Analysis of mandibular jaw movements to assess ventilatory support management of children with obstructive sleep apnea syndrome treated with positive airway pressure therapies.

BACKGROUND: The polysomnography (PSG) is the gold-standard for obstructive sleep apnea (OSA) syndrome diagnosis and assessment under positive airway pressure (PAP) therapies in children. Recently, an innovative digital medicine solution, including a mandibular jaw movement (MJM) sensor coupled with automated analysis, has been validated as an alternative to PSG for pediatric application. OBJECTIVE: This study aimed to assess the reliability of MJM automated analysis for the assessment of residual apnea/hypopnea events during sleep in children with OSA treated with noninvasive ventilation (NIV) or continuous PAP (CPAP). METHODS: In this open-label prospective non-randomized multicentric trial, we included children aged from 5 to 18 years with a diagnosis of severe OSA. The children underwent in-laboratory PSG with simultaneous MJM monitoring and at-home recording with MJM monitoring 3 months later. Agreement between PSG and MJM analysis in measuring the residual apnea-hypopnea index (AHI) was evaluated by the Bland-Altman method. The treatment effect on residual AHI was estimated for both PSG and MJM analysis. RESULTS: Fifteen (60% males) children were included with a median age of 12 years [interquartile range 8-15]. Two (17%) were ventilated with NIV and 13 (83%) with CPAP. There was a good agreement between MJM-AHI and PSG-AHI with a median bias of -0.25 (95% CI: -3.40 to +2.04) events/h. The reduction in AHI under treatment was consistently significant across the three measurement methods: in-laboratory PSG and MJM recordings in the laboratory and at home. CONCLUSION: Automated analysis of MJM is a highly reliable alternative method to assess residual events in a small population treated with PAP therapies.

ERS International Congress 2023: highlights from the Paediatrics Assembly.

Respiratory health in children is essential for general wellbeing and healthy development in the short and long term. It is well known that many respiratory diseases in adulthood have their origins in early life, and therefore research on prevention of respiratory diseases and management of children with respiratory diseases will benefit patients during the full life course. Scientific and clinical advances in the field of respiratory health are moving at a fast pace. This article summarises some of the highlights in paediatric respiratory medicine presented at the hybrid European Respiratory Society (ERS) International Congress 2023 which took place in Milan (Italy). Selected sessions are summarised by Early Career Members of the Paediatrics Assembly (Assembly 7) under the supervision of senior ERS officers, and cover a wide range of research areas in children, including respiratory physiology and sleep, asthma and allergy, cystic fibrosis, respiratory infection and immunology, neonatology and intensive care, respiratory epidemiology and bronchology.

A BEAT-PCD consensus statement: a core outcome set for pulmonary disease interventions in primary ciliary dyskinesia.

Background: Consistent use of reliable and clinically appropriate outcome measures is a priority for clinical trials, with clear definitions to allow comparability. We aimed to develop a core outcome set (COS) for pulmonary disease interventions in primary ciliary dyskinesia (PCD). Methods: A multidisciplinary international PCD expert panel was set up. A list of outcomes was created based on published literature. Using a modified three-round e-Delphi technique, the panel was asked to decide on relevant end-points related to pulmonary disease interventions and how they should be reported. First, inclusion of an outcome in the COS was determined. Second, the minimum information that should be reported per outcome. The third round finalised statements. Consensus was defined as ≥80% agreement among experts. Results: During the first round, experts reached consensus on four out of 24 outcomes to be included in the COS. Five additional outcomes were discussed in subsequent rounds for their use in different subsettings. Consensus on standardised methods of reporting for the COS was reached. Spirometry, health-related quality-of-life scores, microbiology and exacerbations were included in the final COS. Conclusion: This expert consensus resulted in a COS for clinical trials on pulmonary health among people with PCD.

Digital Action Plan (Web App) for Managing Asthma Exacerbations: Randomized Controlled Trial.

