RESUMO Antecedentes: A Pandemia por COVID-19 tem trazido desafios subtanciais para a prática clínica no tratamento das doenças neuromusculares hereditárias (DNMh). A infecção não tem sido a única preocupação para os pacientes. O distanciamento social tem comprometido a assistência multidisciplinar, atividade física e tem trazido problemas mentais em decorrência do próprio isolamento. Nós apresentamos aqui um seguimento de 363 pacientes com DNMh de um centro terciário Brasileiro durante o pico da Pandemia de Covid-19. Objetivos: Mostrar a frequência e gravidade da infecção por Sars-Cov-2 em pacientes com DNMh e demonstrar os efeitos da pandemia nos hábitos de vida, na progressão da doença e no cuidado multidisciplinary. Métodos Trezentos e sessenta e três pacientes (58% homens and 42% mulheres) foram acompanhados por 3 meses através de 3 teleconsultas durante o pico da Pandemia de Covid-19 no Brasil. Resultados Houve um decréscimo no número de pacientes que faziam terapia física, respiratória e fonoaudiológica. Em muitos pacientes, o apetite (33%) e hábitos do sono (25%) se alteraram. Exercícios físicos e terapias foram interrompidas pela maioria dos pacientes. Physical exercises and therapies were interrupted for most of the patients. Eles relataram piora ou aparecimento de fadiga (17%), dor (17%), retrações (14%), e escoliose (7%). Irritabilidade, mudanças no sono, peso e apetite, sendo principalmente diminuição do apetite e peso foram mais frequentemente encontrados em pacientes que apresentaram piora clinica da doença. Houve uma baixa taxa de contaminação por Covid-19 (0.8%), e todos os pacientes infectado apresentaram quadro clinico leve. Conclusão O isolamento por si só se mostrou protetor na perspectiva de infecção por Covid-19, mas pode desencadear um cenário complexo com mudanças nos hábitos de vida e curso desfavorável da doença de base.

ABSTRACT Background The COVID-19 pandemic has brought substantial challenges for current practices in treating hereditary neuromuscular disorders (hNMDs). However, this infection has not been the only concern for these patients. Social distancing has compromised multidisciplinary assistance and physical activity, and has brought about several mental health issues. We presented a follow-up on 363 patients with hNMDs at a Brazilian tertiary center during the peak of the COVID-19 pandemic. Objective We aimed to show the frequency and severity of SARS-CoV-2 infection among hNMD patients and to demonstrate the effects of the pandemic on life habits, disease progression and multidisciplinary supportive care status. Methods Three hundred and sixty-three patients (58% male and 42% female) were followed for three months through three teleconsultations during the peak of the COVID-19 pandemic in Brazil. Results There were decreases in the numbers of patients who underwent physical, respiratory and speech therapies. For several patients, their appetite (33%) and sleep habits (25%) changed. Physical exercises and therapies were interrupted for most of the patients. They reported new onset/worsening of fatigue (17%), pain (17%), contractions (14%) and scoliosis (7%). Irritability and sleep, weight and appetite changes, and especially diminished appetite and weight loss, were more frequent in the group that reported disease worsening. There was a low COVID-19 contamination rate (0.8%), and all infected patients had a mild presentation. Conclusion The isolation by itself was protective from a COVID-19 infection perspective. However, this isolation might also trigger a complex scenario with life habit changes that are associated with an unfavorable course for the NMD.

