Respiratory system parameters in children with low severity cystic fibrosis: is there early involvement in relation to healthy peers? / Parâmetros do sistema respiratório em crianças com fibrose cística de baixa gravidade: há acometimento precoce em relação a pares saudáveis?

ABSTRACT Objective: To compare and analyze pulmonary function and respiratory mechanics parameters between healthy children and children with cystic fibrosis. Methods: This cross-sectional analytical study included healthy children (HSG) and children with cystic fibrosis (CFG), aged 6-13 years, from teaching institutions and a reference center for cystic fibrosis in Florianópolis/SC, Brazil. The patients were paired by age and sex. Initially, an anthropometric evaluation was undertaken to pair the sample characteristics in both groups; the medical records of CFG were consulted for bacterial colonization, genotype, and disease severity (Schwachman-Doershuk Score — SDS) data. Spirometry and impulse oscillometry were used to assess pulmonary function. Results: In total, 110 children were included, 55 in each group. In the CFG group, 58.2% were classified as excellent by SDS, 49.1% showed the ΔF508 heterozygotic genotype, and 67.3% were colonized by some pathogens. Statistical analysis revealed significant differences between both groups (p<0.05) in most pulmonary function parameters and respiratory mechanics. Conclusions: Children with cystic fibrosis showed obstructive ventilatory disorders and compromised peripheral airways compared with healthy children. These findings reinforce the early changes in pulmonary function and mechanics associated with this disease.

RESUMO Objetivo: Comparar e analisar parâmetros de função pulmonar e de mecânica respiratória entre escolares saudáveis e com fibrose cística (FC). Métodos: Estudo transversal que incluiu escolares saudáveis (GES) e com FC (GFC), com idades entre seis e 13 anos, provenientes de instituições de ensino e de um centro de referência da FC em Florianópolis/SC, Brasil, pareados por idade e sexo, respectivamente. Inicialmente, conduziu-se avaliação antropométrica para pareamento e caracterização de ambos os grupos e, no GFC, consultou-se prontuário médico para registro dos dados de colonização bacteriana, genótipo e gravidade da doença (Escore de Schwachman-Doershuk — ESD). Para a avaliação da função pulmonar, realizou-se espirometria e a avaliação da mecânica respiratória foi conduzida por meio do sistema de oscilometria de impulso. Resultados: Participaram 110 escolares, 55 em cada grupo. No GFC, 58,2% foram classificados pelo ESD como excelentes, 49,1% apresentaram genótipo ∆F508 heterozigoto e 67,3% eram colonizados por alguma patógeno. Houve diferença significativa (p<0,05) na maioria dos parâmetros de função pulmonar e de mecânica respiratória entre os grupos. Conclusões: Escolares com FC apresentaram distúrbio ventilatório obstrutivo e com comprometimento de vias aéreas periféricas, em comparação aos escolares hígidos. Esse evento reforça o início precoce da alteração de função pulmonar e de mecânica respiratória nessa enfermidade, evidenciados pelos achados desta investigação.

Respiratory system parameters in children with low severity cystic fibrosis: is there early involvement in relation to healthy peers?

OBJECTIVE: To compare and analyze pulmonary function and respiratory mechanics parameters between healthy children and children with cystic fibrosis. METHODS: This cross-sectional analytical study included healthy children (HSG) and children with cystic fibrosis (CFG), aged 6-13 years, from teaching institutions and a reference center for cystic fibrosis in Florianópolis/SC, Brazil. The patients were paired by age and sex. Initially, an anthropometric evaluation was undertaken to pair the sample characteristics in both groups; the medical records of CFG were consulted for bacterial colonization, genotype, and disease severity (Schwachman-Doershuk Score - SDS) data. Spirometry and impulse oscillometry were used to assess pulmonary function. RESULTS: In total, 110 children were included, 55 in each group. In the CFG group, 58.2% were classified as excellent by SDS, 49.1% showed the ΔF508 heterozygotic genotype, and 67.3% were colonized by some pathogens. Statistical analysis revealed significant differences between both groups (p<0.05) in most pulmonary function parameters and respiratory mechanics. CONCLUSIONS: Children with cystic fibrosis showed obstructive ventilatory disorders and compromised peripheral airways compared with healthy children. These findings reinforce the early changes in pulmonary function and mechanics associated with this disease.

Telefisioterapia durante a COVID-19: Dificuldades e barreiras de familiares e indivíduos com fibrose cística / Telephysiotherapy during COVID-19: Difficulties and barriers individuals with cystic fibrosis and their family members

Descrever dificuldades e barreiras para adesão a telefisioterapia durante a pandemia da COVID-19. Relato de experiência, realizado com responsáveis e indivíduos com fibrose cística (FC) via internet, celular ou computador, para receberem atendimento de telefisioterapia assíncrona, em formato de cartilhas e vídeos. Todos deveriam responder avaliação inicial, questionário de qualidade de vida e, ao final, feedback sobre a assistência remota e a frequência na realização dos exercícios. Nove indivíduos participaram do estudo e poucos concluíram todas as etapas. Cinco participantes fornecerem feedback quanto à assistência oferecida e a maioria das respostas apontou dificuldades para realizar fisioterapia a domicílio. Responsáveis e indivíduos com FC ficaram sobrecarregados com as demandas online, visto que as atividades rotineiras estavam sendo realizadas "via tela" no referido período, o que impactou negativamente na adesão a telefisioterapia. Esse achado atenta para profissionais da saúde refletirem sobre aspectos da adesão ao oferecerem essa modalidade.

To describe difficulties and barriers for adherence to telephysiotherapu durint the COVID-19 pandemic. Experience report, conducted with relatives and individuals with cystic fibrosis (CF) on internet, smarthphone or computer, to receive asynchronous telephysiotherapy care in the form of folders and videos. All participantes were required to complete an initial assessment, a quality of live questionnaire, and at end provide feedback about remote assistance and exercise frequency. Nine individuals participated in this study, and few completed all the stages. Five participantes provided feedback regarding the offered assistance, and the majority of responses indicated difficulties in performing home-based physiotherapy. relatives and individuals with CF became overwhelmed with online demands, as routine activies were being conducted "on screen" during that period, which negatively impacted adherence to tepehysiotherapy. These finding alerts healthcare professionals to reflect on adherence aspects when offering this modality.

