: estudo bibliométrico / Nursing consultation teaching in nurses' training: a bibliometric study / Enseñanza de la consulta de enfermería en la formación de enfermeros: estudio bibliométrico

RESUMO Objetivo identificar a produção científica brasileira acerca do ensino da consulta de enfermagem na formação do enfermeiro. Método estudo descritivo de natureza bibliométrica, realizado no portal da Biblioteca Virtual em Saúde e na PubMed, de abril a maio de 2021, tendo como critérios de inclusão: artigos completos, em português ou inglês, publicados de 2009 a 2020. Resultados identificaram-se 13 estudos, sendo quatro publicados em um periódico B2 da região do Nordeste, contudo, oriundos predominantemente da Região Sul (38,46%), destacando-se os do tipo relato de experiência. As temáticas mais observadas relacionavam-se às metodologias de ensino, sobretudo, as ativas e com uso de ferramentas tecnológicas. A consulta de enfermagem não foi revelada como uma das palavras-chave mais frequentes. Conclusão ao constatar a incipiência da produção relativa à temática, este estudo fornece subsídios para ampliação desta área do conhecimento, reverberando em melhorias no ensino da consulta de enfermagem e na formação do enfermeiro.

ABSTRACT Objective to identify the Brazilian scientific production about Nursing consultation teaching in nurses' training. Method a descriptive study of a bibliometric nature conducted in the portal of Biblioteca Virtual em Saúde and in PubMed from April to May 2021, with the following inclusion criteria: full articles, in Portuguese or English, published from 2009 to 2020. Results a total of 13 studies were identified, of which four were published in a B2 journal from the Northeast region, although with predominance of papers from the South region (38.46%), with the experience report type standing out. The most frequently observed themes were related to the teaching methodologies, especially the active ones with use of technological tools. "Nursing consultation" was not found to be one of the most frequent keywords. Conclusion by verifying the incipience of the production on the theme, this study provides subsidies for expanding this knowledge area, reverberating in improvements in Nursing consultation teaching and in nurses' training.

RESUMEN Objetivo identificar la producción científica de Brasil sobre la enseñanza de la Consulta de Enfermería en la formación de enfermeros. Método estudio descriptivo de carácter bibliométrico realizado en el portal de la Biblioteca Virtual en Salud y en PubMed de abril a mayo de 2021, con los siguientes criterios de inclusión: artículos completos, en portugués o inglés y publicados entre 2009 y 2020. Resultados se identificaron 13 estudios, publicados en una revista académica B2 de la región Noreste, aunque predominantemente provenientes de la región Sur (38,46%), destacándose los del tipo informe de experiencia. Las temáticas más observadas estuvieron relacionadas con las metodologías de enseñanza, especialmente las activas y con utilización de herramientas tecnológicas. "Consulta de Enfermería" no figuró entre las palabras clave más frecuentes. Conclusión al constatar el carácter incipiente de la producción sobre la temática, este estudio proporciona puntos de apoyo para ampliar esta área del conocimiento, con repercusiones en mejoras en la enseñanza de la Consulta de Enfermería y en la formación de enfermeros.

Fatores associados à ansiedade/depressão nos estudantes de Medicina durante distanciamento social devido à Covid-19 / Factors associated with anxiety/depression in medical students during social distancing due to Covid-19

Resumo: Introdução: Os processos formativos na educação médica possuem fatores estressores e possíveis desencadeadores de transtornos mentais. Objetivo: Este estudo teve como objetivo determinar a prevalência e os fatores associados aos sinais e sintomas de ansiedade e depressão nos estudantes de Medicina durante o distanciamento social devido à pandemia pela Covid-19. Método: Realizou-se um estudo de corte transversal entre maio a junho de 2020, com estudantes de Medicina das instituições de ensino superior do estado de Pernambuco, no Brasil. Elaborou-se um questionário on-line por meio do Google Forms contendo características sociodemográficas e clínicas, e a Escala Hospitalar de Ansiedade e Depressão (EHAD). Para análise estatística, realizou-se análise multivariada, e calcularam-se a razão de risco (RR) e o intervalo de confiança de 95% (IC95%), considerando o coeficiente de significância p < 0,05. Resultado: As variáveis associadas ao maior risco de sinais e sintomas de ansiedade foram sexo feminino, diminuir/aumentar o hábito de fumar, aumentar/não alterar o consumo de medicamentos, diminuir/não praticar lazer, não preparação da universidade para o ensino a distância (EAD), ter EAD antes da pandemia e diminuição do rendimento escolar comparado aos períodos anteriores. Quanto à depressão, observaram-se os seguintes fatores: maior risco para aumentar/não alterar o uso de medicamentos, diminuir a prática de lazer, universidade não ter se preparado para o EAD, diminuição do rendimento escolar e cor de pele parda. Conclusão: Identificaram-se diferentes fatores relacionados aos sinais e sintomas de ansiedade e depressão no período de distanciamento social causado pela pandemia da Covid-19.