Abstract Objective: Developing and validating a disease-specific instrument in the Brazilian Portuguese language to assess the Health-Related Quality of Life of children with functional constipation, applied to parents/caregivers. Methods: The process of developing the questionnaire was carried out in the following steps: items generation concerning functional constipation; elaboration of the preliminary questionnaire; assessment by health professionals; identifying problems or inconsistencies by the researchers; improvement of the questions; obtaining a final questionnaire named Pediatric Functional Constipation Questionnaire-Parent Form (PedFCQuest-PR) with 26 questions divided into four domains. Responses options use a Likert scale based on the events of the last four weeks. The process of validation was an observational, cross-sectional study in a sample of 87 parents/caregivers of children from 5 to 15 years of age diagnosed with Functional constipation according to the Rome IV Criteria. The questionnaire was applied simultaneously to the Pediatric Quality of Life Inventory 4.0 (PedsQL TM 4.0) as a control. Results: The questionnaire validation included 87 parents/caregivers. The children’s median age was 8.2 years, with a long time of constipation symptoms associated with fecal incontinence in approximately two-thirds. Internal consistency reliability for the Total Scale Score of PedFC-Quest-PR by Coefficient Alpha of Cronbach score was 0.86. Convergent and divergent validity of PedFCQuest-PR was demonstrated by correlating the domains of both questionnaires. Conclusion: This study provides evidence that PedFCQuest-PR is a reliable instrument. The results showed a high degree of internal consistency and validity of the instrument for future applications.

RESUMO Objetivo: Avaliar os componentes alergênicos da rotulagem de alimentos embalados e responder “Qual é a qualidade da rotulagem?” e “Qual é o grupo de alimentos em que a expressão ‘Pode conter’ é predominante?”. Métodos: Foram obtidas fotografias de 916 produtos, dos quais 518 foram analisados. Os dados dos rótulos foram avaliados de acordo com os grupos da pirâmide alimentar brasileira (Cereais, Frutas & Vegetais; Soja & Derivados; Leite & Laticínios; Carne & Ovos; Gorduras & Óleos; Açúcares & Doces). Dez itens foram analisados nos rótulos: presença de lista de ingredientes; frase de alerta para alérgicos; frase de alerta; local da frase; frase em letras maiúsculas; frase em negrito; a cor da frase de alerta contrastando com o fundo; tamanho de fonte adequado; não alegar ausência de qualquer alérgeno; outros fatores de difícil leitura. Para a segunda questão, foi respondido questionário estruturado, e os produtos classificados em categorias: “Contém” e “Pode conter”. Resultados: A qualidade do rótulo foi adequada e 69% dos alimentos embalados continham pelo menos um alérgeno. A informação “Pode conter” foi mais identificada em: proteína do leite (Cereais e Carnes e ovos), soja (Soja & derivados) e proteína do ovo (Cereais). Soja e derivados foi o grupo alimentar de maior insegurança. Conclusões: Profissionais de saúde podem contar com rotulagem de boa qualidade dos produtos embalados e, consequentemente, promover a educação de pais/cuidadores para consultar os rótulos e gerenciar os riscos em alimentos processados. Soja e produtos são os alimentos com maior insegurança entre os Grupos da Pirâmide Brasileira.

ABSTRACT Objective: The aim of this study was to evaluate allergenic labeling components of packaged foods for “What is the quality of food labels?” and “What is the group of Brazilian Food Pyramid that ‘May contain’ is predominant?.” Methods: The photographs of 916 products were obtained, of which 518 were analyzed. Data from each label were evaluated according to Brazilian Food Pyramid Groups (i.e., Cereals, Fruits, & Vegetables; Soybean & products; Milk & dairy products; Meat & eggs; Fats & oils; and Sugars & sweets). Ten items were analyzed in each label, namely, the presence of a list of ingredients, alert phrase for allergy sufferers, grouping of the alert phrase, phrase location, uppercase phrase, the phrase in bold, the color of alert phrase contrasting to the background, adequate font size, do not claim the absence for any allergen with the ingredients, and others factors that make it difficult to read. For the second question, a structured questionnaire was completed, and products were classified into two categories, namely, “Contain” and “May contain.” Results: The quality of the label was appropriate, and 69% of packaged foods had at least one allergen. The information “May contain” were higher in cow’s milk (Cereals and Meat & eggs), soy (Soybean & products), and egg protein (Cereals). Soybean & products were the highest insecurity group. Conclusions: Brazilian health professionals can count on good-quality labeling of packaged products. Consequently, they could promote patients’ and parents/caregivers’ education to consult the labels and manage the risks in processed foods about precautionary allergen labeling. Soybean & products were the most significant insecurity for food choices between Brazilian Pyramid Groups.