Repercussões Clínicas da Gameterapia no Manejo do Indivíduo com Fibrose Cística / Clinical repercussions of game therapy in the management of individuals with cystic fibrosis

Introdução: A gameterapia (GT) tem sido utilizada como método para estimular a prática de atividade física em indivíduos com fibrose cística (FC), podendo proporcionar melhora no sistema cardiorrespiratório e na adesão ao tratamento. Objetivo: analisar as repercussões clínicas da gameterapia no manejo de pacientes com FC. Métodos:Conduziu-se uma revisão sistemática de literatura nas seguintes bases de dados: PubMed, Embase, Scopus e MEDLINE via Ovid, utilizando-se os descritores:"Cystic fibrosis","video games" e seus cognatos, por meio da qual se incluíram estudos que descrevessem as repercussões do uso da GT em indivíduos com FC. Resultados: Foram identificados 293 estudos, sendo 7 selecionados ao final. Em 6 estudos, a frequência cardíaca alcançada durante a GT apresentou-se dentro dos padrões recomendados para treinamento. A avaliação do consumo máximo de oxigênio (VO2máx) foi realizada em 3 publicações, e este parâmetro apresentou níveis mais elevados em comparação ao identificado no teste de caminhada de seis minutos. Os consoles mais utilizados foram Nintendo Wii TM e Xbox 360 ™ e a intensidade na realização dos exercícios com a GT esteve entre moderada e intensa. A adesão dos indivíduos quanto a esse recurso também foi verificada, e a GT apresentou-se mais aceitável que outros e foi considerada lúdica. Conclusão: a GT se mostra uma intervenção capaz de gerar respostas fisiológicas que correspondem às necessidades de treinamento, além de maior adesão e satisfação dos indivíduos com FC em sua realização (AU),

Game therapy (GT) has been used as a method to encourage the practice of physical activity in individuals with cystic fibrosis (CF), which may provide improvement in the cardiorespiratory system and treatment adherence. Objective: analyze the clinical repercussions of game therapy in the management of patients with CF. Methods: A systematic review of the literature was carried out in the following databases: PubMed, Embase, Scopus and MEDLINE via Ovid, using the descriptors: "Cystic fibrosis", "video games" and their cognates, through which they included Studies describing the repercussions of the use of GT in individuals with CF were sought. Results: 293 studies were identified, seven of which were selected at the end. In six studies, the heart rate reached during the TG was within the recommended standards for training. The assessment of maximum oxygen consumption (VO2max) was performed in three publications, and this parameter showed higher levels compared to that identified in the six-minute walk test. The most used consoles were Nintendo WiiTM and Xbox 360™and the intensity in carrying out the exercises with the TG was moderate and intense. The individuals' adherence to this resource was also verified, and the GT was more acceptable than others and was considered playful. Conclusion: TG proves to be an intervention capable of generating physiological responses that correspond to training needs and greater adherence and satisfaction of individuals with CF in its performance (AU).

Acompanhamento das funções respiratória e motora de crianças com atrofia muscular espinhal e distrofia muscular de Duchenne: um estudo longitudinal / Monitoring of respiratory and motor functions on children with spinal muscular atrophy and Duchenne muscular dystrophy: a longitudinal study

Objetivo: Realizar o acompanhamento de crianças e adolescentes com Atrofia Muscular Espinhal (AME) e Distrofia Muscular de Duchenne (DMD) em um centro de referência, por meio de avaliações de parâmetros respiratórios e motores. Métodos: Conduziu-se 3 avaliações em um período de 24 meses, em pacientes até 15 anos, com DMD e AME. Avaliações respiratórias incluíram: parâmetros cardiorrespiratórios, força muscular respiratória, pico de fluxo de tosse e espirometria. Analisou-se a função motora por meio de escalas especificas: 1) Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) para crianças até 2 anos; 2) Medida da Função Motora (MFM-32) acima de 6 anos; 3) versão reduzida (MFM-20) para 2 a 6 anos. A análise estatística incluiu o teste de Shapiro-Wilk e utilizou-se ANOVA com Post Hoc de Bonferroni ou Friedman, e aplicou-se os coeficientes de Spearman ou Pearson. Resultados: Participaram 16 pacientes com mediana de idade de 6,5 anos, 12 com AME e 4 DMD. Houve diferença entre dados antropométricos, a frequência de crianças que não realizava fisioterapia reduziu (12,5%X6,3%) e houve aumento na adesão para técnica de empilhamento de ar (37,5%X43,8%). Uso de ventilação não invasiva se manteve igual, assim como parâmetros respiratórios e escalas motoras. Verificou-se forte correlação entre valor predito da capacidade vital forçada e escores MFM-20 e MFM-32. Conclusão: O acompanhamento ambulatorial de crianças com AME e DMD evidenciou relativa manutenção em parâmetros respiratórios e de função motora, o que pode ser atribuído a melhora na adesão de rotinas terapêuticas e aos cuidados em um centro de referência.

Objective: The aim of this study was to monitor children and adolescents with Spinal Muscular Atrophy(SMA) and Duchenne Muscular Dystrophy (DMD) at a referral center, through assessments of respiratory and motor parameters. Methods: 3 evaluations were conducted over a period of 24 months, in patients up to 15 years old, with DMD and SMA. Respiratory assessments included: cardiorespiratory parameters, respiratory muscle strength, peak cough flow and spirometry. Motor function was analyzed using specific scales: 1) Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) for children up to 2 years old; 2) Measurement of Motor Function (MFM-32) over 6 years; 3) reduced version (MFM-20) for 2 to 6 years. The statistical analysis included the Shapiro-Wilk test and ANOVA with Bonferroni or Friedman's Post Hoc was used, and the Spearman or Pearson coefficients were applied. Results: 16 patients with a median age of 6.5 years, 12 with SMA and 4 DMD participated. There was a difference between anthropometric data, the frequency of children who did not undergo physical therapy decreased (12.5%X6.3%) and there was an increase in adherence to the air stacking technique (37.5%X43.8%). Use of non-invasive ventilation remained the same, as did respiratory parameters and motor scales. There was a strong correlation between the predicted value of forced vital capacity and scores MFM-20 and MFM-32. Conclusion: Outpatient follow-up of children with SMA and DMD showed a relative maintenance of respiratory and motor function parameters, which can be attributed to the improvement in adherence to therapeutic routines and care in a reference center.

