Resumo Estudos sobre crenças em Linguística Aplicada têm se desenvolvido com base na abordagem metodológica contextual e discursiva, que requer novos instrumentos de pesquisa e métodos de análise de dados para contemplar as histórias, experiências e emoções dos participantes. (CRUZ, 2017; HAUS, 2018; KALAJA et al., 2018) Com base nisso, este trabalho apresenta um recorte de uma pesquisa de doutorado que investigou as crenças de doze acadêmicos do curso de Licenciatura em Letras-Inglês da Universidade Tecnológica Federal do Paraná, Campus Curitiba, professores de inglês em formação inicial, sobre a pronúncia de inglês. Este trabalho tem como objetivo apresentar o círculo de diálogo, concebido nos domínios da Justiça Restaurativa, como uma ferramenta de pesquisa que atua diante das dificuldades estabelecidas pelas interrelações existentes entre crenças e emoções. Ao final de cada ciclo, em posse do objeto de fala, os participantes responderam à pergunta: “Como você se sentiu nesse círculo de diálogo?”. Os dados apontam para uma avaliação positiva dos participantes sobre essa ferramenta, com destaque para aspectos como: respeito à fala, valorização das ideias através da atenção dos ouvintes, possibilidade de falar sem ser interrompido, contato com histórias e opiniões diferentes e contato com as próprias crenças, que até aquele momento eram inconscientes. Os dados também apontam a necessidade de uma reeducação voltada ao comportamento nas diferentes interações sociais em que atuamos. Trata-se de um trabalho que pode enriquecer a pesquisa, o ensino e os relacionamentos humanos em diferentes âmbitos.

Abstract Studies about beliefs in Applied Linguistics have been developed based on contextual and discursive methodological approaches, which require new research instruments and data analysis methods to consider the participants’ histories, experiences, and emotions (CRUZ, 2017; HAUS, 2018; KALAJA et al., 2018). Based on that, this work presents a narrowed portion of a doctoral research that investigated beliefs of twelve undergraduate students of the undergraduate degree in English Language from Universidade Federal do Paraná, campus Curitiba, future English teachers, about English pronunciation. This work aims to present the circle of dialogue, from the domains of Restorative Justice, as a research instrument that contributes in the face of the difficulties established by the interrelations between beliefs and emotions. At the end of each circle, holding the talking piece, the participants answered the question: “How did you feel in this circle of dialogue?”. The results indicate a positive evaluation of the participants about the instrument, mostly related to aspects such as: respect to the speech, appreciation of ideas by receiving the listeners’ attention, possibility of speaking without interruptions, contact with different opinions and histories, and contact with the participants’ own beliefs, which were unconscious up to that point. The data also suggest that behavior reeducation is needed in different social interactions we act in. This is a work that may enrich research, teaching, and human relations in different contexts.

Abstract Fabry disease, caused by deficient alpha-galactosidase A lysosomal enzyme activity, remains challenging to health-care professionals. Laboratory diagnosis in males is carried out by determination of alpha-galactosidase A activity; for females, enzymatic activity determination fails to detect the disease in about two-thirds of the patients, and only the identification of a pathogenic mutation in the GLA gene allows for a definite diagnosis. The hurdle to be overcome in this field is to determine whether a mutation that has never been described determines a ‘‘classic’’ or ‘‘nonclassic’’ phenotype, because this will have an impact on the decision-making for treatment initiation. Besides the enzymatic determination and GLA gene mutation determination, researchers are still searching for a good biomarker, and it seems that plasma lyso-Gb3 is a useful tool that correlates to the degree of substrate storage in organs. The ideal time for treatment initiation for children and nonclassic phenotype remains unclear.

