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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-diabetic» status in these patients has not been addressed and the utility of HbA1c measurement in these patients is under discussion.To study and characterise the different categories of carbohydrate metabolism impairment in paediatric patients with cystic fibrosis.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.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 (p 0.05) with intergroup differences in HbA1c levels (p 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, p 0.05) but not with insulin-AUC.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 120 min in these patients.

Resumen Introduccion El desarrollo de diabetes mellitus en pacientes afectos de fibrosis quistica (DRFQ) se relaciona con peor estado nutricional y funcion pulmonar y aumento de mortalidad. La relevancia del diagnostico de los estados «prediabeticos» es desconocida, cuestionandose la utilidad clinica de la HbA1c en estos pacientes. Objetivo Estudiar y caracterizar las alteraciones del metabolismo hidrocarbonado en pacientes pediatricos afectos de FQ. Pacientes y metodos Caracterizacion antropometrica, clinica y genetica y estudio de prevalencia de las alteraciones del metabolismo hidrocarbonado en 50 pacientes pediatricos afectos de FQ, mediante determinacion de glucosa e insulina en test de tolerancia oral (TTOG) y monitorizacion continua de glucosa subcutanea (MCGS). Resultados Un 6% de pacientes presentaron DRFQ, 26% intolerancia a los hidratos de carbono, 10% alteracion indeterminada de la glucosa y 2% alteracion de glucemia en ayunas. La gravedad de la alteracion glucemica se correlaciono positivamente con la edad y, negativamente, con la talla de los pacientes (p Conclusiones Una mayor edad y necesidad de aportes enzimaticos se relacionan con mayor afectacion del metabolismo hidrocarbonado y menor talla estandarizada en pacientes afectos de FQ, siendo la HbA1c util en la estimacion del tiempo de hiperglucemia. El estudio del AUC de glucemia/insulinemia no aporta informacion adicional frente a la determinacion de glucemia a los 120 min.

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 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 of health encounters and drug consumption before and up to six years after the beginning of the treatment with omalizumab. RESULTS The ICER per avoided MSE was \euro2,107 after one year, and it consistently decreased to \euro656 in those followed up to six years. Similarly, the ICER for the Minimally Important Difference in control tests showed a decrease from \euro2,059 to \euro380 per each 0.5 points of improvement in ACQ5, and from \euro3,141 to \euro2,322 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, mainly those who have frequent exacerbations, showing progressively reduced costs in successive years of treatment.

The receptor for advanced glycation end products (RAGE) has been studied in several respiratory diseases described as an important inflammatory mediator. The RAGE-axis is activated by multiple endogenous ligands related to pro-inflammatory states, upregulate the RAGE expression. The function of soluble RAGE (sRAGE) is not completely understood, it has been hypothesized an anti-inflamatory role as RAGE decoy receptor. Few studies have explored the RAGE-axis in Cystic Fibrosis (CF) with contradictory results. Based on previously, we present this pilot study with the aim of describe the plasma sRAGE levels in children with cystic CF (CFp), compare with the sRAGE levels in a healthy cohort and study its possible correlation with CFp clinical features. We conducted a single-center, cross-sectional observational study. We included 35 clinically stable CF patients (aged < 18 years). The median plasma sRAGE level in CFp was 1494,75 pg/ml [interquartile range (IQR) 708,75pg/ml], compared with 714,20 pg/ml (IQR 490,50 pg/ml)) in the historical cohort of healthy controls (p < 0,001). A positive correlation was found between plasma sRAGE level and forced expiratory volume in 1 second/forced vital capacity ratio (FEV1/FVC) (p 0,004) and forced expiratory flow between 25% and 75% (FEF25%-75%) (p 0,032). In this preliminary study, the plasma sRAGE level were higher in CFp than in healthy controls. Also, we described a positive correlation between FEV1/FVC and FEF25%-75% and plasma sRAGE. To our knowledge, our study is the largest to describe plasma sRAGE values ​​in CFp and the only one carried out in pediatric CF population.

Introduction. Exercise intolerance is common in chronic respiratory diseases (CRD), 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 (VO peak), forced expiratory volume in 1 second (FEV ), breathing reserve (BR), ventilatory equivalent for oxygen consumption (V /VO ) and for carbon dioxide production (V /VCO ), both at the ventilatory threshold (VT ) 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 VO 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 V /VO and V /VCO at VT when compared to healthy individuals. For both V /VO and V /VCO 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 CRD.

[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., [Antecedentes] Las neumopatías intersticiales pediátricas, también conocidas con el acrónimo chILD (del inglés children's Interstitial Lung Diseases), es un grupo heterogéneo de enfermedades raras con morbimortalidad relevante, cuyo diagnóstico y clasificación son complejos. Los estudios epidemiológicos son escasos. El objetivo de este trabajo fue analizar la incidencia y la prevalencia de chILD en España., [Métodos] Estudio prospectivo observacional multicéntrico en pacientes de 0 a 18 años afectos de chILD para analizar la incidencia y la prevalencia en España, a partir de datos recogidos en 2018 y 2019., [Resultados] Se recogieron 381 casos de chILD entre 51 unidades de neumología pediátrica de toda España, que cubrían el 91,7% de la población pediátrica. La incidencia promedio fue 8,18 (IC 95%: 6,28-10,48) casos nuevos/millón de niños por año. La prevalencia promedio fue de 46,53 (IC 95%: 41,81-51,62) casos/millón de niños. El grupo de edad con mayor prevalencia fue el de niños menores de un año. Se observaron diferentes entidades en niños de 2 a 18 años en comparación con niños de 0 a 2 años. Los diagnósticos más frecuentes fueron: glucogenosis intersticial pulmonar primaria en neonatos (17/65), hiperplasia de células neuroendocrinas en lactantes de uno a 12 meses (44/144), hemosiderosis pulmonar idiopática en niños de uno a 5 años (13/74), neumonía por hipersensibilidad en niños de 5 a 10 años (9/51) y esclerodermia en mayores de 10 años (8/47)., [Conclusiones] Encontramos una mayor incidencia y prevalencia de chILD que las descritas previamente, probablemente debido a un mayor conocimiento y detección de estas enfermedades raras., ATV was supported by a grant from the Spanish Society of Paediatric Pulmonology and a Short Term Scientific Mission of the Cost CA 16125 ENTeR-chILD. This work was supported by a grant from the Spanish Society of Pneumology and Thoracic Surgery (SEPAR 2017/492). AMG was supported by a grant from the project HCQ4Surfdefect, in E-Rare-3, the ERA-Net for Research on Rare Diseases (Acciones complementarias en Salud, Instituto Carlos III, Madrid, Spain, AC16/00027) and Cost CA 16125 ENTeR-chILD.

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.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.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, ηA 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.

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.

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

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

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; = 0.083), peak oxygen consumption (P = .008; = 0.116) and test duration (P = .014; = 0.1) presented greater improvements in the IG. In addition, improvements were observed in leg press (P < .001; = 0.36), hamstring curl (P = .001; = 0.217), high row (P = .003; = .167), low row (P = .009; = 0.128) and quadriceps leg extension (P = .015; = 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. Fundación Prandi XOTRIO1729 3.039 JCR (2020) Q2, 36/129 Pediatrics 0.866 SJR (2020) Q1, 61/301 Pediatrics, Perinatology and Child Health No data IDR 2019 UEM

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 influence 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

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.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.Valid measurements were obtained in 82.6% of 460 children (47.6% younger than 7 years old). Reproducibility was very good (ICC= 0.9412; p0.00001), 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.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.

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.