Distrofia muscular congênita merosina negativa: um relato de caso / Congenital muscular dystrophy merosin negative: a case report

A Distrofia muscular laminin subunit alpha 2 (DM LAMA2) é caracterizada pela deficiência da proteína da cadeia laminina α2, apresentando sintomas distróficos que progridem na infância. Objetivo: Apresentar dados da avaliação fisioterapêutica de uma criança com DM LAMA2 em acompanhamento ambulatorial. Métodos: Pesquisou-se em prontuário dados referentes às avaliações fisioterapêuticas de uma criança de 12 anos com diagnóstico de DM LAMA2 atendida em ambulatório especializado. A avaliação caracterizou-se por ausculta pulmonar, espirometria, verificação de parâmetros cardiorrespiratórios, análise do pico de fluxo de tosse (PFT), força muscular respiratória (FMR) e avaliação motora através da escala MFM-32. Resultados: Realizou-se três avaliações durante 9 meses. A paciente possui doença pulmonar restritiva, na primeira avaliação apresentou VEF1= 29% na espirometria e, comparando com a terceira avaliação obteve aumento de 1%, também houve aumento de 2% na relação VEF1/CVF, 5% no PEF e 11% no FEF25-75%. Na FMR, obteve-se valores de PImáx= 17,9% e PEmáx= 7,13% na primeira avaliação com aumento de 16,85% e 5,34% respectivamente, entre primeira e terceira avaliação. O PFT manteve-se em 0L/min em todas avaliações. Na primeira avaliação motora pontuou 25% no escore total da escala MFM-32, aumentando 3,12% na terceira avaliação. Ao longo do acompanhamento iniciou-se o uso da VNI noturna e introduzida a técnica de AS, buscando correção da hipoxemia, apneias noturnas, aumento do PFT e FMR. Conclusão: Paciente apresentou manutenção de parâmetros espirométricos, aumento de variáveis de FMR e função motora, sem piora do quadro. Possíveis resultados decorridos do acompanhamento multiprofissional e especializado.

Laminin subunit alpha 2 muscular dystrophy (DMLAMA2) is characterized by a deficiency of the α2 laminin chain protein, presenting dystrophic symptoms that progress in childhood. Objective: To present data from the physical therapy evaluation of a child with DMLAMA2 in outpatient follow-up. Methods: Medical records were searched for data referring to the physiotherapeutic evaluations of a 12-year-old child diagnosed with DMLAMA2 treated at a specialized outpatient clinic. The evaluation was characterized by pulmonary auscultation, spirometry, verification of cardiorespiratory parameters, analysis of peak cough flow (PFT), respiratory muscle strength (FMR) and motor evaluation using the MFM-32 scale. Results: Three evaluations were carried out during 9 months. The patient has restrictive disease, in the first evaluation she had FEV1= 29% in spirometry, compared to the third evaluation, she had an increase of 1%, there was also an increase of 2% in the FEV1/FVC ratio, 5% in the PEF and 11% in the FEF25-75%. In the FMR, values of MIP=17.9% and MEP= 7.13% were obtained in the first assessment, with an increase of 16.85% and 5.34%, respectively, between the first and third assessments. The PFT remained at 0L/min in all evaluations. In the first motor evaluation, it scored 25% in the total score of the MFM-32 scale, increasing 3.12% in the third evaluation. During the follow-up, the use of nocturnal NIV started and AS technique was introduced, seeking correction of hypoxemia, night apneas, increased PFT and FMR. Conclusion: Patient presented maintenance of spirometric parameters, increased FMR variables and motor function, without worsening the condition. Possible results from the multidisciplinary and specialized monitoring.

Jaw and neck muscle activity during chewing and craniocervical dysfunction index in children, adolescents, and adults with and without cystic fibrosis: a comparative analysis.

OBJECTIVE: To compare masticatory muscle activity between people with cystic fibrosis (pwCF) and healthy controls and to verify whether craniocervical dysfunction is associated with the presence of CF. METHODS: Fifty-six participants were assessed and divided into pwCF and healthy control (HC) groups, each one composed of 13 children and adolescents at 9 (SD 3) years old and 15 adults at 25 (SD 6) years old. Craniocervical Dysfunction Index assessed symptoms of dysfunction and cervical spine mobility. Electromyography was used to evaluate the jaw and neck muscle activity during chewing. RESULTS: Muscle activity during chewing was not statistically different between groups. Prevalence of craniocervical dysfunction was 75% for pwCF vs 64% for healthy controls. Individuals with CF are 1.53 [1.260, 1.870] times more likely to have reduced cervical mobility compared to healthy controls (p = 0.000). CONCLUSION: These results reinforce the need for musculoskeletal disorders treatment in the management of pwCF.

