Reference values for spirometry in Brazilian children.

OBJECTIVE: To generate reference values for spirometry in Brazilian children 3-12 years of age and to compare those values with the values employed in the equations currently in use in Brazil. METHODS: This study involved healthy children, 3-12 years of age, recruited from 14 centers (primary data) and spirometry results from children with the same characteristics in six databases (secondary data). Reference equations by quantile regressions were generated after log transformation of the spirometric and anthropometric data. Skin color was classified as self-reported by the participants. To determine the suitability of the results obtained, they were compared with those predicted by the equations currently in use in Brazil. RESULTS: We included 1,990 individuals from a total of 21 primary and secondary data sources. Of those, 1,059 (53%) were female. Equations for FEV1, FVC, the FEV1/FVC ratio, FEF between 25% and 75% of the FVC (FEF25-75%) and the FEF25-75%/FVC ratio were generated for white-, black-, and brown-skinned children. The logarithms for height and age, together with skin color, were the best predictors of FEV1 and FVC. The reference values obtained were significantly higher than those employed in the equations currently in use in Brazil, for predicted values, as well as for the lower limit of normality, particularly in children with self-reported black or brown skin. CONCLUSIONS: New spirometric equations were generated for Brazilian children 3-12 years of age, in the three skin-color categories defined. The equations currently in use in Brazil seem to underestimate the lung function of Brazilian children 3-12 years of age and should be replaced by the equations proposed in this study.

Assessment of asthma control among different measures and evaluation of functional exercise capacity in children and adolescents with asthma. / Avaliação do controle da asma entre diferentes medidas e avaliação da capacidade de exercício funcional em crianças e adolescentes com asma.

Objective To assess the agreement among asthma control measures and functional exercise capacity in children and adolescents with uncontrolled and controlled asthma. Methods Children and adolescents with asthma from 7-17 years old were selected, and they were attended in the "Pediatric Pulmonology Outpatient Clinic of State University of Campinas", in Brazil. All patients had asthma control level assessed by Global Initiative for Asthma questionnaire (GINAq), Asthma Control Test (ACT), spirometry and six-minute-walk-test (6MWT). Patients were classified as uncontrolled or controlled asthma in each test and agreement among measures was assessed by kappa statistics. The ROC curve was calculated for the 6MWT. The spirometric index obtained from spirometry was composed by FEV1, FEV1/FVC and FEF25-75%. Spirometry and 6MWT results were compared between uncontrolled and controlled asthma group by GINAq. Results Of the 138 subjects included, 78 (56.5%) were male with median age of 11 (7-17) years old. GINAq detected 68.8% of patients with uncontrolled asthma. Moderate agreement (p < 0.001; k = 0.56) and high specificity (100%) was observed between GINAq and ACT. In 6MWT, the cut-off point of 82.03% of predicted distance was able to distinguish patients with controlled and uncontrolled asthma. Spirometric index presented 73.4% of sensitivity according to GINAq. The results for 6MWT in patients with uncontrolled asthma were the worst of all. Conclusion This study highlights the importance of assessing more than one measure to differentiate asthma control level. GINAq identified more patients with uncontrolled asthma and presented moderate agreement with ACT. Spirometric index was associated with uncontrolled asthma according to GINAq. 6MWT was a suitable measure to distinguish patients with controlled and uncontrolled asthma.

Respiratory physical therapy techniques recommended for patients with cystic fibrosis treated in specialized centers.

BACKGROUND: Respiratory therapy is a part of the treatment of patients with cystic fibrosis (CF). However, there is no information about the main factors influencing the recommendation of the use of different techniques or devices by physical therapists from different specialized centers. OBJECTIVE: To determine the respiratory therapy techniques recommended for the treatment of patients with CF seen in specialized treatment centers in Brazil. METHODS: This is a descriptive study including a sample of Brazilian CF specialized treatment centers. Data on demographics, spirometric variables, and recommended respiratory therapy treatment techniques were collected. RESULTS: Twelve specialized treatment centers with a total of 974 patients were included (13.5±11.3 years old and FEV1 (%) 75.7±27.9). The most recommended techniques were huffing (61.1% of patients), high frequency oral oscillation (HFOO) (52.0%), and positive expiratory pressure (PEP) (45.3%). Most often, recommendation was to perform treatment once (54.8% of patients) or twice (34%) a day. There was great variability in the recommendation among the different states. When data were separated by age, there was a predominance of performing conventional and manual techniques in infants and preschool children. There were no significant variations according to pulmonary function. Based on the literature, techniques based on volume, huffing, and PEP were most prevalently performed in international centers. CONCLUSION: The most recommended treatment techniques for patients with CF in Brazil are huffing, HFOO, and PEP, followed by volume-based techniques. There were geographical variations in the preferred treatment techniques, as well as based on patient age, but not based on the level of pulmonary function.

Reference values for spirometry in Brazilian children / Valores de referência de espirometria para crianças brasileiras

ABSTRACT Objective: To generate reference values for spirometry in Brazilian children 3-12 years of age and to compare those values with the values employed in the equations currently in use in Brazil. Methods: This study involved healthy children, 3-12 years of age, recruited from 14 centers (primary data) and spirometry results from children with the same characteristics in six databases (secondary data). Reference equations by quantile regressions were generated after log transformation of the spirometric and anthropometric data. Skin color was classified as self-reported by the participants. To determine the suitability of the results obtained, they were compared with those predicted by the equations currently in use in Brazil. Results: We included 1,990 individuals from a total of 21 primary and secondary data sources. Of those, 1,059 (53%) were female. Equations for FEV1, FVC, the FEV1/FVC ratio, FEF between 25% and 75% of the FVC (FEF25-75%) and the FEF25-75%/FVC ratio were generated for white-, black-, and brown-skinned children. The logarithms for height and age, together with skin color, were the best predictors of FEV1 and FVC. The reference values obtained were significantly higher than those employed in the equations currently in use in Brazil, for predicted values, as well as for the lower limit of normality, particularly in children with self-reported black or brown skin. Conclusions: New spirometric equations were generated for Brazilian children 3-12 years of age, in the three skin-color categories defined. The equations currently in use in Brazil seem to underestimate the lung function of Brazilian children 3-12 years of age and should be replaced by the equations proposed in this study.

