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INTRODUCTION: Among the limited studies on physical exercise interventions in adults with cystic fibrosis (CF), few have specifically addressed the improvement of peripheral muscle strength and body fat-free mass. The aim of this study was to examine the impacts of a remotely supervised, individualized 8-week resistance training program of moderate to high intensity on strength and body composition in these subjects. METHODS: This was a randomized controlled trial performed in adults with CF. The exercise group (EX) performed three 1-h resistance training sessions per week over 8 weeks. The control group (CON) followed the physical activity recommendations of their physician. The main outcomes were muscle strength and body composition, with secondary measures including pulmonary function and quality of life. Two-way repeated measures analysis was used. RESULTS: In 23 participants (age 32.13 ± 7.72 years), the intervention showed a significant beneficial effect on leg press strength, with a large effect size, both in absolute (p = 0.011; η p 2 $$ {\eta}_{\mathrm{p}}^2 $$ = 0.281) and relative (p = 0.007; η p 2 $$ {\eta}_{\mathrm{p}}^2 $$ = 0.310) terms. Large intervention effects were observed on total fat mass (p < 0.001; η p 2 $$ {\eta}_{\mathrm{p}}^2 $$ = 0.415), body adiposity index (p < 0.001; η p 2 $$ {\eta}_{\mathrm{p}}^2 $$ = 0.436), and fat mass index (p < 0.001; η p 2 $$ {\eta}_{\mathrm{p}}^2 $$ = 0.445), all showing reduction in the EX group. In addition, significant large size effects were detected on total fat-free mass (p = 0.046; η p 2 $$ {\eta}_{\mathrm{p}}^2 $$ = 0.177), trunk fat-free mass (p = 0.039; η p 2 $$ {\eta}_{\mathrm{p}}^2 $$ = 0.188), and fat-free mass index (p = 0.048; η p 2 $$ {\eta}_{\mathrm{p}}^2 $$ = 0.174), all favoring exercise. No significant effects were observed on pulmonary function and quality of life. CONCLUSIONS: An 8-week remotely supervised resistance training program, with moderate to high intensity, effectively improved lower limb muscle strength and body composition.
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Fibrosis Quística , Entrenamiento de Fuerza , Adulto , Humanos , Adulto Joven , Fibrosis Quística/terapia , Calidad de Vida , Composición Corporal , Fuerza MuscularRESUMEN
BACKGROUND: Severe pediatric allergic asthma (SPAA) induces a huge economic burden in terms of direct, indirect, and intangible costs. The use of omalizumab for the treatment of these patients has produced a significant improvement in several clinical outcomes, but at the same time, the cost for the management of the disease has also increased. The aim of this report was to evaluate whether the use of omalizumab is cost-effective. METHODS: A sample of 426 children with SPAA from the ANCHORS (Asthma iN CHildren: Omalizumab in Real-life in Spain) study was used to calculate the incremental cost-effectiveness ratio (ICER) for the avoidance of moderate-to-severe exacerbations (MSE) and also for the improvement in childhood Asthma Control Test (c-ACT) or the Asthma Control Questionnaire (ACQ5). We retrospectively collected data on health encounters and drug consumption before and up to 6 years after the beginning of the treatment with omalizumab. RESULTS: The ICER per avoided MSE was 2107 after 1 year, and it consistently decreased to 656 in those followed up to 6 years. Similarly, the ICER for the minimally important difference in control tests showed a decrease from 2059 to 380 per each 0.5 points of improvement in ACQ5 and from 3141 to 2322 per each 3 points improvement in c-ACT, at years 1 and 6, respectively. CONCLUSION: The use of OMZ is a cost-effective option for most children with uncontrolled SPAA, especially those who have frequent exacerbations; the costs are progressively reduced in successive years of treatment.
