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 (

Optical coherence tomography aspects of Stargardt's disease: case report

The term fundus flavimaculatus (Stargardt disease) describes a group of inherited macular dystrophies characterized by multiple yellow to yellow-white flecks at the level of the retinal pigment epithelium. The authors describe findings in the patient with Stargardt's disease using optical coherence tomography (OCT), and suggest the examination to be valid as subsidiary method in the study of the characteristics of the retina in Stargardt's disease patients, but studies involving a series of patients should be able to show the most frequent findings in these cases.O termo fundus flavimaculatus (doença de Stargardt) descreve um grupo de distrofias maculares hereditárias caracterizadas por múltiplos flecks amarelados em nível do epitélio pigmentar da retina. Os autores descrevem os achados de tomografia de coerência óptica (OCT) em paciente portador de doença de Stargardt e sugerem que a OCT tem validade como exame subsidiário no estudo das características da retina de pacientes portadores da doença de Stargardt, embora estudos envolvendo maior número de pacientes sejam indicados para permitir traçar-se o perfil das alterações mais comuns nestes casos.Universidade Federal de São Paulo (UNIFESP) Departamento de Oftalmologia Setor de Ultra-som OcularUNIFESP Departamento de Oftalmologia Setor de Ultra-som OcularUNIFESP Departamento de Oftalmologia Setor de Eletrofisiologia OcularUNIFESP Departamento de Oftalmologia Setor de RetinaUNIFESP, Depto. de Oftalmologia Setor de Ultra-som OcularUNIFESP, Depto. de Oftalmologia Setor de Ultra-som OcularUNIFESP, Depto. de Oftalmologia Setor de Eletrofisiologia OcularUNIFESP, Depto. de Oftalmologia Setor de RetinaSciEL

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

Brazilian mosquito (Diptera: Culicidae) fauna: I. Anopheles species from Porto Velho, Rondônia state, western Amazon, Brazil

Este estudo contribui para o conhecimento de espécies de Anopheles, incluindo vetores de Plasmodium do oeste da Amazônia brasileira, em Porto Velho, no estado de Rondônia. Esta região vem passando por mudanças ambientais, como consequência de agricultura extensiva e projetos hidroelétricos que causam desmatamento, favorecendo o desenvolvimento de algumas espécies de mosquitos. Assim, a proposta deste estudo é registrar a presença de espécies de anofelinos na área, sendo conduzidas coletas de mosquitos em três locais, ao longo de uma linha de transmissão de energia elétrica. Cada uma das localidades foi amostrada três vezes, no período de 2010 a 2011. Os principais mosquitos adultos capturados em armadilhas de Shannon foram Anopheles darlingi, An. triannulatus, An. nuneztovari l.s., An.gilesi e An. costai. Assim como as formas larvárias Anopheles braziliensis, An. triannulatus, An. darlingi, An. deaneorum, An. marajoara, An. peryassui, An. nuneztovari l.s. e An. oswaldoi-konderi, coletadas em criadouros. Anopheles darlingi foi a espécie mais coletada na região. Em adição, discutiu-se sistemática de Culicidae, distribuição de fauna e aspectos da malária em ambientes modificados do oeste da Amazônia brasileira.This study contributes to knowledge of Anopheles species, including vectors of Plasmodium from the western Brazilian Amazon in Porto Velho, Rondônia State. The sampling area has undergone substantial environmental changes as a consequence of agricultural and hydroelectric projects, which have caused intensive deforestation and favored habitats for some mosquito species. The purpose of this study was to diagnose the occurrence of anopheline species from collections in three locations along an electric-power transmission line. Each locality was sampled three times from 2010 to 2011. The principal adult mosquitoes captured in Shannon trap were Anopheles darlingi, An. triannulatus, An. nuneztovari l.s., An.gilesi and An. costai. In addition, larvae were collected in ground breeding sites for Anopheles braziliensis, An. triannulatus, An. darlingi, An. deaneorum, An. marajoara, An. peryassui, An. nuneztovari l.s. and An. oswaldoi-konderi. Anopheles darlingi was the most common mosquito in the region. We discuss Culicidae systematics, fauna distribution, and aspects of malaria in altered habitats of the western Amazon

Correlação entre as alterações osteocondrais evidenciadas à ressonância magnética e a progressão da doença