RESUMO A cultura do milho é altamente responsiva à adubação nitrogenada, no entanto, a aplicação de doses elevadas, geralmente, provoca redução do aproveitamento do fertilizante. Neste sentido, objetivou-se com este estudo determinar a produtividade e o balanço do nitrogênio no milho cultivado em sistema de plantio direto. O experimento consistiu na aplicação de quatro doses de nitrogênio (0, 30; 70 e 95 kg ha-1). Foi aplicado em todas as parcelas do milho híbrido AG 1051, 80 e 56 kg ha-1 de P2O5 e K2O, respectivamente. O experimento foi disposto em delineamento em blocos ao acaso, com cinco repetições. A produtividade teve comportamento linear crescente em função das doses de nitrogênio, a qual atingiu 5.684,79 kg ha-1 de grão. A eficiência da adubação e a extração do N aumentaram linearmente com as doses de nitrogênio, obtendo-se com a maior dose a extração de 114,93 kg ha-1 de N e uma eficiência de adubação de 65%. O estoque de N do solo de 4.489 kg ha-1 disponibiliza às plantas de milho 52,81 kg ha-1 desse nutriente. O milho ajusta a produção de biomassa de acordo com a disponibilidade do nitrogênio, não havendo efeito das doses de N no teor do nutriente nos tecidos da planta.

ABSTRACT The corn crop is highly responsive to nitrogen fertilization; however, the application of high doses generally causes a reduction in the use efficiency of fertilizer. In this sense, this study aimed to determine the yield and nitrogen balance in corn grown under the no-tillage system. The experiment consisted of the application of four nitrogen doses (0, 30; 70 and 95 kg ha-1). In all plots with the AG 1051 hybrid corn, 80 and 56 kg ha-1 of P2O5 and K2O were applied, respectively. The experiment was arranged in a randomized block design with five replications. The grain yield had a linear increasing behavior according to the nitrogen doses, which reached 5,684.79 kg ha-1. The fertilization efficiency and the N extraction increased linearly with the increase of the nitrogen doses, obtaining with the highest dose the extraction of 114.93 kg ha-1 of N and a fertilization efficiency of 65%. The soil N stock of 4,489 kg ha-1 provides corn plants with 52.81 kg ha-1 of this nutrient. Corn adjusts the biomass production according to the availability of nitrogen, with no effect of N doses on the nutrient content in plant tissues.

RESUMO Os efeitos da aplicação de carvão vegetal e nitrogênio sobre a produtividade de arroz de terras altas e feijão-caupi e a fertilidade do solo foram determinados em ambiente protegido em Macaíba, RN, Brasil. O experimento constou de dois cultivos sucessivos em delineamento experimental completamente casualizado em arranjo fatorial, com quatro repetições. Inicialmente, foram testadas quatro doses de carvão vegetal e quatro de nitrogênio para a cultura do arroz. A seguir, foi cultivado feijão-caupi em metade dos vasos (quatro doses de carvão e duas de nitrogênio). O solo foi amostrado após a colheita do arroz, em metade dos vasos, e ao final do experimento, nos vasos restantes. Do arroz foram determinados, massa de cem grãos e, por vaso, massa seca da parte aérea, número de panículas, número de grãos cheios e grãos vazios e produção de grãos. Do feijão-caupi foram determinados: número de vagens por vaso, número de grãos por vagem e produção de grãos por vaso. No solo foram determinados: pH, teores de carbono orgânico, P, K, Ca, Mg e Na, capacidade de troca de cátions e percentagem de sódio trocável. As doses de carvão vegetal não influenciaram nas características de produção do arroz e feijão-caupi, mas favoreceram a retenção de nitrogênio no solo, que se refletiu sobre massa seca da parte aérea do arroz, esterilidade das espiguetas, número de panículas e massa dos grãos. Além disso, promoveram aumento no pH, teor de potássio e percentagem de sódio trocável do solo, com diminuição no teor de cálcio e magnésio.