Comparison of respiratory parameters between singing and non-singing choir children / Comparação dos parâmetros respiratórios de crianças cantoras e não cantoras de coral

Introdução: intervenções musicais, como canto coral, têm caráter artístico e são capazes de promover benefícios psicológicos e fisiológicos. Não há estudos na literatura sobre a repercussão do canto coral em parâmetros do sistema respiratório de escolares cantores, em comparação aos não cantores. Objetivo: comparar parâmetros do sistema respiratório entre crianças e adolescentes cantores de coral e não cantores. Método: estudo observacional transversal quantitativo incluiu escolares de 7 a 14 anos, constituindo o grupo intervenção alunos de canto coral (GCC) pareados com escolares não cantores (GNC) como controles. Realizou-se avaliação antropométrica, seguida de espirometria e manovacuometria, segundo recomendações da American Thoracic Society. Para comparação entre os grupos considerou-se os valores espirométricos absolutos e valores preditos, assim como para força de musculatura respiratória. Aplicou-se teste de Shapiro-Wilk e conduziu-se os testes U de Mann-Witney e Teste-T independente, com nível de significância de 5%. Resultados: participaram 40 crianças (95% meninas), 20 em cada grupo, com idade média de 11,25±1,80 anos no GNC e 11,20±1,64 anos no GCC. Volume expiratório forçado no primeiro segundo em porcentagem do predito (VEF1%) se apresentou maior no GNC (98,58±12,62%), em comparação ao GCC (87,10±8,84%) (p=0,001), por sua vez, GCC apresentou maior valor absoluto de pico de fluxo expiratório (PFE) (GNC:4,21±0,99l/s x GCC: 4,95±1,29l/s; p=0,048). Conclusão: os escolares cantores de canto coral não apresentaram melhores parâmetros de função pulmonar e FMR, em comparação aos não cantores (AU)

Introduction: musical interventions, such as choral singing, are artistic in character and are capable of promoting psychological and physiological benefits. There are no studies in the literature on the repercussion of choir on respiratory system parameters of practicing students compared to non-singers. Objective: compare respiratory system parameters between choir and non-choir children and adolescents. Method: cross-sectional observational study included students from 7 to 14 years old, with the intervention group constituted students of choir singing (GCC) paired with non-singing students (GNC). Anthropometric assessment was performed, followed by spirometry and manovacuometry, according to ATS recommendations. For comparison between groups, absolute spirometric values and predicted values were considered, as well as respiratory muscle strength. Shapiro-Wilk test was applied and the Mann-Witney U test and independent T-test were conducted, with a significance level of 5%. Results: 40 children (95% girls) participated, 20 in each group, with a mean age of 11.25 ± 1.80 years in the GNC and 11.20 ± 1.64 years in the GCC. Forced expiratory volume in the first second as a percentage of predicted (FEV1%) was higher in GNC (98.58 ± 12.62%) compared to GCC (87.10 ± 8.84%) (p = 0.001), GCC presented the highest absolute value of peak expiratory flow (PEF) (GNC: 4.21 ± 0.99l/s x GCC: 4.95 ± 1.29l/s; p=0.048). Conclusion: schoolchildren singing in the choir did not present better parameters of pulmonary function and FMR, compared to non-singers (AU)

Do impulse oscillometry parameters differ between children and adolescents with symptoms of rhinitis and those without?

OBJECTIVE: To compare impulse oscillometry parameters between healthy children and adolescents with symptoms of rhinitis and those without. METHODS: This was a cross-sectional analytical study of healthy individuals 7-14 years of age. Health status was determined through the use of questionnaires. We performed anthropometric measurements, impulse oscillometry, and spirometry. RESULTS: The sample comprised 62 students, with a mean age of 9.58 ± 2.08 years and a mean body mass index (BMI) of 17.96 ± 3.10 kg/m2. The students were divided into two groups: those with symptoms of rhinitis (n = 29) and those without such symptoms (n = 33). The oscillometry results and anthropometric parameters were normal in both groups and did not differ significantly between the two. The variables age, height, and body mass, respectively, correlated negatively and moderately with most of the following parameters: total airway resistance (r = -0.529, r = -0.548, and r = -0.433); central airway resistance (r = -0.441, r = -0.468, and r = -0.439); respiratory impedance (r = -0.549, r = -0.567, and r = -0.455); reactance at 5 Hz (r = 0.506, r = -0.525, and r = -0.414); reactance area (r = -0.459, r = -0.471, and r = -0.358); and resonance frequency (r = -0.353, r = -0.371, and r = -0.293). We found that BMI did not correlate significantly with any of the parameters evaluated. The same was true when we analyzed each group in isolation. CONCLUSIONS: In our sample, impulse oscillometry parameters did not differ between the students who had symptoms of rhinitis and those who did not.

Standard reference and percentiles of maximum respiratory pressures values of healthy children aged 7-10 years.

OBJECTIVE: This study aims to present standard reference for values of maximum respiratory pressures of healthy schoolchildren, according to gender. METHODS: This is a cross-sectional study involving healthy children aged 7-10 years. Data of body mass and height were evaluated to calculate body mass index (BMI). In addition, forced expiratory volume in 1 second (FEV1) and maximal expiratory pressure values were evaluated according to the American Thoracic Society. The maximal inspiratory pressure (MIP) and data of maximal expiratory pressure (MEP) obtained in the study showed normal distribution and curves were built by the Lambda-Mu-Sigma (LMS) method, as well as the values of MIP and MEP percentiles 3, 10, 25, 50, 75, 90, and 97 for each gender. RESULTS: MIP and MEP data were collected from 399 schoolchildren. All schoolchildren in the sample had adequate FEV1 and BMI. The study showed an increase in respiratory pressure values with age progression. The MIP and MEP values of girls were of 53.4±11.0 and 61.8±12.5cmH2O, respectively, and those of boys were 59.9±13.6 and 69.6±15.7cmH2O, respectively. CONCLUSIONS: Normal curves and percentiles were developed for MIP and MEP values of healthy schoolchildren. The extreme percentiles (3rd and 97th) were determined, and a specific graph was elaborated for each group. These graphs may help clinical follow-up and therapeutic monitoring of different pediatric populations.

Impact of Forced Exhalation Maneuvers During Spirometry on Airway Resistance Measured by Oscillometry in Healthy Children.