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Antiasmáticos , Asma , Humanos , Niño , Omalizumab/uso terapéutico , Análisis Costo-Beneficio , Antiasmáticos/uso terapéutico , España , Estudios Retrospectivos , Asma/terapia , Resultado del Tratamiento , Calidad de VidaRESUMEN
BACKGROUND: Various studies have assessed omalizumab outcomes in the clinical practice setting but follow-up and/or number of patients included were limited. We aim to describe the long-term outcomes of pediatric patients with severe persistent allergic asthma receiving omalizumab in the largest real-life cohort reported to date. METHODS: ANCHORS was a multicenter, observational, retrospective cohort study conducted in 25 Pediatric Allergy and Pulmonology units in Spain. We collected data of patients < 18 years and initiating omalizumab between 2006 and 2018, from the year prior to omalizumab initiation to discontinuation or last available follow-up. The primary outcome was the evolution of the annual number of moderate-to-severe exacerbations compared with the baseline period. RESULTS: Of the 484 patients included, 101 (20.9%) reached 6 years of treatment. The mean ± standard deviation number of exacerbations decreased during the first year of treatment (7.9 ± 6.6 to 1.1 ± 2.0, P < .001) and remained likewise for up to 6 years. The other clinical parameters assessed also improved significantly during the first year and stabilized or continued to improve thereafter. The percentage of patients experiencing adverse events was consistently low, and the main reason for discontinuation was good disease evolution. CONCLUSION: In this large, long-term, observational study, moderate-to-severe exacerbations decreased significantly from the first year of treatment with omalizumab. The beneficial effect was maintained in the long term, along with a good safety profile. Our results position omalizumab as an effective long-term treatment in pediatric patients with severe persistent allergic asthma.
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Antiasmáticos/uso terapéutico , Anticuerpos Monoclonales Humanizados/uso terapéutico , Asma , Omalizumab/uso terapéutico , Antiasmáticos/efectos adversos , Anticuerpos Monoclonales Humanizados/efectos adversos , Asma/tratamiento farmacológico , Niño , Humanos , Omalizumab/efectos adversos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
INTRODUCTION: Exercise intolerance is common in chronic airway diseases (CAD), but its mechanisms are still poorly understood. The aim of this study was to evaluate exercise capacity and its association with lung function, ventilatory limitation, and ventilatory efficiency in children and adolescents with cystic fibrosis (CF) and asthma when compared to healthy controls. METHODS: Cross-sectional study including patients with mild-to-moderate asthma, CF and healthy children and adolescents. Anthropometric data, lung function (spirometry) and exercise capacity (cardiopulmonary exercise testing) were evaluated. Primary outcomes were peak oxygen consumption (VO2 peak), forced expiratory volume in 1 s (FEV1 ), breathing reserve (BR), ventilatory equivalent for oxygen consumption (VE /VO2 ) and for carbon dioxide production (VE /VCO2 ), both at the ventilatory threshold (VT1 ) and peak exercise. RESULTS: Mean age of 147 patients included was 11.8 ± 3.0 years. There were differences between asthmatics and CF children when compared to their healthy peers for anthropometric and lung function measurements. Asthmatics showed lower VO2 peak when compared to both healthy and CF subjects, although no differences were found between healthy and CF patients. A lower BR was found when CF patients were compared to both healthy and asthmatic. Both CF and asthmatic patients presented higher values for VE /VO2 and VE /VCO2 at VT1 when compared to healthy individuals. For both VE /VO2 and VE /VCO2 at peak exercise CF patients presented higher values when compared to their healthy peers. CONCLUSION: Patients with CF achieved good exercise capacity despite low ventilatory efficiency, low BR, and reduced lung function. However, asthmatics reported reduced cardiorespiratory capacity and normal ventilatory efficiency at peak exercise. These results demonstrate differences in the mechanisms of ventilatory limitation to maximum exercise testing in children and adolescents with CAD.
