Abstract Background: Mucopolysaccharidosis type II, also known as Hunter syndrome, is a rare X-linked recessive disorder caused by deficiency of the lysosomal enzyme Iduronate-2- Sulfatase (IDS), leading to progressive accumulation of Glycosaminoglycans (GAGs) in several organs. Over the years, Enzyme Replacement Therapy (ERT) has provided significant benefits for patients, retarding the natural progression of the disease. Results: The authors evaluated 17 patients from the same family with a mild form of MPS type II; the proband had developed acute decompensated heart failure refractory to clinical measurements at 23 years and needed a rather urgent heart transplant; however, he died from surgical complications shortly after the procedure. Nevertheless, subsequent to his tragic death, 16 affected male relatives were detected after biochemical tests identifying the low or absent activity of the IDS enzyme and confirmed by molecular analysis of the IDS gene. Following diagnosis, different options of treatment were chosen: 6 patients started ERT with Elaprase® (Idursulfase) soon after, while the other 10 remained without ERT. Eventually, 4 patients in the latter group began ERT with Hunterase® (Idursulfase Beta). None presented adverse effects to either form of the enzyme. Among the 6 individuals without any ERT, two died of natural causes, after reaching 70 years. Despite the variable phenotype within the same family (mainly heart dysfunctions and carpal tunnel syndrome), all 14 remaining patients were alive with an independent lifestyle. Conclusion: Here, the authors report the variable progress of the disease with and without ERT in a large Brazilian family with a slowly progressive form of MPS II, harboring the same missense variant in the IDS gene.

A epidemia covid-19 trouxe inúmeros desafios ao nível das eventuais respostas que o Direito tem de estar preparado para dar. O Direito Internacional dos Direitos Humanos não é exceção, nele se destacando a Convenção Europeia dos Direitos Humanos. Depois da colocação do problema, o presente texto procura saber se o regime das restrições e derrogações de direitos contido na Convenção Europeia dos Direitos Humanos é suscetível de ser aplicado no atual contexto de crise de saúde pública. Ao mesmo tempo, ainda se analisa o eventual papel que o Tribunal Europeu dos Direitos Humanos poderá vir a ter no controlo das medidas estaduais que foram adotadas para combater a epidemia covid-19.

The covid-19 epidemic brought numerous challenges in terms of the possible responses that the Law must be prepared to give. International human rights law is no exception, highlighting the European Convention on Human Rights. After posing the problem, this text seeks to know whether the regime of restrictions and derogations of rights contained in the European Convention on Human Rights is likely to be applied in the current context of public health crisis. At the same time, the possible role that the European Court of Human Rights may have in controlling the state measures that were adopted to combat the covid-19 epidemic will also be analyzed.

RESUMO Objetivo: Descrever a manutenção dos níveis de glicosaminoglicano (GAG) excretados na urina e da estabilização clínica em pacientes com mucopolissacaridose do tipo I (MPS I) com o uso da laronidase num regime de dose alternativo de 1,2 mg/kg a cada duas semanas. Método: Alguns pacientes do nosso serviço participaram de um estudo de otimização de dose da laronidase para o tratamento da MPS I no qual foram comparados quatro esquemas terapêuticos: 0,58 mg/kg/semana, 1,2 mg/kg a cada duas semanas, 1,2 mg/kg/semana e 1,8 mg/kg a cada duas semanas. Após o término do estudo, todos os pacientes passaram a receber a terapia de reposição enzimática (TRE) na dose padrão de bula, que é de 0,58 mg/kg/semana, e nesse regime alguns pais se queixaram da dificuldade em comparecer ao centro todas as semanas, além da dificuldade de se obter acesso para punção venosa. Com base nessas queixas, oito pacientes passaram a receber a TRE no regime alternativo de 1,2 mg/kg a cada duas semanas. Foi feito o estudo retrospectivo de dados de prontuário de pacientes com MPS I que fizeram TRE com laronidase nas doses 0,58 mg/kg/semana e 1,2 mg/kg a cada duas semanas. Resultados: Os pacientes mantiveram-se clinicamente estáveis, não apresentaram aumento dos níveis de GAG urinários nem eventos adversos durante o regime alternativo de dose. Conclusões: A mudança para o esquema de 1,2 mg/kg de laronidase a cada duas semanas foi segura e não acarretou piora clínica nos pacientes que já estavam em TRE na dose padrão.