OBJECTIVE: To analyze the impact of repeated forced spirometry maneuvers on oscillometry parameters of healthy children. METHODS: This is a cross-sectional study with healthy children (6-12 y old) from schools in Florianopolis-SC/Brazil. Good health condition was confirmed through questionnaires, health history, and normal spirometry. Spirometry maneuvers and impulse oscillometry were conducted according to the American Thoracic Society guidelines. The school children were grouped according to the number of spirometry maneuvers performed: 1) 3 maneuvers; 2) 4 maneuvers and 3) 5 to 8 maneuvers. The following oscillometry values were considered: at rest (T0); after the first spirometry maneuver (T1); and after the last maneuver (T2), according to the groups' allocation. The mixed model ANOVA was applied to verify the interaction of oscillometry parameters in all 3 moments and groups. The Friedman test was used for analysis of Fres (p < 0.05). RESULTS: In 149 school children (mean age: 9.13 y old ± 1.98), there was a significant increase in Z5, R5, R20, and X5 values at rest and after the first spirometry maneuver, and values at rest and after the last maneuver in all groups. The effects on analyzed variables were significant in Z5 (F: 12.35; gl: 2; p < 0.001), R5 (F: 11.14; df: 2, p < 0.001), R20 (F: 7.53; df: 2, p < 0.001), and X5 (F: 4.30; df: 2, p = 0.014). CONCLUSION: There were changes in respiratory mechanics after spirometry, like the increase in baseline Z5, R5, R20, and X5 after the first forced spirometry maneuver, and in comparison to the last maneuver obtained.

Espirometria e oscilometria de impulso durante internação por exacerbação pulmonar aguda em escolares com fibrose cística / Spirometry and impulse oscillometry during acute pulmonary exacerbation hospitalization in children with cystic fibrosis

Objetivo: avaliar o comportamento de parâmetros do sistema respiratório durante internação para antibioticoterapia intravenosa (AIV) como tratamento da exacerbação pulmonar aguda (EPA) em escolares com fibrose cística (FC). Métodos: estudo do tipo analítico observacional, before-after, realizado no Hospital Infantil Joana de Gusmão, Florianópolis ­ Santa Catarina. Foram incluídas crianças com diagnóstico de FC, entre seis e 15 anos, em internação para tratamento da exacerbação pulmonar aguda, no início (T1), durante (T2) e ao final (T3) da internação foi conduzida avaliação de escores específicos de EPA, dados antropométricos e realizada avaliação dos parâmetros do sistema respiratório pelo sistema de oscilometria de impulso (IOS) e espirometria. Foram obtidos, em prontuário, dados de colonização bacteriana, genótipo, gravidade da doença (Escore de Schwachman-Doershuk-ESD) e espirometria mais recente em estabilidade clínica. Aplicou-se o teste Shapiro-Wilk para análise da distribuição dos dados e os testes ANOVA de medidas repetidas, teste de Friedman, teste T pareado e Wilcoxon, com nível de significância de 5%. Resultados: participaram 16 crianças/adolescentes (68.8% meninas, 12.88±1.67anos). Houve aumento dos parâmetros da espirometria e dados antropométricos (p<0.005) no T3, bem como redução dos escores de EPA e do X5 (p<0.005) no T3. Conclusão: os dados apresentados nesse trabalho mostram melhora dos escores de EPA, dados antropométricos, parâmetros da espirometria e do parâmetro de recolhimento elástico do IOS (X5).RESUMODescritores: Fibrose cística, Exacerbação dos sintomas, Testes de função pulmonar, Mecânica respiratória (AU)

Objective: Evaluate the respiratory system parameters of children with cystic fibrosis (CF) during hospitalization for acute pulmonary exacerbation (APE) treatment. Methods: observational study before-after that occurred at the CF reference center. There were included children with cystic fibrosis (CF) between six to 15 years old hospitalized due to APE. The registration of the APE clinical scores, anthropometric data, and respiratory system (IOS and spirometry) evaluation occurred at the beginning (T1), during (T2), and at the end (T3) of the hospitalization. There were registered pathogens, genetic mutation, disease severity (Schwachman-Doershuk Score), and the most recent spirometry when they were clinically stable. The Shapiro-Wilk test was applied to analyze data distribution, and the repeated measure ANOVA, Friedman test, Tpaired test, and Wilcoxon test were performed to compare data, with a significance level set at 5%. Results: sixteen children/adolescents participated in the study (68.8% girls, 12.88±1.67 years old). The spirometric parameters, X5 parameter, and anthropometric data increased (p<0.005) and the APE scores decreased (p<0.005) at T3. Conclusion: APE scores, anthropometric data, spirometric parameters, and IOS elastic recoil parameter (X5) improved at the end of hospitalization (AU)

Espirometria e oscilometria de impulso durante internação por exacerbação pulmonar aguda em escolares com fibrose cística / Spirometry and impulse oscillometry during acute pulmonary exacerbation hospitalization in children with cystic fibrosis

RESUMO: Objetivo: avaliar o comportamento de parâmetros do sistema respiratório durante internação para antibioticoterapia intravenosa (AIV) como tratamento da exacerbação pulmonar aguda (EPA) em escolares com fibrose cística (FC). Métodos: estudo do tipo analítico observacional, before-after, realizado no Hospital Infantil Joana de Gusmão, Florianópolis ­ Santa Catarina. Foram incluídas crianças com diagnóstico de FC, entre seis e 15 anos, em internação para tratamento da exacerbação pulmonar aguda, no início (T1), durante (T2) e ao final (T3) da internação foi conduzida avaliação de escores específicos de EPA, dados antropométricos e realizada avaliação dos parâmetros do sistema respiratório pelo sistema de oscilometria de impulso (IOS) e espirometria. Foram obtidos, em prontuário, dados de colonização bacteriana, genótipo, gravidade da doença (Escore de Schwachman-Doershuk-ESD) e espirometria mais recente em estabilidade clínica. Aplicou-se o teste Shapiro-Wilk para análise da distribuição dos dados e os testes ANOVA de medidas repetidas, teste de Friedman, teste T pareado e Wilcoxon, com nível de significância de 5%. Resultados: participaram 16 crianças/adolescentes (68.8% meninas, 12.88±1.67anos). Houve aumento dos parâmetros da espirometria e dados antropométricos (p<0.005) no T3, bem como redução dos escores de EPA e do X5 (p<0.005) no T3. Conclusão: os dados apresentados nesse trabalho mostram melhora dos escores de EPA, dados antropométricos, parâmetros da espirometria e do parâmetro de recolhimento elástico do IOS (X5).RESUMODescritores: Fibrose cística, Exacerbação dos sintomas, Testes de função pulmonar, Mecânica respiratória. (AU)