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Asma , Fibrosis Quística , Humanos , Adolescente , Niño , Anciano de 80 o más Años , Estudios Transversales , Volumen Espiratorio Forzado , Consumo de Oxígeno , Prueba de Esfuerzo/métodos , Tolerancia al EjercicioRESUMEN
INTRODUCTION: Elexacaftor/tezacaftor/ivacaftor (ETI) was used through the early access programme in Spain from December 2019 in cystic fibrosis (CF) patients with homozygous or heterozygous F508del mutation with advanced lung disease. METHODOLOGY: Multicentre, ambispective, observational, study in which 114 patients in follow-up in 16 national CF units were recruited. Clinical data, functional tests, nutritional parameters, quality of life questionnaires, microbiological isolates, number of exacerbations, antibiotic treatments and side effects were collected. The study also compared patients with homozygous and heterozygous F508del mutations. RESULTS: Of the 114 patients, 85 (74.6%) were heterozygous for F508del mutation, and the mean age was 32.2±9.96 years. After 30 months of treatment, lung function measured by FEV1% showed improvement from 37.5 to 48.6 (p<0.001), BMI increased from 20.5 to 22.3 (p<0.001), and all isolated microorganisms decreased significantly. The total number of exacerbations was also significantly reduced from 3.9 (±2.9) to 0.9 (±1.1) (p<0.001). All items in the CFQ-R questionnaire showed improvement, except for the digestive domain. Oxygen therapy use decreased by 40%, and only 20% of patients referred for lung transplantation remained on the active transplant list. ETI was well-tolerated, with only 4 patients discontinuing treatment due to hypertransaminemia. CONCLUSIONS: ETI decreases the number of exacerbations, increases lung function and nutritional parameters, decrease in all isolated microorganisms, for 30 months of treatment. There is an improvement in the CFQ-R questionnaire score except for the digestive item. It is a safe and well-tolerated drug.
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Fibrosis Quística , Adulto , Humanos , Adulto Joven , Aminofenoles/uso terapéutico , Aminofenoles/efectos adversos , Fibrosis Quística/tratamiento farmacológico , Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/efectos adversos , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/uso terapéutico , Mutación , Calidad de VidaRESUMEN
INTRODUCTION: The association between viral infections and pulmonary exacerbations in children with cystic fibrosis (cwCF) is well established. However, the question of whether cwCF are at a higher risk of COVID-19 or its adverse consequences remains controversial. METHODS: We conducted an observational, multicenter, cross-sectional study of cwCF infected by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) between March 2020 and June 2022, (first to sixth COVID-19 pandemic waves) in Spain. The study aimed to describe patients' basal characteristics, SARS-CoV-2 clinical manifestations and outcomes, and whether there were differences across the pandemic waves. RESULTS: During study time, 351 SARS-CoV2 infections were reported among 341 cwCF. Median age was 8.5 years (range 0-17) and 51% were female. Cases were unevenly distributed across the pandemic, with most cases (82%) clustered between November 2021 and June 2022 (sixth wave, also known as Omicron Wave due to the higher prevalence of this strain in that period in Spain). Most cwCF were asymptomatic (24.8%) or presented with mild Covid-19 symptoms (72.9%). Among symptomatic, most prevalent symptoms were fever (62%) and increased cough (53%). Infection occurring along the sixth wave was the only independent risk factor for being symptomatic. Just eight cwCF needed hospital admission. No multisystem inflammatory syndrome, persisting symptoms, long-term sequelae, or deaths were reported. CONCLUSIONS: Spanish current data indicate that cwCF do not experience higher risks of SARS-CoV-2 infection nor worse health outcomes or sequelae. Changes in patients' basal characteristics, clinical courses, and outcomes were detected across waves. While the pandemic continues, a worldwide monitoring of COVID-19 in pediatric CF patients is needed.
