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Objectives: Bronchopulmonary dysplasia (BPD) is a chronic lung disease that affects premature babies and contributes to their morbidity. French BPD guidelines suggest the use of intravenous methylprednisolone pulses (IVMP) for those who require respiratory support (RS) following 36 weeks postmenstrual age (PMA). However, there is scant data on the efficacy and tolerance for this indication. Our objectives were to assess the efficacy and tolerance of IVMP in patients with severe BPD requiring RS., Study and Design: This retrospective observational study included preterm infants (<32 weeks gestational age) treated by IVMP between January 2016 and March 2021, in a single tertiary care hospital. The primary endpoint was signified by changes in the Pulmonary Severity Score (PSS). Our secondary endpoints were changes in RS and the tolerance of IVMP., Results: Data of 14 children with BPD under RS were reviewed. Their median [IQR] gestational age at birth was (26[26-29.5] weeks). After IVMP administration, the PSS did not improve significantly from baseline day 0 (D0) (0.85[0.55-1.15]) to D7 (0.74 [0.48-1.00]) or Month 1 (1.02 [0.57-1.48]). Three children (21%) experienced a decrease in RS in the following month of IVMP. There was no negative short-term impact on growth. Five infants presented infectious complications. The development quotient was measured in 5 children at 2 years of age., Conclusion: In our study, IVMP did not improve PSS and it was associated with a high number of infections. Other studies are needed to better define the place of IVMP in these infants., (© 2024 The Author(s). Pediatric Pulmonology published by Wiley Periodicals LLC.)

Objectives: Bronchopulmonary dysplasia (BPD) is the most common complication in preterm infants. This study aimed at identifying factors associated with early or with late or weaning failure from respiratory support or oxygen (O 2 ) in preterm infants with BPD requiring respiratory support or O 2 therapy after discharge from the neonatal intensive care unit (NICU)., Methods: This retrospective study was conducted in the NICU of a tertiary hospital, in infants born before 32 weeks of gestation between 2012 and 2021, and discharged from the NICU with a respiratory support (tracheostomy [TT], invasive ventilation [IV], Non-IV [NIV], continuous positive airway pressure [CPAP], high flow nasal canula [HFNC]) or O 2 therapy for BPD. Univariate and multivariate analyses were performed to identify factors associated with early weaning (before 6 months postmenstrual age [PMA]) or late (after 6 months PMA) and weaning failure., Results: Among the 53 infants included (2% TT, 2% IV, 11% NIV, 25% CPAP or HFNC, 60% O 2 at NICU discharge), 23 (43%) were weaned from respiratory support or O 2 before 6 months PMA and 39 (73%) before 12 months PMA. IV duration during NICU stay and postnatal steroid treatment were identified as factors associated with a late or weaning failure (OR 1.03, p = .04 and OR 4.11, p = .023, respectively)., Conclusion: In this study, nearly half of preterm infants with severe BPD were weaned from respiratory support or O 2 before 6 months PMA. IV duration and postnatal steroid treatment during NICU stay were associated with a late or weaning failure., (© 2024 The Author(s). Pediatric Pulmonology published by Wiley Periodicals LLC.)

Introduction: Clinical trials for CFTR modulators consider mean changes of clinical status at the cohort level, and thus fail to assess the heterogeneity of the response. We aimed to study the different response profiles to lumacaftor-ivacaftor according to age in children with cystic fibrosis (CF)., Methods: A mathematical framework, including principal component analysis, data clustering, and data completion, was applied to a multicenter cohort of 112 children aged 6-18 years, treated with lumacaftor-ivacaftor. Studied parameters at baseline and 6 months included body mass index (BMI), number of days of antibiotics (ATB), Sweat test (ST), forced expiratory volume in 1 s expressed in percentage predicted (ppFEV 1 ), forced vital capacity (ppFVC), and forced expiratory flow at 25%-75% of FVC (ppFEF 25-75 )., Results: Change in ppFEV 1 was the most significant parameter in characterizing response heterogeneity among the 12-18-year-old patients. Patients with minimal changes in ppFEV 1 were further separated by change in BMI and ATB course. In the 6-12-year-old children both BMI and ppFEV 1 evolution were the most relevant. ST change was not associated with a clinical response., Conclusions: Change in ppFEV 1 , BMI, and ATB course are the most relevant outcomes to discriminate clinical response profiles in children treated with lumacaftor-ivacaftor. Prepubertal and pubertal children display different response profiles., (© 2022 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Introduction: The prognosis of people diagnosed with cystic fibrosis (CF) has dramatically improved over the past decade in France, largely due to advances in CF care management, including an emphasis on chronic maintenance medications. Currently, the majority of French CF patients are adults, which means that they went through a transition process from receiving care at a pediatric CF center to receiving care at an adult CF center. To determine the impact of the transfer on clinical evolution, we report the transition procedure of our CF center in Lyon., Materials and Methods: From January 2006 to December 2016, 97 CF patients underwent a standardized process of transitioning from the pediatric to the adult CF center in Lyon. We compared the clinical evolution of these patients during three periods, starting the year before transition and ending the year after transition. Clinical data taken into account were forced expiratory volume in 1 s (FEV1 in liters), body mass index (BMI in kg/m 2 ), pulmonary colonization, number of antibiotic courses, number of days of hospitalization per year, and outpatient visits per year., Results: No significant differences were observed between respiratory and nutritional status, respiratory microbiome, number of antibiotic courses, or number of hospitalizations or visits when comparing the threeperiods of observation around transition (the year before, the first year after, and the second year after transfer)., Conclusion: The standardized transition procedure used in Lyon is associated with the clinical stability of our CF patients., (© 2021 Wiley Periodicals LLC.)