ABSTRACT: Objective: Evaluate the respiratory system parameters of children with cystic fibrosis (CF) during hospitalization for acute pulmonary exacerbation (APE) treatment. Methods: observational study before-after that occurred at the CF reference center. There were included children with cystic fibrosis (CF) between six to 15 years old hospitalized due to APE. The registration of the APE clinical scores, anthropometric data, and respiratory system (IOS and spirometry) evaluation occurred at the beginning (T1), during (T2), and at the end (T3) of the hospitalization. There were registered pathogens, genetic mutation, disease severity (Schwachman-Doershuk Score), and the most recent spirometry when they were clinically stable. The Shapiro-Wilk test was applied to analyze data distribution, and the repeated measure ANOVA, Friedman test, Tpaired test, and Wilcoxon test were performed to compare data, with a significance level set at 5%. Results: sixteen children/adolescents participated in the study (68.8% girls, 12.88±1.67 years old). The spirometric parameters, X5 parameter, and anthropometric data increased (p<0.005) and the APE scores decreased (p<0.005) at T3. Conclusion: APE scores, anthropometric data, spirometric parameters, and IOS elastic recoil parameter (X5) improved at the end of hospitalization.ABSTRACTKeywords: Cystic fibrosis, Symptom flare up, Respiratory function tests, Respiratory mechanics.1. Universidade do Estado de Santa Catarina ­ UDESC ­ Florianópolis, (SC) ­ Brasil https://doi.org/10.11606/issn.2176-7262.rmrp.2022.183755Tayná Castilho1, Renata Maba Gonçalves Wamosy1, Camila Isabel Santos Schivinski1Este é um artigo publicado em acesso aberto (Open Access) sob a licença Creative Commons Attribution, que permite uso, distribuição e reprodução em qualquer meio, sem restrições, desde que o trabalho original seja corretamente citado. (AU)

"Test-retest reliability and minimal detectable change in TGlittre-P test in children and adolescents with cystic fibrosis".

OBJECTIVE: to provide test-retest reliability for the TGlittre-P in children and adolescents with cystic fibrosis (CFG) and healthy controls (HCG), to establish the minimal detectable change for time in TGlittre-P and comparing the performance in the TGlittre-P test between these populations. METHOD: A cross-sectional study evaluated 36 children and adolescents aged 6 to 13. Anthropometric and spirometric evaluation was performed, as well as, on the same day, two TGlittre-P tests with a 30-minute interval between them. RESULTS: TGlittre-P time test-retest reliability was excellent for both groups (CFG: intraclass correlation coefficient [ICC] = 0.849, p < 0.001 and HCG: ICC = 0.913, p < 0.001). As concerning absolute reliability, the time spent presented a small variability with a standard error of measurement of 8.4 s (s) to CFG and 5.3 s to HCG. The minimal detectable change at 95% confidence level (MDC95) was 23.2 s and 14.6 s, respectively. There was no difference between the groups regarding performance in the TGlittre-P test (CFG 179.1 s ± 25.7 s vs. HCG 174.7 s ± 22.3 s) p = 0.589. CONCLUSION: The TGlittre-P is a reliable tool in children and adolescents with CF and healthy controls. The TGlittre-P appears not to be sensitive enough to discriminate a group of children and adolescents with mild cystic fibrosis from healthy counterparts.IMPLICATIONS FOR REHABILITATIONTGlittre-P is a multitasking test that has been used to assess the functional capacity of children and adolescents with chronic diseases.TGlittre-P has excellent reliability in children and adolescents with and without CF.TGlittre-P differences time greater than 12% could indicate changes in the functional capacity of children and adolescents with CF.Other functional capacity tests may be preferred to detect continuous increases in functional capacity through rehabilitation or training, whether children and adolescents obtain performance values close to 100% of predicted.

Standard reference and percentiles of maximum respiratory pressures values of healthy children aged 7-10 years / Distribuição padrão e percentuais dos valores de pressão respiratória máxima de crianças saudáveis de 7 a 10 anos de idade

Abstract Objective: This study aims to present standard reference for values of maximum respiratory pressures of healthy schoolchildren, according to gender. Methods: This is a cross-sectional study involving healthy children aged 7-10 years. Data of body mass and height were evaluated to calculate body mass index (BMI). In addition, forced expiratory volume in 1 second (FEV1) and maximal expiratory pressure values were evaluated according to the American Thoracic Society. The maximal inspiratory pressure (MIP) and data of maximal expiratory pressure (MEP) obtained in the study showed normal distribution and curves were built by the Lambda-Mu-Sigma (LMS) method, as well as the values of MIP and MEP percentiles 3, 10, 25, 50, 75, 90, and 97 for each gender. Results: MIP and MEP data were collected from 399 schoolchildren. All schoolchildren in the sample had adequate FEV1 and BMI. The study showed an increase in respiratory pressure values with age progression. The MIP and MEP values of girls were of 53.4±11.0 and 61.8±12.5cmH2O, respectively, and those of boys were 59.9±13.6 and 69.6±15.7cmH2O, respectively. Conclusions: Normal curves and percentiles were developed for MIP and MEP values of healthy schoolchildren. The extreme percentiles (3rd and 97th) were determined, and a specific graph was elaborated for each group. These graphs may help clinical follow-up and therapeutic monitoring of different pediatric populations.

