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Simple clinical indices, complex composite phenotyping/endotyping and telehome monitoring now allow us to evaluate the risk of preschool wheezing on a population or individual level. We herein discuss the usefulness of clinical markers which involve underlying issues such as in utero smoke exposure, the pathophysiology of viral-immunological-allergic interactions, and changes in airway epithelium, mesenchyme and smooth muscle cells. The most important unmet need in this age group remains disease-modifying interventions., (Pediatric Pulmonology© 2024 The Author(s). Pediatric Pulmonology published by Wiley Periodicals LLC.)

Background: Pulmonary exacerbations (PEx) are strong predictors of respiratory disease progression in children with cystic fibrosis (CwCF) and may be associated with persistent decreased lung function after acute management. Telemonitoring devices can be used for early detection and monitoring of PEx, but its utility is debated., Research Question: Which symptoms and telemonitoring spirometry characterics are related to outcome dynamics following initial PEx management?, Methods: This retrospective study included CwCF followed at Bordeaux University Hospital, France. All severe PEx episodes treated with intravenous (IV) antibiotics (ATB) between 1 January 2017 and 31 December 2021 in CwCF using home telemonitoring were analyzed. Symptoms and home spirometry data were collected 45 days before and up to 60 days after each IV ATB course. We defined three response profiles based on terciles of baseline forced expiratory volume in 1 s (FEV 1 ) recovery., Results: A total of 346 IV ATB courses for PEx were administered to 65 CwCF during the study period. The drop in FEV 1 became significant 8 days before IV ATB initiation. Forty-one percent of IV ATB courses failed to restore baseline FEV 1 . The magnitude of FEV 1 drop and a greater delay in the initiation of treatment correlated with a low response level. On the 14th day of the IV treatment, a FEV 1 recovery less than 94% of baseline was associated with a nonresponder profile., Interpretation: Home spirometry may facilitate the early recognition of PEx to implement earlier interventions. This study also provides an outcome lung function threshold which identifies low responders to IV ATB., (© 2023 Wiley Periodicals LLC.)

Background: Bacterial infection early in life may increase structural lung lesions in children with cystic fibrosis (CF)., Methods: A 9-year monocentric (Bordeaux University Hospital, France) retrospective study in children with CF to evaluate the impact of the early-onset (at 1 year of age, Y1) of chronic meticillin-sensitive Staphylococcus aureus (MSSA) infection on the severity of bronchiectasis and Bhalla score on CT scan, clinical status, lung function tests, and serum immunoglobulins (IgG) at the age of 6 years (Y6)., Results: A total of 37 children were included: 10 had contracted chronic MSSA infection at Y1 and 27 at a later date. Children with MSSA infection at Y1 showed increased Y6 CT scan bronchiectasis severity scores vs late MSSA infection (mean ± SD: 4.7 ± 0.8 vs 2.5 ± 0.5, P < .05) and Bhalla scores (7.3 ± 1.1 vs 4.7 ± 0.8, P < .05), but no significant decrease in lung function ([% reference values] FEV1: 83.7 ± 6 vs 90.6 ± 2.2, P = .21; FEF25-75: 67.8 ± 8.9 vs 76.3 ± 3.9, P = .18). In addition, Y6 serum IgG was greater in the early chronic Y1 MSSA group (11.3 ± 0.7 vs 8.9 ± 0.7 g/L, P < .05). Clinical symptoms or nutritional status were similar in both infection groups., Conclusion: Early chronic MSSA infection may enhance the progression of structural lung disease in CF at 6 years., (© 2020 Wiley Periodicals LLC.)

Introduction: Interstitial lung disease in children (chILD) is a highly heterogeneous group of rare and severe respiratory disorders. The disease by itself, the burden of the treatments (oxygen therapy, corticosteroid pulses, nutritional support) and recurrent hospitalizations may impair the quality of life (QoL) of these children. The aim of the study was to compare the health-related QoL (HR-QoL) in chILD compared to a healthy population and to find out the predictive factors of an altered QoL., Methods: Patients aged 1 month to 18 years with ILD of known or unknown etiology were prospectively included. Parents and children over 8 years old were asked to fill the PedsQL 4.0 Generic Core Scale ranging from 0 to 100 points., Results: A total of 78 children were recruited in 13 French pediatric centers. Total scores were 11.94 points (P = 0.0003) less for child self-report and 14.08 points ( P < 0.0001) less for parent proxy-report with respect to the healthy population. The clinical factors associated with a lower total score were: extrapulmonary expression of the disease, higher Fan severity score, long-term oxygen therapy, nutritional support, and a number of oral treatments., Conclusion: Using a validated quality of life (QoL) scale, we showed that health-related-QoL is significantly impaired in chILD compared with a healthy population. Factors altering QoL score are easy to recognize and could help identify children at a heightened risk of low QoL., (© 2019 Wiley Periodicals, Inc.)

