Title: Comparison of respiratory function in school age children Objectives: The main aims of this study were to estimate influence of age, anthropometric measures, and a type of physical activity on forced vital capacity (FVC), and additionally to create reference values of FVC for children aged 5 to 10 years. Methods: 281 girls and 345 boys were enrolled in this cross-sectional study. Data were obtained during the sport propagation event called Sportacek 2016. Descriptive statistics and regression models were carried out in data processing. Results: Statistically significant influence of body height as well as influence of physical activity in boys' gymnastics on FVC was found. Keywords: respiration, dependence, body height, forced vital capacity

A Distrofia Muscular de Duchenne (DMD) é a distrofia muscular mais comum e severa da infância, resultando em fraqueza e perda de massa muscular progressiva, que leva a múltiplas incapacidades e diminuição da sobrevivência. Os tratamentos disponíveis incluem corticoterapia e gerenciamento das funções ventilatória e motora com o objetivo de postergar os acometimentos provocados pela doença. Objetivo: Avaliar a função respiratória de crianças com DMD. Método: Foi realizado um estudo do tipo transversal, sendo coletados dados referentes a avaliações de função respiratória realizadas em 101 pacientes com DMD. Foram considerados elegíveis, todos os pacientes, do sexo masculino, que estavam em seguimento clínico pelo Ambulatório de DMD e com diagnóstico confirmado por método molecular ou por análise imunohistoquímica da distrofina na biópsia muscular e que tivessem passado por avaliação da função respiratória durante as visitas de seguimento clínico entre janeiro de 2012 e junho de 2013. Resultados: Os pacientes avaliados tinham em média 14,4 anos (dp=4,4 anos), 147cm de altura (dp=17,1), com mediana de peso de 45kg e capacidade vital forçada de 65,1 por cento (dp=24,6) em média em relação ao predito e aproximadamente 45 por cento dos avaliados possuiam entre 60 a 89 por cento de capacidade vital forçada em relação ao seu predito. Sobre as demais variáveis espirométricas, os valores mais altos foram obtidos na faixa etária de 13 e 15 anos, seguida de um declínio da função respiratória. Após os 18 anos de idade, todos os parâmetros foram menores do que 50 por cento em relação ao predito. Conclusão: a casuística estudada apresentou resultados de avaliação da função respiratória semelhantes aos de grandes centros de pesquisa e seguimento de pacientes com DMD em países desenvolvidos, demonstrando a boa qualidade do serviço oferecido aos pacientes. / Duchenne Muscular Dystrophy (DMD) is the most common and severe muscular dystrophy of childhood, resulting in weakness and progressive loss of muscle mass, which leads to multiple disabilities and decrease of the survival. The available treatments include corticosteroids and management of respiratory and motor functions in order to postpone the damages caused by the disease. Objective: To evaluate the respiratory function of children with DMD. Method: A cross-sectional study was made in order to collect data based on the evaluation of the respiratory function applied in 101 patients with DMD. It was considered eligible all the patients, males, who were being followed up by the clinical ambulatory DMD, with the diagnosis confirmed by molecular method or by the immunohistochemical analysis of dystrophin in muscle biopsy and for the once who had passed by an evaluation of the respiratory function during the visits between January 2012 and June 2013. Results : All patients had an average of 14.4 years ( SD = 4.4 years ) , height 147cm ( SD = 17.1 ) with a median weight of 45kg and forced vital capacity of 65.1 per cent (SD = 24.6) related to the predicted and approximately 45 per cent of the evaluated had 60 per cent to 89 per cent FVC related to its predicted. About the other spirometric variables, the highest values were obtained from the age of 13 to 15 years, followed by a decline in respiratory function. After the age of 18, all parameters were lower than 50 per cent compared to predicted. Conclusion: the population studied showed similar results of respiratory function assessment with major centers of research and follow-up of patients with DMD in developed countries, showing the good quality of the service offered to the patients

A Distrofia Muscular de Duchenne (DMD) é a distrofia muscular mais comum e severa da infância, resultando em fraqueza e perda de massa muscular progressiva, que leva a múltiplas incapacidades e diminuição da sobrevivência. Os tratamentos disponíveis incluem corticoterapia e gerenciamento das funções ventilatória e motora com o objetivo de postergar os acometimentos provocados pela doença. Objetivo: Avaliar a função respiratória de crianças com DMD. Método: Foi realizado um estudo do tipo transversal, sendo coletados dados referentes a avaliações de função respiratória realizadas em 101 pacientes com DMD. Foram considerados elegíveis, todos os pacientes, do sexo masculino, que estavam em seguimento clínico pelo Ambulatório de DMD e com diagnóstico confirmado por método molecular ou por análise imunohistoquímica da distrofina na biópsia muscular e que tivessem passado por avaliação da função respiratória durante as visitas de seguimento clínico entre janeiro de 2012 e junho de 2013. Resultados: Os pacientes avaliados tinham em média 14,4 anos (dp=4,4 anos), 147cm de altura (dp=17,1), com mediana de peso de 45kg e capacidade vital forçada de 65,1 por cento (dp=24,6) em média em relação ao predito e aproximadamente 45 por cento dos avaliados possuiam entre 60 a 89 por cento de capacidade vital forçada em relação ao seu predito. Sobre as demais variáveis espirométricas, os valores mais altos foram obtidos na faixa etária de 13 e 15 anos, seguida de um declínio da função respiratória. Após os 18 anos de idade, todos os parâmetros foram menores do que 50 por cento em relação ao predito. Conclusão: a casuística estudada apresentou resultados de avaliação da função respiratória semelhantes aos de grandes centros de pesquisa e seguimento de pacientes com DMD em países desenvolvidos, demonstrando a boa qualidade do serviço oferecido aos pacientes. / Duchenne Muscular Dystrophy (DMD) is the most common and severe muscular dystrophy of childhood, resulting in weakness and progressive loss of muscle mass, which leads to multiple disabilities and decrease of the survival. The available treatments include corticosteroids and management of respiratory and motor functions in order to postpone the damages caused by the disease. Objective: To evaluate the respiratory function of children with DMD. Method: A cross-sectional study was made in order to collect data based on the evaluation of the respiratory function applied in 101 patients with DMD. It was considered eligible all the patients, males, who were being followed up by the clinical ambulatory DMD, with the diagnosis confirmed by molecular method or by the immunohistochemical analysis of dystrophin in muscle biopsy and for the once who had passed by an evaluation of the respiratory function during the visits between January 2012 and June 2013. Results : All patients had an average of 14.4 years ( SD = 4.4 years ) , height 147cm ( SD = 17.1 ) with a median weight of 45kg and forced vital capacity of 65.1 per cent (SD = 24.6) related to the predicted and approximately 45 per cent of the evaluated had 60 per cent to 89 per cent FVC related to its predicted. About the other spirometric variables, the highest values were obtained from the age of 13 to 15 years, followed by a decline in respiratory function. After the age of 18, all parameters were lower than 50 per cent compared to predicted. Conclusion: the population studied showed similar results of respiratory function assessment with major centers of research and follow-up of patients with DMD in developed countries, showing the good quality of the service offered to the patients

Spirometry is the most important tool in diagnosing pulmonary disease and is the most frequently performed pulmonary function test. Respiratory disease is also one of the greatest causes for morbidity and mortality on the Hopi Nation, but no specific reference equations exist for this unique population. The purpose of this study was to determine if population reference equations were necessary for these children and, if needed, to create new age and race-specific pulmonary nomograms for Hopi children. Two hundred and ninety-two healthy children, ages 4-13 years, attending Hopi Nation elementary schools in Arizona, were asked to perform spirometry for a full battery of pulmonary volumes and capacities of which the following were analyzed: forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1), FEV1 % (FEV1/FVC), FEF25-75% and peak expiratory flow rate (PEFR). Spirometric data from Navajo children living in the same geographical region as the Hopi children were compared as well as spirometric data from common reference values used for other ethnic groups in the USA. Spirometry tests from 165 girls and 127 boys met American Thoracic Society quality control standards. We found that the natural log of height, body mass and age were significant predictors of FEV1, FVC, and FEF25-75% in the gender-specific models and that lung function values all increased with height and age as expected. The predictions using the equations derived for Navajo, Caucasian, Mexican-American, African-American youth were significantly different (P ≤ 0.05) from the predictions derived from the Hopi equations for all of the variables across both genders, with the exceptions of Hopi versus Navajo FEV1/FVC in the males and Hopi versus Caucasians FEF25-75% in the females. Thus it would appear for this population important to have specific formulae to provide more accurate reference values.

Spirometry is the most important tool in diagnosing pulmonary disease and is the most frequently performed pulmonary function test. Since respiratory disease is the single greatest cause for morbidity and mortality on the Navajo Nation, the purpose of this study was to create newage and race-specific pulmonary nomograms for Navajo children. Five hundred fifty-eight healthy children, ages 6-14 years, attending Navajo Nation elementary schools in Arizona, were asked to perform spirometry to develop population-specific and tribe-specific nomograms for forced vitalcapacity (FVC), forced expiratory volume in 1 sec (FEV1), and FEV1 Ratio (FEV1/FVC). Spirometry tests from 284 girls and 274 boys met American Thoracic Society quality control standards. Lung function values, except for FEV1/FVC, all increased with height. The lower limit of the normal range for FEV1/FVC was 80%. The spirometry reference equations from the healthyboys and girls were developed. Height and the natural log of height were significant predictors of FEV1, FVC, and FEF25-75% in the gender-specific models. The resulting population-specific spirometry reference equations should be used when testing Navajo children ages 6-14 years. However, the use of the NHANES III spirometry reference equations for Caucasian children may not result in significant misclassification in clinical settings providing that a maximal effort is given by the Navajo child being tested.

Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease with no known cause or cure. IPF has an incidence of 75/1,000,000 of the population, predominately in men over the age of 60. This relatively rare disease develops in a chronic and progressive way, ultimately leading to death within two to five years of diagnosis. Our use of translatable methodologies in the bleomycin mouse model of IPF led to the novel identification of the similarities between the average percent loss of lung function in previous human clinical trials to that of our mouse model data. There is no treatment for IPF outside of lung transplantation, therefore our goal is to develop a protein therapy to halt the progression of IPF. B6_BP_dslf is a small, 93.36 kDa minibinder protein with a nanomolar affinity to αvβ6, an integrin of therapeutic potential for IPF when inhibited by halting αvβ6/TGF-β signaling. Our hypothesis is that B6_BP_dslf will halt the progression of pulmonary fibrosis induced in a mouse model of IPF. To test this hypothesis, a de novo design method was used resulting in the B6_BP_dslf minibinder having high β unit selectivity and nanomolar affinity for αvβ6, and maintenance of its secondary structure after aerosolization. These attributes led to testing in the bleomycin mouse model for IPF as an inhaled therapy. We found that B6_BP_dslf inhalation by mice with induced pulmonary fibrosis had reduced pathogenesis through the quantification of biomarkers for αvβ6/TGF-β mediated fibrosis, lowered histopathological scores, and improved lung function. These positive results from standard biochemical analysis and clinically translatable methods show that BP_B6_dslf has clinical potential as an inhaled therapy for IPF. Additionally, we tested the use of lung function tests in an animal model of chronic obstructive pulmonary disease (COPD), using secondhand smoke exposure to induce the disease and to identify inflammatory pathways. We found that smoke exposure increased inflammatory signaling through receptors for advanced glycation end-products, and inhibition of these receptors using a novel therapy of semi-synthetic glycosaminoglycan ethers (SAGEs) reduced inflammation and improved lung function. Together, the data from two different lung disease models supports the use of lung function as a preclinical efficacy variable for experimental drugs. The combination of biochemical and functional assessments of B6_BP_dslf and SAGEs gives weight to their therapeutic potential.

Introduktion: Spirometri är en av de vanligaste undersökningsmetoderna som utförs vid bedömning av lungfunktion. Vid en dynamisk spirometri mäts lungvolymsförändringar över tid vid detektion av obstruktiva lungsjukdom. Syftet med denna studie var att validera en konventionell spirometriutrustning, Jaeger MasterScreen PFT(Carefusion, Hoechburg, Tyskland), med en ny handhållen utrustning, COPD-6 mätare (Vitalograph COPD-6 400, Ennis, Irland), för att undersöka om det fanns statistisk signifikanta skillnader på mätresultatet mellan dessa två utrustningar.  Metod och material: 22 lungfriska testpersoner mellan åldrarna 20–33 år varav 17 kvinnor och 5 män deltog i denna studie som utfördes vid Örebro universitet. De spirometrivariabler som undersöktes för Jaegerutrustningen var den forcerade exspiratoriska volymen under första sekunden (FEV1), den forcerade vitalkapaciteten (FVC) samt hur stor andel av hela lungvolymen som en person kan andas ut på första sekunden (FEV1/FVC). Med COPD-6 mätaren mättes FEV1, den forcerade exspiratoriska volymen under sex sekunder (FEV6), samt kvoten av dessa (FEV1/FEV6). För att undersöka om det förelåg en statistisk signifikant skillnad användes ett parat t-test med signifikansnivån α=0,05. Resultat: Resultatet från denna studie visade att det förelåg en statistik signifikant skillnad mellan Jaeger MasterScreen PFT och COPD-6 mätaren med p <0,05 för samtliga undersökta variabler. Bland-Altmananalysen visade en spridning inom konfidensintervallet hos samtliga variabler. Slutsats: I denna studie påvisades en statisk signifikant skillnad mellan utrustningarna där COPD-6 mätare visade lägre mätresultat än Jaegerutrustningen för variablerna FEV1 samt FEV6 alternative FVC. Dock kan inte resultatet från denna studie vara representativt för en större population då endast ett få observationer gjordes. / Introduction: Spirometry is one of the most common examination methods performed when assessing lung function. In dynamic spirometry, changes in lung volume are measured over time for the detection of obstructive pulmonary disease. The purpose of this study was to validate a conventional spirometry equipment, Jaeger MasterScreen PFT(Carefusion, Hoechburg, Germany), with a new handheld equipment, COPD-6 screener (Vitalograph COPD-6 400, Ennis, Irland), to investigate whether there were statistically significant differences in the measurement result between these two equipments. Method and material: 22 lung-healthy test subjects between the ages of 20–33 years of whom 17 women and 5 men, participated in this study, which was conducted at Örebro University. The spirometry variables examined for Jaeger equipment were the forced expiratory volume during the first second (FEV1), the forced vital capacity (FVC) and the percentage of total lung volume that a person can exhale at the first second (FEV1 / FVC). With the COPD-6 screener FEV1, forced expiratory volume for six seconds (FEV6), and the ratio of these (FEV1 / FEV6) were measured. To investigate whether there was a statistically significant difference, a paired t-test with the significance level α = 0.05 was used. Results: The results from this study showed that there was a statistically significant difference between the Jaeger MasterScreen PFT and the COPD-6 screener with p <0.05 for all variables examined. The Bland-Altman analysis showed a spread within the confidence interval for all variables. Conclusion: In this study, a statically significant difference was demonstrated between the equipments where COPD-6 screener showed lower measurement results than the Jaeger equipments for the variables FEV1 and FEV6 alternative FVC. However, the results from this study cannot be representative of a larger population as only a few observations were made.

With the rapid somatic growth and development in infants, simultaneous accurate measurements of lung volume and airway function are essential. Raised volume rapid thoracoabdominal compression (RTC) is widely used to generate forced expiration from an airway opening pressure of 30 cmH2O (V30). The (dynamic) functional residual capacity (FRCdyn) remains the lung volume most routinely measured. The aim of this study was to develop comprehensive integrated spirometry that included all subdivisions of lung volume at V30 or total lung capacity (TLC30). Measurements were performed on 17 healthy infants aged 8.6-119.7 weeks. A commercial system for multiple-breath nitrogen washout (MBNW) to measure lung volumes and a custom made system to perform RTC were used in unison. A refined automated raised volume RTC and the following two novel single maneuvers with dual volume measurements were performed from V30 during a brief post-hyperventilation apneic pause: (1) the passive expiratory flow was integrated to produce the inspiratory capacity (IC) and the static (passive) FRC (FRCst) was estimated by initiating MBNW after end-passive expiration; (2) RTC was initiated late during passive expiration, flow was integrated to produce the slow vital capacity (jSVC) and the residual volume (RV) was measured by initiating MBNW after end-expiration while the jacket (j) was inflated. Intrasubject FRCdyn and FRCst measurements overlapped (p = 0.6420) but neither did with the RV (p < 0.0001). Means (95% confidence interval) of FRCdyn, IC, FRCst, jSVC, RV, forced vital capacity and tidal volume were 21.2 (19.7-22.7), 36.7 (33.0-40.4), 21.2 (19.6-22.8), 40.7 (37.2-44.2), 18.1 (16.6-19.7), 40.7 (37.1-44.2) and 10.2 (9.6-10.7) ml/kg, respectively. Static lung volumes and capacities at V30 and variables from the best forced expiratory flow-volume curve were dependent on age, body length and weight. In conclusion, we developed a comprehensive physiologically integrated approach for in-depth investigation of lung function at V30 in infants.

Introduktion  Genom dynamisk spirometri går det att beräkna kvoten mellan forcerad exspiratorisk volym under en sekund och forcerad vitalkapacitet (FEV1/FVC) där ett värde på ≤0,7 tyder på obstruktiv lungsjukdom. Mobil spirometriutrustning skulle kunna möjliggöra tidigare diagnostisering av obstruktiva lungsjukdomar men även fungera som ett kostnadseffektivt alternativ vid enklare undersökningar. Syftet med studien var att jämföra resultat erhållna vid dynamisk spirometri utförd på en stationär respektive en mobil spirometriutrustning. Material och metod  I studien deltog totalt 36 försökspersoner. Data samlades in mellan februari-mars 2023 och bestod av FVC och FEV1 erhållna från dynamisk spirometri på Vyntus™ One (stationär spirometriutrustning) och Aiocare™ Vyaire (mobil spirometriutrustning). Ett parat t-test (signifikansnivå 0,05) utfördes för att avgöra om det förelåg någon signifikant systematisk skillnad för data mellan de båda utrustningarna och förekomsten av slumpfel illustrerades med Bland-Altman-diagram. Intraklasskorrelationskoefficient (ICC) beräknades för samtliga variabler. Resultat och slutsats  En mycket god korrelation mellan utrustningarna förelåg med ett ICC&gt;0.9 för samtliga variabler. Ingen systematisk skillnad kunde påvisas för uppmätt FVC, FEV1 eller kvoten FEV1/FVC mellan Vyntus™ One och Aiocare™ Vyaire (p &gt;0,05) och slumpfelet bedömdes acceptabelt. / Introduction  Dynamic spirometry enables calculating the ratio of forced expiratory volume under one second and forced vital capacity (FEV1/FVC) where ≤0,7 suggests obstructive lung disease. Mobile spirometer equipment could assist in earlier detection of obstructive lung diseases, but also function as a cost-efficient alternative. The purpose of the study is to compare results obtained with dynamic spirometry performed on a stationary and a mobile spirometry equipment. Material and method  A total of 36 subjects participated in the study. Data collected between February-March 2023 consisted of FVC and FEV1 obtained from dynamic spirometry on Vyntus™ One (stationary spirometry equipment) and Aiocare™ Vyaire (mobile spirometry equipment). A paired t-test (0,05 level of significance) determined if there was any significant systematic difference between data from the two types of equipment and the magnitude of sampling error was visualized by Bland-Altman plots. Intraclass correlation coefficient (ICC) was calculated for all variables. Result and conclusion  Excellent correlation with ICC&gt;0,9 for all variables. There was no significant difference for measured FVC, FEV1 or FEV1/FVC between Vyntus™ One and Aiocare™ Vyaire (p &gt;0,05) and the sampling error was considered acceptable.