Findings in the anterior segment on ultrasound biomicroscopy in Maroteaux-Lamy syndrome

Purpose. Maroteaux-Lamy syndrome is one of the mucopolysaccharidoses caused by enzyme deficiency (arylsulfatase B) that leads to incomplete degradation and storage of dermatan sulfate. We report a case of mucopolysaccharidosis type VI (MPS VI; Maroteaux-Lamy syndrome) with corneal involvement and introduce ultrasound biomicroscopy (UBM) as an examination with which to follow disease progression in relation to deposition in cornea, angle, and iris, Methods. We describe a Ii-year-old boy with a clinical and laboratorial diagnosis of MPS VI who developed increasing bilateral corneal opacification and decreased visual acuity. He underwent two seriate UBM (50-MHz transducer) evaluations. Results. UBM examination showed diffuse and homogeneous stromal hyper-reflective deposit in both eyes and an increase in peripheral corneal thickness throughout time. Conclusion. High-frequency ultrasound documentation of corneal deposit and anterior segment involvement in a patient with Maroteaux-Lamy syndrome is unique, and follow-up revealed thickening of the corneal periphery, which may be related to the progression of the disease (continuous mucopolysaccharide deposits in corneal stroma). UBM was used to locate and document the deposit, as well as to accompany the deposit's evolution, characterizing corneal changes and angle structure involvement.Universidade Federal de São Paulo, Dept Ophthalmol, Paulista Sch Med, BR-04023062 São Paulo, BrazilUniversidade Federal de São Paulo, Dept Ophthalmol, Paulista Sch Med, BR-04023062 São Paulo, BrazilWeb of Scienc

Bilateral deep keratitis caused by systemic lupus erythematosus

We report the optical and ultrasonic biomicroscopy and confocal microscopy findings in bilateral stromal keratitis (keratoendotheliitis), a rare ocular manifestation of systemic lupus erythematosus (SLE). Examination revealed deposits with polyrefringent crystals. Topical corticosteroid produced regression of the corneal edema, but there was an increase in corneal opacity. Ultrasound biomicroscopy images confirmed the deep location of the corneal opacities, and confocal microscopy showed a disruption of the corneal stroma and crystal-like bodies.Universidade Federal de São Paulo, Dept Ophthalmol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Reumatol, São Paulo, BrazilUniv Santo Amaro, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Dermatol, São Paulo, BrazilUniv Calif Davis, Dept Ophthalmol, Davis, CA 95616 USAUniversidade Federal de São Paulo, Dept Ophthalmol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Reumatol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Dermatol, São Paulo, BrazilWeb of Scienc

Comitê de Ética em Pesquisa da Escola de Enfermagem da USP: experiência dos primeiros seis anos Comité de Ética en Investigación de la Escuela de Enfermería de la USP: experiencia de los primeros seis años de funcionamiento Research Ethics Committee of the University of São Paulo Nursing School: experience of the first six years

O objetivo do estudo foi conhecer a experiência do Comitê de Ética em Pesquisa da Escola de Enfermagem da USP (CEP-EEUSP), nos seus primeiros seis anos. O estudo exploratório, descritivo, retrospectivo e de abordagem quantitativa foi realizado pelo levantamento dos registros do CEP-EEUSP e dos 401 protocolos de pesquisa submetidos à apreciação, no período de 31 de agosto de 1998 a 17 de setembro de 2004. Os resultados evidenciaram que 98,7% dos projetos submetidos eram de pesquisadores da EEUSP; 31,4% eram de dissertações de mestrado e 29,1% de iniciação científica. Quanto ao método, 43,8% propunham a utilização do método qualitativo. Além disso, 99,2% dos projetos pertenciam à área temática do Grupo III; 58,8% previam a utilização de instituições públicas e 31,7% tinham profissionais, principalmente enfermeiros, como sujeitos das pesquisas. O Termo de Consentimento Livre e Esclarecido foi responsável por 56,4% das pendências. A experiência do CEP-EEUSP mostra sua progressiva consolidação.<br>El objetivo de este estudio fue conocer la experiencia del Comité de Ética en Investigación de la Escuela de Enfermería de la USP en los primeros seis años de funcionamiento. Se trata de un estudio exploratorio, descriptivo, retrospectivo e de abordaje cuantitativo realizado con base en los registros del CEP-EEUSP y de los 401 protocolos de investigación presentados para evaluación en el período del 31 de agosto de 1998 hasta 17 de septiembre de 2004. Los resultados mostraron que 98,7% de los proyectos fueron presentados por investigadores de la EEUSP; 31,4% eran de trabajos de maestría y 29,1% de iniciación científica. Cuanto a los métodos utilizados, 43,8% de los proyectos eran relativos al abordaje cualitativo. Además, 99,2% pertenecían a la área temática del Grupo III; 58,8% propusieron la utilización de instituciones públicas y 31,7% tenían profesionales, principalmente enfermeros, como sujetos investigados. El Termo de Consentimiento Libre e Informado fue el responsable por 56,4% de las pendencias. La experiencia del CEP-EEUSP muestra su progresiva consolidación.<br>The aim of this study was to know the experience of the first six years of the Research Ethics Committee (REC) of the University of São Paulo Nursing School activities. The exploratory, descriptive, retrospective and quantitative study analised the records of the REC and of all the 401 research protocols submitted for appreciation from 31st August, 1998 to 17th September, 2004. The results showed that 98.7% of protocols were from in-house researchers; 31.4% were master degree dissertations and 29.1% were graduate students scientific initiation. Qualitative methods were used by 43.8%. Furthemore, 99.2% were classified as Group III thematic area; 58.8% researches were conducted in public institutions and 31.7% enrolled professionals, mostly nurses as research subjects. Informed Consent accounts for 56.4% of the REC's querries. The experience of the REC of the University of São Paulo Nursing School shows its progressive consolidation

