Mean Platelet Volume and Immature Platelet Fraction in Autoimmune Disorders.

Mean platelet volume (MPV), measured using automated blood analysers, has been appraised as a potential biomarker in cardiovascular disease, diabetes mellitus, and cancer. The test, a useful tool in differentiation of thrombocytopenic states, has now been carried out for autoimmune disorders, but data are yet scarce. Controversial results have been obtained in systemic and organ-specific autoimmune disorders. Another test, the immature platelet fraction (IPF) reflects the amount of young, reticulated platelets. IPF is calculated by automated hematology analysis or flow cytometry, and it is usually high in patients with rapid platelet destruction. For both MPV and IPF, standardization of cutoff is a major need. In this review, we focus the current applicability of MPV and IPF as biomarkers in patients with autoimmune diseases.

Arterial stiffness by oscillometric device and telomere lenght in juvenile idiopathic artrhitis with no cardiovascular risk factors: a cross-sectional study.

BACKGROUND: Advances in juvenile idiopathic arthritis (JIA) treatment is promoting free disease survival. Cardiovascular disease (CVD) may emerge as an important cause of morbidity and mortality. Pulse wave velocity (PWV), a surrogate marker of arterial stiffness, and telomere length (TL) are considered as potential predictors of CVD and its outcomes. The study aim was to assess PWV, TL in a JIA population and to test its correlation. In a cross sectional study, 24 JIA patients, 21 controls for TL and 20 controls for PWV were included. PWV was assessed by an oscillometric device. TL was assessed by qPCR. JIA activity was accessed by JADAS-27. Smoking, diabetes, obesity, renal impairment, hypertension, dyslipidemia and inflammatory diseases were excluded. FINDINGS: Between cases and controls for TL, there was significant difference in age. No differences in gender, ethnics and bone mass index between JIA and control groups for PWV and TL. The JADAS-27 median was 8. TL was significantly reduced in JIA (0.85 ± 0.34 vs. 1. 67 ± 1.38, P = 0.025). When age adjusted by ANCOVA, the difference remained significant (P = 0,032). PWV was normal in all patients (5.1 ± 0.20 m/s vs. 4.98 ± 0.06 m/s, P = 0, 66). There was no correlation between TL, PWV or JADAS-27. CONCLUSION: Compared to controls, JIA with high disease activity and no CVD risk factors have shorter telomeres and normal PWV. As far as we know, this first time this correlation is being tested in rheumatic disease and in paediatrics.

Juvenile idiopatic arthritis in orthodontics: Case report with a 6-year follow-up.

Juvenile idiopathic arthritis is a childhood disease that causes joint swelling and pain, and limitation in the range of joint movements. It is characterized by progressive destruction of the joints. Juvenile idiopathic arthritis is the most frequent systemic inflammatory disease of the temporomandibular joint. When the temporomandibular joint is involved, orthodontic treatment becomes more challenging. This case report shows the treatment of a young patient with a Class II subdivision malocclusion and juvenile idiopathic arthritis. Excellent results were achieved and maintained at the 6-year follow-up, when neither clinical symptoms nor radiographic changes in the temporomandibular joint were seen.

Doença de Behçet na adolescência: relato de um caso com boa resposta à sulfasalazina / Behçet's disease in adolescence: case report with a good response to sulfasalazine

As autoras relatam a história, o diagnóstico e a evolução de um caso de um menino de 14 anos com doença de Behçet. Trata-se de uma vasculite crônica, multissistêmica, de causa desconhecida, que se caracteriza por úlceras orais recorrentes, úlceras genitais, uveíte e lesões de pele. O tratamento desta doença varia de acordo com os autores, dependendo das manifestações clínicas do paciente. Dentre as drogas utilizadas incluem-se esteróides tópicos, colchicina e agentes citotóxicos tais como azatioprina, clorambucil e ciclofosfamida. O interferon e, mais recentemente, um bloqueador TNF-α também vêm sendo prescritos. Os benefícios da sulfasalazina no tratamento da vasculite intestinal têm sido relatados há bastante tempo (2,19,20,21). No entanto, na literatura revisada, não se encontrou nenhum artigo que referisse o uso desta droga em adolescentes portadores de Behçet. A boa resposta à sulfasalazina e a baixa ocorrência de efeitos colaterais motivaram o relato deste caso (AU)

The authors report a case of Behçet's disease in a 14 year-old boy with history, diagnosis and evolution of this pathology. Behçet's disease is a multistemic vasculitis of unknow cause, with recurrent oral and genital ulcers, uveitis and skin lesions. The treatment varies according to different authors, taking into consideration the patients' clinical manifestations. The drugs that can be used in these cases are topic steroids, colchicine, cytotoxic agents such as azatioprine, clorambucil and ciclofosfamide. Interferon and a TNF-α blocker have also been used. The benefits of sulfasalazine in the treatment of patients with intestinal vasculitis have been reported before (2,19,20,21). Nevertheless, no data was found referring to the use of this drug in adolescents with Behçet's disease. The satisfactory response to sulfalazine and the rare occurrence of side effects were the motivations to report this case (AU)

Anticorpos anticardiolipina em pacientes com abortamento recorrentes / Anticardiolipin antibodies in patients with recurrent abortions

Objetivo: Avaliar a prevalência de anticorpos anticardiolipina (aCL) em populaçao selecionada de pacientes com abortamento recorrentes de causa indefinida. Material e métodos: Quarenta e três pacientes com histórico de no mínimo dois abortamentos espontâneos foram estudadas quanto à prevalência de aCL no soro. Presença de causas gineco-obstétricas de abortamentos e evidência de doenças difusas do tecido conjuntivo foram consideradas critérios de exclusao. A quantificaçao de IgG e IgM aCL foi realizada através de ELISA. Vinte soros de doadores de banco de sangue pareados por sexo e idade foram utilizados como controles. Resultados: Nenhuma paciente teve IgM aCL em níveis consideráveis no soro. Três pacientes (6,9 por cento) apresentaram níveis moderados ou altos de IgG aCL. Nenhum dos soros-controles apresentou aCL em níveis relevantes. A diferença de positividade para aCL entre pacientes e controles nao foi estatisticamente significante (p>0,54, teste de Fisher). Conclusoes: A prevalência de níveis significantes de aCL em nossa populaçao-alvo foi baixa. No entanto, o eventual diagnóstico de síndrome antifosfolipídica (SAFp), como confirmado em três de nossas pacientes, pode fazer perspectivas terapêuticas em futuras gestaçoes. Este dado corrobora a utilidade do teste aCL em casos de abortamentos repetidos de origem indefinida

Esclerose sistêmica e púrpura trombocitopênica / Systemic sclerosis and thrombocytopenic purpura

Na esclerose sistêmica, os estudos hematológicos usualmente nao se mostram alterados; anormalidades sugerem complicaçoes ou doenças associadas. Os autores descrevem o caso de uma paciente com esclerose sistêmica com alteraçoes cutâneas, pulmonares e cardíacas que desenvolveu púrpura trombocitopênica de características auto-imunes