BACKGROUND: A written action plan (WAP) for managing asthma exacerbations is recommended. OBJECTIVE: We aimed to compare the effect on unscheduled medical contacts (UMCs) of a digital action plan (DAP) accessed via a smartphone web app combined with a WAP on paper versus that of the same WAP alone. METHODS: This randomized, unblinded, multicenter (offline recruitment in private offices and public hospitals), and parallel-group trial included children (aged 6-12 years) or adults (aged 18-60 years) with asthma who had experienced at least 1 severe exacerbation in the previous year. They were randomized to a WAP or DAP+WAP group in a 1:1 ratio. The DAP (fully automated) provided treatment advice according to the severity and previous pharmacotherapy of the exacerbation. The DAP was an algorithm that recorded 3 to 9 clinical descriptors. In the app, the participant first assessed the severity of their current symptoms on a 10-point scale and then entered the symptom descriptors. Before the trial, the wordings and ordering of these descriptors were validated by 50 parents of children with asthma and 50 adults with asthma; the app was not modified during the trial. Participants were interviewed at 3, 6, 9, and 12 months to record exacerbations, UMCs, and WAP and DAP use, including the subjective evaluation (availability and usefulness) of the action plans, by a research nurse. RESULTS: Overall, 280 participants were randomized, of whom 33 (11.8%) were excluded because of the absence of follow-up data after randomization, leaving 247 (88.2%) participants (children: n=93, 37.7%; adults: n=154, 62.3%). The WAP group had 49.8% (123/247) of participants (children: n=45, 36.6%; mean age 8.3, SD 2.0 years; adults: n=78, 63.4%; mean age 36.3, SD 12.7 years), and the DAP+WAP group had 50.2% (124/247) of participants (children: n=48, 38.7%; mean age 9.0, SD 1.9 years; adults: n=76, 61.3%; mean age 34.5, SD 11.3 years). Overall, the annual severe exacerbation rate was 0.53 and not different between the 2 groups of participants. The mean number of UMCs per year was 0.31 (SD 0.62) in the WAP group and 0.37 (SD 0.82) in the DAP+WAP group (mean difference 0.06, 95% CI -0.12 to 0.24; P=.82). Use per patient with at least 1 moderate or severe exacerbation was higher for the WAP (33/65, 51% vs 15/63, 24% for the DAP; P=.002). Thus, participants were more likely to use the WAP than the DAP despite the nonsignificant difference between the action plans in the subjective evaluation. Median symptom severity of the self-evaluated exacerbation was 4 out of 10 and not significantly different from the symptom severity assessed by the app. CONCLUSIONS: The DAP was used less often than the WAP and did not decrease the number of UMCs compared with the WAP alone. TRIAL REGISTRATION: ClinicalTrials.gov NCT02869958; https://clinicaltrials.gov/ct2/show/NCT02869958.

Bronchodilator response by interrupter technique to guide management of preschool wheeze.

OBJECTIVE: We examined relationships between clinical features and pulmonary function before and after inhaled corticosteroid (ICS) treatment in wheezy preschool children, and specifically, whether measuring bronchodilator response (BDR) could predict benefit from ICS. DESIGN: Clinical non-randomised intervention study SETTING: Secondary care. PATIENTS: Preschool children (2 years to <6 years) with recurrent wheeze. INTERVENTIONS: Inhaled beta-agonist, ICS. OUTCOME MEASURES: We measured prebronchodilator and postbronchodilator interrupter resistance (Rint) and symptom scores at 0 (V1), 4 (V2) and 12 (V3) weeks. At V2, those with a predetermined symptom level commenced ICS. Modified Asthma Predictive Index (mAPI) and parental perception of response to bronchodilator were recorded. Response to ICS was defined as a reduction in daily symptom score of >0.26. Positive BDR was defined as fall in Rint of ≥0.26 kPa.s/L, ≥35% predicted or ≥1.25 Z Scores. RESULTS: Out of 138 recruited children, 67 completed the full study. Mean (SD) prebronchodilator Rint at V2 was 1.22 (0.35) kPa.s/L, and fell after starting ICS (V3) to 1.09 (0.33) kPa.s/L (p<0.001), while mean (SD) daily symptom score fell from 0.56 (0.36) to 0.28 (0.36) after ICS (p<0.001). Positive Rint BDR before ICS (at V1 and/or V2), using all three threshold criteria, was significantly associated with response to ICS on symptom scores at V3 (p<0.05). mAPI was not significantly associated with response to ICS, and parents' perception of response to bronchodilator was not related to measured Rint BDR . CONCLUSIONS: Rint BDR may be helpful in selecting which wheezy preschool children are likely to benefit from ICS.