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COVID-19 , Fibrosis Quística , Humanos , Niño , Femenino , Recién Nacido , Lactante , Preescolar , Adolescente , Masculino , COVID-19/complicaciones , COVID-19/epidemiología , SARS-CoV-2 , Estudios Transversales , España/epidemiología , Pandemias , Fibrosis Quística/complicaciones , Fibrosis Quística/epidemiología , ARN ViralRESUMEN
INTRODUCTION: Development of cystic fibrosis-related diabetes (CFRD) is associated with worsening of nutritional status and lung function, as well as increased mortality. The relevance of diagnosing the «pre-diabetic¼ status in these patients has not been addressed and the utility of HbA1c measurement in these patients is under discussion. AIM: To study and characterise the different categories of carbohydrate metabolism impairment in paediatric patients with cystic fibrosis. PATIENTS AND METHODS: A transversal study for characterisation of carbohydrate metabolism impairment according to clinical and anthropometric status and genetic background in 50 paediatric patients with cystic fibrosis (CF) was undertaken. Oral glucose tolerance tests (OGTT) for determination of glucose and insulin levels measurement and continuous subcutaneous glucose monitoring (CSGM) were performed. RESULTS: 6% of patients presented with CFRD, 26% impaired glucose tolerance, 10% an indeterminate glucose alteration and 2% impaired fasting glucose. The severity of glycaemic impairment correlated positively with age and negatively with standardised height (pâ¯<â¯0.05) with intergroup differences in HbA1c levels (pâ¯<â¯0.01), with the latter correlating with the duration of hyperglycaemia throughout CSGM. No intergroup differences in mutation prevalence, pulmonary function test, nutritional status or disease exacerbations in the previous year were found. The daily enzyme replacement dose correlated with the glucose area under the curve (AUC, pâ¯<â¯0.05) but not with insulin-AUC. CONCLUSIONS: An older age and greater enzyme replacement need are correlated with more severe carbohydrate metabolism impairment and lower standardized height in paediatric CF patients, with HbA1c correlating with the duration of hyperglycaemia. The study of the full glucose/insulin AUCs throughout the OGTT affords no additional information compared to glucose determination at 120â¯min in these patients.
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Fibrosis Quística , Diabetes Mellitus , Intolerancia a la Glucosa , Estado Prediabético , Humanos , Adolescente , Niño , Fibrosis Quística/complicaciones , Glucemia/metabolismo , Automonitorización de la Glucosa Sanguínea , Hemoglobina Glucada , Intolerancia a la Glucosa/diagnóstico , Insulina , Diabetes Mellitus/diagnóstico , Metabolismo de los Hidratos de CarbonoRESUMEN
BACKGROUND: Evidence on resistance-training programs for cystic fibrosis (CF) is limited and the possible benefits of the addition of neuromuscular electrical stimulation (NMES) are unknown. This study aimed to evaluate the effects of a supervised resistance-training program, associated or not with NMES, on muscle strength, aerobic fitness, lung function and quality of life in children with CF presenting mild-to-moderate pulmonary impairment. METHODS: This is a randomized controlled trial including CF patients aged between 6 and 17years. Subjects were randomly allocated to control (CON); exercise (EX); or exercise and NMES (EX + NMES) groups, and evaluated at baseline and at the end of an 8-week individualized exercise-program (3 days/week, 60min/session). NMES was applied in the quadriceps and the interscapular region, simultaneously to the exercises. CON group followed the CF team recommendations. The main outcome measures were lung function, cardiorespiratory fitness, functional capacity, quality of life and muscle strength. RESULTS: Twenty-seven patients, aged 12.6 ± 3.0 years, were analyzed. No significant interactions were found for cardiorespiratory fitness. Functional capacity presented significant differences, indicating a better performance in both EX and EX + NMES. No significant changes between groups were seen for quality of life and lung function. As for muscle strength, EX and EX + NMES presented large effect sizes and significant differences, compared to CON, for quadriceps (p = 0.004, η2p = 0.401), pectoral (p = 0.001, η2p = 0.487), dorsal (p = 0.009, η2p = 0.333) and handgrip (p = 0.028, η2p = 0.278). CONCLUSION: A resistance exercise-training program led to improvements in muscle strength and functional capacity in CF patients with mild-to-moderate pulmonary impairment. The addition of NMES to the training program resulted in no extra favorable effects.