RESUMO Objetivo: Gerar valores de referência para espirometria em crianças brasileiras de 3-12 anos de idade e comparar os resultados obtidos com as equações em uso no Brasil. Métodos: Foram incluídas crianças sadias de 3-12 anos recrutadas em 14 centros (dados primários) e resultados de espirometria de crianças com as mesmas características de seis bancos de dados (dados secundários). As equações quantílicas foram geradas após transformações logarítmicas dos dados espirométricos e antropométricos. A classificação por cor da pele foi autodeclarada. Os resultados obtidos foram comparados com os previstos nas equações em uso no Brasil para testar sua adequação. Resultados: Foram incluídos 1.990 indivíduos de 21 fontes de dados primários e secundários, sendo 1.059 (53%) do sexo feminino. Equações para VEF1, CVF, VEF1/CVF, FEF25-75% e FEF25-75%/CVF foram geradas para crianças brancas e para crianças negras e pardas. Os logaritmos da estatura e da idade e a cor da pele foram os melhores preditores para VEF1 e CVF. Os resultados obtidos foram significativamente maiores do que as estimativas geradas pelas equações em uso no Brasil, tanto para valores previstos quanto para o limite inferior da normalidade, particularmente em crianças negras e pardas. Conclusões: Novas equações espirométricas foram geradas para crianças brasileiras de 3-12 anos de cor branca, negra e parda. As equações atualmente em uso no Brasil parecem subestimar a função pulmonar de crianças brasileiras menores de 12 anos de idade e deveriam ser substituídas pelas equações propostas neste estudo.

Avaliação do controle da asma entre diferentes medidas e avaliação da capacidade de exercício funcional em crianças e adolescentes com asma / Assessment of asthma control among different measures and evaluation of functional exercise capacity in children and adolescents with asthma

RESUMO Objetivo Avaliar a concordância entre as medidas de controle da asma e a capacidade de exercício funcional em crianças e adolescentes com asma não controlada e controlada. Métodos Selecionamos crianças e adolescentes com asma de 7 a 17 anos, que foram atendidos no Ambulatório de Pneumologia Pediátrica da Universidade Estadual de Campinas. Todos os pacientes tiveram nível de controle da asma avaliado pelo questionário da Global Initiative for Asthma (GINAq), Teste de Controle da Asma (TCA), espirometria e teste de caminhada de seis minutos (TC6M). Os pacientes foram classificados como asma não controlada ou controlada em cada teste e a concordância entre as medidas foi avaliada pelas estatísticas do Kappa. A curva ROC foi calculada para o TC6M. O índice espirométrico da espirometria foi composto por FEV1, FEV1/FVC e FEF25-75%. Os resultados da espirometria e do TC6M foram comparados entre o grupo de asma não controlada e controlada pela GINAq. Resultados Dos 138 sujeitos incluídos, 78 (56,5%) eram do sexo masculino com idade média de 11,00 (7-17) anos. O GINAq detectou 68,8% dos pacientes com asma não controlada. Foi observada concordância moderada (p<0,001; k=0,56) e alta especificidade (100%) entre o GINAq e o TCA. No TC6M, o ponto de corte de 82,03% da distância prevista possibilitou a distinção de pacientes com asma controlada e não controlada. O índice espirométrico apresentou 73,4% de sensibilidade com o GINAq. Foram observados resultados piores no TC6M em pacientes com asma não controlada. Conclusões Este estudo destaca a importância de avaliar mais de uma medida para diferenciar o nível de controle da asma. O GINAq identificou mais pacientes com asma não controlada e apresentou concordância moderada com o TCA. O índice espirométrico foi associado à asma não controlada de acordo com o GINAq. O TC6M foi uma boa medida para distinguir pacientes com asma controlada e não controlada.

ABSTRACT Objective To assess the agreement among asthma control measures and functional exercise capacity in children and adolescents with uncontrolled and controlled asthma. Methods Children and adolescents with asthma from 7-17 years old were selected, and they were attended in the "Pediatric Pulmonology Outpatient Clinic of State University of Campinas", in Brazil. All patients had asthma control level assessed by Global Initiative for Asthma questionnaire (GINAq), Asthma Control Test (ACT), spirometry and six-minute-walk-test (6MWT). Patients were classified as uncontrolled or controlled asthma in each test and agreement among measures was assessed by kappa statistics. The ROC curve was calculated for the 6MWT. The spirometric index obtained from spirometry was composed by FEV1, FEV1/FVC and FEF25-75%. Spirometry and 6MWT results were compared between uncontrolled and controlled asthma group by GINAq. Results Of the 138 subjects included, 78 (56.5%) were male with median age of 11 (7-17) years old. GINAq detected 68.8% of patients with uncontrolled asthma. Moderate agreement (p < 0.001; k = 0.56) and high specificity (100%) was observed between GINAq and ACT. In 6MWT, the cut-off point of 82.03% of predicted distance was able to distinguish patients with controlled and uncontrolled asthma. Spirometric index presented 73.4% of sensitivity according to GINAq. The results for 6MWT in patients with uncontrolled asthma were the worst of all. Conclusion This study highlights the importance of assessing more than one measure to differentiate asthma control level. GINAq identified more patients with uncontrolled asthma and presented moderate agreement with ACT. Spirometric index was associated with uncontrolled asthma according to GINAq. 6MWT was a suitable measure to distinguish patients with controlled and uncontrolled asthma.

