Cuidando para a criança crescer apesar da dor: a experiência da família

This study aimed to understand the meaning of the experience of families having a child experiencing pain due to Juvenile Rheumatoid Arthritis and to construct a theoretical model representing this experience. Grounded Theory and Symbolic Interactionism were used as methodological framework and theoretical framework, respectively. Data were collected by semistructured interviews with 12 families. Data analysis allowed for the construction of the theoretical model Caring for the child to grow despite the pain, which describes an experience based on motivational elements: wanting to see the child without pain and wanting to see the child live a normal life, reviewing how the family lives the transition in its development cycles, retaking and integrating them in the family dynamic with the appearance of the disease and pain in the child. This theoretical model provides a framework for teaching, research and care, permitting advances in terms of theoretical nursing knowledge.Este trabajo tuvo como objetivos comprender el cotidiano de la familia del niño que vivencia la situación de dolor consecuente de la Artritis Reumatoidea Juvenil y construir un modelo teórico representativo de esa experiencia. La Teoría Fundamentada en los Datos y el Interacionismo Simbólico fueron utilizados como referenciales metodológico y teórico, respectivamente. Los datos fueron obtenidos por intermedio de entrevistas semi-estructuradas a 12 familias. El análisis de los datos permitió construir el modelo teórico Cuidando para que el niño crezca a pesar del dolor, que describe una experiencia estructurada en torno a los elementos motivadores: queriendo ver el niño libre del dolor y queriendo ver el niño llevar una vida normal, revelando como la familia vivencia las transiciones en sus ciclos de desarrollo, integrándolos en la dinámica familiar con la llegada de la enfermedad y del dolor en el niño. Este modelo proporciona un referencial que ayuda a la enseñanza, investigación y atención, permitiendo avanzar en el conocimiento teórico en enfermería.Os objetivos desse estudo foram compreender o significado da experiência da família da criança com dor decorrente de artrite reumatóide juvenil e construir um modelo teórico representativo dessa experiência. A Teoria Fundamentada nos Dados e o Interacionismo Simbólico foram utilizados como referencial metodológico e teórico, respectivamente. Foram realizadas 12 entrevistas semi-estruturadas com famílias das crianças. A partir da análise dos dados, emergiu o modelo teórico Cuidando para a criança crescer, apesar da dor, constituído por elementos motivacionais: querendo ver a criança livre da dor e querendo ver a criança levar uma vida normal, além de elementos intervenientes, revelando como a família vivencia as transições em seus ciclos de desenvolvimento, retomando-os e integrando-os na sua dinâmica com a chegada da doença e da dor na criança. Esse modelo teórico proporciona um referencial para as práticas de ensino, pesquisa e assistência, permitindo avançar em termos de conhecimento teórico para a enfermagem

O espectro desafiador e imprevisível da COVID-19 em crianças e adolescentes

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Skeletal muscle major histocompatibility complex class I and II expression differences in adult and juvenile dermatomyositis

OBJECTIVE: To analyze major histocompatibility complex expression in the muscle fibers of juvenile and adult dermatomyositis. METHOD: In total, 28 untreated adult dermatomyositis patients, 28 juvenile dermatomyositis patients (Bohan and Peter's criteria) and a control group consisting of four dystrophic and five Pompe's disease patients were analyzed. Routine histological and immunohistochemical (major histocompatibility complex I and II, StreptoABComplex/HRP, Dakopatts) analyses were performed on serial frozen muscle sections. Inflammatory cells, fiber damage, perifascicular atrophy and increased connective tissue were analyzed relative to the expression of major histocompatibility complexes I and II, which were assessed as negatively or positively stained fibers in 10 fields (200X). RESULTS: The mean ages at disease onset were 42.0±15.9 and 7.3±3.4 years in adult and juvenile dermatomyositis, respectively, and the symptom durations before muscle biopsy were similar in both groups. No significant differences were observed regarding gender, ethnicity and frequency of organ involvement, except for higher creatine kinase and lactate dehydrogenase levels in adult dermatomyositis (