ABSTRACT Objective: To report the stabilization of urinary glycosaminoglicans (GAG) excretion and clinical improvements in patients with mucopolysaccharidosis type I (MPS I) under an alternative dose regimen of laronidase of 1.2 mg/kg every other week. Methods: We participated in a dose-optimization trial for laronidase in MPS-I patients using four alternative regimens: 0.58 mg/kg every week, 1.2 mg/kg every two weeks, 1.2 mg/kg every week and 1.8 mg/kg every other week (EOW). After the trial ended, the patients resumed the recommended dose and regimen of 0.58 mg/kg every week. Under this regimen, some patients presented difficulties in venous access and were unable to commute weekly to the treatment center. Therefore, we used an alternative regimen that consisted of 1.2 mg/kg EOW in eight patients. A retrospective study of medical records of MPS-I patients who underwent both enzyme replacement therapy (ERT) regimens, of 0.58 mg/kg every week and 1.2 mg/kg EOW, was done. Results: Patients remained clinically stable under the alternative regimen, did not present elevation of urinary GAG nor any adverse event. Conclusions: The switch of dose regimen to 1.2 mg/kg EOW of laronidase was safe, and did not cause any clinical worsening in patients who had been previously under standard dose ERT.

Abstract We assessed levels of plasma selenium (Se), selenoproteins and their change after Se supplementation in patients with mucopolysaccharidosis (MPS) types I, II and VI. This was done in a retrospective study of the medical records of 30 patients with MPS I (n=13), MPS II (n=9) and MPS VI (n=8) who were being treated with enzyme replacement therapy. As part of routine nutritional monitoring, Se levels were measured, revealing that 28 patients (93.3%) had values below the normal range. Therefore, they received supplementation for 12 months, and Se was measured after 6 and 12 months. Glutathione peroxidase (GPx) activity, total glutathione (GSHt), oxidized glutathione (GSSG) and reduced glutathione (GSH) were measured at baseline and 6 months after Se supplementation. The mean GSHt at baseline was 7.90 ± 2.36 μmol/g Hb, and after Se supplementation it was 5.76 ± 1.13 μmol/g Hb; GSH/GSSG was 2.3 ± 1.16 at baseline and 0.58 ± 0.38 after supplementation. GPx activity was 16.46 ± 3.31 U/g Hb at baseline and 4.53 ± 4.92 U/g Hb after Se supplementation. The difference was shown to be statistically significant by paired t-test. In conclusion, our study demonstrated that oxidative stress parameters were altered by Se supplementation in patients with MPS I, II and VI who were previously deficient in Se.

A abordagem do nexo urbano corresponde à compreensão e à busca de soluções integradas mediante o reconhecimento das interdependências entre água, energia e alimentos, recursos cuja escassez configura iniquidades em saúde. A hipótese central deste artigo considera que o contexto de escassez corrobora práticas sociais que podem ser sinérgicas ou contraditórias em relação aos desafios da sustentabilidade e dos direitos sociais. O objetivo é investigar sinergias e contradições a partir de práticas sociais mediante o nexo urbano no bairro Novo Recreio, na cidade de Guarulhos, Região Metropolitana de São Paulo, Brasil. A metodologia consiste em um estudo qualitativo e de base etnográfica com referência à Teoria das Práticas, com observações diretas de campo e narrativas. Os resultados apresentaram práticas sociais associadas a falta sistemática de água, precariedades na iluminação pública e no transporte, bem como dificuldade de acesso a alimentos frescos e saudáveis. O estudo das práticas sociais entre sinergias e contradições permitiu verificar que, nesse processo espontâneo de busca de solução para problemas locais, é constatada a necessidade de integrar práticas e saberes locais a políticas públicas e demandas globais. Com isso, denominamos nexos de exclusão a condição periférica de impossibilidade de opções conscientes que permitam orientar conjuntamente a redução da escassez e de iniquidades com alternativas para a sustentabilidade.