Protocolo de atendimento e seguimento fisioterapêutico na fibrose cí­stica / Protocol for physical therapy follow-up and monitoring in cystic fibrosis patients

A Fibrose Cí­stica (FC) requer uma abordagem multidisciplinar culminando em um complexo regime terapêutico. A fisioterapia tem papel fundamental na depuração das vias aéreas, melhora da ventilação pulmonar e da tolerância ao exercí­cio. No entanto, aspectos como acompanhamento da evolução clí­nica do paciente, identificação de perí­odos de exacerbação pulmonar e avaliação da adesão ao tratamento também fazem parte da rotina fisioterapêutica. Com o objetivo de contemplar os principais instrumentos disponí­veis que auxiliam em um melhor controle, acompanhamento e avaliação de pacientes com FC, elaborou-se o PASFIC - Protocolo de Atendimento e Seguimento Fisioterapêutico na Fibrose Cí­stica. Este protocolo inclui 10 critérios: 1) fisioterapia respiratória, 2) escore de gravidade, 3) escores de exacerbação pulmonar, 4) avaliação do peso e da estatura, 5) avaliação respiratória, 6) espirometria, 7) coleta de secreção das vias respiratórias, 8) aderência, 9) habilidades funcionais, 10) anormalidades posturais. A finalidade do PASFIC é caracterizar a população, identificar as alterações clí­nicas e acompanhar a evolução clí­nica e o atendimento fisioterapêutico de pacientes com FC, viabilizando suporte assistencial individualizado, uma melhor adesão ao tratamento e intervenção precoce durante as agudizações pulmonares, facilitando a monitorização pela equipe multiprofissional, otimizando o atendimento de pacientes com FC. (AU)

Cystic Fibrosis (CF) requires multidisciplinary approach culminating in a complex treatment regimen. Physical therapy is an important factor for better management of the disease, playing a key role in airway clearance, lung ventilation and exercise tolerance improvement. However, aspects such as monitoring of patients clinical evolution, identification of periods of acute pulmonary exacerbations and assessment of treatment adherence are also part of the physical therapy routine. In order to cover all instruments that help improve control, monitoring and evaluation of CF patients, a systematic protocol has been developed to qualify and quantify CF patients` interventions data. It includes 10 criteria: 1) Monitoring of physiotherapy techniques, 2) Application of severity score, 3) Application of pulmonary exacerbation scores, 4) Weight and height measurements, 5) Evaluation of respiratory system, 6) Systematic spirometry, 7) Airway secretion collection and register, 8) Monitoring of adherence to treatment, 9) Assessment of functional abilities, and 10) Postural abnormalities. The aim is to characterize the CF population, to identify clinical alterations and to follow up on the disease evolution and physiotherapy assistance for CF patients, as well as to allow individualized care and support, better treatment adherence, and early intervention for pulmonary exacerbations. (AU)

Teste de caminhada de seis minutos em pediatria: discutindo evidências em situações específicas / Six minutes walk test in pediatrics: discussing evidence in specific situations

Teste de caminhada de seis minutos (TC6) tem se mostrado uma ferramenta bem tolerada, confiá- vel e de baixo custo para monitorar a capacidade funcional de crianças e adolescentes saudáveis e em diferentes situações clínicas. Objetivo: Verificar e discutir as evidências científicas do TC6 utilizado em 4 situações específicas da criança com: 1) asma; 2) fibrose cística 3) obesidade e 4) higidez. Método: A busca nas bases de dados foi conduzida utilizando-se as palavras-chaves: teste de caminhada de seis minutos, crianças, adolescentes, obesos, fibrose cística e asma. Consultou-se as bases Pubmed (Medline), Lilacs e PEDro. Foram considerados os ensaios clínicos em português, inglês e espanhol, publicados no período de 2005 a 2016 e incluídos os estudos que abordam o TC6como método de avaliação, monitorização e prognóstico de crianças e adolescentes saudáveis, com diagnósticos de asma, fibrose cística e obesidade. Resultados: Identificou-se 97 artigos, sendo 48 duplicados. Conduziu-se a pré-seleção de 43 estudos dos quais 6foram excluídos, pelo título ou resumo, por não atenderem aos critérios de inclusão. A seleção final totalizou 39 manuscritos para a apreciação na íntegra e discussão na presente revisão. Conclusão: TC6 é reprodutível e validado para a população pediátrica, sendo considerado um instrumento importante para avaliar as implicações das doenças crônicas na capacidade funcional. 1) TC6 tem se mostrado útil pra identificação do prejuízo das atividades de vida diária durante a crise de asma e fora dela, assim como do comprometimento da capacidade funcional diante do hábito de vida sedentário. 2) É adequado para avaliação de programas de reeducação alimentar na obesidade. 3) Na fibrose cística é uma boa ferramenta para avaliação de programas de reabilitação pulmonar e acompanhamento da progressão da doença. 4) Entre os saudáveis observa-se a busca por valores de referência e falta de um consenso sobre a forma de aplicabilidade do teste

The six-minute walk test (6MWT) is regarded as a well-tolerated, reliable and cost-effective tool to monitor the functional capacity of healthy children and adolescents and in different clinical situations. Objective: To verify and discuss the scientific evidence of the 6MWT in four specific situations of children with: 1) asthma; 2) cystic fibrosis 3) obesity and 4) healthy controls. Method: A search was conducted in Pubmed (Medline), Lilacs and PEDro databases with the key words: sixminute walk test, children, adolescents, obesity, cystic fibrosis and asthma. The selected articles were those that reported clinical trials in Portuguese, English or Spanish, published between 2005- 2016 that included studies that assessed the 6MWT as a method of evaluation for monitoring and prognosis of healthy or diseased children and adolescents. Results: We identified 97 articles, 48 of them were duplicate. A pre-selection of 43 remaining studies based on title or summary excluded 6 articles for not meeting the inclusion criteria. Finally, 39 manuscripts were included in this review. Conclusion: 6MWT is reproducible and validated for the pediatric population and is considered an important instrument to assess the implications of chronic diseases on functional status. 1) 6MWT has proven useful to identify function losses to the activities of daily living during or in-between asthma attacks, as well as the functional jeopardy of sedentary habit; 2) It is suitable for assessing nutritional education programs in obesity; 3) In cystic fibrosis, it is a good tool for evaluating pulmonary rehabilitation programs and monitoring of the disease progression; and 4) Among healthy subjects, however, there is still lack of consensus of a reference as well as the most suitable applicability of this test

Sweat test and cystic fibrosis: overview of test performance at public and private centers in the state of São Paulo, Brazil.

