Interference of oropharyngeal dysphagia in food consumption in patients with mucopolysaccharidosis II

PURPOSE: this study aimed to relate the degree of dysphagia and food consumption of individuals with mucopolysaccharidosis II (MPS II). METHOD: it was included individuals with MPS II of the genetics department at the State University of Alagoas and excluded those with other types of mucopolysaccharidosis and in use of alternative way of supply. There were performed anthropometric, dietetic, speech therapy for dysphagia,clinical evaluation and otorrinolarigologic videoendoscopy swallowing. RESULTS: there were studied 07 individuals, male, between 5 and 14 years old: from them, more than 50% were taking anti-hypertensive and 42.8% had the severe form of disease. Six had serious deficit height / age and over 70% were obese according to Body Mass Index (BMI). Dysphagia was observed in five subjects, with daily average caloric intake 920.15 ± 244.09 calories, against 1264.94 ± 106.85 calories for those without such changes, with significantly greater intraindividual variation in the group of patients with dysphagia (p <0.05). In addition, individuals without dysphagia had higher food intake of carbohydrates, proteins and lipids. As for micronutrients, except average daily intake of iron and vitamin E, all other assessed had higher average daily consumption in the group without dysphagia (p < 0.05). CONCLUSION: it was observed a high frequency of dysphagia in the studied patients with MPS II, and this was associated with low caloric food consumption and imbalance in the proportion and amount of macronutrients and part of micronutrients.OBJETIVO: o presente estudo visou relacionar o grau de disfagia com o consumo alimentar de indivíduos com mucopolissacaridose II (MPS II). MÉTODO: foram incluídos indivíduos com MPS II do departamento de genética da Universidade Estadual de Alagoas e excluídos aqueles com outros tipos de mucopolissacaridoses, bem como que estivessem em uso de via alternativa de alimentação. Realizadas avaliações antropométrica, dietética, fonoaudiológica para disfagia, clínica otorrinolaringológica e a videoendoscopia da deglutição. RESULTADOS: foram estudados 07 indivíduos, do gênero masculino, entre 5 e 14 anos de idade, dos quais mais de 50% faziam uso de anti-hipertensivo e 42,8% manifestavam a forma grave da doença. Seis deles apresentaram déficit de altura/ idade e mais de 70% encontravam-se obesos segundo o Índice de Massa Corporal (IMC). Foi observada disfagia em cinco deles, com média diária de consumo calórico de 920,15 ± 244,09 calorias, contra 1264,94 ± 106,85 calorias para aqueles sem disfagia, com variação intra-individual significativamente maior no grupo de portadores de disfagia (p < 0,05). Além disso, os indivíduos sem disfagia apresentaram consumo alimentar mais elevado de carboidratos, proteínas e lipídios. Já para os micronutrientes, com exceção da média de consumo diária de ferro e vitamina E, todos os outros avaliados apresentaram médias diárias de consumo maiores no grupo sem disfagia (p < 0,05). CONCLUSÃO: foi observada uma elevada frequência de disfagia nos portadores de MPS II estudados, e isso foi associado ao baixo consumo alimentar calórico e desequilíbrio na proporção e quantidade de macronutrientes e de parte dos micronutrientes.UNCISAL Faculdade de Fonoaudiologia de AlagoasUniversidade Federal de AlagoasCentro de Estudos Superiores de Maceió Escola de Ciências da SaúdeUniversidade Federal de São Paulo (UNIFESP)UNIFESP, EPMSciEL

Interferência da disfagia orofaríngea no consumo alimentar de indivíduos com mucopolissacaridose II Interference of oropharyngeal dysphagia in food consumption in patients with mucopolysaccharidosis II