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Capacidad Cardiovascular , Fibrosis Quística , Terapia por Estimulación Eléctrica , Adolescente , Niño , Fibrosis Quística/terapia , Terapia por Estimulación Eléctrica/métodos , Fuerza de la Mano , Humanos , Pulmón , Fuerza Muscular/fisiología , Músculo Cuádriceps , Calidad de VidaRESUMEN
BACKGROUND: Children's diffuse lung disease, also known as children's Interstitial Lung Diseases (chILD), are a heterogeneous group of rare diseases with relevant morbidity and mortality, which diagnosis and classification are very complex. Epidemiological data are scarce. The aim of this study was to analyse incidence and prevalence of chILD in Spain. METHODS: Multicentre observational prospective study in patients from 0 to 18 years of age with chILD to analyse its incidence and prevalence in Spain, based on data reported in 2018 and 2019. RESULTS: A total of 381 cases with chILD were notified from 51 paediatric pulmonology units all over Spain, covering the 91.7% of the paediatric population. The average incidence of chILD was 8.18 (CI 95% 6.28-10.48) new cases/million of children per year. The average prevalence of chILD was 46.53 (CI 95% 41.81-51.62) cases/million of children. The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2-18 years of age compared with children 0-2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (17/65), neuroendocrine cell hyperplasia of infancy in infants from 1 to 12 months (44/144), idiopathic pulmonary haemosiderosis in children from 1 to 5 years old (13/74), hypersensitivity pneumonitis in children from 5 to 10 years old (9/51), and scleroderma in older than 10 years old (8/47). CONCLUSIONS: We found a higher incidence and prevalence of chILD than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.
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OBJECTIVE: Asthma is a chronic disease that may affect physical fitness, although its primary effects on exercise capacity, muscle strength, functionality and lifestyle, in children and adolescents, are still poorly understood. This study aimed to evaluate the differences in cardiorespiratory fitness, muscle strength, lifestyle, lung function, and functionality between asthmatics with exercise symptoms and healthy children. In addition, we have analyzed the association between clinical history and the presence of asthma. STUDY DESIGN: Cross-sectional study including 71 patients with a diagnosis of asthma and 71 healthy children and adolescents (7-17 years of age). Anthropometric data, clinical history, disease control, lifestyle (KIDMED and physical activity questionnaires), lung function (spirometry), exercise-induced bronchoconstriction test, aerobic fitness (cardiopulmonary exercise test), muscle strength and functionality (timed up and go; timed up and down stairs) were evaluated. RESULTS: Seventy-one patients with asthma (mean age 11.5 ± 2.7) and 71 healthy subjects (mean age 10.7 ± 2.5) were included. All asthmatic children had mild to moderate and stable asthma. EIB occurred in 56.3% of asthmatic children. Lung function was significantly (p < .05) lower in the asthmatic group when compared to healthy peers, as well as the cardiorespiratory fitness, muscle strength, lifestyle and functionality. Moreover, asthmatic children were more likely to have atopic dermatitis, allergic reactions, food allergies, and a family history of asthma when compared to healthy children. CONCLUSIONS: Children with mild-to-moderate asthma presenting exercise symptoms show a reduction in cardiorespiratory fitness, muscle strength, lung function, functionality, and lifestyle when compared to healthy peers. The study provides data for pediatricians to support exercise practice aiming to improve prognosis and quality of life in asthmatic children.