Respiratory function and sleep in children with myotonic dystrophy type 1.

Myotonic dystrophy type 1 (DM1) is a rare neuromuscular disease in children causing sleep and respiratory disorders that are poorly described in the literature compared to adult forms. This retrospective observational study was performed at the Armand Trousseau University Hospital, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France. We retrospectively collected data from lung function tests, nocturnal gas exchange recordings, and polysomnography of 24 children with DM1. 39% of the children with DM1 reported respiratory symptoms indicative of sleep disordered breathing. Three patients (12%) presented with a restrictive respiratory pattern, 10 (42%) with a sleep apnoea syndrome, mainly of obstructive origin (2/10 with severe obstructive sleep apnea syndrome), and 11 (45%) with nocturnal alveolar hypoventilation. Non-invasive ventilation (NIV) was indicated in 9 (37.5%) children, although tolerance was poor. No significant deterioration in respiratory function or nocturnal gas exchange was observed during the NIV-free period. This study provides new and useful insights into DM1 disease evolution in children to better adapt for respiratory follow-up and management. This highlights the need for future research to better understand the origin of respiratory and sleep disorders in patients with DM1.

Nasal nitric oxide measurement in children for the diagnosis of primary ciliary dyskinesia: European Respiratory Society technical standard.

Nasal nitric oxide (nNO) is extremely low in most people with primary ciliary dyskinesia (PCD) and its measurement is an important contributor to making the diagnosis. Existing guidelines and technical standards focus on nNO measurements in older, cooperative children using chemiluminescence analysers. However, measurements of nNO in pre-school-age children (age 2-5 years) may facilitate early diagnosis and electrochemical rather than chemiluminescence analysers are widely used. Pre-schoolers often need different methods to be employed when measuring nNO. Hence, a European Respiratory Society Task Force has developed this technical standard as the first step towards standardising sampling, analysis and reporting of nNO measured as part of the diagnostic testing for PCD in all age groups, including pre-school-age children. Furthermore, we considered both chemiluminescence and electrochemical analysers that are in use worldwide. There was a paucity of quality evidence for electrochemical analysers and sampling methods used in young children, and the Task Force proposes future research priorities to allow updates of this technical standard.

Longitudinal assessment of loss and gain of lung function in childhood asthma.

OBJECTIVE: The Childhood Asthma Management Program revealed that 25.7% of children with mild to moderate asthma exhibit loss of lung function. The objective was to assess the trajectories of function by means of serial FEV1 in asthmatic children participating in out-of-hospital follow-up. METHODS: A total of 295 children (199 boys) who had undergone at least 10 spirometry tests from the age of 8 were selected from a single-center open cohort. The annualized rate of change (slope) for prebronchodilator FEV1 (percent predicted) was estimated for each participant and three patterns were defined: significantly positive slope, significantly negative slope, and null slope (non-significant P-value; Pearson test). The standard deviation (SD) of each individual slope was recorded as a variability criterion of FEV1. RESULTS: The median (25th; 75th percentile) age at inclusion and the last visit was 8.5 (8.2; 9.3) and 15.4 (14.8, 16.0) years, respectively. Tracking of function (null slope) was observed in 68.8% of the children, while 27.8% showed a loss of function or reduced growth (negative slope) and 3.4% showed a gain in function (positive slope). The children characterized by loss of function depicted a better initial function and a lower FEV1 variability during their follow-up than children with tracking or gain of lung function. At the last visit, these children were characterized by a lower lung function than children with tracking or gain of lung function. CONCLUSION: Better initial FEV1 value and less FEV1 variability are associated with loss of lung function or reduced lung growth in asthmatic children.