The urban nexus approach involves the investigation and elucidation of integrated solutions through the recognition of tradeoffs between water, energy, and food, namely resources whose shortage leads to inequalities in health. The article’s central hypothesis is that the context of shortage corroborates social practices that can be synergic or contradictory in relation to the challenges of sustainability and social rights. The objective is to investigate synergies and contradictions based on social practices in the urban nexus in the neighborhood of Novo Recreio in the city of Guarulhos, Greater Metropolitan São Paulo, Brazil. The methodology consists of a qualitative ethnographic study drawing on practice theory as the reference, with direct field observations and narratives. The results featured social practices associated with systematic lack of water, precarious public lighting and transportation, and difficult access to fresh and healthy foods. The study of social practices between synergies and contradictions allowed verifying that this spontaneous process of search for solutions to local problems reveals the need to incorporate local practices and knowledge into public policies and global demands. We define nexus of exclusion as the peripheral condition of impossibility of conscious options that allow jointly orienting the reduction of shortage and iniquities through alternatives for sustainability.

El abordaje del nexo urbano se corresponde con la comprensión y búsqueda de soluciones integradas, mediante el reconocimiento de las interdependencias entre agua, energía, alimentos y recursos, cuya escasez configura inequidades en salud. La hipótesis central de este artículo considera que el contexto de escasez corrobora prácticas sociales que pueden ser sinérgicas o contradictorias, a través de los desafíos en sostenibilidad y derechos sociales. Los objetivos son investigar sinergias y contradicciones, a partir de prácticas sociales mediante el nexo urbano en el barrio Novo Recreio, en la ciudad de Guarulhos, región metropolitana de São Paulo, Brasil. La metodología consiste en un estudio cualitativo y de base etnográfica, vinculado a la teoría de las prácticas, analizando observaciones de campo directas y relatos. Los resultados presentaron prácticas sociales asociadas a la falta sistemática de agua, precariedad en la iluminación pública y el transporte, dificultad de acceso a alimentos frescos y saludables. El estudio de las prácticas sociales entre sinergias y contradicciones permitió verificar que, en ese proceso espontáneo de búsqueda de soluciones para problemas locales, se constata la necesidad de integrar prácticas y saberes locales con políticas públicas y demandas globales. De esta forma, denominamos nexos de exclusión a las condiciones periféricas de imposibilidad, en cuanto a opciones conscientes que permitan orientar conjuntamente la reducción de la escasez e iniquidades con alternativas para la sostenibilidad.

El abordaje del nexo urbano se corresponde con la comprensión y búsqueda de soluciones integradas, mediante el reconocimiento de las interdependencias entre agua, energía, alimentos y recursos, cuya escasez configura inequidades en salud. La hipótesis central de este artículo considera que el contexto de escasez corrobora prácticas sociales que pueden ser sinérgicas o contradictorias, a través de los desafíos en sostenibilidad y derechos sociales. Los objetivos son investigar sinergias y contradicciones, a partir de prácticas sociales mediante el nexo urbano en el barrio Novo Recreio, en la ciudad de Guarulhos, región metropolitana de São Paulo, Brasil. La metodología consiste en un estudio cualitativo y de base etnográfica, vinculado a la teoría de las prácticas, analizando observaciones de campo directas y relatos. Los resultados presentaron prácticas sociales asociadas a la falta sistemática de agua, precariedad en la iluminación pública y el transporte, dificultad de acceso a alimentos frescos y saludables. El estudio de las prácticas sociales entre sinergias y contradicciones permitió verificar que, en ese proceso espontáneo de búsqueda de soluciones para problemas locales, se constata la necesidad de integrar prácticas y saberes locales con políticas públicas y demandas globales. De esta forma, denominamos nexos de exclusión a las condiciones periféricas de imposibilidad, en cuanto a opciones conscientes que permitan orientar conjuntamente la reducción de la escasez e iniquidades con alternativas para la sostenibilidad.

A abordagem do nexo urbano corresponde à compreensão e à busca de soluções integradas mediante o reconhecimento das interdependências entre água, energia e alimentos, recursos cuja escassez configura iniquidades em saúde. A hipótese central deste artigo considera que o contexto de escassez corrobora práticas sociais que podem ser sinérgicas ou contraditórias em relação aos desafios da sustentabilidade e dos direitos sociais. O objetivo é investigar sinergias e contradições a partir de práticas sociais mediante o nexo urbano no bairro Novo Recreio, na cidade de Guarulhos, Região Metropolitana de São Paulo, Brasil. A metodologia consiste em um estudo qualitativo e de base etnográfica com referência à Teoria das Práticas, com observações diretas de campo e narrativas. Os resultados apresentaram práticas sociais associadas a falta sistemática de água, precariedades na iluminação pública e no transporte, bem como dificuldade de acesso a alimentos frescos e saudáveis. O estudo das práticas sociais entre sinergias e contradições permitiu verificar que, nesse processo espontâneo de busca de solução para problemas locais, é constatada a necessidade de integrar práticas e saberes locais a políticas públicas e demandas globais. Com isso, denominamos nexos de exclusão a condição periférica de impossibilidade de opções conscientes que permitam orientar conjuntamente a redução da escassez e de iniquidades com alternativas para a sustentabilidade.