OBJECTIVE:: The sweat test (ST) measures chloride levels in sweat and is considered the gold standard for the diagnosis of cystic fibrosis (CF). However, the reliability of a ST depends on their being performed by experienced technicians and in accordance with strict guidelines. Our aim was to evaluate how sweat stimulation, sweat collection, and chloride measurement are performed at 14 centers (9 public centers and 5 private centers) that routinely perform STs in the state of São Paulo, which has the highest frequency of CF in Brazil. METHODS:: This was a cross-sectional cohort study, using a standardized questionnaire administered in loco to the staff responsible for conducting STs. RESULTS:: No uniformity regarding the procedures was found among the centers. Most centers were noncompliant with the international guidelines, especially regarding the collection of sweat (the samples were insufficient in 10-50% of the subjects tested); availability of stimulation equipment (which was limited at 2 centers); modernity and certification of stimulation equipment (most of the equipment having been used for 3-23 years); and written protocols (which were lacking at 12 centers). Knowledge of ST guidelines was evaluated at only 1 center. CONCLUSIONS:: Our results show that STs largely deviate from internationally accepted guidelines at the participating centers. Therefore, there is an urgent need for standardization of STs, training of qualified personnel, and acquisition/certification of suitable equipment. These are essential conditions for a reliable diagnosis of CF, especially with the increasing demand due to newborn screening nationwide, and for the assessment of a possible clinical benefit from the use of modulator drugs. OBJETIVO:: O teste do suor (TS) mede os níveis de cloro no suor e é considerado o padrão ouro para o diagnóstico da fibrose cística (FC). Contudo, a confiabilidade do TS depende de sua realização por técnicos experientes e segundo diretrizes rígidas. Nosso objetivo foi avaliar como são realizadas a estimulação e coleta do suor e a dosagem de cloro em 14 centros (9 públicos e 5 privados) que realizam TS rotineiramente no estado de São Paulo, que possui a maior frequência de FC do Brasil. MÉTODOS:: Estudo de coorte transversal utilizando um questionário padronizado aplicado in loco ao pessoal responsável pela realização dos TS. RESULTADOS:: Não houve uniformidade entre os centros quanto aos procedimentos. A maioria dos centros não era aderente às diretrizes internacionais, especialmente quanto à coleta do suor (amostras insuficientes em 10-50% dos indivíduos testados), disponibilidade de equipamentos de estimulação (limitada em 2 centros), modernidade e certificação dos mesmos (a maioria utilizada há 3-23 anos) e protocolos escritos (ausentes em 12 centros). Avaliou-se o conhecimento sobre diretrizes para TS em apenas 1 centro. CONCLUSÕES:: Nossos resultados mostram que, nos centros participantes, os TS estão muito distantes das diretrizes internacionalmente aceitas. Portanto, há necessidade urgente de padronização dos TS, de treinamento de pessoal qualificado e de aquisição/certificação de equipamentos adequados. Essas são condições essenciais para um diagnóstico confiável de FC, especialmente com a crescente demanda resultante da triagem neonatal em todo o país, e para a avaliação do possível benefício clínico do uso de moduladores.

Sweat test and cystic fibrosis: overview of test performance at public and private centers in the state of São Paulo, Brazil / Teste do suor e fibrose cística: panorama da realização do teste em centros públicos e privados do estado de São Paulo

ABSTRACT Objective: The sweat test (ST) measures chloride levels in sweat and is considered the gold standard for the diagnosis of cystic fibrosis (CF). However, the reliability of a ST depends on their being performed by experienced technicians and in accordance with strict guidelines. Our aim was to evaluate how sweat stimulation, sweat collection, and chloride measurement are performed at 14 centers (9 public centers and 5 private centers) that routinely perform STs in the state of São Paulo, which has the highest frequency of CF in Brazil. Methods: This was a cross-sectional cohort study, using a standardized questionnaire administered in loco to the staff responsible for conducting STs. Results: No uniformity regarding the procedures was found among the centers. Most centers were noncompliant with the international guidelines, especially regarding the collection of sweat (the samples were insufficient in 10-50% of the subjects tested); availability of stimulation equipment (which was limited at 2 centers); modernity and certification of stimulation equipment (most of the equipment having been used for 3-23 years); and written protocols (which were lacking at 12 centers). Knowledge of ST guidelines was evaluated at only 1 center. Conclusions: Our results show that STs largely deviate from internationally accepted guidelines at the participating centers. Therefore, there is an urgent need for standardization of STs, training of qualified personnel, and acquisition/certification of suitable equipment. These are essential conditions for a reliable diagnosis of CF, especially with the increasing demand due to newborn screening nationwide, and for the assessment of a possible clinical benefit from the use of modulator drugs.