Nasal nitric oxide measurement variability to establish a standard for reliable results.

A repeatability of 10% for NO measurements obtained with the velum closed in the same or both nostrils is relevant, while measurements taken during tidal breathing should aim for a repeatability of 20% and 30%, respectively https://bit.ly/3sMnug6.

Prediction of height using ulna length in African-Caribbean children.

Standing height is an essential anthropometric measurement in pediatrics. In children unable to stand upright, measurement of ulna to predict standing height is recommended, but height prediction equations based on the ulna have not been established in children of African ancestry. We hypothesized that such equations would result in lower predicted height compared to using equations derived from non-African children. We measured prospectively standing height and both ulna in 358 African-Caribbean children without bone deformity or muscle disease, referred to two pulmonary function test laboratories. Interobserver variability was low for standing height (n = 54) and ulna measurement (n = 51) (mean biases [95%CI]: -0.02 [-0.99; 0.95] and 0.05 [-0.91; 1.01] cm, respectively), as well as inter-ulna variability (n = 352; mean bias 0.03 95%CI [-0.66; 0.73] cm). We used the mean value of 247 bilateral ulna measurements to calculate prediction equations using a generalized linear model including age, sex, ulna length, and geographic origin group, the latter showing no influence on the model. In the validation population of 107 children, the median difference [inter-quartile range] between standing height and ulna-predicted height was -0.4 [-2.7; 1.0] cm. Of 260 reliable baseline spirometry, there was a strong concordance between bronchial obstruction diagnosis established using standing height or ulna-predicted height (kappa coefficient: 0.85 [0.77; 0.94]) with only 11 (4.3%) children misclassified. The ulna predicted height calculated from African-Caribbean prediction equations resulted in a smaller height than the height calculated using equations derived from non-African children.

High Nasal Nitric Oxide, Cilia Analyses, and Genotypes in a Retrospective Cohort of Children with Primary Ciliary Dyskinesia.

Rationale: Although children with primary ciliary dyskinesia (PCD) typically have low nasal nitric oxide (nNO), some children with indisputable PCD may have unexplained high nNO concentrations. Objectives: To look for relationships between nNO measures and genetic findings (and cilia motility or ultrastructure when available) in children with PCD with known genotypes. Methods: We retrospectively studied 73 children with PCD (median age, 9.5 [range, 2.1-18.2] yr). nNO was the mean value of a plateau reached while the velum was closed (nNO-VC; threshold, 77 nl ⋅ min-1) or was calculated as the average of five peaks obtained during tidal breathing (threshold, 40 nl ⋅ min-1). Ciliary beat was classified as either motile (including dyskinetic pattern) or immotile, depending on whether motility was present or absent in all cilia, or as a mixture of motile and immotile cilia. Genotypes were classified as pathogenic mutations in genes known to be associated with high nNO (mild genotype), biallelic truncating mutations in other genes (severe mutations), or putative hypomorphic pathogenic mutations (missense, single amino acid deletion, or moderate splicing mutations) in at least one allele believed to be possibly associated with residual production of a functional protein. Results: nNO was above the discriminant threshold in 16 of 73 (21.9%) children (11 nNO-VC and 5 nNO during tidal breathing). High nNO was less frequent in children with severe mutations (2 of 42) than in those with mild genotypes (7 of 10) or at least one hypomorphic mutation (7 of 21) (P < 0.0001). Median (interquartile range) nNO-VC values (n = 60) were significantly different in the three genotype groups: severe mutations, 18 (10-26) nl ⋅ min-1 (n = 36); possible residual functional protein production (putative hypomorphic mutations), 23 (16-68) nl ⋅ min-1 (n = 17); and mild genotypes, 139 (57-216) nl ⋅ min-1 (n = 7) (P = 0.0002). The higher the cilia motility, the higher the nNO-VC (16 [10-23], 23 [17-56], and 78 [45-93] nl ⋅ min-1 in patients with immotile, dyskinetic motile and immotile, and dyskinetic motile cilia, respectively; P < 0.0001), while nNO values were scattered across different ultrastructure defects (P = 0.07). Conclusions: In children with PCD, high nNO values were linked not only to specific genes but also to potentially hypomorphic mutations in other genes (with possible functional protein production). nNO values increased with the proportion of motile cilia.