The urban nexus approach involves the investigation and elucidation of integrated solutions through the recognition of tradeoffs between water, energy, and food, namely resources whose shortage leads to inequalities in health. The article’s central hypothesis is that the context of shortage corroborates social practices that can be synergic or contradictory in relation to the challenges of sustainability and social rights. The objective is to investigate synergies and contradictions based on social practices in the urban nexus in the neighborhood of Novo Recreio in the city of Guarulhos, Greater Metropolitan São Paulo, Brazil. The methodology consists of a qualitative ethnographic study drawing on practice theory as the reference, with direct field observations and narratives. The results featured social practices associated with systematic lack of water, precarious public lighting and transportation, and difficult access to fresh and healthy foods. The study of social practices between synergies and contradictions allowed verifying that this spontaneous process of search for solutions to local problems reveals the need to incorporate local practices and knowledge into public policies and global demands. We define nexus of exclusion as the peripheral condition of impossibility of conscious options that allow jointly orienting the reduction of shortage and iniquities through alternatives for sustainability.

Abstract: Fabry disease is a rare lysosomal storage disorder, inherited in an X-linked manner. It is characterized by the deficiency of the enzyme alpha-galactosidase, leading to a buildup of glycosphingolipids in the cells. Angiokeratoma is one of the cutaneous manifestations of this condition, and it helps making the diagnosis. The typical site involves the genital area in men and lumbosacral, buttocks and trunk region in both sexes. We report a case of genital angiokeratoma in a woman with Fabry disease. The diagnosis is through molecular analysis and, when made early, starting treatment reduces the morbidity and mortality of the disease. Thus, the dermatologist has an important role in the identification of angiokeratoma as a cutaneous marker, and the knowledge of its different presentations is essential for the early diagnosis and management of Fabry disease.

OBJECTIVES: To characterize the audiometric evaluation and acoustic immittance measures in different types of mucopolysaccharidosis. METHOD: Fifty-three mucopolysaccharidosis patients were evaluated. The classification consisted of type I (Hurler syndrome, Hurler-Scheie and Scheie syndrome), type II (Hunter syndrome), type III (Sanfilippo syndrome), type IV (Morquio syndrome), and type VI (Maroteaux-Lamy syndrome). Immittance audiometry and play or conventional threshold tone audiometry were used to obtain auditory thresholds and were chosen according to the patient’s chronological age and ability to understand/respond to the procedure. The findings were analyzed using descriptive statistics and considering the recommendations for research involving human beings contained in Resolution CNE N° 466/2012. RESULTS: Fifty-one subjects (96.2%) had hearing loss, and the conductive type was the most frequent. Only two (3.8%) patients presented bilateral thresholds within normal limits, one with type IV mucopolysaccharidosis and the other with type VI. There were 11 individuals (20.8%) with mucopolysaccharidosis type I with mixed hearing loss, 9 (16.9%) individuals with type I with conductive hearing loss and 9 (16.9%) with type VI with conductive hearing loss. Mild hearing loss was most common (37.3%), followed by moderately severe hearing loss (36.3%). The type B tympanometric curve (80.4%) was the most frequent. CONCLUSIONS: Most of the individuals with mucopolysaccharidosis types I, II, III, IV and VI presented mixed or conductive hearing losses of mild to moderately severe degree, type B tympanograms and an absence of contralateral acoustic reflexes.