RESUMO Objetivo: O teste do suor (TS) mede os níveis de cloro no suor e é considerado o padrão ouro para o diagnóstico da fibrose cística (FC). Contudo, a confiabilidade do TS depende de sua realização por técnicos experientes e segundo diretrizes rígidas. Nosso objetivo foi avaliar como são realizadas a estimulação e coleta do suor e a dosagem de cloro em 14 centros (9 públicos e 5 privados) que realizam TS rotineiramente no estado de São Paulo, que possui a maior frequência de FC do Brasil. Métodos: Estudo de coorte transversal utilizando um questionário padronizado aplicado in loco ao pessoal responsável pela realização dos TS. Resultados: Não houve uniformidade entre os centros quanto aos procedimentos. A maioria dos centros não era aderente às diretrizes internacionais, especialmente quanto à coleta do suor (amostras insuficientes em 10-50% dos indivíduos testados), disponibilidade de equipamentos de estimulação (limitada em 2 centros), modernidade e certificação dos mesmos (a maioria utilizada há 3-23 anos) e protocolos escritos (ausentes em 12 centros). Avaliou-se o conhecimento sobre diretrizes para TS em apenas 1 centro. Conclusões: Nossos resultados mostram que, nos centros participantes, os TS estão muito distantes das diretrizes internacionalmente aceitas. Portanto, há necessidade urgente de padronização dos TS, de treinamento de pessoal qualificado e de aquisição/certificação de equipamentos adequados. Essas são condições essenciais para um diagnóstico confiável de FC, especialmente com a crescente demanda resultante da triagem neonatal em todo o país, e para a avaliação do possível benefício clínico do uso de moduladores.

Quality of sweat test (ST) based on the proportion of sweat sodium (Na) and sweat chloride (Cl) as diagnostic parameter of cystic fibrosis: are we on the right way?

BACKGROUND: To assess the quality of sweat test (ST) based on the proportion of sweat sodium and sweat chloride as diagnostic parameter of cystic fibrosis (CF). METHODS: A retrospective study of 5,721 sweat samples and subsequent descriptive analysis were carried out. The test was considered "of good quality" (correct) when: (i) sweat chloride was lower than 60 mEq/L, and sweat sodium was higher than sweat chloride; (ii) sweat chloride was higher than 60 mEq/L, and sweat sodium was lower than sweat chloride. RESULTS: The study included 5,692/5,721 sweat samples of ST which had been requested due to clinical presentations compatible with CF and/or neonatal screenings with altered immunoreactive trypsinogen values. Considering the proportion of sweat sodium and sweat chloride as ST quality parameter, the test was performed correctly in 5,023/5,692 (88.2 %) sweat samples. The sweat chloride test results were grouped into four reference ranges for chloride (i) chloride < 30 mEq/L: 3,651/5,692 (64.1 %); (ii) chloride ≥ 30 mEq/L to < 40 mEq/L: 652/5,692 (11.5 %); (iii) ≥ 40 mEq/L to < 60 mEq/L: 673/5,692 (11.8 %); (iv) ≥ 60 mEq/L: 716/5,692 (12.6 %). In the comparative analysis, there was no association between ST quality and: (i) symptoms to indicate a ST [respiratory (p = 0.084), digestive (p = 0.753), nutritional (p = 0.824), and others (p = 0.136)], (ii) sweat weight (p = 0.416). However, there was a positive association with: (i) gender, (ii) results of ST (p < 0.001), (iii) chloride/sodium ratio (p < 0.001), (iv) subject's age at the time of ST [grouped according to category (p < 0.001) and numerical order (p < 0.001)]. For the subset of 169 patients with CF and two CFTR mutations Class I, II and/or III, in comparative analysis, there was a positive association with: (i) sweat chloride/sodium ratio (p < 0.001), (ii) sweat chloride values (p = 0.047), (iii) subject's age at the time of the ST grouped by numerical order (p = 0.001). CONCLUSIONS: Considering that the quality of ST can be assessed by levels of sweat sodium and sweat chloride, an increasing number of low-quality tests could be observed in our sweat samples. The quality of the test was associated with important factors, such as gender, CF diagnosis, and subjects' age.

Association between clinical variables related to asthma in schoolchildren born with very low birth weight with and without bronchopulmonary dysplasia / Associação entre variáveis clínicas relacionadas à asma em escolares nascidos com muito baixo peso com e sem displasia broncopulmonar

OBJECTIVE: To assess the prevalence, spirometry findings and risk factors for asthma in schoolchildren who were very low birth weight infants with and without bronchopulmonary dysplasia. METHODS: Observational and cross-sectional study. The parents and/or tutors answered the International Study of Asthma and Allergies in Childhood questionnaire. The schoolchildren were submitted to the skin prick test and spirometry assessment. RESULTS: 54 schoolchildren who were very low birth weight infants were assessed and 43 met the criteria for spirometry. Age at the assessment (bronchopulmonary dysplasia=9.5±0.85; without bronchopulmonary dysplasia=10.1±0.86 years) and birth weight (bronchopulmonary dysplasia=916.7±251.2; without bronchopulmonary dysplasia=1171.3±190.5g) were lower in the group with bronchopulmonary dysplasia (p<0.05). The prevalence of asthma among very low birth weight infants was 17/54 (31.5%), being 6/18 (33.3%) in the group with bronchopulmonary dysplasia. There was an association between wool blanket use in the first year of life (p=0.026) with the presence of asthma at school age. The skin prick test was positive in 13/17 (76.5%) and 23/37 (62.2%) of patients with and without asthma, respectively. The schoolchildren with asthma had lower z-score values of forced expiratory flow between 25% and 75% of forced vital capacity (n=16;−1.04±1.19) when compared to the group of patients without asthma (n=27;−0.380.93) (p=0.049). There was no difference between the spirometry variables in the groups regarding the presence or absence of bronchopulmonary dysplasia. CONCLUSIONS: Very low birth weight infants with and without bronchopulmonary dysplasia showed a high prevalence of asthma (33.3% and 30.6%, respectively). Pulmonary flow in the small airways was lower in children with asthma.