Objectives: To describe the characteristics of pediatric cases of eosinophilic granulomatosis with polyangiitis (EGPA), a systemic necrotizing vasculitis rarely diagnosed in children, retrieved from the French Reference Center for rare pediatric lung diseases and compared with adult cases included in the French Vasculitis Study Group cohort., Methods: We collected information on pediatric EGPA disease presentation, management, and outcome. Cases met the Lanham criteria and/or American College of Rheumatology classification criteria., Results: Fourteen cases of pediatric EGPA were included, from 1980 to 2012, with a median follow-up of 58.5 months. Median age at diagnosis was 12.3 years. All cases had respiratory involvement. The organ systems most frequently involved were the upper airway (85%), skin (71%), digestive tract (64%), and heart (57%). Neurological and renal involvement were rare. Four of the fourteen children were positive for ANCA (30.7%). During follow-up, three children required intensive care and one child died. The relapse rate was 64%. In comparison with an adult cohort, we found more ENT, heart, and digestive-tract involvement, and fewer neurological manifestations. In children, the delay between asthma onset and diagnosis was shorter, and biopsies showed fewer features of vasculitis., Conclusion: This French cohort is the biggest pediatric EGPA series described to date, with a long follow-up period. The findings confirm that pediatric EGPA has specific clinical, radiological, and histological characteristics that differ from adult EGPA. Development of systemic symptoms, and consequently diagnosis, occur with a shorter delay in children, mainly during the eosinophilic phase and leading to a specific presentation., (© 2018 Wiley Periodicals, Inc.)

Asthma is characterized by airway hyperresponsiveness (AHR) and inflammation leading to airway remodeling (AR). In children, AR may occur very early prior to the age of 6 years. Treatments to prevent or reverse AR are unknown., Aim: We sought to determine (i) whether short allergenic sensitization at a young age in a mouse model may induce enhanced AR and inflammation compared to adults; (ii) the effect of Montelukast on such AR., Methods: Immature and adult Balb/c mice were sensitized and challenged with ovalbumin. AHR and AR were measured using cultured precision-cut lung slices and inflammation by bronchoalveolar lavage. Experiments were repeated after administration of Montelukast., Results: OVA-challenged mice developed AHR to methacholine regardless of age of first exposure to OVA. Young mice developed greater thickened basement membrane, increased smooth muscle mass, and increased area of bronchovascular fibrosis compared with adult mice. Cellular infiltrates in BAL differed depending upon animal age at first exposure with higher eosinophilia measured in younger animals. Montelukast decreased ASM mass, BAL cellularity., Conclusion: We provide thus evidence for a greater degree of AR after allergenic sensitization and challenge in younger mice versus adults. This study provides proof of concept that airway remodeling can be prevented and reversed in this case by anti-asthmatic drug Montelukast in this model., (© 2018 Wiley Periodicals, Inc.)

Objectives: To describe diffusing capacity for carbon monoxide (DLCO) and its components, that is, membrane diffusing capacity (DmCO) and pulmonary capillary blood volume (Vc) in children with Crohn's disease (CD), and to investigate the correlation between these parameters and disease activity., Working Hypothesis: The most common lung function abnormalities are a reduced pulmonary DLCO and small airways disorders which are in many instances, clinically silent. No valid explanations have been proposed regarding the modifications in gas transfer capacity in active CD., Methods: DLCO, DmCO, and Vc were measured in 25 CD children by the simultaneous single breath lung diffusing capacity method using nitric oxide (NO) and carbon monoxide (CO) transfer. These parameters were analyzed in relation to the CD disease activity index., Results: DLCO (90.7 ± 4.5% vs 128.5 ± 4.7%; P < 0.001), Dm (92.4 ± 5.9% vs 125.6 ± 6.3%; P < 0.001), and Vc (72.6 ± 3.7% vs 104.4 ± 4.0%; P < 0.001) were significantly decreased in the active CD group in comparison with the inactive CD group. DLCO (r = -0.60; P < 0.01), DmCO (r = -0.45; P < 0.01), and Vc (r = -0.60; P < 0.01) were inversely correlated to the PCDAI. In 8 patients who participated to the study at initial diagnosis then during remission, DmCO and Vc increased significantly between the active and the inactive period of the disease., Conclusion: Pulmonary diffusing capacity is impaired in children with active CD, mainly because of a decrease of the pulmonary capillary volume., (© 2017 Wiley Periodicals, Inc.)

