RESUMO Objetivo: Destacar a importância do conhecimento sobre os novos critérios de classificação para síndrome de ativação macrofágica (SAM) na artrite idiopática juvenil sistêmica para reduzir a morbidade e mortalidade desse desfecho. Descrição do caso: Adolescente do sexo feminino de 12 anos de idade, em terapia imunossupressora por diagnóstico de artrite idiopática juvenil sistêmica há 2 anos, com quadro de tosse, dor precordial aguda, taquipneia, taquicardia e hipoxemia há 2 dias. A tomografia de tórax evidenciou efusão pleural laminar bilateral com consolidação bibasal. O eletrocardiograma foi compatível com pericardite aguda, e o ecocardiograma foi normal. Os exames laboratoriais revelaram anemia, leucocitose e aumento da velocidade de hemossedimentação, proteína C-reativa e marcadores séricos de lesão miocárdica. Infecção sistêmica e/ou doença sistêmica em atividade foram consideradas. A paciente foi tratada com antibióticos e glicocorticoide. Entretanto, dez dias depois, evoluiu com doença sistêmica em atividade (febre, exantema e miopericardite com insuficiência cardíaca) associada à SAM, de acordo com o 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis, e necessitou de cinco dias de pulsoterapia com glicocorticoide, imunoglobulina e ciclosporina A, com melhora de todos os parâmetros clínicos e laboratoriais. Comentários: A miopericardite com sinais de insuficiência cardíaca associada à SAM é uma apresentação clínica rara da artrite idiopática juvenil sistêmica, que ocorre principalmente em períodos de atividade sistêmica da doença e pode ser deflagrada por infecções. O conhecimento sobre essa síndrome é fundamental para reduzir morbidade e mortalidade desse grave desfecho.

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

Abstract The "racket" lesion is a rare presentation of tuberculoid leprosy, which consists of a thickened nerve branch emerging from a tuberculoid plaque. It results from centripetal damage to cutaneous nerves caused by granuloma formation. We describe a typical case of tuberculoid leprosy presenting as a "racket" lesion. The lesion persisted after treatment with paucibacillary multidrug therapy.

We conducted a transversal retrospective study with secondary data collection from 25 cases of sporotrichosis, treated at a teaching unit in inner São Paulo (Brazil), between the years 2003-2013. We found that the prevalence was higher in men (72%), rural workers (44%) and those living in rural areas (60%), with an average age of 42.48 years. The median between the onset of lesions and diagnosis was six weeks. Lesions predominated in the upper limbs (92%), and were classified as lymphocutaneous (80%) and fixed cutaneous (20%) forms. Clinical cure was observed in 62.5% of the cases treated with potassium iodide and 100% of cases treated with itraconazole.

Sleep disorders have an increased incidence after menopause. The objective of this work was to evaluate the effects of isoflavone on some oxidative stress markers in postmenopausal women complaining of insomnia. Women aged between 50-65 years (n=38) were recruited and assigned to a double-blind placebo controlled study for 4 months. The treated group received 100 mg/day of isoflavones. Blood collections were conducted on three different occasions to assess total glutathione; superoxide dismutase and catalase in erythrocytes; lipid peroxidation; and homocysteine plasma concentrations. No differences between the groups were observed. However, all the patients seem to improve their oxidative stress status and homocysteine concentration after treatment. Superoxide dismutase activity was correlated with age and time of menopause at the beginning of the treatment, but these correlations were no longer observed by the end of the study. Soy isoflavones were not able to overcome the placebo effect for either oxidative stress parameters or homocysteine concentrations.

A alcaptonúria (ocronose) é um erro inato do metabolismo da fenilalanina e tirosina, transmitido de forma autossômica recessiva. Resulta da deficiência completa da enzima ácido homogentísico oxidase (HGO), causada por mutação no gene 3q (3q21 - q23), levando ao acúmulo do ácido em diversos órgãos e tecidos, com aumento de sua excreção urinária. Os achados clínicos característicos incluem artropatia ocronótica, pigmentação anormal da cartilagem de outros tecidos conjuntivos e urina enegrecida. A incidência é rara, estimada em 1-4: 1.000.000 indivíduos, tendo maior prevalência em populações com alto grau de consangüinidade. A seguir, relataremos o caso de dois irmãos (um homem e uma mulher) com ocronose, discutindo sua patogênese, manifestações clínicas, diagnóstico e tratamento(1, 2).