ABSTRACT The effects of biochar and nitrogen application on yields of upland rice and cowpea and on soil fertility were determined in a greenhouse in Macaíba, RN, Brazil. The trial consisted of the succession of two crops in a completely randomized design and a factorial scheme, with four replicates. Initially, four doses of biochar and four doses of nitrogen were tested for cultivation of rice. Subsequently, four doses of biochar and two doses of nitrogen were tested in half of the pots maintained for planting cowpea. Soil was sampled after rice harvest for half of the pots and at end of the trial for the remaining pots. We evaluated the following parameters: mass of hundred grains of rice, dry shoot mass, panicle number, number of filled spikelets and of empty spikelets, and grain production. Determinations for cowpea were: pod number per pot, grain number per pod, and grain production per pot. Measured soil parameters were: pH, contents of organic carbon, P, K, Ca, Mg, Na, cation exchange capacity, and exchangeable sodium percentage. Biochar addition did not influence yield components of upland rice and cowpea, but resulted in increased soil N retention, which influenced rice dry shoot mass, spikelets sterility, panicle number, and grain mass. Biochar also promoted increased soil pH, potassium content, and exchangeable sodium percentage and decreased calcium and magnesium concentrations.

RESUMO O Brasil é o terceiro maior produtor de frutas do mundo, porém muitas fruteiras nativas continuam subexploradas, como ocorre com a mangabeira (Hancornia speciosa Gomes). Pouco se conhece sobre os aspectos gerais da espécie em seus ambientes de ocorrência natural. Desta forma, objetivou-se caracterizar a morfologia da planta e do fruto de mangabeiras silvestres selecionadas em áreas de ocorrência natural da espécie, em vegetação de transição entre o Cerrado e a Restinga. O experimento foi realizado no município de Morros-Maranhão, nos povoados Patizal, Recanto e Recurso, caracterizados por extensas áreas de vegetação de transição: Cerrado e Restinga, com ocorrência natural de mangabeira. Nos três povoados, selecionaram-se 45 mangabeiras-matrizes, das quais se mensuraram a altura total, altura do fuste, diâmetro do caule, volume de copa, número de ramos principais e secundários, e número de frutos. Coletaram-se 20 frutos por matriz e determinaram-se a massa, comprimento e diâmetro dos frutos, massa da polpa mais casca, rendimento de polpa, número e massa de sementes. Houve elevado grau de variabilidade para todas as variáveis estudadas, exceto para o rendimento de polpa (média = 86,69%, mínimo = 76,75% e máximo = 91,11%; CV = 3,51%). Há correlação positiva significativa de alta magnitude entre as variáveis massa do fruto e massa da polpa mais casca com o número de sementes (r=0,01; ?=0,78 e ?=0,74, respectivamente).

ABSTRACT Brazil is the third largest fruit producer in the world, but many native fruits remain underexploited, as is the case for mangabeira (Hancornia speciosa Gomes). Little is known about general aspects of this species in its naturally occurring environment. Thus, this study aimed to characterize plant and fruit morphologies from native mangabeira selections in the Savannah-Restinga vegetation transition zone. The experiment was carried out in Patizal, Recanto and Recurso villages located in the Morros municipality, Maranhão State, dominated by extensive Savannah-Restinga vegetation transition zone with naturally occurring mangabeiras. Forty five mangabeira trees were randomly chosen from native selections in the three villages. Data collected were plant height, stem height, stem diameter, canopy volume, number of main and secondary branches and number of fruits. Twenty fruits were collected per plant to determine the mass, length and diameter, pulp and skin mass, pulp yield, seed number and seed mass. There was a high degree of variability for all assessed variables except for the pulp yield (average = 86.69%, minimum = 76.75% and maximum = 91.11%; CV= 3.51%). Significant and positive high magnitude correlations were noted among the variables of fruit mass and pulp mass plus skin with seed number (r=0,01; ?=0,78 e ?=0,74, respectively).