An international survey on nasal nitric oxide measurement practices for the diagnosis of primary ciliary dyskinesia.

Nasal nitric oxide (nNO) measurements are used in the assessment of patients suspected of having primary ciliary dyskinesia (PCD), but recommendations for performing such measurements have not focused on children and do not include all current practices. To guide the development of a European Respiratory Society-supported technical standard for nNO measurement in children, an international online survey was conducted to better understand current measurement practices among providers involved in PCD diagnostics. 78 professionals responded, representing 65 centres across 18 countries, mainly in Europe and North America. Nearly all centres measured nNO in children and more than half performed measurements before 5 years of age. The test was often postponed in children with signs of acute airway infection. In Europe, the electrochemical technique was more frequently used than chemiluminescence. A similar proportion of centres performed measurements during exhalation against a resistance (49 out of 65) or during tidal breathing (50 out of 65); 15 centres used only exhalation against a resistance and 15 used only tidal breathing. The cut-off values used to discriminate PCD were consistent across centres using chemiluminescence analysers; these centres reported results as an output (nL·min-1). Cut-off values were highly variable across centres using electrochemical devices, and nNO concentrations were typically reported as ppb. This survey is the first to determine real-world use of nNO measurements globally and revealed remarkable variability in methodology, equipment and interpretation. These findings will help standardise methods and training.

International consensus on lung function testing during the COVID-19 pandemic and beyond.

Coronavirus disease 2019 (COVID-19) has negatively affected the delivery of respiratory diagnostic services across the world due to the potential risk of disease transmission during lung function testing. Community prevalence, reoccurrence of COVID-19 surges and the emergence of different variants of SARS-CoV-2 have impeded attempts to restore services. Finding consensus on how to deliver safe lung function services for both patients attending and for staff performing the tests are of paramount importance. This international statement presents the consensus opinion of 23 experts in the field of lung function and respiratory physiology balanced with evidence from the reviewed literature. It describes a robust roadmap for restoration and continuity of lung function testing services during the COVID-19 pandemic and beyond. Important strategies presented in this consensus statement relate to the patient journey when attending for lung function tests. We discuss appointment preparation, operational and environmental issues, testing room requirements including mitigation strategies for transmission risk, requirement for improved ventilation, maintaining physical distance and use of personal protection equipment. We also provide consensus opinion on precautions relating to specific tests, filters, management of special patient groups and alternative options to testing in hospitals. The pandemic has highlighted how vulnerable lung function services are and forces us to re-think how long-term mitigation strategies can protect our services during this and any possible future pandemic. This statement aspires to address the safety concerns that exist and provide strategies to make lung function tests and the testing environment safer when tests are required.

Evaluation of obesity and asthma as risk factors for moderate to severe obstructive sleep apnea in children.