ABSTRACT The objective of this study was to compare the histopathological aspects of the liver of free-living and farmed bullfrogs (Lithobates catesbeianus). Thirty liver samples were collected from young and adult bullfrogs in the states of Minas Gerais and Rio de Janeiro, Brazil. Marked blood congestion and even hemorrhage and a large number of melanomacrophages were observed in most of the livers from free-living bullfrogs. Additionally, hepatocytes showed cytoplasmic eosinophilia and some showed nuclear hypertrophy and presence of microvesicular steatosis; small foci of infiltrates of mononuclear cells were observed in some samples, as well as a thicker capsule. In the farmed bullfrogs, cytoplasmic rarefaction was normal. Morphologically, unlike the livers of animals reared on farms, the livers of free-living animals did not show any evidence of protein-mineral deficiency, characterized by cytoplasmic vacuolation and a larger number of melanomacrophages, suggesting greater aggression to the liver parenchyma, probably due to environmental toxicity. Free-living bullfrogs feed on proteins of better biological value, derived from animals preyed in the natural environment.

RESUMO No campo das Dimensões Humanas das Mudanças Climáticas existe um rico debate ancorado particularmente em arcabouços teóricos das ciências sociais sobre como as respostas que emergem para lidar com a questão climática estão intrinsecamente relacionadas a aspectos e configurações locais que interferem, em maior ou menor grau, nos processos adaptativos de diferentes sistemas. Outras possibilidades teórico-analíticas, também focadas em adaptação, vulnerabilidade e capacidade adaptativa, buscam intersecções com a literatura sobre resiliência e desenvolvimento sustentável. Neste artigo, as autoras partem de uma revisão crítica dessas perspectivas para pensar o contexto brasileiro e os processos de ajustamentos necessários para antecipar e responder aos impactos associados às mudanças climáticas nas cidades. A partir de uma reflexão sobre estudos conduzidos em diferentes localidades no país, indica-se a necessidade de um olhar sobre um conjunto de variáveis críticas (e as possíveis associações entre elas) em futuras pesquisas focadas na análise do contexto brasileiro.

ABSTRACT The field of the Human Dimensions of Climate Change there is a rich debate grounded in the theoretical frameworks of the Social Science on how responses to climate change are intrinsically related to local factors and determinants that shape adaptive processes in different systems. Other theoretical-analytical possibilities, also focused on adaptation, vulnerability and adaptive capacity, have sought to explore intersections with the literatures on resilience and sustainable development. In this paper, the authors provide a critical review of these theoretical perspectives, and propose a reflection on the Brazilian context and the adjustment processes needed to anticipate and respond to climate impact in the cities. Drawing on empirical studies in Brazil, the authors of this paper advocate the need for a set of critical variables (and an analysis of possible synergies and tradeoffs amongst them) in future studies focused on analyzing the Brazilian context.

Resumo Introdução: Mucopolissacaridose (MPS) é uma doença de depósito lisossômico causada pela deficiência de a-l-iduronidase. Os achados otorrinolaringológicos incluem perda auditiva, otorreia, otites de repetição, hipertrofia adenotonsilar, rinossinusite recorrente, distúrbios da fala, roncos, respiração bucal e obstrução nasal. Objetivo: Avaliar o impacto da terapia de reposição enzimática com laronidase (Aldurazyme(r)) em pacientes com mucopolissacaridose I (MPS I) em relação ao sono, distúrbios auditivos e manifestações clínicas do trato respiratório superior (TRS). Método: Nove pacientes com MPS I (oito com fenótipo Hurler-Scheie e um com fenótipo Scheie), de ambos os sexos, com idades variando entre 3 e 20 anos, foram incluídos neste estudo. Os pacientes foram avaliados entre 7 e 11 meses antes do tratamento e entre 16 e 22 meses após o início da substituição enzimática. Todos foram submetidos a uma avaliação clínica e otorrinolaringológica, incluindo nasofibroscopia, polissonografia e exames radiológicos. Resultados: Os dados dos resultados mostraram diminuição da frequência de infecções de orelha, nariz e garganta, com melhora da rinorreia e da qualidade respiratória. Mudanças significativas não foram observadas em relação à macroglossia e à hipertrofia adenotonsilar. Avaliações audiométricas e polissonográficas não apresentaram significância estatística. Conclusão: A terapia de reposição enzimática em pacientes com mucopolissacaridose I fornece controle de infecções recorrentes do TRS, rinorreia e qualidade respiratória, porém, não parece melhorar os parâmetros audiológicos e polissonográficos, ou exercer efeito sobre a hipertrofia adenotonsilar e macroglossia.