Centronuclear myopathy (CNM) is a rare congenital muscle disease characterized by fibers with prominent centralized nuclei in muscle biopsies. The disease is clinically heterogeneous, ranging from severe neonatal hypotonic phenotypes to adult-onset mild muscle weakness, and can have multiple modes of inheritance in association with various genes, including MTM1, DNM2, BIN1 and RYR1. Here we analyzed 18 sporadic patients with clinical and histological diagnosis of CNM and sequenced the DNM2 gene, which codes for the dynamin 2 protein. We found DNM2 missense mutations in two patients, both in exon 8, one known (p.E368K) and one novel (p.F372C), which is found in a position of presumed pathogenicity and appeared de novo. The patients had similar phenotypes characterized by neonatal signs followed by improvement and late childhood reemergence of slowly progressive generalized muscle weakness, elongated face with ptosis and ophthalmoparesis, and histology showing fibers with radiating sarcoplasmic strands (RSS). These patients were the only ones in the series to present this histological marker, which together with previous reports in the literature suggest that, when RSS are present, direct sequencing of DNM2 mutation hot spot regions should be the first step in the molecular diagnosis of CNM, even in sporadic cases.

OBJECTIVE: This paper assesses the quality of the underlying cause of death (COD) statistics in Hermosillo, Mexico in a random sample of 300 in-hospital adult deaths. MATERIAL AND METHODS: A "gold standard" COD, determined by a systematic review of hospital medical charts, was compared to the COD reported by the vital registry system. RESULTS: Overall agreement between the reviewer and original COD at the ICD-10 chapter block was 69.2%, with a weighted kappa of 0.62. Agreement varied greatly by ICD-10 chapter. Mutual misclassification among common co-morbidities,such as diabetes mellitus and circulatory disease, minimized the net change in the mortality fraction assigned to each ICD-10 chapter after physician review. CONCLUSIONS: The ICD-10 chapter level underlying COD codes can be used to estimate disease burden in the population. Caution is recommended for use of vital registry statistics in Hermosillo for individual level or disease-specific analyses.

OBJETIVO: Examinar la validez de la causa básica de muerte (CBM) de certificados de defunción de un sistema digital de vigilancia epidemiológica en Hermosillo, México. MATERIAL Y MÉTODOS: Se comparó la CBM originalmente codificada en una muestra aleatoria de 300 certificados de defunción de muertes adultas ocurridas en al año 2005, con la CBM determinada por una revisión de expedientes médicos. RESULTADOS: Se identificó una concordancia global de 69.2% entre la CBM original y la determinada por la revisión, con un coeficiente de kappa de 0.62. La concordancia varió,con errores de codificación en padecimientos como diabetes mellitus, enfermedades circulatorias y respiratorias y neoplasias. El efecto de esos errores fue compensado por una mutua asignación equivocada en el proceso de codificación de la CBM empleado por el sistema. CONCLUSIONES: Las estadísticas generadas por el sistema examinado pueden usarse con cautela para estimar la carga poblacional de mortalidad en Hermosillo, México.

OBJECTIVE: This paper assesses the quality of the underlying cause of death (COD) statistics in Hermosillo, Mexico in a random sample of 300 in-hospital adult deaths. MATERIAL AND METHODS: A "gold standard" COD, determined by a systematic review of hospital medical charts, was compared to the COD reported by the vital registry system. RESULTS: Overall agreement between the reviewer and original COD at the ICD-10 chapter block was 69.2%, with a weighted kappa of 0.62. Agreement varied greatly by ICD-10 chapter. Mutual misclassification among common co-morbidities,such as diabetes mellitus and circulatory disease, minimized the net change in the mortality fraction assigned to each ICD-10 chapter after physician review. CONCLUSIONS: The ICD-10 chapter level underlying COD codes can be used to estimate disease burden in the population. Caution is recommended for use of vital registry statistics in Hermosillo for individual level or disease-specific analyses.