STUDY OBJECTIVES: Asthma and obesity are risk factors for obstructive sleep apnea (OSA) in children but their link to OSA severity is uncertain. We aimed at determining whether asthma or obesity was associated with an increased risk of moderate/severe OSA. METHODS: Children undergoing a one-night polysomnography for suspicion of OSA were retrospectively included. Univariate and multivariate analyses were conducted to assess the clinical and demographic characteristics linked to moderate/severe OSA (obstructive apnea-hypopnea index ≥ 5 events/h of sleep) with odds ratio and 95% confidence interval reported. RESULTS: Four hundred ninety children (311 [64%] boys) were included with a median [25th; 75th percentile] age of 8.7 [5.4; 12.9] years, 164 (33%) nonasthmatics nonobese, 122 (25%) obese nonasthmatics, 125 (26%) asthmatics nonobese, 79 (16%) asthmatics and obese. Moderate/severe OSA was present in 157 (32%) children (75/157 [48%] obese and 52/157 [33%] asthmatics). Independent factors associated with increased or decreased risk of moderate/severe OSA were obesity and male sex (odds ratio 1.82 [1.16; 2.87], P = .01, and 1.55 [1.02; 2.36], P = .04, respectively), and current asthma, age > 6 years, or behavioral disorders (odds ratio 0.45 [0.29; 0.70], P < .001; 0.44 [0.27; 0.73], P < .001; and 0.55 [0.33; 0.92], P = .02, respectively). Abnormal resistance of the respiratory system (measured in 241 children), but not abnormal spirometry (measured in 213 children), increased the risk of moderate/severe OSA (odds ratio 2.95 [1.46-5.96], P = .003). CONCLUSIONS: In our cohort enriched in obese and asthmatic children, obesity was associated with higher risk of moderate/severe OSA whereas current asthma was not. CITATION: Ioan I, Mulier G, Taytard J, Césaire A, Beydon N. Evaluation of obesity and asthma as risk factors for moderate to severe obstructive sleep apnea in children. J Clin Sleep Med. 2022;18(6):1639-1648.

Estimating pulmonary function after surgery for adolescent idiopathic scoliosis using biplanar radiographs of the chest with 3D reconstruction.

AIMS: This study addressed two questions: first, does surgical correction of an idiopathic scoliosis increase the volume of the rib cage, and second, is it possible to evaluate the change in lung function after corrective surgery for adolescent idiopathic scoliosis (AIS) using biplanar radiographs of the ribcage with 3D reconstruction? METHODS: A total of 45 patients with a thoracic AIS which needed surgical correction and fusion were included in a prospective study. All patients underwent pulmonary function testing (PFT) and low-dose biplanar radiographs both preoperatively and one year after surgery. The following measurements were recorded: forced vital capacity (FVC), slow vital capacity (SVC), and total lung capacity (TLC). Rib cage volume (RCV), maximum rib hump, main thoracic curve Cobb angle (MCCA), medial-lateral and anteroposterior diameter, and T4-T12 kyphosis were calculated from 3D reconstructions of the biplanar radiographs. RESULTS: All spinal and thoracic measurements improved significantly after surgery (p < 0.001). RCV increased from 4.9 l (SD 1) preoperatively to 5.3 l (SD 0.9) (p < 0.001) while TLC increased from 4.1 l (SD 0.9) preoperatively to 4.3 l (SD 0.8) (p < 0.001). RCV was correlated with all functional indexes before and after correction of the deformity. Improvement in RCV was weakly correlated with correction of the mean thoracic Cobb angle (p = 0.006). The difference in TLC was significantly correlated with changes in RCV (p = 0.041). It was possible to predict postoperative TLC from the postoperative RCV. CONCLUSION: 3D rib cage assessment from biplanar radiographs could be a minimally invasive method of estimating pulmonary function before and after spinal fusion in patients with an AIS. The 3D RCV reflects virtual chest capacity and hence pulmonary function in this group of patients. Cite this article: Bone Joint J 2022;104-B(1):112-119.