Treatment with biologic agents in child and adolescent

OBJETIVO: Revisar os mecanismos fisiopatológicos e novos alvos terapêuticos, os agentes biológicos disponíveis, principais indicações e a evidência científica atual para o uso de terapias biológicas na população pediátrica. FONTES DE DADOS: Pesquisa na base de dados Medline e SciELO, nas línguas inglesa e portuguesa, entre 2000 e 2009. As palavras-chave usadas foram "agentes biológicos", "crianças" e "adolescentes". SÍNTESE DOS DADOS: Os agentes biológicos são uma importante opção terapêutica para tratar as doenças autoimunes refratárias às terapias convencionais na infância e na adolescência. Com exceção da artrite idiopática juvenil, a maioria dos estudos em outras doenças autoimunes não é controlada. CONCLUSÕES: Os agentes biológicos têm demonstrado eficácia no tratamento de doenças autoimunes pediátricas como artrite idiopática juvenil, miopatias idiopáticas inflamatórias, lúpus eritematoso juvenil, vasculites, uveítes crônicas, doenças inflamatórias intestinais e púrpura trombocitopênica imune crônica, assim como no linfoma não-Hodgkin. Considerando-se o custo elevado e os potenciais eventos adversos, o uso desses agentes deve ser individualizado e acompanhado por especialista.OBJECTIVE: To review the physiopathology and new therapeutical targets, the available biologic agents, the main indications and the current scientific evidence for the use of biological therapies in the pediatric population. DATA SOURCES: A bibliographical search was obtained from Medline and SciELO databases in English and Portuguese from 2000 to 2009. The key-words included were "biologic agent", "children" and "adolescent". DATA SYNTHESIS: Biologic agents are important therapeutic options to treat refractory autoimmune diseases to conventional therapies in childhood and adolescence. Excluding juvenile idiopathic arthritis, the majority of studies in other autoimmune diseases are uncontrolled trials. CONCLUSIONS: Biologic agents have shown efficacy in the treatment of pediatric autoimmune diseases such as juvenile idiopathic arthritis, idiopathic inflammatory myositis, juvenile systemic lupus erythematosus, vasculitis, chronic uveitis, inflammatory bowel diseases, and chronic immune thrombocytopenic purpura, as well as in non-Hodgkin lymphoma. Considering the high cost and the potential adverse events, the choice to use them must be individualized and followed by a specialist

Skeletal muscle major histocompatibility complex class I and II expression differences in adult and juvenile dermatomyositis

OBJECTIVE: To analyze major histocompatibility complex expression in the muscle fibers of juvenile and adult dermatomyositis. METHOD: In total, 28 untreated adult dermatomyositis patients, 28 juvenile dermatomyositis patients (Bohan and Peter's criteria) and a control group consisting of four dystrophic and five Pompe's disease patients were analyzed. Routine histological and immunohistochemical (major histocompatibility complex I and II, StreptoABComplex/HRP, Dakopatts) analyses were performed on serial frozen muscle sections. Inflammatory cells, fiber damage, perifascicular atrophy and increased connective tissue were analyzed relative to the expression of major histocompatibility complexes I and II, which were assessed as negatively or positively stained fibers in 10 fields (200X). RESULTS: The mean ages at disease onset were 42.0 +/- 15.9 and 7.3 +/- 3.4 years in adult and juvenile dermatomyositis, respectively, and the symptom durations before muscle biopsy were similar in both groups. No significant differences were observed regarding gender, ethnicity and frequency of organ involvement, except for higher creatine kinase and lactate dehydrogenase levels in adult dermatomyositis (p<0.050). Moreover, a significantly higher frequency of major histocompatibility complex I (96.4% vs. 50.0%, p<0.001) compared with major histocompatibility complex II expression (14.3% vs. 53.6%, p = 0.004) was observed in juvenile dermatomyositis. Fiber damage (p = 0.006) and increased connective tissue (p<0.001) were significantly higher in adult dermatomyositis compared with the presence of perifascicular atrophy (p<0.001). The results of the histochemical and histological data did not correlate with the demographic data or with the clinical and laboratory features. CONCLUSION: The overexpression of major histocompatibility complex I was an important finding for the diagnosis of both groups, particularly for juvenile dermatomyositis, whereas there was lower levels of expression of major histocompatibility complex II than major histocompatibility complex I. This finding was particularly apparent in juvenile dermatomyositis.Conselho Nacional de Desenvolvimento Cientifico e Tecnologico - CNPQ [300248/2008-3]Conselho Nacional de Desenvolvimento Cientifico e Tecnologico (CNPq)Federico FoundationFederico Foundatio