OBJETIVO: o presente estudo visou relacionar o grau de disfagia com o consumo alimentar de indivíduos com mucopolissacaridose II (MPS II). MÉTODO: foram incluídos indivíduos com MPS II do departamento de genética da Universidade Estadual de Alagoas e excluídos aqueles com outros tipos de mucopolissacaridoses, bem como que estivessem em uso de via alternativa de alimentação. Realizadas avaliações antropométrica, dietética, fonoaudiológica para disfagia, clínica otorrinolaringológica e a videoendoscopia da deglutição. RESULTADOS: foram estudados 07 indivíduos, do gênero masculino, entre 5 e 14 anos de idade, dos quais mais de 50% faziam uso de anti-hipertensivo e 42,8% manifestavam a forma grave da doença. Seis deles apresentaram déficit de altura/ idade e mais de 70% encontravam-se obesos segundo o Índice de Massa Corporal (IMC). Foi observada disfagia em cinco deles, com média diária de consumo calórico de 920,15 ± 244,09 calorias, contra 1264,94 ± 106,85 calorias para aqueles sem disfagia, com variação intra-individual significativamente maior no grupo de portadores de disfagia (p PURPOSE: this study aimed to relate the degree of dysphagia and food consumption of individuals with mucopolysaccharidosis II (MPS II). METHOD: it was included individuals with MPS II of the genetics department at the State University of Alagoas and excluded those with other types of mucopolysaccharidosis and in use of alternative way of supply. There were performed anthropometric, dietetic, speech therapy for dysphagia,clinical evaluation and otorrinolarigologic videoendoscopy swallowing. RESULTS: there were studied 07 individuals, male, between 5 and 14 years old: from them, more than 50% were taking anti-hypertensive and 42.8% had the severe form of disease. Six had serious deficit height / age and over 70% were obese according to Body Mass Index (BMI). Dysphagia was observed in five subjects, with daily average caloric intake 920.15 ± 244.09 calories, against 1264.94 ± 106.85 calories for those without such changes, with significantly greater intraindividual variation in the group of patients with dysphagia (p <0.05). In addition, individuals without dysphagia had higher food intake of carbohydrates, proteins and lipids. As for micronutrients, except average daily intake of iron and vitamin E, all other assessed had higher average daily consumption in the group without dysphagia (p < 0.05). CONCLUSION: it was observed a high frequency of dysphagia in the studied patients with MPS II, and this was associated with low caloric food consumption and imbalance in the proportion and amount of macronutrients and part of micronutrients

Brazilian Flora 2020: Leveraging the power of a collaborative scientific network

International audienceThe shortage of reliable primary taxonomic data limits the description of biological taxa and the understanding of biodiversity patterns and processes, complicating biogeographical, ecological, and evolutionary studies. This deficit creates a significant taxonomic impediment to biodiversity research and conservation planning. The taxonomic impediment and the biodiversity crisis are widely recognized, highlighting the urgent need for reliable taxonomic data. Over the past decade, numerous countries worldwide have devoted considerable effort to Target 1 of the Global Strategy for Plant Conservation (GSPC), which called for the preparation of a working list of all known plant species by 2010 and an online world Flora by 2020. Brazil is a megadiverse country, home to more of the world's known plant species than any other country. Despite that, Flora Brasiliensis, concluded in 1906, was the last comprehensive treatment of the Brazilian flora. The lack of accurate estimates of the number of species of algae, fungi, and plants occurring in Brazil contributes to the prevailing taxonomic impediment and delays progress towards the GSPC targets. Over the past 12 years, a legion of taxonomists motivated to meet Target 1 of the GSPC, worked together to gather and integrate knowledge on the algal, plant, and fungal diversity of Brazil. Overall, a team of about 980 taxonomists joined efforts in a highly collaborative project that used cybertaxonomy to prepare an updated Flora of Brazil, showing the power of scientific collaboration to reach ambitious goals. This paper presents an overview of the Brazilian Flora 2020 and provides taxonomic and spatial updates on the algae, fungi, and plants found in one of the world's most biodiverse countries. We further identify collection gaps and summarize future goals that extend beyond 2020. Our results show that Brazil is home to 46,975 native species of algae, fungi, and plants, of which 19,669 are endemic to the country. The data compiled to date suggests that the Atlantic Rainforest might be the most diverse Brazilian domain for all plant groups except gymnosperms, which are most diverse in the Amazon. However, scientific knowledge of Brazilian diversity is still unequally distributed, with the Atlantic Rainforest and the Cerrado being the most intensively sampled and studied biomes in the country. In times of “scientific reductionism”, with botanical and mycological sciences suffering pervasive depreciation in recent decades, the first online Flora of Brazil 2020 significantly enhanced the quality and quantity of taxonomic data available for algae, fungi, and plants from Brazil. This project also made all the information freely available online, providing a firm foundation for future research and for the management, conservation, and sustainable use of the Brazilian funga and flora