PURPOSE: To determine the consequences of the chronic use of systemic corticosteroids in children with juvenile rheumatoid arthritis by means of evaluating osteochondral effects depicted by magnetic resonance imaging. PATIENTS AND METHODS: We reviewed clinical and magnetic resonance imaging findings in 69 children (72 knees) with juvenile rheumatoid arthritis. Two groups were studied. Group I: 34 (49.3%) children had previous or current use of systemic corticotherapy (22 girls; 12 boys; mean age: 11.3 years; mean disease duration: 5.9 years; mean corticotherapy duration: 2.9 years; mean cumulative dose of previous corticosteroids: 5000 mg); Group II: 35 (50.7%) children had no previous use of corticosteroids (27 girls; 8 boys; mean age: 11.7 years; mean disease duration: 5.3 years). The groups were compared statistically. RESULTS: In the group that had received corticotherapy (Group I), osteochondral abnormalities were significantly correlated to long-standing disease (>;3.5 years; pOBJETIVO: Determinar as conseqüências do uso crônico de corticosteróides sistêmicos em crianças com artrite reumatóide juvenil através da avaliação dos efeitos osteocondrais à ressonância magnética. PACIENTES E MÉTODOS: Achados clínicos e imaginológicos (ressonância magnética) de 72 joelhos em 69 crianças com artrite reumatóide juvenil foram revisados. Trinta e quatro (49.3%) pacientes fizeram uso prévio de corticoterapia sistêmica (22 pacientes do sexo feminino; 12 pacientes do sexo masculino; idade média: 11.3 anos; duração média da doença: 5.9 anos; duração média da corticoterapia: 2.9 anos; dose média cumulativa de corticosteróides: 5000 mg); 35 (50.7%) pacientes não haviam feito uso prévio de corticoterapia sistêmica (27 pacientes do sexo feminino; 8 pacientes do sexo masculino; idade média: 11.7 anos; duração média da doença: 5.3 anos). RESULTADOS: No grupo que recebeu corticoterapia sistêmica prévia (Grupo I) a presença de alterações osteocondrais à ressonância magnética relacionou-se de uma forma estatisticamente significativa com longo tempo de duração da doença (>;3.5 years;

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

Risk factors associated with calcinosis of juvenile dermatomyositis

OBJETIVO: Identificar fatores de risco associados à calcinose em crianças e adolescentes com dermatomiosite juvenil. MÉTODOS: Prontuários de 54 pacientes com dermatomiosite juvenil foram estudados. Foram avaliados dados demográficos; características clínicas: grau de força muscular (I a V do Medical Research Council), presença de comprometimentos pulmonar (distúrbio ventilatório restritivo com presença ou ausência do anticorpo anti-Jo-1), gastrointestinal (refluxo gastroesofágico) e cardíaco (pericardite e/ou miocardite); exames laboratoriais: elevação de enzimas musculares (creatinoquinase, aspartato aminotransferase, alanina aminotransferase e desidrogenase lática) e terapias utilizadas: corticoterapia isolada ou associada à cloroquina e/ou imunossupressor. Os pacientes foram divididos em dois grupos de acordo com a presença ou ausência de calcinose e foram avaliados através de análise univariada e multivariada. RESULTADOS: Calcinose foi evidenciada em 23 (43%) pacientes, sendo em seis (26%) antes do diagnóstico e em 17 (74%) após. A análise univariada revelou que comprometimentos cardíaco (p = 0,01) e pulmonar (p = 0,02) e necessidade da utilização de um ou mais imunossupressores (metotrexato, ciclosporina A e/ou pulsoterapia com ciclofosfamida endovenosa) no tratamento da dermatomiosite juvenil (p = 0,03) foram associados com uma maior incidência de calcinose. A análise multivariada mostrou que comprometimento cardíaco (OR = 15,56; IC95% 1,59-152,2) e uso de um ou mais imunossupressores (OR = 4,01; IC95% 1,08-14,87) foram as únicas variáveis independentes associadas à presença de calcinose. CONCLUSÕES: O aparecimento da calcinose foi freqüente na dermatomiosite juvenil, habitualmente na evolução da doença. A calcinose foi associada aos casos mais graves, que apresentaram envolvimento cardíaco e necessitaram da utilização de imunossupressores no seu tratamento.OBJECTIVE: To identify risk factors associated with calcinosis in children and adolescents with juvenile dermatomyositis. METHODS: A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale), pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies), gastrointestinal problems (gastroesophageal reflux) and/or heart disease (pericarditis and/or myocarditis); laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase); and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses. RESULTS: Calcinosis was identified in 23 (43%) patients, and in six (26%) patients it had emerged prior to diagnosis while in 17 (74%) it was post diagnosis. The univariate analysis revealed that cardiac (p = 0.01) and pulmonary (p = 0.02) involvement and the need for one or more immunosuppressor (methotrexate, cyclosporine A and/or pulse therapy with intravenous cyclophosphamide) to treat juvenile dermatomyositis (p = 0.03) were all associated with an increased incidence of calcinosis. The multivariate analysis then demonstrated that only cardiac involvement (OR = 15.56; 95%CI 1.59-152.2) and the use of one or more immunosuppressor (OR = 4.01; 95%CI 1.08-14.87) were independently associated with the presence of calcinosis. CONCLUSIONS: Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with the more severe cases that also had cardiac involvement and where immunosuppressors had to be included in the treatment