Alkaptonuria (ochronosis) is an autossomal recessive disorder which results from an innate error of phenylalanine and tyrosine’s metabolism. This defect causes accumulation of homogentisic acid on the tissue and usually is excreted in the urine. This disorder results from a complete deficiency of the homogentisate 1,2 - dioxygenase (HGO) caused by chromosome 3q(3q21-q23) mutation. The clinical features include: ochronotic artropathy, black-gray pigmentation of the cartilage and the urine turns black. This is a rare disorder and incidence is about 1-4 in 1.000.000 of people and the prevalence is higher in consanguineous groups. The author will describe two affected brothers and discuss the pathophysiology, clinical features, diagnosis and treatment of ochronosis.

A osteonecrose é uma terminologia que se refere à morte celular de dois componentes do osso: a medula hematopoiética e os osteócitos. É mais freqüente em homens do que em mulheres (8:1) e a maioria dos casos acontece em pacientes abaixo dos 50 anos de idade. Pacientes infectados pelo vírus HIV encontram-se em maior risco de desenvolver osteonecrose, estando implicados como fatores predisponentes a terapêutica antiviral, ao uso de corticosteróides e outros fatores associados, comuns à população geral. Os autores fazem uma revisão dos aspectos etiológicos, clínicos, patogênicos e diagnóstico, além de aspectos relacionados a esta entidade nos enfermos infectados pelo HIV.

Osteonecrosis is a generic term that refers to cell death in the two components of the bone: hematopoietic fat marrow and osteocytes. It is more frequent in men than women (8:1), with the vast majority of the cases being reported in patients under age fifty. HIV-infected patients appears to be at major risk to the development of osteonecrosis, and the predisposing factors listed are the antiretroviral therapy, corticosteroids use and others risk factors that can be encountered on the general population. The authors get thorough a review of the etiology, clinical picture, pathogenesis, diagnosis, and the selected aspects of this entity in HIV-infected individuals.

O objetivo deste estudo foi comparar a resistência à compressão e o limite de fadiga compressiva de três resinas compostas indicadas para dentes posteriores (Filtek P-60, Surefil e Prodigy Condensable) e uma universal (Z-100). Corpos-de-prova cilíndricos (8 mm de altura x 4 mm de diâmetro) foram usados. O teste dinâmico foi realizado usando-se o método escada e a relação entre limite de fadiga compressiva, e resistência à compressão também foi calculada (n = 15). Os dados de resistência à compressão e de limite de fadiga compressiva foram submetidos à Anova e ao teste de Tukey. O compósito Z-100 apresentou maior resistência à compressão (307,20 MPa) que Surefil (266,93 MPa) e Prodigy Condensable (222,08 MPa). A resistência de Filtek P-60 (270,44 MPa) foi similar à de Z-100 e à de Surefil, enquanto Prodigy Condensable apresentou a menor resistência à compressão. No teste de limite de fadiga compressiva, Filtek P-60 mostrou maior valor (184,20 MPa) que Prodigy Condensable (155,50 MPa). Os compósitos Surefil (165,74 MPa) e Z-100 (161,22 MPa) mostraram-se similares a Filtek P-60 e Prodigy Condensable. A relação limite de fadiga compressiva/resistência à compressão foi de 70,01% para Prodigy Condensable, 68,11% para Filtek P-60, 62,09% para Surefil e 52,48% para Z-100. Foi concluído que o compósito universal Z-100 foi mais sensível ao teste dinâmico que os materiais de alta viscosidade.

The purpose of this study was to compare the compressive strengths and compressive fatigue limits of three posterior composite resins (Filtek P-60, Surefil and Prodigy Condensable) and a universal restorative composite (Z-100). Cylindrical specimens (8 mm in length x 4 mm in diameter) were used. The dynamic test was performed using the staircase method, and the ratio between compressive fatigue limit and compressive resistance was also calculated (n = 15). The compressive strength and compressive fatigue limit data were analyzed by Anova and Tukey’s test. The Z-100 composite demonstrated higher compression strength (307.20 MPa) than Surefil (266.93 MPa) and Prodigy Condensable (222.08 MPa). The resistance of Filtek P-60 (270.44 MPa) was similar to the resistances of Z-100 and Surefil, while Prodigy Condensable presented the lowest compressive strength. In the compressive fatigue limit tests, Filtek P-60 demonstrated a higher value (184.20 MPa) than Prodigy Condensable (155.50 MPa). Surefil (165.74 MPa) and Z-100 (161.22 MPa) presented limits similar to those of Filtek P-60 and Prodigy Condensable. The compressive fatigue limit/compressive strength ratio was 70.01% for Prodigy Condensable, 68.11% for Filtek P-60, 62.09% for Surefil and 52.48% for Z-100. It was concluded that the Z-100 universal composite was more sensitive to the dynamic test than the high viscosity materials.