OBJETIVO: Examinar la validez de la causa básica de muerte (CBM) de certificados de defunción de un sistema digital de vigilancia epidemiológica en Hermosillo, México. MATERIAL Y MÉTODOS: Se comparó la CBM originalmente codificada en una muestra aleatoria de 300 certificados de defunción de muertes adultas ocurridas en al año 2005, con la CBM determinada por una revisión de expedientes médicos. RESULTADOS: Se identificó una concordancia global de 69.2% entre la CBM original y la determinada por la revisión, con un coeficiente de kappa de 0.62. La concordancia varió,con errores de codificación en padecimientos como diabetes mellitus, enfermedades circulatorias y respiratorias y neoplasias. El efecto de esos errores fue compensado por una mutua asignación equivocada en el proceso de codificación de la CBM empleado por el sistema. CONCLUSIONES: Las estadísticas generadas por el sistema examinado pueden usarse con cautela para estimar la carga poblacional de mortalidad en Hermosillo, México.

A Doença do Refluxo Gastroesofágico (DRGE) é uma afecção comum na infância, aumentando as evidências de que o refluxo gastroesofágico seja um cofator importante que contribui para as desordens de vias aéreas, principalmente na população pediátrica. É muito comum serem observadas manifestações em vias aéreas superiores e inferiores. Nosso objetivo é avaliar a presença de sintomas otorrinolaringológicos em crianças com idade de um a 12 anos e suspeita de doença do refluxo gastroesofágico. MATERIAIS E MÉTODO: Foram avaliados dados de prontuários de pacientes de até 12 anos submetidos à pHmetria de 24 horas de um ou dois canais, locados a 2 e 5 cm do EEI para confirmação de diagnóstico de Doença do Refluxo Gastroesofágico. RESULTADOS: Foram analisados 143 prontuários de crianças que realizaram pHmetria de 24 horas para investigação de DRGE; porém 65 foram incluídas. Os sintomas mais prevalentes nas crianças eram os broncopulmonares, encontrados em 89,2%, de sintomas nasossinusais (72,3%) , otológicos (46,1%) e de infecções de VAS de repetição (44,6%). Quando comparada a presença de cada grupo de sintomas com o resultado da pHmetria, não foi encontrada diferença significativa entre os sintomas e o resultado da pHmetria. CONCLUSÃO: DRGE pode se manifestar de diversas maneiras e os sintomas otorrinolaringológicos são frequentes em crianças.

Gastroesophageal reflux disease (GERD) is a common ailment in children, adding up to the evidence that gastroesophageal reflux is an important cofactor in upper airway disorders, especially in the pediatric population. It is very common for it to impact the upper and lower airways. Our goal was to assess the presence of otorhinolaryngological symptoms in children aged between one and twelve years in whom gastroesophageal reflux is suspected. MATERIALS AND METHODS: We assessed data from the charts of patients up to 12 years of age submitted to 24 hour pH measuring of one of two channels, placed at 2 and 5 cm from the LEE in order to confirm the diagnosis of Gastroesophageal Reflux Disease. RESULTS: We studied 143 charts from children who underwent 24 hour pH measuring to investigate GERD; however, only 65 were included. The most prevalent symptoms in the children were bronchopulmonary, found in 89.2%, of sinonasal symptoms (72.3%), otologic (46.1%) and repetition UAW infections (44.6%). When we compared the presence of each group of symptoms of the results from the pH measuring, no significant differences were found between the symptoms and the pH measuring results. CONCLUSION: GERD can manifest in different ways and otorhinolaryngological symptoms are frequent in children.