ABSTRACT INTRODUCTION: Mucopolysaccharidosis (MPS) is a lysosomal storage disease caused by deficiency of a-l-iduronidase. The otolaryngological findings include hearing loss, otorrhea, recurrent otitis, hypertrophy of tonsils and adenoid, recurrent rhinosinusitis, speech disorders, snoring, oral breathing and nasal obstruction. OBJECTIVE: To evaluate the impact of enzymatic replacement therapy with laronidase (Aldurazyme(r)) in patients with mucopolysaccharidosis (MPS I), regarding sleep and hearing disorders, and clinical manifestations in the upper respiratory tract (URT). METHODS: Nine patients with MPS I (8 Hurler-Scheie, and 1 Scheie phenotypes) of both sexes, ages ranging between 3 and 20 years, were included in this study. Patients were evaluated between seven and 11 months before the treatment and between 16 and 22 months after the onset of the enzymatic replacement. They were all submitted to a clinical and otolaryngological evaluation, including nasofibroscopical, polysomnographic and audiologic exams. RESULTS: The results' data showed decreasing of the frequency of ear, nose and throat infections, with improvement of the rhinorrhea and respiratory quality. No remarkable changes were observed regarding macroglossia and tonsil and adenoid hypertrophy. Audiometric and polysomnographic evaluations did not show statistical significance. CONCLUSION: Enzymatic replacement therapy in patients with mucopolysaccharidosis I provides control of recurrent URT infections, rhinorrhea and respiratory quality, however it is does not seem to improve audiologic and polisomnographic parameters, with no effect on adenoid and tonsils hypertrophy and macroglossia.

Este trabalho trata do contraste interpretativo que resulta da colocação pré-nominal ou pós-nominal do quantificador indefinido algum (e.g. alguma coisa vs. coisa alguma) e procura explicar, numa perspetiva formal, diacrónica e comparativa, a relação entre ordem de palavras e polaridade (i.e., a oposição positivo/negativo). A análise proposta explora a hipótese de paralelismo estrutural entre frases e expressões nominais (especificamente quanto à categoria NegP) e leva à identificação de um conjunto de mudanças sintáticas correlacionadas que ocorrem no português a partir do século XVIII. Entre elas está a expansão das estruturas de inversão nominal negativa (que passam a incluir também o quantificador indefinido nenhum), a substituição dos pronomes algum e nenhum, com referência [+humana], por alguém e ninguém, e a generalização do uso do artigo definido antes de possessivo. As diferenças e semelhanças entre o português e o espanhol no que diz respeito à inversão nominal com algum/alguno são analisadas numa perspetiva teórica e diacrónica.

This work deals with the interpretive contrast that results from placing the indefinite quantifier algum in prenominal or postnominal position (e.g. alguma coisa ‘something' vs. coisa alguma ‘nothing') and seeks to explain the observed relationship between word order and polarity (i.e. the opposition positive/negative) in a formal, diachronic and comparative perspective. The proposed analysis explores the hypothesis of structural parallelism between clauses and nominal expressions (specifically vis-à-vis NegP) and leads to the identification of a set of related syntactic changes taking place in Portuguese from the 18th century. These changes include the expansion of nominal negative inversion from algum to nenhum (‘none'), the loss of algum and nenhum as strong pronouns with a [+human] feature, and the generalization of the use of the definite article before possessives. Last but not least, the paper describes the differences and similarities between Portuguese and Spanish nominal negative inversion with algum/alguno and accounts for them in a theoretical and diachronic perspective.

O Brasil se destaca na produção mundial de alimentos de origem animal, sendo que cada vez mais os mercados consumidores buscam alimentos seguros e inócuos para a saúde. Nesse contexto, torna-se fundamental a prevenção das zoonoses, que podem ser transmitidas através dos alimentos de origem animal, que muitas vezes são consumidos sem o devido preparo ou por populações mais vulneráveis. Nesta revisão bibliográfica, serão abordadas importantes enfermidades parasitárias de caráter zoonótico que ocorrem atualmente pela via de transmissão alimentar: a toxoplasmose, o complexo teníase-cisticercose, criptosporidiose, anisacose, difilobotriose e triquinelose.

