Concentração de ferro anormalmente aumentada nos núcleos da base na síndrome de Shy-Drager

Report of an early case of Shy-Drager syndrome in a 67 year-old woman patient. Autonomic failure was diagnosed by functional evaluation as well as laboratory tests. MR imaging disclosed a prominent putamina hypodensity in T2-weighted images at high field strength due to iron increased depositing in this basal ganglia. MR imaging evidences confirm Shy-Drager syndrome diagnosis, and contributes for differential diagnosis of idiopathic hypotension (pure autonomic failure) in special in SDS early cases.É relatado o caso de unm paciente de 67 anos de idade com quadro inicial da síndrome de Shy-Drager. O diagnóstico foi possível por provas funcionais autonômicas e exames laboratoriais. A ressonância magnética cerebral (contraste baseado na densidade de prótons e em T2) objetivou proeminente hipodensidade putaminal em T2, secundária ao aumento do depósito do ferro nesta região. Esse achado da RM confirma o diagnóstico da síndrome de Shy-Drager e permite diferenciá-la da hipotensão ortostática idiopática, particularmente na fase de início da SSD quando os sinais de comprometimento do SNC são discretos ou estão ausentes.34234

Skaken baby syndrome: case report

The authors report the case of a 50-day-old baby who was brought to the Pediatric Emergency Room of the Hospital de Clinicas da UNICAMP with seizures. At the examination she presented without external signs of trauma, hypoactive and with generalized seizures. Numerous hemorrhages were found in the ocular fundi. CT scan showed interhemisferic hemorrhage and brain swelling. The child was admitted to the Pediatric Intensive Care Unit, kept under controlled ventilation and hydantal. Although these measures, she died three days later. Since the beginning there was suspicion of child abuse. One day after the admission the father told that he had shaken the baby because she was crying too much. The present paper discuss social and epidemiological aspects, and about the difficulties in the diagnosis of this syndrome that sometimes may be fatal, as in this case.Os autores relatam o caso de uma lactente de 50 dias de vida que foi trazida ao Pronto Socorro Pediátrico do Hospital de Clínicas da UNICAMP com crises convulsivas. Ao exame apresentava-se sem sinais externos de trauma, hipoativa e com crises convulsivas generalizadas. A fundoscopia evidenciou hemorragia retiniana difusa bilateral. Foi submetida a tomografia de crânio que mostrou hemorragia inter-hemisférica e swelling cerebral. A criança foi internada na UTI pediátrica, mantida sob ventilação assistida e recebendo hidantal porém, apesar do tratamento, evoluiu para óbito três dias depois. Desde o início havia suspeita de maus-tratos, o que foi confirmado um dia após a internação quando o pai relatou que chacoalhou a criança porque ela chorava bastante. O presente estudo tem como objetivo ressaltar aspectos sociais, epidemiológicos e a dificuldade diagnostica desta síndrome que algumas vezes pode ser fatal, como neste caso.64965

T-Cell Memory Responses Elicited by Yellow Fever Vaccine are Targeted to Overlapping Epitopes Containing Multiple HLA-I and -II Binding Motifs

The yellow fever vaccines (YF-17D-204 and 17DD) are considered to be among the safest vaccines and the presence of neutralizing antibodies is correlated with protection, although other immune effector mechanisms are known to be involved. T-cell responses are known to play an important role modulating antibody production and the killing of infected cells. However, little is known about the repertoire of T-cell responses elicited by the YF-17DD vaccine in humans. In this report, a library of 653 partially overlapping 15-mer peptides covering the envelope (Env) and nonstructural (NS) proteins 1 to 5 of the vaccine was utilized to perform a comprehensive analysis of the virus-specific CD4+ and CD8+ T-cell responses. The T-cell responses were screened ex-vivo by IFN-γ ELISPOT assays using blood samples from 220 YF-17DD vaccinees collected two months to four years after immunization. Each peptide was tested in 75 to 208 separate individuals of the cohort. The screening identified sixteen immunodominant antigens that elicited activation of circulating memory T-cells in 10% to 33% of the individuals. Biochemical in-vitro binding assays and immunogenetic and immunogenicity studies indicated that each of the sixteen immunogenic 15-mer peptides contained two or more partially overlapping epitopes that could bind with high affinity to molecules of different HLAs. The prevalence of the immunogenicity of a peptide in the cohort was correlated with the diversity of HLA-II alleles that they could bind. These findings suggest that overlapping of HLA binding motifs within a peptide enhances its T-cell immunogenicity and the prevalence of the response in the population. In summary, the results suggests that in addition to factors of the innate immunity, "promiscuous" T-cell antigens might contribute to the high efficacy of the yellow fever vaccines. © 2013 de Melo et al