O valor da ética e da formação no ser e fazer polícia no Brasil

Joviano Conceição de Lima é coronel da Polícia Militar do Estado de São Paulo. Exerceu as funções de comandante doPoliciamento de Choque (CPChq), de 2005 a 2008, diretor de Telemática (Dir Tel), em 2005, e chefe da Inteligência – 2ª Seçãodo EM/PM e Agência Central (AC), entre 2003 e 2005. Atuou como professor de Ética Profissional em cursos de habilitação deoficiais, em 1995, Curso de Formação de Oficiais da Academia de Polícia Militar do Barro Branco, de 1995 a 1996, e no Cursode Aperfeiçoamento de Oficiais, de 1996 a 2004. Foi professor de Deontologia Policial Militar (Ética Profissional) no CursoSuperior de Polícia Integrado, de 2005 a 2008, no Centro de Aperfeiçoamento e Estudos Superiores

Validation of a score tool for measurement of histological severity in juvenile dermatomyositis and association with clinical severity of disease.

OBJECTIVES: To study muscle biopsy tissue from patients with juvenile dermatomyositis (JDM) in order to test the reliability of a score tool designed to quantify the severity of histological abnormalities when applied to biceps humeri in addition to quadriceps femoris. Additionally, to evaluate whether elements of the tool correlate with clinical measures of disease severity. METHODS: 55 patients with JDM with muscle biopsy tissue and clinical data available were included. Biopsy samples (33 quadriceps, 22 biceps) were prepared and stained using standardised protocols. A Latin square design was used by the International Juvenile Dermatomyositis Biopsy Consensus Group to score cases using our previously published score tool. Reliability was assessed by intraclass correlation coefficient (ICC) and scorer agreement (α) by assessing variation in scorers' ratings. Scores from the most reliable tool items correlated with clinical measures of disease activity at the time of biopsy. RESULTS: Inter- and intraobserver agreement was good or high for many tool items, including overall assessment of severity using a Visual Analogue Scale. The tool functioned equally well on biceps and quadriceps samples. A modified tool using the most reliable score items showed good correlation with measures of disease activity. CONCLUSIONS: The JDM biopsy score tool has high inter- and intraobserver agreement and can be used on both biceps and quadriceps muscle tissue. Importantly, the modified tool correlates well with clinical measures of disease activity. We propose that standardised assessment of muscle biopsy tissue should be considered in diagnostic investigation and clinical trials in JDM

Responsiveness to exercise training in juvenile dermatomyositis: a twin case study

<p>Abstract</p> <p>Background</p> <p>Patients with juvenile dermatomyositis (JDM) often present strong exercise intolerance and muscle weakness. However, the role of exercise training in this disease has not been investigated.</p> <p>Purpose</p> <p>this longitudinal case study reports on the effects of exercise training on a 7-year-old patient with JDM and on her unaffected monozygotic twin sister, who served as a control.</p> <p>Methods</p> <p>Both the patient who was diagnosed with JDM as well as her healthy twin underwent a 16-week exercise training program comprising aerobic and strengthening exercises. We assessed one repetition-maximum (1-RM) leg-press and bench-press strength, balance, mobility and muscle function, blood markers of inflammation and muscle enzymes, aerobic conditioning, and disease activity scores. As a result, the healthy child had an overall greater absolute strength, muscle function and aerobic conditioning compared to her JDM twin pair at baseline and after the trial. However, the twins presented comparable relative improvements in 1-RM bench press, 1-RM leg press, VO_2peak, and time-to-exhaustion. The healthy child had greater relative increments in low-back strength and handgrip, whereas the child with JDM presented a higher relative increase in ventilatory anaerobic threshold parameters and functional tests. Quality of life, inflammation, muscle damage and disease activity scores remained unchanged.</p> <p>Results and Conclusion</p> <p>this was the first report to describe the training response of a patient with non-active JDM following an exercise training regimen. The child with JDM exhibited improved strength, muscle function and aerobic conditioning without presenting an exacerbation of the disease.</p