10 research outputs found
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Chloride Conductance, Nasal Potential Difference and Cystic Fibrosis Pathophysiology.
PurposeCystic fibrosis (CF) is a multisystem genetic disease caused by dysfunction of the epithelial anionic channel Cystic Fibrosis Transmembrane conductance Regulator (CFTR). Decreased mucociliary clearance because of thickened mucus is part of the pulmonary disease pathophysiology. It is controversial if the thickened airway surface liquid (ASL) is caused by the deficient chloride secretion and excessive sodium (through ENaC) and water hyperabsorption from the periciliar fluid or by the lack of bicarbonate secretion with relative acidification of the ASL. Correlations between the magnitude of in vivo chloride conductance with phenotypic characteristics and CF genotype can help to elucidate these mechanisms and direct to new treatments.MethodsNasal potential difference was measured in 28 CF patients (age from 0.3 to 28 year) and correlated with pulmonary function, pancreatic phenotype, pulmonary colonization and genotype severity.ResultsThe CFTR-chloride conductance was better in older patients (r = 0.40; P = 0.03), in patients with better pulmonary function (r = 0.48; P = 0.01), and was associated with genotype severity. Higher chloride diffusion in the presence of a favorable chemical gradient was associated with Pseudomonas aeruginosa negativity (P < 0.05). More negative NPDmax was associated with pancreatic insufficiency (P < 0.01) as well with genotype severity, but not with the pulmonary function.ConclusionsThe anion permeability through CFTR, mainly chloride, but bicarbonate as well, is the most critical factor in CF airway pathophysiology. Treatments primarily directed to correct CFTR function and/or airway acidity are clearly a priority
Is willingness to exercise programmed in utero? Reviewing sedentary behavior and the benefits of physical activity in intrauterine growth restricted individuals
Objective: The literature suggests that a fetus will adapt to surrounding adversities by optimizing its use of energy to improve survival, ultimately leading to the programming of the individual's energy intake and expenditure. While recent reviews focused on the fetal programming of energy intake and food preferences, there is also some evidence that fetal adversity is associated with diminished physical activity levels. Therefore, we aimed to review (a) the evidence for an association between being born with intrauterine growth restriction and sedentarism over the life-course and (b) the potential benefits of physical activity over cardiometabolic risk factors for this population. Sources: PubMed, Scielo, Scopus and Embase. Summary of findings: Most clinical studies that used objective measures found no association between intrauterine growth restriction and physical activity levels, while most studies that used self-reported questionnaires revealed such relationships, particularly leisure time physical activity. Experimental studies support the existence of fetal programming of physical activity, and show that exposure to exercise during IUGR individuals’ life improves metabolic outcomes but less effect was seen on muscle architecture or function. Conclusions: Alterations in muscle strength and metabolism, as well as altered aerobic performance, may predispose IUGR individuals to be spontaneously less physically active, suggesting that this population may be an important target for preventive interventions. Although very heterogeneous, the different studies allow us to infer that physical activity may have beneficial effects especially for individuals that are more vulnerable to metabolic modifications such as those with IUGR. Resumo: Objetivo: A literatura sugere que um feto se adaptará às adversidades externas ao aprimorar seu gasto energético para melhorar a sobrevida, o que leva, em última instância, à programação do consumo e gasto energético do indivíduo. Apesar de análises recentes terem focado na programação fetal do consumo energético e preferências alimentares, ainda há alguma comprovação de que as adversidades fetais estão associadas aos baixos níveis de atividade física. Portanto, visamos analisar: a) a comprovação de uma associação entre nascer com restrição de crescimento intrauterino (RCIU) e sedentarismo durante o curso de vida e b) os possíveis benefícios da atividade física sobre os fatores de risco cardiometabólico dessa população. Fontes: PubMed, Scielo, Scopus e Embase. Resumo dos achados: A maior parte dos estudos clínicos que usaram medidas objetivas não constatou associação entre RCIU e os níveis de atividade física, ao passo que a maior parte dos estudos que usaram questionários de autorrelato revelou essas relações, principalmente no que diz respeito à atividade física de lazer. Estudos experimentais corroboram a existência de programação fetal de atividade física e mostram que a exposição a exercícios durante a vida de indivíduos com RCIU melhora os resultados metabólicos, porém menos efeito foi visto sobre a arquitetura ou função muscular. Conclusões: Alterações na força muscular e no metabolismo, bem como o desempenho aeróbico alterado, podem predispor indivíduos com RCIU a serem espontaneamente menos ativos fisicamente, sugere que essa população pode ser um importante alvo de intervenções preventivas. Apesar de muito heterogêneos, os diferentes estudos nos possibilitam deduzir que a atividade física pode ter efeitos benéficos principalmente em indivíduos mais vulneráveis a modificações metabólicas, como aqueles com RCIU. Keywords: DOHaD, IUGR, Physical activity, Sedentary behavior, Palavras-chave: DOHaD, RCIU, Atividade física, Comportamento sedentári