Fabry Disease, an X-linked inborn error of metabolism, is characterized by progressive renal insufficiency, with cardio and cerebrovascular involvement. Homocysteine (Hcy) is considered a risk factor for vascular diseases, but the mechanisms by which it produces cardiovascular damage are still poorly understood. Regarding the vascular involvement in FD patients, the analysis of factors related to thromboembolic events could be useful to improving our understanding of the disease. The aim of this study was to evaluate plasma Hcy and other parameters involved in the methionine cycle, as well as oxidative stress markers. The sample consisted of a group of 10 male FD patients and a control group of 8 healthy individuals, paired by age. Venous blood was collected for Hcy determination, molecular analysis, identification of thiobarbituric acid reactive substances, total glutathione and antioxidant enzymes activity, as well as vitamins quantification. Comparative analysis of FD patients versus the control group indicated hyperhomocysteinemia in 8 of the 10 FD patients, as well as a significant increase in overall glutathione levels and catalase activity. It is inferred that FD patients, apart from activation of the antioxidant system, present increased levels of plasma Hcy, although this is probably unrelated to common alterations in the methionine cycle.

A doença de Gaucher (DG) é um erro inato do metabolismo do grupo das doenças lisossômicas de depósito, sendo a mais freqüente do referido grupo. É de herança autossômica recessiva, portanto com risco de 25% a cada gestação de casal heterozigoto. A doença é resultante da deficiência da beta-glicosidase ácida ou beta-glicocerebrosidase, que leva ao acúmulo de glicolipídios nos macrófagos principalmente em baço, fígado, medula óssea e pulmão. As manifestações clínicas ou fenotípicas da DG vão depender do grau de deficiência da enzima, existindo três tipos: Tipo I, forma não neuropática, afeta crianças e adultos com hepatoesplenomegalia, anemia, trombocitopenia, leucopenia e lesões ósseas; Tipo II, forma neuropática aguda, afeta crianças com 4-5 meses com quadro neurológico grave, hepatoesplenomegalia e comprometimento pulmonar e o Tipo III, forma neuropática crônica, afeta crianças e adolescentes com quadro neurológico menos grave que o Tipo II e ainda pode comprometer fígado, baço e ossos. Um grupo de catorze médicos com experiência no tratamento da DG com reposição enzimática realizaram extensa revisão da literatura, confrontaram com os dados evolutivos dos pacientes brasileiros e chegaram a um consenso quanto aos critérios para iniciar o tratamento, a dose da enzima e freqüência das infusões, do acompanhamento ambulatorial, laboratorial e radiológico. O Grupo Brasileiro de Estudos em Doença de Gaucher e outras Doenças de Depósito Lisossômico (GBDDL) tem o objetivo de estabelecer diretrizes para o diagnostico, tratamento e acompanhamento de pacientes com doença de Gaucher no Brasil. Esta iniciativa pioneira visa uniformizar a conduta no país com relação ao tratamento de DG com reposição enzimática, tratamento de alto custo porém de grande eficácia.

Gaucher disease is one of the inborn errors of metabolism from lysossomal storage diseases and the most frequent of this group. It iss an autosomal recessive disease with reoccurrence risk of 25% in each pregnancy of hetrozygotes parents. The disease results from an b-glucosidase acid or b-glucocerebrosidase deficiency leading to accumulation of glucolipids mainly in the spleen, liver, bone marrow, and lung macrophages. The clinical manifestations of Gaucher disease depend on the enzyme deficiency level. There are three types of the disease, namely, Type I, corresponding to a non-neuropathic form affecting both adults and children with hepatosplenomegaly, anemia, thrombocytopenia, leukopenia, and bone lesions; Type II, corresponding to an acute neuropathic form affecting 4- to 5-month-old babies with a severe neurological condition, hepatosplenomegaly, and lung damage; Type III, corresponding to a chronic neuropathic form affecting both children and teenagers with less severe neurological conditions than Type II, besides being able to cause damage to liver, spleen and bones. A group of fourteen physicians experienced in treating Gaucher disease by enzyme replacement therapy has performed an extensive literature review on the subject and compared data of the evolution of Brazilian patients, having reached a consensus as regards the criteria to start treatment, the enzyme doses and frequency of infusions, as well as medical evaluations and laboratory and radiological monitoring. The purpose of this pioneering initiative is to standardize the conduct within the country, of Gaucher disease treatment using enzyme replacement therapy, which is an expensive treatment but with an expressive efficacy.