Abstract: Introduction: Medical training involves stressors and possible triggers of mental disorders. Objective: Our study aims to determine the prevalence and factors associated with signs and symptoms of anxiety and depression in medical students during social distancing measures imposed due to the COVID-19 pandemic. Method: A cross-sectional study was conducted between May and June 2020 among medical students from higher education institutions in Pernambuco state, Brazil. An online questionnaire was created using Google Forms containing sociodemographic and clinical characteristics and the Hospital Anxiety and Depression Scale (EHAD). For statistical analysis, multivariate analysis was performed and the risk ratio (RR) and its 95% confidence interval (95%CI) were calculated, considering the significance coefficient (p) <0.05. Result: The variables associated with a higher risk of anxiety signs and symptoms were female gender, decreasing/increasing smoking habit, increasing/not changing drug consumption, decreasing/not practicing leisure, lack of preparation by the university for distance learning, distance learning in place before the pandemic and decline in academic performance compared to previous periods. As for depression, there was a greater risk of increasing/not changing the use of medications, decreasing leisure time, university not being prepared for distance learning, declining academic performance and brown skin colour. Conclusion: Different factors related to the signs and symptoms of anxiety and depression were identified in the period of social distancing resulting from the COVID-19 pandemic.

Legislation and quality of nursing education in the context of the COVID-19 pandemic / Legislación y calidad de la educación en enfermería en el contexto de la pandemia de COVID-19 / Legislação e qualidade da educação em enfermagem no contexto da pandemia de COVID-19

ABSTRACT Objectives: to reflect on the governmental normative acts issued for higher education during the COVID-19 pandemic and on the repercussions of these acts on the quality of nursing education in Brazil. Methods: this is a reflection on the repercussions for the quality of nursing education, based on the normative acts applied to higher education, enacted from March to December 2020, available on the Ministry of Education website and in the current literature on the subject anchored in the National Curriculum Guidelines for the Undergraduate Nursing Course. Results: educational legislation, in the context of the pandemic, distanced education from the world of work, as well as from the quality of training established in the guidelines. Final Considerations: remote teaching in nursing makes it difficult to train professionals with the capacity to effectively teach and learn in the real world of care and health promotion in the Unified Health System.

RESUMEN Objetivos: reflexionar sobre actos normativos gubernamentales editados para la educación superior durante la pandemia de COVID-19 y las repercusiones de estos en la calidad de la educación en enfermería en Brasil. Métodos: reflexión sobre las repercusiones para la calidad de la enseñanza de enfermería, basada en actos normativos aplicados a educación superior, promulgados entre marzo a diciembre de 2020, disponibles en sitio del Ministerio de la Educación y en la literatura vigente sobre la temática, ancorada en Directrices Curriculares Nacionales de la Graduación en Enfermería. Resultados: la legislación educacional, durante la pandemia, alejó la enseñanza del mundo del trabajo, así como de la calidad de la formación establecida en las directrices. Consideraciones Finales: la enseñanza remota en enfermería dificulta la formación de profesionales con capacidad para efectividad del proceso de enseñar y aprender en el mundo real del cuidado y promoción de salud en el Sistema Único de Salud.

RESUMO Objetivos: refletir sobre os atos normativos governamentais editados para a educação superior durante a pandemia de COVID-19 e sobre as repercussões desses atos na qualidade da educação em enfermagem no Brasil. Métodos: trata-se de uma reflexão acerca das repercussões para a qualidade do ensino de enfermagem, baseada nos atos normativos aplicados ao ensino superior, promulgados no período de março a dezembro de 2020, disponíveis no site do Ministério da Educação e na literatura vigente sobre a temática, ancorada nas Diretrizes Curriculares Nacionais do Curso de Graduação em Enfermagem. Resultados: a legislação educacional, no contexto da pandemia, distanciou o ensino do mundo do trabalho, assim como da qualidade da formação estabelecida nas diretrizes. Considerações Finais: o ensino remoto na enfermagem dificulta a formação de profissionais com capacidade para a efetividade do processo de ensinar e aprender no mundo real do cuidado e da promoção da saúde no Sistema Único de Saúde.

Granulomatosis with polyangiitis - the incomplete puzzle.

Granulomatous with polyangiitis (GPA) is a necrotizing granulomatous vasculitis that mostly affects small-sized vessels. The disease can affect many organs, although renal and respiratory tract involvement are the most frequent and distinguishing features. Musculoskeletal manifestations have been reported in about 50% of patients and can occur as myalgia, oligoarthralgia/arthritis of large joints or polyarthralgia/arthritis of small joints. Infrequently musculoskeletal symptoms can be the first disease manifestation, and in this clinical scenario GPA diagnosis might be delayed or mistaken by other rheumatic diseases. The authors describe three patients with musculoskeletal symptoms as earliest GPA manifestations, illustrating the clinical challenge.