Resumo Objetivo: Apresentar distribuição normal para os valores das pressões respiratórias máximas de escolares saudáveis, de acordo com o gênero. Métodos: Estudo transversal envolvendo crianças saudáveis de 7 a 10 anos. Foram avaliados os dados de massa corporal e estatura, para o cálculo do índice de massa corporal (IMC). Os valores de volume expiratório forçado no primeiro segundo (VEF1) e pressão expiratória máxima foram avaliados de acordo com a American Thoracic Society. Os dados de pressão inspiratória máxima (PImáx) e pressão expiratória máxima (PEmáx) obtidos no estudo deram origem à distribuição normal, cuja construção foi elaborada pelo método the Lambda-Mu-Sigma (LMS), bem como os percentis 3, 10, 25, 50, 75, 90 e 97 de PImáx e PImáx de cada sexo. Resultados: Os dados de PImáx e PImáx foram coletados de 399 escolares. Todos os escolares da amostra apresentavam VEF1 e índice de massa corporal adequados. O estudo mostrou aumento dos valores de pressão respiratória com o avançar da idade. Os valores de PImáx e PImáx das meninas foram de 53,49±11,07 e 61,80±12,51cmH2O, respectivamente; e os meninos de 59,96±13,66 e 69,68±15,72 cmH2O, respectivamente. Conclusões: Foram desenvolvidas curvas e percentis normais para os valores de PImáx e PImáx de escolares saudáveis o que poderia auxiliar no acompanhamento clínico e terapêutico de diferentes populações pediátricas.

Evaluation of cardiopulmonary system outcomes in children with developmental coordination disorder: A systematic review.

BACKGROUND: children with Developmental Coordination Disorder (DCD) tend to avoid physical activity, which can affect their health and well-being. AIM: Conduct a systematic review to identify, synthesize and compile data from the literature on the evaluation of the cardiopulmonary system in children with DCD. METHOD: According to PRISMA guidelines, we searched for articles indexed in PubMed, LILACS, Scopus, Web of Science, and Cochrane Library. The strategy was directed at the Population (children), Exposure (DCD), Outcomes (descriptors related to cardiopulmonary evaluation). RESULTS: A total of 59 articles were identified through the databases. In the end, after analyzing the titles, abstracts, and full articles, including articles through manual search in the lists of bibliographic references, 13 articles relevant to the topic were included. All selected studies evaluated cardiorespiratory fitness / aerobic capacity parameters, and only three studies included the assessment of lung function. CONCLUSION: Although very heterogeneous, the evidence found in this review suggests that children with DCD have less cardiorespiratory fitness and lower lung function when compared to children with typical development. However, new studies are suggested to investigate and strengthen the evidence found in the present study.

Coughing and diaphragmatic breathing exercise: What is the repercussion on respiratory mechanics of children and adolescents with cystic fibrosis?

INTRODUCTION: The aim of this study was to investigate the immediate effect of coughing episodes and diaphragmatic breathing exercise (DBE) on respiratory mechanics of children/adolescents with cystic fibrosis (CF). METHODS: It is a cross-sectional analytical study that occurred in a reference center for children with CF. Forty-five children/adolescents with CF (60% male; mean age 10.22 ± 2.84 years old; mean forced expiratory volume in 1 second 73.74 ± 21.38% predicted) were divided into 3 groups according to the R5 parameter response to the DBE: G1 (increased R5), G2 (no change R5), and G3 (decreased R5). The children/adolescents performed 5 successive coughs and 10 DBE. The main outcome measures were the impulse oscillometry system (IOS) parameters evaluated before, during, and after the interventions. RESULTS: In the total sample, the IOS parameters (Z5, R5, and R20) were worse after coughing, and they did not change after the DBE. In the G1, the parameters were progressively worsening during the interventions. In the G2, they worsened after coughing and after the DBE. In the G3, they worsened after coughing; however, after the DBE, the IOS parameters have improved and returned close to the baseline. CONCLUSIONS: The children/adolescents with CF airway resistance got worse after coughing episodes, and 10 DBE repetitions did not affect the respiratory mechanics in most of the sample. Meanwhile, in the group with older children, the DBE worsened the respiratory mechanics, yet in the younger group it improved.

Immediate effect of inhalation therapy combined with oscillatory positive expiratory pressure on the respiratory system of children with cystic fibrosis.

INTRODUCTION: It is recommended the association of inhalation therapies and physiotherapy on the management of cystic fibrosis (CF); however, it is still necessary to understand the effect on respiratory mechanics of these therapies combined. This study aimed to evaluate the immediate effect of inhalation with Dornase-Alfa (DNase) and hypertonic saline solution (HSS), as well as the impact of these inhalation therapies associated with an oral high-frequency oscillation (OHFO) physiotherapy device, on the respiratory mechanics of children and adolescents with CF. METHOD: Children/adolescents with CF were allocated into two groups (DNaseG and HSSG), where they performed inhalation therapy before using the OHFO device for physiotherapy. In each group, the Impulse Oscillometry System was conducted before and after inhalation therapy, and after OHFO. ANOVA was carried out to analyse the respiratory mechanics at different moments of DNaseG and HSSG. The Mann-Whitney test compared the immediate effect of each inhalation therapy and after OHFO. RESULTS: 30 children (6-14 years old) were studied. In DNaseG, the mean value of most oscillometric parameters decreased in the evaluated moments; in HSSG, only reactance showed an immediate increase. CONCLUSION: Children/adolescents with CF showed an immediate decrease in airway resistance and reactance after the use of DNase and associated with OHFO, indicating improvement. The inhalation with HSS has an immediate effect on peripheral airways.