INTRODUCTION: Limb-girdle muscular dystrophy presents with heterogeneous clinical and molecular features. The primary characteristic of this disorder is proximal muscular weakness with variable age of onset, speed of progression, and intensity of symptoms. Sarcoglycanopathies, which are a subgroup of the limb-girdle muscular dystrophies, are caused by mutations in sarcoglycan genes. Mutations in these genes cause secondary deficiencies in other proteins, due to the instability of the dystrophin-glycoprotein complex. Therefore, determining the etiology of a given sarcoglycanopathy requires costly and occasionally inaccessible molecular methods. OBJECTIVE: The aim of this study was to identify phenotypic differences among limb-girdle muscular dystrophy patients who were grouped according to the immunohistochemical phenotypes for the four sarcoglycans. METHODS: To identify phenotypic differences among patients with different types of sarcoglycanopathies, a questionnaire was used and the muscle strength and range of motion of nine joints in 45 patients recruited from the Department of Neurology - HC-FMUSP (Clinics Hospital of the Faculty of Medicine of the University of São Paulo) were evaluated. The findings obtained from these analyses were compared with the results of the immunohistochemical findings. RESULTS: The patients were divided into the following groups based on the immunohistochemical findings: a-sarcoglycanopathies (16 patients), b-sarcoglycanopathies (1 patient), y-sarcoglycanopathies (5 patients), and nonsarcoglycanopathies (23 patients). The muscle strength analysis revealed significant differences for both upper and lower limb muscles, particularly the shoulder and hip muscles, as expected. No pattern of joint contractures was found among the four groups analyzed, even within the same family. However, a high frequency of tiptoe gait was observed in patients with a-sarcoglycanopathies, while calf pseudo-hypertrophy was most common in patients with non-sarcoglycanopathies. The a-sarcoglycanopathy patients presented with more severe muscle weakness than did y-sarcoglycanopathy patients. CONCLUSION: The clinical differences observed in this study, which were associated with the immunohistochemical findings, may help to prioritize the mutational investigation of sarcoglycan genes.

OBJETIVO: Comparar força muscular e função motora de pacientes com distrofia muscular de Duchenne (DMD) em corticoterapia com a evolução natural da doença descrita por Scott et al. MÉTODO: Noventa pacientes, entre 5 e 12 anos de idade, em corticoterapia por um até sete anos, foram avaliados quanto à força muscular (FM) (escala MRC) e função motora (Hammersmith motor ability score). A relação entre idade, FM e função motora e a comparação com o estudo de Scott et al foram determinadas estatisticamente. RESULTADOS: a relação idade/escore Hammersmith diminuiu 0,76 pontos a cada ano de aumento da idade (2,23 pontos na história natural). A relação idade/MRC decresceu 0,80 pontos a cada ano de aumento da idade (3,65 pontos na história natural). CONCLUSÃO: Nos pacientes em corticoterapia, a progressão da doença é mais lenta que na evolução natural em todas as faixas etárias avaliadas, sendo a FM mais comprometida que a função motora.

OBJECTIVE: To compare muscle strength (MS) and motor function in patients with Duchenne muscular dystrophy (DMD) receiving steroids for different times against the natural evolution of DMD described by Scott et al. METHOD: 90 patients with DMD (aged 5- 12 years), receiving steroids for one to seven years, were evaluated by Medical Research Council Scale (MRC) and Hammersmith motor ability score. The relation between MS and motor abilities measurement from our data and Scott's ones were ascertained statistically. RESULTS: The relation between patient's age and Hammersmith scores revealed decrease of 0.76 point per year for age against decrease of 2.23 points on Scott's study. The relation between MRC scale and patient's age showed decrease of 0.80 point per year of age against decrease of 3.65 points on Scott's study. CONCLUSION: In patients with DMD aged five to 12 years the progression of the disease is delayed by steroids and the motor function is less reduced than muscular strength.