OBJETIVO: Avaliar prevalência, espirometria e fatores de risco para asma em escolares que foram recém-nascidos de muito baixo peso com e sem displasia broncopulmonar. MÉTODOS: Estudo observacional e transversal. Aplicou-se aos pais e/ou responsáveis o questionário International Study of Asthma and Allergies in Childhood. Foi feito teste cutâneo de hipersensibilidade imediata e espirometria nos escolares. RESULTADOS: Avaliados 54 escolares que foram recém-nascidos de muito baixo peso e 43 preencheram critérios para espirometria. A idade na avaliação (displasia broncopulmonar=9,5±0,85; sem displasia broncopulmonar=10,1±0,86 anos) e o peso de nascimento (displasia broncopulmonar=916,7±251,2; sem displasia broncopulmonar=1.171,3±190,5 g) foram menores no grupo com displasia broncopulmonar (p<0,05). A prevalência de asma entre os recém-nascidos de muito baixo peso foi de 17/54 (31,5%); no grupo com displasia broncopulmonar, de 6/18 (33,3%). Houve associação entre uso de cobertor de lã no primeiro ano de vida (p=0,026) com presença de asma na idade escolar. O teste cutâneo de hipersensibilidade imediata foi positivo em 13/17 (76,5%) e 23/37 (62,2%) nos grupos com e sem asma, respectivamente. Os escolares com asma apresentaram menores valores em z-score do fluxo expiratório forçado entre 25% e 75% da capacidade vital forçada (n=16; −1,04±1,19) comparados com os do grupo de pacientes sem asma (n=27; −0,38±0,93) (p=0,049). Não houve diferença entre as variáveis da espirometria no grupo com relação à presença ou não de displasia broncopulmonar. CONCLUSÕES: Os recém-nascidos de muito baixo peso, com e sem displasia broncopulmonar, apresentaram prevalência elevada de asma (33,3% e 30,6%, respectivamente). Os fluxos pulmonares das pequenas vias aéreas foram menores nos escolares com asma.

Association between clinical variables related to asthma in schoolchildren born with very low birth weight with and without bronchopulmonary dysplasia. / Associação entre variáveis clínicas relacionadas à asma em escolares nascidos com muito baixo peso com e sem displasia broncopulmonar.

OBJECTIVE: to assess the prevalence, spirometry findings and risk factors for asthma in schoolchildren who were very low birth weight infants with and without bronchopulmonary dysplasia. METHODS: Observational and cross-sectional study. The parents and/or tutors answered the International Study of Asthma and Allergies in Childhood questionnaire. The schoolchildren were submitted to the skin prick test and spirometry assessment. RESULTS: 54 schoolchildren who were very low birth weight infants were assessed and 43 met the criteria for spirometry. Age at the assessment (bronchopulmonary dysplasia=9.5±0.85; without bronchopulmonary dysplasia=10.1±0.86 years) and birth weight (bronchopulmonary dysplasia=916.7±251.2; without bronchopulmonary dysplasia=1,171.3±190.5g) were lower in the group with bronchopulmonary dysplasia (p<0.05). The prevalence of asthma among very low birth weight infants was 17/54 (31.5%), being 6/18 (33.3%) in the group with bronchopulmonary dysplasia. There was an association between wool blanket use in the first year of life (p=0.026) with the presence of asthma at school age. The skin prick test was positive in 13/17 (76.5%) and 23/37 (62.2%) of patients with and without asthma, respectively. The schoolchildren with asthma had lower z-score values of forced expiratory flow between 25% and 75% of forced vital capacity (n=16; -1.04±1.19) when compared to the group of patients without asthma (n=27; -0.38±0.93) (p=0.049). There was no difference between the spirometry variables in the groups regarding the presence or absence of bronchopulmonary dysplasia. CONCLUSIONS: Very low birth weight infants with and without bronchopulmonary dysplasia showed a high prevalence of asthma (33.3% and 30.6%, respectively). Pulmonary flow in the small airways was lower in children with asthma.

Evaluation of quality of life according to asthma control and asthma severity in children and adolescents / Avaliação da qualidade de vida de acordo com o nível de controle e gravidade da asma em crianças e adolescentes

ABSTRACT OBJECTIVE: To evaluate quality of life according to the level of asthma control and degree of asthma severity in children and adolescents. METHODS: We selected children and adolescents with asthma (7-17 years of age) from the Pediatric Pulmonology Outpatient Clinic of the State University of Campinas Hospital de Clínicas, located in the city of Campinas, Brazil. Asthma control and asthma severity were assessed by the Asthma Control Test and by the questionnaire based on the Global Initiative for Asthma, respectively. The patients also completed the Paediatric Asthma Quality of Life Questionnaire (PAQLQ), validated for use in Brazil, in order to evaluate their quality of life. RESULTS: The mean age of the patients was 11.22 ± 2.91 years, with a median of 11.20 (7.00-17.60) years. We selected 100 patients, of whom 27, 33, and 40 were classified as having controlled asthma (CA), partially controlled asthma (PCA), and uncontrolled asthma (UA), respectively. As for asthma severity, 34, 19, and 47 were classified as having mild asthma (MiA), moderate asthma (MoA), and severe asthma (SA), respectively. The CA and the PCA groups, when compared with the NCA group, showed higher values for the overall PAQLQ score and all PAQLQ domains (activity limitation, symptoms, and emotional function; p < 0.001 for all). The MiA group showed higher scores for all of the PAQLQ components than did the MoA and SA groups. CONCLUSIONS: Quality of life appears to be directly related to asthma control and asthma severity in children and adolescents, being better when asthma is well controlled and asthma severity is lower.