Atividade física e força muscular respiratória em crianças saudáveis / Actividad física y fuerza muscular respiratoria en niños sanos / Level of physical activity and respiratory muscle force in healthy children

RESUMO Este artigo tem como objetivo verificar a relação entre a idade e o nível de atividade física de crianças não saudáveis (NAF) com parâmetros de força muscular respiratória (FMR) de crianças saudáveis e comparar os dados entre gêneros. Participaram da pesquisa escolares saudáveis de 6 a 12 anos provenientes da Grande Florianópolis (SC), Brasil. A higidez foi controlada com um questionário, um recordatório de saúde e parâmetros espirométricos. Avaliaram-se parâmetros de FMR por manovacuometria e seus valores preditos (%) e foi aplicado um questionário sobre NAF. Aplicou-se também o teste Kolmogorov-Smirnov e, na sequência, foram realizados os testes de correlação de Spearman, T e U-Mann-Whitney para comparação dos dados de FMR entre gêneros e NAF. Denominou-se GA para o grupo ativo e GI para o grupo inativo e se adotou 5% como nível de significância. Participaram da pesquisa 76 crianças (10,1±1,7 anos) com média de pressão inspiratória (PIMÁX) de −89,7±41,4 cmH2O e de pressão expiratória máximas (PEMÁX) de 86,6±22,6cmH2O. Os resultados mostraram que os parâmetros de FMR e NAF não apresentaram relação, enquanto os valores de PIMÁX, PEMÁX e %PEMÁX se relacionaram significativamente com a idade. Além disso, o GA apresentou maior valor da PIMÁX em comparação ao GI e não houve diferença entre gêneros para NAF, somente maior PEMÁX nos meninos. Os parâmetros de FMR e NAF não se associaram, mas ambos apresentaram relação com a idade. Na comparação entre os grupos, as crianças ativas apresentaram maiores valores de PIMÁX que as inativas. Não houve diferença no NAF entre gêneros, mas os meninos apresentaram maiores valores absolutos de PEMÁX.

RESUMEN Este artículo tiene como objetivo comprobar la relación entre la edad y el nivel de actividad física de niños no sanos (NAF) con los parámetros de fuerza muscular respiratoria (FMR) de niños sanos, así como comparar los datos entre los géneros. En el estudio participaron escolares sanos de 6 a 12 años de edad de la gran Florianópolis (Brasil). Para analizar la buena salud se utilizaron cuestionario, recordatorio clínico y parámetros espirométricos. Los parámetros de FMR se evaluaron por manovacuometría y sus valores predichos (%), y se aplicó un cuestionario sobre NAF. También se aplicó la prueba de Kolmogorov-Smirnov y, posteriormente, se realizaron los test de correlación de Spearman, t y U-Mann-Whitney para comparar los datos de FMR entre géneros y NAF. Se denominó GA al grupo activo y GI al grupo inactivo, y el nivel de significación adoptado fue del 5%. En el estudio participaron 76 niños (10,1±1,7 años) con presión inspiratoria media (PIMÁX) de -89,7±41,4 cmH2O y con presión espiratoria máxima (PEMÁX) de 86,6±22,6 cmH2O. Los resultados mostraron que los parámetros de FMR y NAF no estaban relacionados, mientras que los valores de PIMáX, PEMÁX y %PEMÁX se relacionaron significativamente con la edad. Además, el GA tuvo un valor de PIMÁX más alto que el GI y no hubo diferencias entre los géneros para NAF, solo mayor PEMÁX en los niños. Los parámetros de FMR y NAF no estuvieron asociados, pero ambos se relacionaron con la edad. Al comparar los grupos, los niños activos tuvieron valores de PIMÁX más altos que los niños inactivos. No hubo diferencia en NAF entre los géneros, pero los niños tuvieron valores de PEMÁX más altos.

ABSTRACT This study aimed to verify the relationship between the level of physical activity (LPA) and age with respiratory muscle strength (RMS) parameters of healthy children, as well as to compare this data between boys and girls. Healthy schoolchildren (6 to 12 years old) from the metropolitan area of Florianópolis-Santa Catarina/Brazil were the subjects of this study. Health was controlled by questionnaire, health record and spirometric parameters. Manovacuometry was performed to evaluate the RMS parameters and a questionnaire was applied to assess the LPA. In the sequence of the Kolmogorov-Smirnov test, the Spearman's correlation test was applied to verify the relationship between variables, and U-Mann-Whitney test to compare the RMS parameters between genders and LPA (active group-AG and inactive group-IG). The level of significance adopted was 5%. In total, 76 children (10.1±1.7 years) participated in this study, with mean maximum inspiratory pressure (MIP) of −89.7±41.4cmH2O and maximum expiratory pressure (MEP) 86.6±22.6cmH2O. The parameters of RMS and LPA level were not related. Values of MIP, MEP and predicted percentage of MEP were significantly related to age. AG showed a higher MIP value compared with IG. There was no difference between genders for LPA, there was only a difference for MEP in boys. RMS parameters and LPA were not related, but both were related to age. Active children had a higher MIP value compared with inactive children. Between genders, there was no difference in the LPA and boys showed higher MEP values.

Which factors can influence the number of forced expiratory manoeuvres on spirometry in schoolchildren?

INTRODUCTION: Spirometry is of great value for understanding respiratory function and management of lung diseases. Adaptations in the exam were made to meet paediatric population since the forced expiratory manoeuvres (FEM) present in the exam require effort and cooperation; therefore, its use should be reconsidered. OBJECTIVE: To analyse factors that may influence the number of FEM required for successful spirometry in schoolchildren. METHOD: Healthy children aged between 6 and 12 years were tested. FEM were conducted according to the American Thoracic Society/European Respiratory Society guideline. The children were divided into three groups according to the number of attempts: G3M if child completed the test in three FEM; G4M if child completed in four attempts FEM; and G5/8M if child completed the test in five to eight FEM. Factors that potentially influenced the number of FEM included: age; impulse oscillometry parameters; slow vital capacity; respiratory muscle strength; orofacial motor function, school performance, physical activity level and quality of life. The Kolmogorov-Smirnov test was performed, followed by the Chi-Square, repeated measures ANOVA and Kruskal-Wallis tests; thereafter, a multinomial logistic regression was applied. RESULTS: One hundred and forty-nine schoolchildren (80 girls) with mean age of 9.13 years (±1.98) were included, age was related to the required number of FEM (F = 3.38(2), P = .03) and children with poor school performance had a 2.84-fold greater chance of completing the exam in more than five attempts. CONCLUSION: Age and school performance influenced the number of FEM required for a successful spirometry in schoolchildren.