Background: To better understand the mechanisms of infection with nontuberculous mycobacteria (NTM) in patients with cystic fibrosis (CF), we explore different risk factors associated with NTM positivity in a meta-analysis., Methods: Studies published before 31 July 2019 were selected from MEDLINE. Combined odds ratios (ORs) were calculated by pooling the ORs of each study. The weighted mean difference (WMD) was used for continuous numerical measurements. Summary data were pooled using fixed- or random-effects models according to the presence of heterogeneity (P < .1 or I 2  > 50%)., Results: Nineteen studies with a total of 23 418 patients, of whom 1421 (6%) were diagnosed as NTM positive, were included. Older age was significantly associated with NTM positivity (WMD = 2.12, 95% confidence interval [CI]: 1.11-3.13; P < .01, fixed-effects model). The OR for Staphylococcus aureus colonization was 1.66 (95% CI: 1.21-2.26; P = .001) in 11 studies (8091 patients), the OR for Aspergillus fumigatus colonization was 3.59 (95% CI: 3.05-4.23; P < .001) in 11 studies (20 480 patients), and the OR for Stenotrophomonas maltophilia colonization was 3.41 (95% CI: 2.66-4.39; P < .01) in seven studies (14 935 patients). Oral corticosteroids were significantly associated with NTM positivity (OR = 1.98, 95% CI: 1.24-3.16; P < .01, 6 studies, 1936 patients). No other factor showed a significant association., Conclusion: Older age, S. aureus, S. maltophilia, and A. fumigatus chronic colonization, and oral corticosteroids were significantly associated with an increased risk of NTM positivity. CF patients with more severe conditions should be closely monitored for NTM., (© 2020 Wiley Periodicals LLC.)

Background: Hypersensitivity pneumonitis (HP) is a rare interstitial lung disease in children, and very little data are available on the frequency, diagnosis, and outcomes of HP. In a pediatric cohort with HP, the characteristics of the CD4/CD8 lymphocyte ratio are often described as nonspecific., Methods: We used the National French Database (RespiRare) to collect data from the last decade on HP. The diagnosis of HP was defined by the presence of a relevant exposure, clinical symptoms, and compatible lung imaging radiology and was usually defined by positive precipitins antibodies., Results: A total of 16 children with a mean age of 10 years (4-13) presented with HP. All children presented with dyspnea on exertion. Diffuse ground-glass opacity was present in all computed tomography (CT) scans. Research guided by a questionnaire and precipitins antibodies against the corresponding antigens showed that patients were positive for contact with birds with or without fungi. Bronchoalveolar lavage (BAL) was performed in 12 children. The total cell counts were elevated in BAL fluid, with a mean value of 36% lymphocytes. The CD4/CD8 lymphocyte ratio was below one for all children., Conclusion: BAL in our pediatric cohort with HP had the same characteristics as that of adults with HP. An HP diagnosis must be considered when dyspnea on exertion and diffuse ground-glass opacity are observed. Carrying out BAL and serological tests can help diagnose and avoid lung biopsy., (© 2019 Wiley Periodicals, Inc.)