Consentimento livre e esclarecido: experiência de pesquisadores brasileiros na área da regulação da fecundidade Free informed consent: the experience of Brazilian researchers in the area of fertility regulation

O objetivo deste estudo foi avaliar a experiência de pesquisadores com a apreciação do protocolo por um Comitê de Ética em Pesquisa (CEP); com a elaboração do Termo de Consentimento Livre e Esclarecido (TCLE) e com a obtenção do consentimento, e verificar se o conteúdo do TCLE estava de acordo com as exigências da Resolução 196/96. Trinta e quatro pesquisadores completaram um questionário auto-respondido, enviado por correio eletrônico. Quase todos os participantes (91%) relataram que o protocolo desse estudo havia sido avaliado por um CEP. Mais da metade deles referiram que o TCLE utilizado havia sido recebido pronto; que o consentimento do possível sujeito havia sido obtido pelo pesquisador responsável, e que os sujeitos haviam recebido uma cópia do Termo assinada. Cerca da metade dos participantes afirmaram que a forma de ressarcimento das despesas dos sujeitos, decorrentes da participação na pesquisa, e 44% disseram que as formas de indenização, no caso de eventuais danos, constavam no Termo. De acordo com o relato dos participantes, em geral as exigências da Resolução avaliadas haviam sido cumpridas.<br>This study aimed to evaluate the experience of Brazilian researchers in relation to: assessment of the research protocol by a research ethics committee with the elaboration of a free informed term of consent (FITC) and the process of obtaining consent, as well as to verify whether the content and wording of the term of consent complies with Federal Ruling 196/96. Thirty-four researchers filled out a questionnaire and returned it by electronic mail. The vast majority of the respondents (91%) reported that the respective study protocol had been evaluated by a research ethics committee. More than half reported that they had received a previously prepared FITC; that consent from potential study subjects had been obtained by the researcher in charge; and that the subjects had received a copy of their signed FITC. Approximately one half of participants stated that the form of reimbursement of subjects was specified in the FITC, and 44% reported the same in relation to occasional damages resulting from the study. According to participants, in general the provisions of Ruling 196/96 had been met

Newly discovered reefs in the southern Abrolhos Bank, Brazil: Anthropogenic impacts and urgent conservation needs

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)CNPq: GDE 202475/ 2011-5CNPq: 54515130/2011The Abrolhos Bank is an area of high ecological, socio-economic importance and harbour the richest and most extensive coral reefs in the South Atlantic. Here we report the discovery of shallow (12-25 m depth) reef complex with ten large biogenic structures, intermediate between the typical mushroom-shaped pinnacles of the northern Abrolhos Bank (17 degrees-18 degrees S) and the small patch reefs found on the central/southern coast of the Espirito Santo State (19 degrees-20 degrees S). The newly discovered reefs harbour a relatively rich and abundant reef community, with 73 fish and 14 benthic cnidarian species, including endangered and commercially important ones. We discuss on urgent needs of properly mapping and understanding the ecological functioning of this reef system. Information provided here is a baseline for future impact evaluations, particularly considering the recent worst environmental disaster of Brazil from a dam collapse in Doce river that affected the region. (C) 2016 Elsevier Ltd. All rights reserved