Redução na força muscular e capacidade funcional em pacientes fisicamente inativos com lúpus eritematoso sistêmico de início juvenil, apesar de doença muito leve

ResumoObjetivoComparar a força muscular (ou seja, a força muscular dos membros superiores e inferiores) e a capacidade funcional de pacientes fisicamente inativos com lúpus eritematoso sistêmico de início juvenil (LESJ) com controles saudáveis (CTRL).MétodosEstudo transversal cuja amostra foi composta por 19 pacientes com LESJ (entre 9 e 18 anos) e 15 CTRL pareados por idade, sexo, índice de massa corporal (IMC) e nível de atividade física (avaliada através do uso de acelerômetros). A força dos membros superiores e inferiores foi avaliada pelo teste de uma repetição máxima (1‐RM). A força isométrica foi avaliada através do uso de um dinamômetro. A capacidade funcional foi avaliada pelo Timed‐stands test (TST) e Timed‐up‐and‐go test (TUG).ResultadosQuando comparados com os CTRL, os pacientes com LESJ apresentaram menor força em 1‐RM no Leg press e supino (p=0,026 e p=0,008, respectivamente) e uma tendência a menor força de preensão manual (p=0,052). Os pacientes com LESJ apresentaram menores escores no TST (p=0,036) e uma tendência a maior pontuação no TUG (p=0,070), quando comparados com o grupo CTRL.ConclusãoPacientes com LESJ, fisicamente inativos, com doença muito leve mostraram redução na força muscular e capacidade funcional quando comparados com controles saudáveis pareados por níveis de atividade física. Esses achados sugerem que pacientes com LESJ podem apresentar mais efeitos deletérios por manter um estilo de vida fisicamente inativo do que controles saudáveis. Além disso, alguns efeitos “residuais” subclínicos da doença ou do tratamento farmacológico parecem afetar pacientes com LESJ, mesmo com uma doença bem controlada.AbstractObjectiveTo compare muscle strength (i.e. lower‐ and upper‐body strength) and function between physically inactive childhood‐onset systemic lupus erythematosus patients (C‐SLE) and healthy controls (CTRL).MethodsThis was a cross‐sectional study and the sample consisted of 19 C‐SLE (age between 9 to 18 years) and 15 CTRL matched by age, sex, body mass index (BMI), and physical activity levels (assessed by accelerometry). Lower‐ and upper‐body strength was assess by the one‐repetition‐maximum (1‐RM) test. Isometric strength was assessed through a handgrip dynamometer. Muscle function was evaluated by the timed‐stands test (TST) and the timed‐up‐and‐go test (TUG).ResultsWhen compared with CTRL, C‐SLE showed lower leg‐press and bench‐press 1‐RM (p=0.026 and p=0.008, respectively), and a tendency towards lower handgrip strength (p=0.052). C‐SLE showed lower TST scores (p=0.036) and a tendency towards higher TUG scores (p=0.070) when compared with CTRL.ConclusionPhysically inactive C‐SLE patients with very mild disease showed reduced muscle strength and functionality when compared with healthy controls matched by physical activity levels. These findings suggest C‐SLE patients may greatly suffer from a physically inactive lifestyle than healthy controls do. Moreover, some sub‐clinical “residual” effect of the disease or its pharmacological treatment seems to affect C‐SLE patients even with a well‐controlled disease

Acute petrified myocardium associated with meningococcal sepsis in childhood-onset systemic lupus erythematous: a fatal case

Acute petrified myocardium associated with septic shock, diagnosed by autopsy has rarely been described. A 15-year-old adolescent male was diagnosed with childhood-onset systemic lupus erythematosus. One year later, he was hospitalized with fever, myalgia, headache, arthritis, vomiting, dyspnea and was diagnosed with sepsis secondary to bronchopneumonia and meningitis. Blood culture identified Neisseria meningitidis serogroup Y. Despite antibiotics and intensive therapeutic measures, he died after 29 days of hospitalization. The autopsy revealed necrotic cardiomyocytes with dystrophic calcification and interstitial fibrosis