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Asma , Calidad de Vida , Adolescente , Asma/epidemiología , Niño , Estudios Transversales , Ejercicio Físico , Prueba de Esfuerzo , Humanos , Aptitud FísicaRESUMEN
Background: Cystic fibrosis (CF) affects the autonomic nervous system (ANS) and exercise in healthy children modulates the interaction between sympathetic and parasympathetic activity. This study aimed to evaluate the effects of a short-term resistance exercise program on heart rate variability (HRV) in children and adolescents with CF. Methods: A randomized controlled trial was carried out in children diagnosed with CF aged 6-18 years. Individuals were divided into two groups: control (CON) and resistance-training (EX). Individuals in the EX group completed an individualized guided resistance program (5-RM-60-80%) for 8 weeks (3 sessions of 60 min/week). Upper and lower limbs exercises (seated bench press, seated lateral row, and leg press) were used. HRV was measured using a Suunto watch with subjects in lying position. Results: Nineteen subjects (13 boys) were included (CON = 11; and EX = 8). Mean age was 12.2 ± 3.3, FEV1 (forced expiratory volume in the first second) z-score was 1.72 ± 1.54 and peak oxygen consumption (VO2peak) 42.7 ± 7.4 mL.Kg-1.min-1. Exercise induced significant changes in the frequency-domain variables, including a decrease in LF power (p = 0.001, d = 0.98) and LF/HF ratio (p = 0.020, d = 0.92), and an increase in HF power (p = 0.001, d = -0.97), compared to the CON group. No significant changes were found for time-domain variables, although increases with a moderate effect size were seen for SDNN (p = 0.152, d = -0.41) and RMSSD (p = 0.059, d = -0.49) compared to the CON group. Conclusion: A short-term resistance exercise-training program was able to modulate HRV in children and adolescents with CF presenting mild to moderate lung function impairment and good physical condition. Clinical Trial Registration: www.ClinicalTrials.gov, identifier NCT04293926.
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BACKGROUND: Asthmatic patients may benefit from exercise training, although the effects of a combined aerobic and resistance training program are still poorly investigated in children and adolescents. OBJECTIVE: To analyze the effects of a combined exercise training (resistance and aerobic) program on aerobic fitness, lung function, asthma control and quality of life in a group of mild-moderate asthmatic children with exercise symptoms. METHODS: This was a 12-week randomized controlled trial including children and adolescents diagnosed with mild-moderate asthma and presenting exercise-induced symptoms. The intervention group (IG) performed the exercise training (resistance and aerobic) 3 days/week, for 60 minutes. The control group (CG) followed routine clinical orientations. The main outcomes were cardiorespiratory fitness, muscle strength, lung function, quality of life, asthma control, and functional tests after 3 months of the intervention. RESULTS: Fifty-three patients (IG = 25 and CG = 28) with a mean age of 11.5 ± 2.6 years were included. No significant differences were found between groups regarding lung function, asthma control, quality of life, and functional tests. Ventilatory equivalent for oxygen consumption at ventilatory threshold (P = .025; ηp2 = 0.083), peak oxygen consumption (P = .008; ηp2 = 0.116) and test duration (P = .014; ηp2 = 0.1) presented greater improvements in the IG. In addition, improvements were observed in leg press (P < .001; ηp2 = 0.36), hamstring curl (P = .001; ηp2 = 0.217), high row (P = .003; ηp2 = .167), low row (P = .009; ηp2 = 0.128) and quadriceps leg extension (P = .015; ηp2 = 0.108) in the IG. CONCLUSION: Combined exercise training (resistance and aerobic) improved cardiorespiratory fitness and muscle strength in children and adolescents with controlled asthma and exercise symptoms.