Is willingness to exercise programmed in utero? Reviewing sedentary behavior and the benefits of physical activity in intrauterine growth restricted individuals
Objective: The literature suggests that a fetus will adapt to surrounding adversities by optimizing its use of energy to improve survival, ultimately leading to the programming of the individual's energy intake and expenditure. While recent reviews focused on the fetal programming of energy intake and food preferences, there is also some evidence that fetal adversity is associated with diminished physical activity levels. Therefore, we aimed to review (a) the evidence for an association between being born with intrauterine growth restriction and sedentarism over the life‐course and (b) the potential benefits of physical activity over cardiometabolic risk factors for this population. Sources: PubMed, Scielo, Scopus and Embase. Summary of findings: Most clinical studies that used objective measures found no association between intrauterine growth restriction and physical activity levels, while most studies that used self‐reported questionnaires revealed such relationships, particularly leisure time physical activity. Experimental studies support the existence of fetal programming of physical activity, and show that exposure to exercise during IUGR individuals’ life improves metabolic outcomes but less effect was seen on muscle architecture or function. Conclusions: Alterations in muscle strength and metabolism, as well as altered aerobic performance, may predispose IUGR individuals to be spontaneously less physically active, suggesting that this population may be an important target for preventive interventions. Although very heterogeneous, the different studies allow us to infer that physical activity may have beneficial effects especially for individuals that are more vulnerable to metabolic modifications such as those with IUGR. Resumo: Objetivo: A literatura sugere que um feto se adaptará às adversidades externas ao aprimorar seu gasto energético para melhorar a sobrevida, o que leva, em última instância, à programação do consumo e gasto energético do indivíduo. Apesar de análises recentes terem focado na programação fetal do consumo energético e preferências alimentares, ainda há alguma comprovação de que as adversidades fetais estão associadas aos baixos níveis de atividade física. Portanto, visamos a analisar: a) a comprovação de uma associação entre nascer com restrição de crescimento intrauterino (RCIU) e sedentarismo durante o curso de vida e b) os possíveis benefícios da atividade física sobre os fatores de risco cardiometabólico dessa população. Fontes: PubMed, Scielo, Scopus e Embase. Resumo dos achados: A maior parte dos estudos clínicos que usaram medidas objetivas não constatou associação entre RCIU e os níveis de atividade física, ao passo que a maior parte dos estudos que usaram questionários de autorrelato revelou essas relações, principalmente no que diz respeito à atividade física de lazer. Estudos experimentais corroboram a existência de programação fetal de atividade física e mostram que a exposição a exercícios durante a vida de indivíduos com RCIU melhora os resultados metabólicos, porém menos efeito foi visto sobre a arquitetura ou função muscular. Conclusões: Alterações na força muscular e no metabolismo, bem como o desempenho aeróbico alterado, podem predispor indivíduos com RCIU a serem espontaneamente menos ativos fisicamente, sugere que essa população pode ser um importante alvo de intervenções preventivas. Apesar de muito heterogêneos, os diferentes estudos nos possibilitam deduzir que a atividade física pode ter efeitos benéficos principalmente em indivíduos mais vulneráveis a modificações metabólicas, como aqueles com RCIU. Keywords: DOHaD, IUGR, Physical activity, Sedentary behavior, Palavras‐chave: DOHaD, RCIU, Atividade física, Comportamento sedentári
A eficácia do uso de videogames e programas baseados em software na educação e autogestão em asma para crianças e adolescentes
Introduction: Asthma is a chronic disease of the lower
airways characterized by usually reversible airflow
obstruction, inflammation, and hyperreactivity to various
types of stimuli. It is one of the most common chronic
respiratory diseases among children and adults, with an
incidence of 300 million people worldwide. Objective:
To evaluate the quality of the existent evidence in the
literature in order to support the use of videogames and
software-based programson asthmatic children and
teenager’s knowledge capacity and self-management
compared with standard education. Methods: Search,
selection and analysis (starting March 2016) of all the
original articles on virtual reality (VR) concerning asthmatic
children and adolescents (ages 3 to 18), published up to
October 2017, in Portuguese, English and Spanish, at the
electronic databases Pubmed, Web of Science, MedlLine
and LILACS, obtained by using the descriptors: asthma,
video game, virtual reality, pulmonary rehabilitation,
physical training. Results: Only six original articles were
obtained. Of these, five (80%) presented level of evidence
A, and one (20%) presented level of evidence B. All authors
point out the treatment of asthma from VR as a safe and
innovative therapy considering that the motivation and
intensity of treatment from the use of VR improves the selfmanagement
capacity and aerobic capacity of asthmatic
patients. Conclusion: The present study seeks to contribute
to the literature by demonstrating that videogames and
other software-based systemscan be used to improve
knowledge capacity and self-management skills in children
and teenagers with asthma diagnosis.A asma é uma doença crônica das vias aéreas
inferiores, caracterizada por obstrução ao fluxo aéreo
geralmente reversível, inflamação e hiper-reatividade a vários
tipos de estímulos. É uma das doenças respiratórias crônicas
mais comuns entre crianças e adultos, com incidência de 300
milhões de pessoas em todo o mundo. Objetivo: Avaliar a
qualidade das evidências existentes na literatura a fim de apoiar
o uso de videogames e programas baseados em software na
capacidade de conhecimento e autogestão de crianças e
adolescentes asmáticos em comparação com a educação
padrão. Métodos: Pesquisa, seleção e análise (a partir de março
de 2016) de todos os artigos originais sobre realidade virtual
(RV) utlizada com crianças e adolescentes asmáticos (idades de
3 a 18), publicados até outubro de 2017, em português, inglês
e espanhol, nas bases de dados eletrônicas Pubmed, Web of
Science, MedlLine e LILACS, obtidas por meio dos descritores:
asma, videogame, realidade virtual, reabilitação pulmonar,
treinamento físico. Resultados: Apenas seis artigos originais
foram obtidos. Destes, cinco (80%) apresentaram nível de
evidência A e um (20%) apresentou nível de evidência B. Todos
os autores apontam o tratamento da asma por RV como uma
terapia segura e inovadora, considerando que a motivação
e intensidade do tratamento com o uso da RV melhora a
capacidade de autogerenciamento e capacidade aeróbia
do paciente asmático. Conclusão: O presente estudo busca
contribuir com a literatura ao demonstrar que videogames e
outros sistemas baseados em software podem ser utilizados
para melhorar a capacidade de conhecimento e autogerenciamento
de crianças e adolescentes com diagnóstico de asma
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Chloride Conductance, Nasal Potential Difference and Cystic Fibrosis Pathophysiology.
PurposeCystic fibrosis (CF) is a multisystem genetic disease caused by dysfunction of the epithelial anionic channel Cystic Fibrosis Transmembrane conductance Regulator (CFTR). Decreased mucociliary clearance because of thickened mucus is part of the pulmonary disease pathophysiology. It is controversial if the thickened airway surface liquid (ASL) is caused by the deficient chloride secretion and excessive sodium (through ENaC) and water hyperabsorption from the periciliar fluid or by the lack of bicarbonate secretion with relative acidification of the ASL. Correlations between the magnitude of in vivo chloride conductance with phenotypic characteristics and CF genotype can help to elucidate these mechanisms and direct to new treatments.MethodsNasal potential difference was measured in 28 CF patients (age from 0.3 to 28 year) and correlated with pulmonary function, pancreatic phenotype, pulmonary colonization and genotype severity.ResultsThe CFTR-chloride conductance was better in older patients (r = 0.40; P = 0.03), in patients with better pulmonary function (r = 0.48; P = 0.01), and was associated with genotype severity. Higher chloride diffusion in the presence of a favorable chemical gradient was associated with Pseudomonas aeruginosa negativity (P < 0.05). More negative NPDmax was associated with pancreatic insufficiency (P < 0.01) as well with genotype severity, but not with the pulmonary function.ConclusionsThe anion permeability through CFTR, mainly chloride, but bicarbonate as well, is the most critical factor in CF airway pathophysiology. Treatments primarily directed to correct CFTR function and/or airway acidity are clearly a priority
Can the data of microbiome be used to predict the presence of Burkholderia spp in pulmonary microbiota of cystic fibrosis patients?
Brazilian guidelines for the diagnosis and treatment of cystic fibrosis
ABSTRACT Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions