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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., (Copyright © 2021 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Primary ciliary dyskinesia (PCD) is characterized by an alteration in the ciliary structure causing difficulty in the clearance of respiratory secretions. Diagnosis is complex and based on a combination of techniques. The objective of this study was to design a gene panel including all known causative genes, and to corroborate their diagnostic utility in a cohort of Spanish patients., Methods: This was a multicenter cross-sectional study of patients with a high suspicion of PCD, according to European Respiratory Society criteria, designed around a gene panel for massive sequencing using SeqCap EZ capture technology that included 44 genes associated with PCD., Results: We included 79 patients, 53 of whom had a diagnosis of confirmed or highly probable PCD. The sensitivity of the gene panel was 81.1%, with a specificity of 100%. Candidate variants were found in some of the genes of the panel in 43 patients with PCD, 51.2% (22/43) of whom were homozygotes and 48.8% (21/43) compound heterozygotes. The most common causative genes were DNAH5 and CCDC39. We found 52 different variants, 36 of which were not previously described in the literature., Conclusions: The design and implementation of a tailored gene panel produces a high yield in the genetic diagnosis of PCD. This panel provides a better understanding of the causative factors involved in these patients and lays down the groundwork for future therapeutic approaches., (Copyright © 2020 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Recent publication of multi-ethnic spirometry reference equations for subjects aged from 3-95 years aim to avoid age-related discontinuities and provide a worldwide standard for interpreting spirometric test results., Objectives: To assess the agreement of the Global Lung Function Initiative (GLI-2012) and All ages (FEV 0.5 ) reference equations with the Spanish preschool lung function data. To verify the appropriateness of these reference values for clinical use in Spanish preschool children., Methods: Spirometric measurements were obtained from children aged 3 to 6 years attending 10 randomly selected schools in Barcelona (Spain). Stanojevic's quality control criteria were applied. Z-scores were calculated for the spirometry outcomes based on the GLI equations. If the z-score (mean) of each parameter was close to 0, with a maximum variance of ± 0.5 from the mean and a standard deviation of 1, the GLI-2012 equations would be applicable in our population., Results: Of 543 children recruited, 405 (74.6%) were 'healthy', and of these, 380 were Caucasians. Of these 380, 81.6% (169 females, 141 males) performed technically acceptable and reproducible maneuvers to assess FEVt, and 69.5% achieved a clear end-expiratory plateau. Z-scores for FVC, FEV 1 , FEV 1 /FVC, FEV 0.75 , FEV 0.75 /FVC, FEV 0.5 , FEF 75 and FEF 25-75 all fell within ± 0.5, except for FEV 1 /FVC (0.53 z-scores)., Conclusions: GLI equations are appropriate for Spanish preschool children. These data provide further evidence to support widespread application of the GLI reference equations., (Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Pulmonary hypoplasia is the most frequent congenital anomaly associated with perinatal mortality., Material and Methods: A retrospective and descriptive review was conducted on cases of patients diagnosed with pulmonary hypoplasia between 1995 and 2014 in a tertiary university hospital. An analysis was made of the prenatal imaging, clinical manifestations, post-natal diagnostic tests, treatment and management, long-term follow up, and survival data., Results: A total of 60 cases were identified, all of them with prenatal imaging. Sixteen patients required foetal surgery. Congenital diaphragmatic hernia was the most frequent diagnosis. Main clinical presentation was respiratory distress with severe hypoxemia and high requirements of mechanical ventilation. Mortality rate was 47% within first 60 days of life, and 75% for the first day of life. Pneumonia and recurrent bronchitis episodes were observed during follow-up. They had a lung function obstructive pattern, and their quality of life and exercise tolerance was good., Conclusions: High neonatal mortality and significant long-term morbidity associated with pulmonary hypoplasia requires an early diagnosis and a specialised multidisciplinary team management., (Copyright © 2015 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.)

Pseudomonas aeruginosa is the main pathogen in bronchopulmonary infections in cystic fibrosis (CF) patients. It can only be eradicated at early infection stages while reduction of its bacterial load is the therapeutic goal during chronic infection or exacerbations. Neonatal screening and pharmacokinetic/pharmacodynamic knowledge has modified the management of CF-patients. A culture based microbiological follow-up should be performed in patients with no infection with P.aeruginosa. At initial infection, inhaled colistin (0,5-2MU/tid), tobramycin (300mg/bid) or aztreonam (75mg/tid) with or without oral ciprofloxacin (15-20mg/kg/bid, 2-3weeks) are recommended. In chronic infections, treatment is based on continuous administration of colistin or with a 28-day on-off regimen with tobramycin or aztreonam. During mild-moderate exacerbations oral ciprofloxacin (2-3weeks) can be administered while serious exacerbations must be treated with intravenous combination therapy (beta-lactam with an aminoglycoside or a fluoroquinolone). Future studies will support antibiotic rotation and/or new combination therapies. Epidemiological measures are also recommended to avoid new P.aeruginosa infections and "patient-to-patient transmission" of this pathogen., (Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved.)

Objective: To evaluate bronchial hyperresponsiveness in children under 4 years of age with recurrent wheezing bronchitis, and to determine if its presence or absence can predict the subsequent progression to a transient or persistent wheezing bronchitis phenotype., Population and Methods: A bronchial challenge test was performed with methacholine using a modified tidal volume method, without sedation in a group of patients from 8 to 47 months of age with recurrent wheezing bronchitis and a control group of healthy children. A decrease in oxygen saturation of ≥ 5% or an increase in respiration rate of >50% [PCwheeze (PCw)] was considered a positive response. The patients were subsequently clinically followed up to assess their progress., Results: A total of 63 patients and 16 controls were studied (mean age 23.9 vs. 25.2 months). The PCw was lower than the control group (≤ 4 mg/ml) in 43 (68%) children from the bronchitis group (P<0.001). No significant adverse effects were observed on performing the test. After a mean follow up of 28.5 months, completed in 49 of the patients, no differences were seen between the presence of bronchial hyperresponsiveness at the beginning of the study and the subsequent progression to transient, infrequent and frequent wheezing (P=0.63)., Conclusions: A high percentage of children under 4 years of age affected by wheezing bronchitis had a bronchial hyperresponse. The subsequent progression to transient or persistent wheezing bronchitis phenotype is not associated with bronchial hyperresponsiveness., (Copyright © 2010 SEPAR. Published by Elsevier Espana. All rights reserved.)

Objective: To compare low and high flow nebulizers performance (total of samples) and its side effects on sputum induction in asthmatic children., Patients and Methods: Sputum induction was performed by inhalation of a hypertonic saline solution at increasing concentrations (3%, 4% and 5%) using low flow (OMRON NE-U07; flow rate 1ml/min), or high flow (OMRON NE-U12; flow rate 3ml/min, and DeVilbiss Ultraneb 3000; flow rate 2.5ml/min) ultrasonic nebulizers., Results: We performed 49 inductions in 49 patients from 7 to 15 years old (in 15 children we used a low flow nebulizer (Omron NE-U07) and in 34 children a high flow nebulizer (OMRON NEU12, 6 patients, and DeVilbiss Ultraneb 3000, 28 patients). We obtained 37 samples of which 36 had less than 20% of squamous cells, and 26 had a viability > or =60%. The test performance was higher with high-flow nebulizers, obtaining 85.3% of samples compared to 53% (p=0.04). A total of 69% of samples obtained with the high flow nebulizer were valid, compared to 62.5% (p=0.7) with the low flow nebulizers. With high flow rate nebulizers the incidence of cough (17.6%, p=0.08) and itchy eyes (0%, p=0.02) decreased with the low flow nebulizer (47% and 20% respectively), but bad taste (82.3%, p <0.001) and salivation (14.7%, p=0.3) increased., Conclusions: With high flow rate ultrasonic nebulizers we obtain a higher performance of the technique without an increase in significant side effects., (2009 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)

Background: The objective of the study was to assess bronchial inflammation in preschool children with recurrent bronchitis by measuring exhaled nitric oxide., Patients and Methods: The study included patients under 4 years of age with at least 3 episodes of wheezing in the past year (n=63) and a control group (n=30). Exhaled nitric oxide was measured in samples collected offline during spontaneous tidal breathing with a face mask and stored in Mylar balloons., Results: The fractional exhaled nitric oxide concentration (FE(NO)) was higher in the group with bronchitis (mean [SD], 5.3 [1.3] parts per billion [ppb]) than in the control group (4.6 [1.1]ppb) (P=.02). There was a significant difference between the control group and children in the bronchitis group not treated with inhaled corticosteroids (P<.05), but not between controls and corticosteroid-treated patients. A relationship with eosinophil count was observed in that those with higher counts (>400 microL) had higher FE(NO) levels (P<.01). No relationship was observed between FE(NO) and a positive methacholine challenge test. Follow-up lasted at least 20 months. The initial FE(NO) level did not differ significantly according to whether patients were subsequently transient, infrequent, or frequent wheezers (5.2 [0.98]ppb, 5.6 [1.5]ppb, and 4.8 [1.34]ppb, respectively; P=.36)., Conclusions: In children under 4 years of age with recurrent wheezing bronchitis who were asymptomatic at study entry, a small increase in FE(NO) was observed although there was a good deal of overlap with the control group.

The aspiration of lipoid material following the accidental ingestion of lipid formulations is the most frequent cause of exogenous lipoid pneumonia in paediatrics. The presence of cough, increasing dyspnea and chest pain, together with alveolar infiltrates in the chest radiography and the previous accidental intake of a lipid substance and vomiting should make us suspect this diagnosis. We present two cases of aspiration lipoid pneumonia in paediatric patients, with a different clinical presentation and radiological outcome, pointing out in one of them the appearance of pneumatoceles as a consequence of aspiration.

This article is an academic review of the application in children of the measurement of fractional exhaled nitric oxide (FENO). We outline the joint American Thoracic Society/European Respiratory Society recommendations for online measurement of FENO in both cooperating children and children unable to cooperate, offline measurement with uncontrolled exhalation flow rate, offline measurement with controlled exhalation flow rate using a dynamic flow restrictor, and offline measurement during tidal breathing in children unable to cooperate. This is followed by a review of the normal range of values for single-breath online measurements obtained with a chemiluminescence FENO analyzer (geometric mean, 9.7 parts per billion [ppb]; upper limit of the 95% confidence interval, 25.2 ppb). FENO values above 17 ppb have a sensitivity of 81% and a specificity of 80% for predicting asthma of an eosinophilic phenotype. We discuss the response of FENO values to anti-inflammatory treatment and the use of this marker in the management of asthma. Results obtained with chemiluminescence and portable electrochemical analyzers are compared. The portable devices offer the possibility--in children over 5 years of age--of accurate and universal monitoring of exhaled nitric oxide concentrations, an emerging marker of eosinophilic inflammation in asthma that facilitates diagnosis, monitoring of disease progression, and assessment of response to therapy.

Objective: To assess the safety of the tracheal auscultation method for measuring bronchial hyperresponsiveness in healthy unsedated children aged less than 4 years and to establish a range of normal bronchial hyperresponsiveness values., Population and Methods: The study population consisted of healthy children aged between 6 months and 4 years. A methacholine bronchial provocation test was administered to unsedated children, using the tidal volume breathing technique and applying an abbreviated protocol. The test was considered positive when wheezing was heard in the trachea, arterial oxygen saturation (SaO2) fell by 5% or more, or respiratory rate increased by 50% or more., Results: A total of 16 children were studied. Ages ranged from 8 to 47 months, with a mean (SD) of 23.5 (12.2) months. There was no response to the methacholine in 11 children. In the other 5 children, there was a positive response at a concentration of 8 mg/mL. Response to the test was considered positive on the basis of tracheal wheezing in 3 cases, tracheal wheezing and a fall in SaO2 in 1 case, and a fall of SaO2 of 5% or more in 1 case. SaO2 never fell below 93%., Conclusions: As a means for assessing bronchial hyperresponsiveness, the tracheal auscultation method is appropriate, is simple to apply, and can be safely administered to unsedated children aged less than 4 years. The lowest concentration at which a response to methacholine occurs in healthy children of this age group is 8 mg/mL.

Objectives: To assess the efficacy and tolerability of oral deflazacort versus oral prednisolone in acute moderate asthma in children., Patients and Methods: We performed a prospective, randomized, parallel group trial of children aged 6 to 14 years old with a diagnosis of asthma who presented to the pediatric emergency department for moderate asthma exacerbation. All patients were administered short-acting beta2-adrenergic agonists. The intervention groups received either oral deflazacort (1.5 mg/kg) or prednisolone (1 mg/kg) for 7 days. The primary outcome measure was forced expiratory volume in 1 second (FEV1) and secondary outcome measures were pulmonary symptom score index, peak expiratory flow rate (PEFR), hospitalization rate and the use of rescue beta2-agonists. Patients were evaluated at the start of treatment (visit 1), on day 2 (visit 2) and on day 7 (visit 3)., Results: Of the 54 children enrolled, two were hospitalized on visit 2 (one from each group). Baseline clinical data were similar in both groups: FEV1: 53 and 51 %; bronchodilator test: 119 and 121 %; PEFR: 169 and 165 L/min; symptom score: 6 and 6.5 for the deflazacort and prednisolone groups, respectively. On visit 2, all measures improved: FEV1: 122.2 and 126.5 % (p < 0.05); PEFR: 164 and 149 L/min (p < 0.05); symptom score: -4.4 and -3.8 (p < 0.05), without significant differences between groups. On visit 3 all variables continued to show improvement: FEV1: 133.2 and 132.5 % (p < 0.05); PEFR: 1115.7 and 187.6 L/min (p < 0.05); symptom score: -5.4 and -5.9 (p < 0.05), without significant differences between groups. No adverse effects were reported., Conclusions: Deflazacort and prednisolone show similar efficacy in improving pulmonary function and in producing clinical improvement in the management of acute moderate asthma in children.