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Asma/terapia , Terapia por Ejercicio , Adolescente , Asma/fisiopatología , Niño , Ejercicio Físico/fisiología , Femenino , Humanos , Masculino , Fuerza Muscular , Aptitud Física/fisiología , Calidad de Vida , Pruebas de Función RespiratoriaAsunto(s)
Bronquiectasia , Sistema de Registros , Humanos , España/epidemiología , Bronquiectasia/epidemiología , Niño , Preescolar , AdolescenteRESUMEN
INTRODUCTION: Pulmonary function tests allow an objective assessment of the degree of bronchial obstruction in collaborative subjects. The measurement of airway resistance using passive methods is very helpful in non-collaborative subjects. The objective of this study was to assess the applicability of measuring airway resistance by the interrupter technique (Rint) in pediatric subjects, determining its reproducibility, reliability and accuracy versus other techniques to measure airway resistance. MATERIAL AND METHODS: Cross-sectional study in healthy children and in children with an obstructive airway disease, all aged 2-18 years old. The Rint was measured using a portable device and results were compared to airway resistance measured by oscillometry and plethysmography. The reproducibility of measurements and the infuence of the different outcome measures (use of mask or mouthpiece, cheek support, or nose clip) were assessed. RESULTS: Valid measurements were obtained in 82.6% of 460 children (47.6% younger than 7 years old). Reproducibility was very good (ICC= 0.9412; p <0.00001), and no differences were found among the measurements obtained at separate time intervals (0.75 ± 0.3 versus 0.74 ± 0.28; p= 0.435). None of the factors indicated before had an effect on the reproducibility of measurements. Resistance values obtained by plethysmography and oscillometry were higher than those obtained using the Rint, with a positive correlation between them. The higher the degree of airway obstruction, the worse the correlation with plethysmography. CONCLUSIONS: The Rint measurement is a plausible and reproducible technique, and has an adequate correlation with the resistance measurements obtained using oscillometry or plethysmography, thus making it useful for non-collaborative patients. In patients with airway obstruction, this technique could underestimate resistance, so it would be a hurdle to use it to follow-up subjects with a moderate to severe obstructive disease or in bronchial challenge tests.
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Resistencia de las Vías Respiratorias/fisiología , Adolescente , Asma/fisiopatología , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Masculino , Reproducibilidad de los Resultados , Pruebas de Función Respiratoria/métodosRESUMEN
Cystic adenomatoid malformation of the lung is a rare malformation of the lung airway which often performed diagnosed in the prenatal period by ultrasound. Ultrasound monitoring should be performed during pregnancy to assess lung development. We report the case of a 4-year-old patient with prenatal diagnosis of cystic adenomatoid malformation of the lung, not confirmed by chest radiograph at birth. The patient underwent surgery at 4 years of age after diagnosis was made for presenting recurrent pneumonia. A normal chest radiograph at birth does not exclude this malformation and a computerized tomography at 4 weeks of birth must be done to confirm or rule out this anomaly. Once the diagnosis is made, surgical treatment should be prompted to avoid complications.
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Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico por imagen , Ultrasonografía Prenatal , Preescolar , Femenino , Humanos , EmbarazoRESUMEN
Introducción. Las pruebas de función pulmonar permiten objetivar el grado de obstrucción bronquial en sujetos colaboradores. La medición de las resistencias pulmonares por métodos pasivos es muy útil en sujetos no colaboradores. El objetivo de este estudio fue evaluar la aplicabilidad de la medición de resistencias por interrupción (Rint) en la edad pediátrica, valorando su reproducibilidad, fabilidad y exactitud en relación con otras técnicas de medición de resistencias. Material y métodos. Estudio transversal en niños de entre 2 y 18 años, sanos y con patología obstructiva. Se midió la Rint con un aparato portátil y se comparó con las resistencias medidas por oscilometría y pletismografía. Se valoró la reproducibilidad de las mediciones y la infuencia de diferentes variables (uso de mascarilla o boquilla, sujeción de mejillas o uso de pinza nasal). Resultados. De 460 niños (47,6% menores de 7 años) se obtuvieron medidas válidas en el 82,6%. La reproducibilidad fue muy buena (CCI= 0,9412; p < 0,00001), sin encontrar diferencias entre las medidas obtenidas separadas en el tiempo (0,75 ± 0,3 contra 0,74 ± 0,28; p= 0,435). Ninguno de los factores antes mencionados afectó la reproducibilidad de las medidas. Los valores de resistencia obtenidos por pletismografía y oscilometría fueron superiores a los obtenidos por Rint, con una correlación positiva entre ambos. La correlación con la pletismografía disminuía cuanto mayor era el grado de obstrucción. Conclusiones. La medición de Rint es factible y reproducible, y se correlaciona bien con las resistencias medidas por oscilometría o pletismografía, por lo que sería de utilidad en los pacientes poco colaboradores. En los pacientes obstruidos, podría infraestimar la resistencia, difcultando su uso para el seguimiento de una patología obstructiva moderada-grave o en las pruebas de provocación bronquial.