RESUMO OBJETIVO: Avaliar a qualidade de vida de acordo com o nível de controle e gravidade da asma em crianças e adolescentes. MÉTODOS: Foram selecionados crianças e adolescentes com asma (7-17 anos de idade) acompanhados no Ambulatório de Pneumologia Pediátrica do Hospital de Clínicas da Universidade Estadual de Campinas, localizado na cidade de Campinas (SP). O nível de controle e a gravidade da asma foram avaliados pelo Teste de Controle da Asma e pelo questionário baseado na Global Initiative for Asthma, respectivamente. Os pacientes responderam o Paediatric Asthma Quality of Life Questionnaire (PAQLQ), validado para uso no Brasil, para a avaliação de sua qualidade de vida. RESULTADOS: A média de idade dos asmáticos foi de 11,22 ± 2,91 anos, com mediana de 11,20 (7,00-17,60) anos. Foram selecionados 100 pacientes, dos quais 27, 33 e 40, respectivamente, foram classificados com asma controlada (AC), asma parcialmente controlada (APC) e asma não controlada (ANC). Quanto à gravidade da asma, 34, 19 e 47 foram classificados, respectivamente, com asma leve (AL), asma moderada (AM) e asma grave (AG). Os grupos AC e APC, quando comparados ao grupo ANC, apresentaram maiores valores no escore geral do PAQLQ e em seus domínios (limitação de atividades, sintomas e função emocional; p < 0,001 para todos). O grupo AL apresentou os maiores escores em todos os componentes do PAQLQ quando comparado aos grupos AM e AG. CONCLUSÕES: A qualidade de vida parece estar diretamente relacionada com o nível de controle e a gravidade da asma, uma vez que as crianças e adolescentes com maior controle e menor gravidade da doença apresentaram melhor qualidade de vida.

Ventilatory Efficiency in Children and Adolescents: A Systematic Review.

INTRODUCTION: The index of ventilatory efficiency (VE/VCO2) obtained by the progressive exercise test has been considered the gold standard in the prognosis of adults with heart failure, but few studies have evaluated this approach in children. OBJECTIVE: To verify the scientific evidence about the VE/VCO2 in pediatric and adolescents patients. METHODS: A systematic literature review was carried out using the key words VE/VCO2, children, and adolescents using the PEDro and PubMed/MedLine databases. Clinical trials published from 1987 to 2014, including children, adolescents, and young adults up to 25 years, addressing the VE/VCO2 index as a method of evaluation, monitoring, and prognosis were considered. RESULTS: Initially, 95 articles were found; 12 were excluded as the title/abstract did not contain the VE/VCO2 index or because they included patients greater than 25 years of age. From the remaining 83, 58 were repeated between the databases. The final sample consisted of 32 studies including healthy children and children with respiratory and other diseases. CONCLUSION: There are few studies involving cardiorespiratory assessment by ventilatory efficiency. The studies highlight the fact that high VE/VCO2 values are associated with a worse prognosis of patients due to the relationship with the decrease in pulmonary perfusion and cardiac output.

Oscilação oral de alta frequência e fibrose cística: comparação entre instrumentais / High-frequency oral oscillation and cystic fibrosis: a comparison between instruments

Objetivos: Avaliar e comparar o efeito do Flutter VRP1® e do Shaker® referente à quantidade de secreção pulmonar expectorada e aos parâmetros cardiorrespiratórios de frequência cardíaca (fc) e de saturação periférica de oxigênio (SpO2). Método: Neste estudo randomizado do tipo intervencional, 16 pacientes, com idades entre 7 e 21 anos (12 anos ±4,11), realizaram sessões de fisioterapia em dois dias diferentes com ambos os dispositivos. Na primeira sessão, os participantes utilizaram um dos dispositivos, conforme o resultado da randomização. E, após sete dias sem intervenção, na segunda sessão, usaram o outro dispositivo. Verificaram-se os parâmetros cardiorrespiratórios de fc e SpO2 e registraram-se tais valores antes e ao final de cada coleta em cada dia de terapia. Colheu-se secreção pulmonar durante e após a terapia para determinação dos pesos seco e úmido. Resultados: Não houve diferença entre os dispositivos quanto a peso úmido (p=0,589) e seco (p=0,719) e parâmetros cardiorrespiratórios de fc e SpO2. Conclusão: Os dispositivos foram semelhantes em relação às variáveis analisadas

Objectives: To evaluate and compare the effect of Flutter VRP1® and Shaker® on the amount of expectorated pulmonary secretions and on heart rate (HR) and peripheral oxygen saturation (SO2) cardiorespiratory parameters. Method: In this randomized interventional study, 16 patients, aged between 7 and 21 years (12 years±4.11), performed physiotherapy sessions on two different days with both devices. In the first session, participants used one of the devices, according to the randomization. After seven days without intervention, they underwent a second session with the other device. The cardiorespiratory parameters of HR and SO2 were recorded immediately before and after each session on every day of therapy. The collection of pulmonary secretions was conducted during and after therapy to determine the dry and wet weights. Results: There was no difference between the devices as to the wet weight (p=0.589) and dry weight (p=0.719) nor HR and SO2 cardiorespiratory parameters. Conclusion: The devices were similar in relation to the analyzed variables.

Ventilatory abnormalities in patients with cystic fibrosis undergoing the submaximal treadmill exercise test.