Diaphragmatic paralysis (DP) is a rare cause of respiratory distress in young children. In the first years of life, the main cause is phrenic nerve injury after cardiothoracic surgery or obstetrical trauma. DP usually presents as respiratory distress. Asymmetrical thorax elevation, difficulty weaning from mechanical ventilation, pulmonary atelectasis, and repeated pulmonary infections are other suggestive signs or complications. DP is usually suspected on chest X-ray showing abnormal hemidiaphragm elevation. Although fluoroscopy was considered the gold standard for DP confirmation, it has gradually been replaced by ultrasound, which can be done at the bedside. Some electrophysiological tools may be useful for a better characterization of phrenic nerve injury and chance of recovery. The management of DP is mainly based on clinical severity. In mild asymptomatic cases, DP may only require close monitoring. In more severe cases, adequate ventilatory support and/or surgical diaphragmatic plication may be needed. Electrophysiological tools may help clinicians assess the ideal timing for diaphragmatic plication., (© 2019 Wiley Periodicals, Inc.)

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.)

Background: Among the different techniques used to monitor lung disease progression in infants with CF diagnosed by Newborn screening (NBS), raised volume-rapid thoracic compression (RVRTC) remains a promising tool. However, the need of sedation and positive pressure ventilation considerably limits its clinical use. We recently described a semi-quantitative method to evaluate air trapping by chest tomography during quite breathing without sedation (CTqb score). This parameter is the radiological sign of airway obstruction and could be also used for lung disease follow-up in infants with CF. However, its discriminative power compared with RVRTC and correlation with lung function parameters are not known., Objectives: To compare the discriminative powers of the CTqb score and RVRTC parameters and to determine their correlation during the first year of life of infants with CF., Methods: In this multicenter longitudinal study, infants with CF diagnosed by NBS underwent RVRTC and CT during quite breathing at 10 ± 4 weeks (n = 30) and then at 13 ± 1 months of age (n = 28)., Results: All RVRTC parameters and the CTqb score remained stable between evaluations. The CTqb score showed a higher discriminative power than forced expiratory volume in 0.5 s (FEV 0.5 ; the main RVRTC parameter) at both visits (66% and 50% of abnormal values vs 30% and 28%, respectively). No correlation was found between CTqb score and , the different RVRTC parameters or the plethysmographic functional residual capacity, indicating that they evaluate different aspect of CF lung disease., (© 2017 Wiley Periodicals, Inc.)

Acute chest pain is common in patients with cystic fibrosis (CF). Here we report the case of an adolescent who suffered acute chest pain in September after an history of acute abdominal pain and fever. The reason for this clinical sceneriao was found to be Coxsackievirus B3, known to be responsible of Bornholm disease, a frequent but under recognized viral myositis. The diagnosis is mainly clinical, but evocating this diagnosis may avoid unnecessary exam., (© 2017 Wiley Periodicals, Inc.)

Allergic bronchopulmonary aspergillosis (ABPA) affects up to 15% of patients with cystic fibrosis (CF). Corticosteroids are used as first-line therapy, but relapse and adverse effects commonly occur. Case reports have suggested the efficacy of the anti-IgE recombinant humanized monoclonal antibody omalizumab. A retrospective multicenter observational French study retrieved 32 CF patients (11 children and 21 adults) who have received omalizumab for more than 3 months in the context of ABPA. Clinical characteristics, concomitant medications (inhaled and oral corticosteroids, antifungal drugs), lung function, body mass index (BMI), and serum IgE were compared at the start and during the first year of omalizumab therapy. Omalizumab-related adverse effects and costs were also evaluated. No significant difference with omalizumab could be demonstrated with regard to lung function, BMI, or the number of patients receiving oral corticosteroids. At the time of initiation of omalizumab, 56% of patients were receiving oral corticosteroids. Five patients were able to discontinue corticosteroids during follow-up and nine patients were able to reduce their daily dose. A total of 78% of the patients had received antifungal therapy at the time of the initiation of omalizumab. Treatment tolerance was good (12.5% of patients experienced side effects). The median cost of omalizumab treatment was €3,620 per patient per month. Omalizumab may represent a steroid-sparing therapy in CF patients with ABPA. A randomized-controlled trial is urgently required to provide higher level of evidence regarding the efficacy and cost-effectiveness of omalizumab in CF patients with ABPA. Pediatr Pulmonol. 2017;52:190-197. © 2016 Wiley Periodicals, Inc., (© 2016 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.)