Introduction. Pulmonary function tests allow an objective assessment of the degree of bronchial obstruction in collaborative subjects. The measurement of airway resistance using passive methods is very helpful in non-collaborative subjects. The objective of this study was to assess the applicability of measuring airway resistance by the interrupter technique (Rint) in pediatric subjects, determining its reproducibility, reliability and accuracy versus other techniques to measure airway resistance. Material and Methods. Cross-sectional study in healthy children and in children with an obstructive airway disease, all aged 2-18 years old. The Rint was measured using a portable device and results were compared to airway resistance measured by oscillometry and plethysmography. The reproducibility of measurements and the infuence of the different outcome measures (use of mask or mouthpiece, cheek support, or nose clip) were assessed. Results. Valid measurements were obtained in 82.6% of 460 children (47.6% younger than 7 years old). Reproducibility was very good (ICC= 0.9412; p <0.00001), and no differences were found among the measurements obtained at separate time intervals (0.75 ± 0.3 versus 0.74 ± 0.28; p= 0.435). None of the factors indicated before had an effect on the reproducibility of measurements. Resistance values obtained by plethysmography and oscillometry were higher than those obtained using the Rint, with a positive correlation between them. The higher the degree of airway obstruction, the worse the correlation with plethysmography. Conclusions. The Rint measurement is a plausible and reproducible technique, and has an adequate correlation with the resistance measurements obtained using oscillometry or plethysmography, thus making it useful for non-collaborative patients. In patients with airway obstruction, this technique could underestimate resistance, so it would be a hurdle to use it to follow-up subjects with a moderate to severe obstructive disease or in bronchial challenge tests.
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Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Resistencia de las Vías Respiratorias/fisiología , Asma/fisiopatología , Estudios Transversales , Reproducibilidad de los Resultados , Pruebas de Función Respiratoria/métodosRESUMEN
La malformación adenomatoidea quística pulmonar (MAQP)es una anomalía de la vía aérea pulmonar poco frecuente cuyodiagnóstico suele realizarse en el período prenatal medianteecografía. Durante el embarazo, debe realizarse seguimiento ecográfico para valorar el desarrollo pulmonar. Presentamos el caso clínico de una paciente de 4 años con diagnóstico prenatal de MAQP, no confirmado mediante radiografía de tóraxrealizada al nacimiento, lo cual retrasó el diagnóstico definitivo; fue intervenida con 4 años de edad tras haber presentado varias neumonías a repetición. Una radiografía de tórax normal realizada al nacimiento no descarta la presencia de estamalformación, por lo que es necesario realizar una tomografía computarizada a las 4 semanas del nacimiento para confirmar o descartar la MAQP. Una vez diagnosticada, el tratamiento quirúrgico debe ser precoz para evitar complicaciones.
Cystic adenomatoid malformation of the lung is a rare malformation of the lung airway which often performed diagnosed in the prenatal period by ultrasound. Ultrasound monitoring should be performed during pregnancy to assess lung development. We report the case of a 4-year-old patient with prenatal diagnosis of cystic adenomatoid malformation of the lung, not confirmed by chest radiograph at birth. The patient underwent surgery at 4 years of age after diagnosis was made for presenting recurrent pneumonia. A normal chest radiograph at birth does not exclude this malformation and a computerized tomography at 4 weeks of birth must be done to confirm or rule out this anomaly. Once the diagnosis is made, surgical treatment should be prompted to avoid complications.