Introduction: There is a lack of evidence concerning the effectiveness of immunoprophylaxis with palivizumab in children with childhood interstitial lung disease (chILD). In this retrospective study, we evaluated the effectiveness of palivizumab for decreasing the rate of RSV-related hospitalizations in children under the age of 24 months with chILD treated with corticosteroids., Methods: A retrospective national study was conducted in France. Patients born between 2007 and 2013, diagnosed with chILD and on corticosteroid treatment were identified through the French online database for pediatric interstitial lung disease (Respirare(®) ). Data were collected for the etiology and severity of chILD, risk factors and preventive measures for bronchiolitis, palivizumab immunoprophylaxis, and hospitalizations for bronchiolitis and RSV-bronchiolitis., Results: We included and evaluated 24 children during their first two RSV seasons, corresponding to 36 patient-seasons. The observed rate of RSV-related hospitalization (305/1000 patient-seasons), and the median length of stay (7 days), were higher than those for the general population. RSV-related hospitalization rates did not differ significantly between children with and without palivizumab prophylaxis (5/16 vs. 4/18, respectively, P = 0.70)., Conclusion: Children with chILD on corticosteroid treatment are at high risk of hospitalization for RSV-bronchiolitis, which tends to be more severe in these children than in the general population. The effectiveness of palivizumab prophylaxis in this population remains to be demonstrated. Pediatr Pulmonol. 2016;51:688-695. © 2015 Wiley Periodicals, Inc., (© 2015 Wiley Periodicals, Inc.)

Pediatric eosinophilic pneumonias (EPs) are characterized by a significant infiltration of the alveolar spaces and lung interstitium by eosinophils, with conservation of the lung structure. In developed countries, EPs constitute exceptional entities in pediatric care. Clinical symptoms may be transient (Löffler syndrome), acute (<1 month and mostly <7 days), or chronic (>1 month). Diagnosis relies on demonstration of alveolar eosinophilia on bronchoalveolar lavage, whether or not associated with blood eosinophilia. EPs are a heterogeneous group of disorders divided into: (i) secondary forms (seen mainly in parasitic infections, allergic bronchopulmonary aspergillosis, and drug reactions); and (ii) primary forms (eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome, idiopathic chronic eosinophilic pneumonia, and idiopathic acute eosinophilic pneumonia). Despite their rarity, the etiological approach to EP must be well-defined as some causes can be rapidly life-threatening without initiation of the proper treatment. This approach (i) eliminates secondary forms, with comprehensive history taking and minimal biological assessment, (ii) is oriented in primary forms by the acute or chronic setting, and the existence of extrapulmonary symptoms. Treatment of primary forms has traditionally relied on corticosteroids, usually with a dramatic response. Specific treatments or the adjunction of corticosteroid-sparing treatment or immunosuppressors are currently being evaluated in order to improve the prognosis and the side effects associated with corticosteroid treatment in a pediatric setting., (© 2015 Wiley Periodicals, Inc.)