Brazil is one of the most important countries in the production of animal source foods, and the consumers are looking for safe and innocuous products for health. In this context, it is necessary to prevent zoonosis that can be transmitted by animal source foods, which are sometimes consumed without proper cooking or made by vulnerable populations. On this review, important parasitic zoonotic diseases will be covered, which currently occur by this transmission route: toxoplasmosis, taeniasis-cysticercosis complex, cryptosporidiosis, anisakiasis, diphyllobothriasis and trichinosis.

This review aims to provide clinicians in Latin America with the most current information on the clinical aspects, diagnosis, and management of Hunter syndrome, a serious and progressive disease for which specific treatment is available. Hunter syndrome is a genetic disorder where iduronate-2-sulfatase (I2S), an enzyme that degrades glycosaminoglycans, is absent or deficient. Clinical manifestations vary widely in severity and involve multiple organs and tissues. An attenuated and a severe phenotype are recognized depending on the degree of cognitive impairment. Early diagnosis is vital for disease management. Clinical signs common to children with Hunter syndrome include inguinal hernia, frequent ear and respiratory infections, facial dysmorphisms, macrocephaly, bone dysplasia, short stature, sleep apnea, and behavior problems. Diagnosis is based on screening urinary glycosaminoglycans and confirmation by measuring I2S activity and analyzing I2S gene mutations. Idursulfase (recombinant I2S) (Elaprase®, Shire) enzyme replacement therapy (ERT), designed to address the underlying enzyme deficiency, is approved treatment and improves walking capacity and respiratory function, and reduces spleen and liver size and urinary glycosaminoglycan levels. Additional measures, responding to the multi-organ manifestations, such as abdominal/inguinal hernia repair, carpal tunnel surgery, and cardiac valve replacement, should also be considered. Investigational treatment options such as intrathecal ERT are active areas of research, and bone marrow transplantation is in clinical practice. Communication among care providers, social workers, patients and families is essential to inform and guide their decisions, establish realistic expectations, and assess patients' responses.

O bem-estar animal é uma prática que implica na manutenção das liberdades psicológicas e fisiológicas dos animais, para que seus organismos permaneçam saudáveis até o abate, proporcionando uma carcaça com qualidade e evitando sofrimentos desnecessários aos mesmos. Uma forma de avaliar o bem-estar é através da monitoração da ocorrência de contusões nas carcaças, um indicativo de manejo inadequado dos mesmos. Objetivou-se avaliar a ocorrência de contusões em carcaças bovinas em um matadouro-frigorífico localizado na Região Centro-oeste do Estado de São Paulo. Assim, foi aplicada "check-list", para determinação da presença de contusões, o local e a gravidade das mesmas, classificando-as entre escore I a III. Do total de animais avaliados, 98% (880/898) apresentaram contusão. As contusões de grau I na região do coxão das meias carcaças tiveram maior incidência com média de 61,8% entre os lados direito e esquerdo. Também foram observadas múltiplas lesões em diversos animais abatidos. Em 133 animais avaliados foram retirados 15,6 kg de material lesionado na região do coxão apenas, demonstrando grandes prejuízos econômicos aos produtores e às indústrias por falhas em bem-estar animal. O manejo pré-abate inadequado compromete o bem-estar animal e a qualidade das carcaças, desprestigiando nossos produtos no comércio internacional da carne bovina e consistindo em perdas econômicas à pecuária bovina nacional.

The animal welfare is a series of practices that involves the maintenance of psychological and physiological freedoms of animals, to them remain healthy until slaughter and provide a satisfactory quality carcass and to avoid unnecessary suffering to animals. One way to evaluate the animal welfare is monitoring the occurrence of bruises on carcasses that is an indicative of inadequate management of the animals. The present study aimed to evaluate the occurrence of bruises in cattle carcasses at a slaughterhouse located in the Midwest Region of the State of São Paulo, Brazil. Was applied a "check-list" that determined the presence of bruises, the location and severity of them, ranking among scores I to III. Was observed in 98% (880/898) of the animals the presence of contusion. The Grade I injuries in the region of the hips at half carcasses had higher incidence with an average of 61.8% between the right and left sides. Could be observed multiple lesions in several animals slaughtered. In 133 animals observed were removed 15.6 kilograms of material only the injured region only of the hips, demonstrating economic losses to cattle farmers and industries for failures in animal welfare practices. The inappropriate pre-slaughter management compromises animal welfare and the quality of carcasses, discrediting our products on international meat trade and consisting in economic losses to the national cattle herds.