A literature search identified one retrospective study on the responsiveness of impulse oscillometry (IOS) in pediatric patients with cystic fibrosis. The aim of this prospective observational study was to assess this property in an adequately powered study after intravenous antibiotic therapy (IVAT) administered for an acute episode of pulmonary exacerbation. Spirometry and IOS were done on the same day as the start and the end of IVAT. Data from 34 patients' of mean age 11.9 years (range, 5-17 years) were studied. The mean FEV1 at the start and at the end of the IVAT was 73.1 ± 23.8% (range, 23.4-122%) and 88.3 ± 21.3% (range, 29.4-131%), respectively. The mean relative change (mean ± SD) was 20.2 ± 14.2% for FEV1 (ΔFEV1 ), -21.9 ± 23.8% for reactance at 5 Hz (ΔX5) and -13.4 ± 18.9% for resistance at 5 Hz (Δ R5) (all P-values <0.05). There was a weak but significant correlation between ΔFEV1 and ΔX5 (r =-0.473; p = 0.01). The magnitude of improvement of ΔX5 was not statistically different between patients with normal versus abnormal lung function at the start of IVAT. Furthermore, using ΔX5 alone as an outcome measure of IVAT efficiency resulted in a significant improvement in 44% of the patients, while it was 79% with ΔFEV1 . These results indicate that IOS may track changes after IVAT, but that this improvement may be insufficiently evaluated using IOS alone., (© 2015 Wiley Periodicals, Inc.)

Congenital chondroid lesions of the lung are rare pathological findings. They are a constant feature of lung malformations such as giant cystic pulmonary chondroid hamartoma, chondroid cystic malformation, and in the "cartilaginous variant" of congenital adenomatoid malformation. All of these present as a large single thoracic mass.We present the cases of three males and two females with hitherto undescribed diffuse chondroid lung disease, all but one of whom had neonatal respiratory distress syndrome with interstitial syndrome on chest radiograph. The pathological findings were similar in all patients, showing large areas of disorganized lung parenchyma containing diffusely distributed mature cartilage islands. With a mean follow-up of 6 years, all patients had a favorable outcome. This diffuse chondroid lung disease appears to be a new entity whose initial presentation mimicked interstitial lung disease without the usual clinical, radiological, and histological features. We speculate that it could be part of a clinical spectrum between malformative chondroid lung cyst and congenital pulmonary airway malformation.

Aims: To review the middle- and long-term effects of partial splenectomy (PS) on portal hypertension (PHT) and its complications in patients with cystic fibrosis (CF) related liver disease risky PHT., Method: Over a 20 years period, 19 patients aged 7-23 years underwent partial PS for massive splenomegaly, hypersplenism, and/ or severe PHT., Results: In all but three cases, PHT and hypersplenism have improved for long periods. Noticeable improvement of hepatic tests occurred simultaneously. In all patients PS resolved abdominal discomfort. Fifteen patients are alive and a stabilization of the liver disease occurred with a follow-up of 1-20 years (mean 7.9). One patient died following respiratory insufficiency 10 years after PS although PHT was stable. Manifestations recurred in 2 patients 5 and 6 years after PS. In two patients, the course of the disease evolved to hepatic insufficiency without recurrence of PHT 3 and 8 years after PS. PS did not give the expected results in three cases only, in which PHT was not modified or reoccurred during the following year. No severe complication was observed. Early (three patients) or late (one patient) eventration required surgical procedure., Conclusions: Our results show that PS is a reliable and well-tolerated technique. Therefore, it is a therapeutic option for the management of PHT in CF patients with a preserved liver function. It can prevent and significantly delay a liver transplantation and its constraints.

Laryngeal sensitivity in the newborn has been a subject of great interest for both researchers and clinicians for a number of years. From a clinical standpoint, laryngeal sensitivity is essential for both preventing foreign substances from entering into the lower airway and for finely tuning upper airway resistance. However, heightened reflexes originating from the laryngeal receptors in newborns and infants, due to neural immaturity, can lead to potentially dangerous cardiorespiratory events. The latter have been linked to apneas of prematurity, apparent life-threatening events, and sudden infant death syndrome (SIDS). From a physiological standpoint, many mechanisms pertaining to reflexes originating from laryngeal receptors are yet to be fully understood. This short review is an attempt to summarize current knowledge on laryngeal sensitivity and its potential consequences upon control of breathing abnormalities encountered within the first weeks of life., (2007 Wiley-Liss, Inc.)