BACKGROUND: Exercise has been studied as a prognostic marker for patients with cystic fibrosis (CF), as well as a tool for improving their quality of life and analyzing lung disease. In this context, the aim of the present study was to evaluate and compare variables of lung functioning. Our data included: (i) volumetric capnography (VCAP) parameters: expiratory minute volume (VE), volume of exhaled carbon dioxide (VCO2), VE/VCO2, ratio of dead space to tidal volume (VD/VT), and end-tidal carbon dioxide (PetCO2); (ii) spirometry parameters: forced vital capacity (FVC), percent forced expiratory volume in the first second of the FVC (FEV1%), and FEV1/FVC%; and (iii) cardiorespiratory parameters: heart rate (HR), respiratory rate, oxygen saturation (SpO2), and Borg scale rating at rest and during exercise. The subjects comprised children, adolescents, and young adults aged 6-25 years with CF (CF group [CFG]) and without CF (control group [CG]). METHODS: This was a clinical, prospective, controlled study involving 128 male and female patients (64 with CF) of a university hospital. All patients underwent treadmill exercise tests and provided informed consent after study approval by the institutional ethics committee. Linear regression, Kruskal-Wallis test, and Mann-Whitney test were performed to compare the CFG and CG. The α value was set at 0.05. RESULTS: Patients in the CFG showed significantly different VCAP values and spirometry variables throughout the exercise test. Before, during, and after exercise, several variables were different between the two groups; statistically significant differences were seen in the spirometry parameters, SpO2, HR, VCO2, VE/VCO2, PetCO2, and Borg scale rating. VCAP variables changed at each time point analyzed during the exercise test in both groups. CONCLUSION: VCAP can be used to analyze ventilatory parameters during exercise. All cardiorespiratory, spirometry, and VCAP variables differed between patients in the CFG and CG before, during, and after exercise.

Nasal potential difference in cystic fibrosis considering severe CFTR mutations.

The gold standard for diagnosing cystic fibrosis (CF) is a sweat chloride value above 60 mEq/L. However, this historical and important tool has limitations; other techniques should be studied, including the nasal potential difference (NPD) test. CFTR gene sequencing can identify CFTR mutations, but this method is time-consuming and too expensive to be used in all CF centers. The present study compared CF patients with two classes I-III CFTR mutations (10 patients) (G1), CF patients with classes IV-VI CFTR mutations (five patients) (G2), and 21 healthy subjects (G3). The CF patients and healthy subjects also underwent the NPD test. A statistical analysis was performed using the Mann-Whitney, Kruskal-Wallis, χ(2), and Fisher's exact tests, α = 0.05. No differences were observed between the CF patients and healthy controls for the PDMax, Δamiloride, and Δchloride + free + amiloride markers from the NPD test. For the finger value, a difference between G2 and G3 was described. The Wilschanski index values were different between G1 and G3. In conclusion, our data showed that NPD is useful for CF diagnosis when classes I-III CFTR mutations are screened. However, if classes IV-VI are considered, the NPD test showed an overlap in values with healthy subjects.

Evaluation of quality of life according to asthma control and asthma severity in children and adolescents.

OBJECTIVE: To evaluate quality of life according to the level of asthma control and degree of asthma severity in children and adolescents. METHODS: We selected children and adolescents with asthma (7-17 years of age) from the Pediatric Pulmonology Outpatient Clinic of the State University of Campinas Hospital de Clínicas, located in the city of Campinas, Brazil. Asthma control and asthma severity were assessed by the Asthma Control Test and by the questionnaire based on the Global Initiative for Asthma, respectively. The patients also completed the Paediatric Asthma Quality of Life Questionnaire (PAQLQ), validated for use in Brazil, in order to evaluate their quality of life. RESULTS: The mean age of the patients was 11.22 ± 2.91 years, with a median of 11.20 (7.00-17.60) years. We selected 100 patients, of whom 27, 33, and 40 were classified as having controlled asthma (CA), partially controlled asthma (PCA), and uncontrolled asthma (UA), respectively. As for asthma severity, 34, 19, and 47 were classified as having mild asthma (MiA), moderate asthma (MoA), and severe asthma (SA), respectively. The CA and the PCA groups, when compared with the NCA group, showed higher values for the overall PAQLQ score and all PAQLQ domains (activity limitation, symptoms, and emotional function; p < 0.001 for all). The MiA group showed higher scores for all of the PAQLQ components than did the MoA and SA groups. CONCLUSIONS: Quality of life appears to be directly related to asthma control and asthma severity in children and adolescents, being better when asthma is well controlled and asthma severity is lower.

Pulsed direct and constant direct currents in the pilocarpine iontophoresis sweat chloride test.

BACKGROUND: The classic sweat test (CST) is the golden standard for cystic fibrosis (CF) diagnosis. Then, our aim was compare the production and volume of sweat, and side effects caused by pulsed direct current (PDC) and constant direct current (CDC). To determine the optimal stimulation time (ST) for the sweat collection. To verify the PDC as CF diagnosis option. METHODS: Prospective study with cross-sectional experimental intervention. Experiment 1 (right arm): PDC and CDC. ST at 10 min and sweat collected at 30 min. Currents of 0.5; 0.75; 1.0 and 1.5 mA and frequencies of 0, 200, 1,000 and 5,000 Hz applied. Experiment 2 (left arm): current of 1.0 mA, ST at 5 and 10 min and sweat collected at 15 and 30 min with frequencies of 0; 200; 1,000 and 5,000 Hz applied Experiments 1 and 2 were performed with current density (CD) from 0.07 to 0.21 mA/cm2. Experiment 3: PDC was used in typical CF patients with two CFTR mutations screened and or with CF diagnosis by rectal biopsy and patients with atypical CF. RESULTS: 48 subjects (79.16% female) with average of 29.54 ± 8.87 years old were enrolled. There was no statistical difference between the interaction of frequency and current in the sweat weight (p = 0.7488). Individually, positive association was achieved between weight sweat and stimulation frequency (p = 0.0088); and current (p = 0.0025). The sweat production was higher for 10 min of stimulation (p = 0.0023). The sweat collection was better for 30 min (p = 0.0019). The skin impedance was not influenced by ST and sweat collection (p > 0.05). The current frequency was inversely associated with the skin impedance (p < 0.0001). The skin temperature measured before stimulation was higher than after (p < 0.0001). In Experiment 3 (29 subjects) the PDC showed better kappa index compared to CDC (0.9218 versus 0.5205, respectively). CONCLUSIONS: The performance of the CST with CDC and PDC with CD of 0.14 to 0.21 mA/cm2 showed efficacy in steps of stimulation and collection of sweat, without side effects. The optimal stimulation time and sweat collection were, respectively, 10 and 30 min.