Background: Meconium aspiration syndrome (MAS) remains a frequent cause of morbidity and mortality in term newborns. Our objective was to compare two modes of high-frequency ventilation, high-frequency oscillation (HFOV), and high-frequency percussive ventilation (HFPV) with conventional mechanical ventilation (CMV) in a piglet model of MAS., Methods: Fifteen newborn piglets were anesthetized, paralyzed, and intubated. Following the instillation of a 3 ml/kg solution of meconium diluted to 30%, the piglets were randomized to one of three groups: high-frequency oscillation (HFOV; Sensormedics®), HFPV (Percussionaire®), or CMV (Siemens®). Animals were ventilated for 6 hr to maintain arterial blood gases within a normal range, that is, pH 7.35-7.45, PaO(2) 10-16 kPa, PaCO(2) 4-6.6 kPa. Arterial blood gas measurements, dynCrs and dynRrs, ventilator settings, and vital signs (heart rate, arterial blood pressure, transcutaneous pulse oxygen saturation, and temperature) were collected at 30, 60, 90, 120, 180, 240, 300, and 360 min after meconium instillation. Oxygenation index (OI) ([(fraction of inspired oxygen)(mean airway pressure)(100)]/PaO(2) ), mean airway pressure, dynamic lung function, secretions cleared and histological alterations were studied in all groups., Results: Mean airway pressure and OI were significantly lower in the CV and HFPV groups compared to the HFOV group (P < 0.05). There was no significant difference between groups regarding lung function, amount of secretions and histological alterations., Conclusion: In our model of MAS in piglets, whilst effective gas exchange with a lower mean airway pressure was possible with both CMV and HFPV compared with HFOV there was no apparent difference in lung histology or secretions., (Copyright © 2012 Wiley Periodicals, Inc.)

Silicone stents were inserted into the trachea or left main-stem bronchus in 14 children aged 2-69 months (median, 7 months). Indications were as follows: tracheomalacia or airway kinking (7 cases), vascular compression (5 cases), and surgically corrected congenital tracheal stenoses (2 cases). The best results were obtained in tracheomalacia. Overall, 6 cases out of 14 (43%) were considered successful, with a stent placement duration of 3-15 months (median, 7 months). Two cases were considered a technical success, although they were clinical failures. Five cases were considered failures primarily due to stent migration. A retrospective analysis of failures suggests that most of these could have been avoided by the use of larger stents. One patient died of stent obstruction. No wall erosion was observed, and the development of granulation tissue was infrequent. Endoscopic removal of the prostheses was uneventful. The biocompatibility of silicone stents appears to be better than what is reported for metal ones, although the stability of the former is less satisfactory. The present study shows the feasibility of silicone stent placement in infants. These stents should be considered as a possible therapeutic option in certain types of childhood airway disorders, although further studies are required.

Impaired lung development has been demonstrated in neonatal animals exposed to hyperoxia. High lung cys-leukotriene levels may be a contributing factor towards the increase in oxygen toxicity. We investigated the effect of cysteinyl-leukotriene inhibition using the receptor antagonist, montelukast (MK, Singulair), on hyperoxia-induced changes in lung parenchymal structure in neonatal rat pups. Rat pups were exposed to 21% O(2) (air) or 50% O(2) (moderate hyperoxia) from days 1-14 after birth, and were administered the cys-leukotriene receptor antagonist MK (1 mg/kg/day) or normal saline from days 4-14. Somatic growth and morphometric measurements were done on day 15. There was a significant increase in bronchoalveolar lavage fluid cysteinyl-leukotriene levels (+61.9%) when animals were exposed to hyperoxia. O(2) exposure significantly decreased the specific internal surface area by 13%. There was a nonsignificant 5.8% and 19.6% increase in mean chord length and mean alveolar diameter, respectively, as well as an 8.6% decrease in lung volume to body weight ratio. Inhibition of only one arm of the arachidonic-acid cascade by MK was not sufficient to prevent these oxygen-induced changes., (Copyright 2003 Wiley-Liss, Inc.)

Glucocorticoids are widely used in perinatology, since they decrease the incidence of respiratory distress syndrome and chronic lung disease. However, evidence is now increasing that their use in this age group may result in impaired alveolar lung growth and general development. The aim of this study was to determine whether a low dose of hydrocortisone (1 mg/kg/day for 11 days) was deleterious to lung growth in rat pups, as compared to an equivalent dose of dexamethasone. While both dexamethasone and hydrocortisone increased alveolar diameter with thinning of the interairspace walls, only dexamethasone reduced the overall internal surface area of the lung available for respiratory exchange. Changes were more marked with dexamethasone as compared to hydrocortisone, which did not appear to affect alveolar septation. In conclusion, a prolonged course of low-dose hydrocortisone may be deleterious for alveolar lung growth in rat pups, but the changes are less marked than those caused by dexamethasone., (Copyright 2002 Wiley-Liss, Inc.)