OBJETIVO: Avaliar a prevalência do sobrepeso/obesidade em um grupo de crianças com constipação crônica funcional. MÉTODOS: Estudo retrospectivo com 257 crianças de dois a 15 anos com constipação crônica funcional diagnosticada segundo critérios de Roma II, atendidas consecutivamente em clínica terciária de gastroenterologia pediátrica. Informações demográficas, clínicas, diagnósticas e antropométricas foram obtidas de prontuários. O índice de massa corporal (IMC) foi calculado e utilizou-se a referência do Centers for Disease Control para determinar o percentil de IMC para idade e gênero. Crianças com percentil de IMC>85 foram consideradas com sobrepeso/obesidade; as com percentis > 5 e <85 foram classificadas como eutróficas. RESULTADOS: Para as variáveis sociodemográficas e clínicas, não houve diferença estatisticamente significante entre os subgrupos sobrepeso/obesidade e eutrofia. A prevalência de crianças constipadas com sobrepeso/obesidade foi de 24,4%. A média dos percentis do subgrupo sobrepeso/obesidade foi 94,4±4,3; a das eutróficas foi 45,3±24,2. A prevalência de sobrepeso/obesidade foi similar em ambos os sexos e grupos etários (<7 anos e >7 anos). CONCLUSÕES: Os subgrupos dos eutróficos e sobrepeso/obesidade foram homogêneos nas características sociodemográficas e clínicas, sugerindo que tais variáveis não influenciaram na prevalência do excesso de peso. A prevalência estimada de sobrepeso/obesidade entre as crianças com constipação crônica funcional está dentro dos valores esperados para a população pediátrica brasileira.

OBJECTIVE: To determine the prevalence of overweight/obesity in a group of children with chronic functional constipation. METHODS: Retrospective chart review of 257 children with chronic functional constipation as defined by Rome II criteria, 2-15 years old, assisted in a tertiary pediatric gastroenterology clinic. Demographic, clinical, diagnostic and anthropometric information was extracted from the charts. Body mass index (BMI) was calculated and The Centers for Disease Control's BMI growth reference was used to determine age and gender-specific BMI percentiles of the children. Overweight/obesity was classified as a BMI of >85th percentile, and euthrophy as BMI >5th and <85th percentile. RESULTS: There was no statistically significant difference for demographics and clinical variables between the subgroups overweight/obesity and eutrophy. In constipated children, prevalence of overweight/obesity was 24.4%. Prevalence of overweight/obesity did not differ between boys and girls or across age groups (<7 years and >7 years). CONCLUSIONS: Demographics and clinical characteristics were similar between the overweight/obese and eutrophic subgroups, which suggests that these characteristics do not influence on the prevalence of overweight/obesity in constipated children. The estimated prevalence of overweight/obesity in this group was similar to the prevalence of age-matched reference for Brazilian children.

OBJETIVO: Um protocolo de avaliação foi aplicado com o objetivo de quantificar e descrever evolutivamente a força muscular e as habilidades motoras de 32 pacientes com distrofia muscular de Duchenne (DMD), com idades variando de 5 a 12 anos, em corticoterapia. MÉTODO: As avaliações foram aplicadas mensalmente durante os primeiros seis meses e bimensais até completar um período de 14 meses. Os testes empregados foram: escala da "Medical Research Council" (MRC); Hammersmith "motor ability score"; levantamento da carga máxima de peso; cronometragem do tempo para levantar-se do chão e percorrer nove metros. RESULTADOS: Os resultados demonstraram que a perda da força muscular e das habilidades motoras foi mais lenta do que a observada na evolução natural da doença, como descrito na literatura internacional. CONCLUSÃO: Concluímos que uma rápida e objetiva avaliação pode ser executada utilizando a escala MRC para membros inferiores e tronco, Hammersmith motor ability score, cronometragem do tempo para percorrer 9 metros.e o levantamento de peso.

OBJECTIVE: An assessment protocol was applied to quantify and describe muscular strength and motor abilities of 32 patients with Duchenne muscular dystrophy (DMD), aged between 5 and 12 years on steroid therapy. METHOD: Assessments were made monthly for the first six months and with intervals of two months thereafter until the 14-month end point. The tests employed included: the Medical Research Council (MRC) scale; the Hammersmith motor ability score; maximum weight lift; timed rise from floor and nine-meter walk. RESULTS: The results showed that loss of muscular strength and motor abilities were slowed in comparison to that observed in the natural evolution of the disease according to the literature. CONCLUSION: We conclude that a swift and objective assessment may be performed using the MRC scale for lower limbs and trunk, the Hammersmith motor ability score, timed nine-meter walk and weight lifts.