Skin-centered strategies in food allergy prevention.

While the early introduction of food allergens in the infant diet has been shown to be effective at preventing the development of food allergy (FA), its implementation in real life has been associated with various challenges. Interventions aimed at correcting skin barrier dysfunction have been explored in recent decades as a distinct or complementary mean to prevent allergic sensitization through the skin and subsequent development of FA. Studies assessing the application of emollient from birth have yielded conflicting results, and meta-analyses have demonstrated either no effect or only a slight positive effect on FA prevention. However, a careful review of the clinical trials reveals that different emollients were used, which may have explained some of the discrepancies between study results. Emollient application protocols also varied widely between studies. While firm conclusions cannot be drawn with regard to their overall efficacy at preventing FA, the available data provide valuable insight into the characteristics that could be associated with a more effective intervention. Namely, successful trials tended to use emollients with an acidic pH of 5.5, applied over the entire body, and combined with topical corticosteroids in affected areas. Consensus on the optimal strategy to restore skin barrier function could help improve the homogeneity and clinical relevance of future trials on this topic. In the meantime, clinicians should avoid products associated with worse outcomes.

Persistent and symptomatic periodic breathing beyond the neonatal period in full-term infants: A case series.

INTRODUCTION: Periodic breathing (PB) is considered physiological in the neonatal period and usually disappears in the first months of life. There are few data available on persistent PB after the neonatal period. The objective of this study was to characterize infants born at term with persistent PB after the age of 1 month through polysomnography (PSG) performed during symptoms. METHODS: This retrospective case series included infants born at term between 2012 and 2021, without an underlying disease, who presented with symptoms of persistent PB during a PSG. Persistent PB was defined as more than 1 % of total sleep time (TST) of PB after 1 month of life, and PB was defined as a succession of at least three episodes of central apnea lasting more than 3 s and separated by less than 20 s of normal breathing. RESULTS: A total of 10 infants born at term were included. They underwent PSG for brief resolved unexplained events, desaturation, pauses in breathing, cyanosis, and/or signs of respiratory distress. The percentage of TST spent with PB was 18.1 % before 3 months of age (n = 7), and 4.7 % between 3 and 6 months of age (n = 10). During the first PSG, ≥3 % of desaturation events were observed in 77-100 % of the PB episodes. At the first PSG, nine of the 10 infants had an obstructive apnea-hypopnea index of >10/h and five of 10 infants had a central apnea index of >5/h. Gastroesophageal reflux (GER) was suspected in eight infants. All infants showed improvement in the initial symptoms during the first year of life. CONCLUSION: This study presents cases of persistent and symptomatic PB after 1 month of life in infants born at term. The interesting finding was the presence of obstructive sleep apnea syndrome and/or central apnea syndrome in the majority of children, along with GER.

Gas exchange parameters for the prediction of obstructive sleep apnea in infants.

STUDY OBJECTIVES: Sleep laboratory polysomnography (PSG) is the gold standard for obstructive sleep apnea (OSA) diagnosis in infants, but its access remains limited. Oximetry-capnography is another simple and widely used tool that can provide information on the presence of desaturations and alveolar hypoventilation. However, its reliability is debated. This study aimed at examining its use in determining OSA severity in infants. METHODS: This retrospective study was conducted in a sleep unit in a tertiary hospital, in infants < 4 months old with clinical signs of OSA or Pierre Robin Sequence (PRS) who underwent a one-night PSG coupled with oximetry-capnography. RESULTS: Among the 78 infants included (median [IQR] age: 61 [45-89] days at PSG), 44 presented with PRS, and 34 presented with isolated airway obstruction. The clinical, sleep and respiratory characteristics were not significantly different between the two subgroups. In the entire cohort, 63.5% had severe OSA. Median OAHI was 14.5 events/h [7.4-5.9], SpO2 was 97.4% [96.5-98.1], and PtcCO2 was 41.1 mmHg [38.3-44.9]. The optimal threshold to predict OAHI > 10 events/h was 6 events/h for ODI3% (sensitivity 95.7%, specificity 51.9%) and 2 events/h for ODI4% (sensitivity 95.7%, specificity 48.1%). CONCLUSIONS: Whereas transcutaneous capnography does not appear to be sufficient in predicting severe OSA in infants < 4 months old with PRS or clinical signs of OSA, oximetry may be a useful alternative for the screening of severe OSA in infants in the absence of PSG.

Hypoventilation in patients with Prader-Willi syndrome across the pediatric age.

OBJECTIVES: Few data on alveolar hypoventilation in Prader-Willi syndrome (PWS) are available and the respiratory follow-up of these patients is not standardized. The objectives of this study were to evaluate the prevalence of alveolar hypoventilation in children with PWS and identify potential risk factors. STUDY DESIGN: This retrospective study included children with PWS recorded by polysomnography (PSG) with transcutaneous carbon dioxide pressure (PtcCO2) or end-tidal CO2 (ETCO2) measurements, between 2007 and 2021, in a tertiary hospital center. The primary outcome was the presence of alveolar hypoventilation defined as partial pressure of carbon dioxide (pCO2) ≥ 50 mmHg during ≥2% of total sleep time (TST) or more than five consecutive minutes. RESULTS: Among the 57 included children (38 boys, median age 4.8 years, range 0.1-15.6, 60% treated with growth hormone [GH], 37% obese), 19 (33%) had moderate-to-severe obstructive sleep apnea syndrome (defined as obstructive apnea-hypopnea index ≥5/h) and 20 (35%) had hypoventilation. The median (range) pCO2 max was 49 mmHg (38-69). Among the children with hypoventilation, 25% were asymptomatic. Median age and GH treatment were significantly higher in children with hypoventilation compared to those without. There was no significant difference in terms of sex, BMI, obstructive or central apnea-hypopnea index between both groups. CONCLUSION: The frequency of alveolar hypoventilation in children and adolescents with PWS is of concern and may increase with age and GH treatment. A regular screening by oximetry-capnography appears to be indicated whatever the sex, BMI, and rate of obstructive or central apneas.

Unattended home sleep studies for the diagnosis of obstructive sleep apnea in a population of French children.

BACKGROUND: Ambulatory exams were preferred in children during the COVID-19 pandemic. Polysomnography (PSG), the gold standard for obstructive sleep apnea (OSA) diagnosis, requires several leads and sensors to be attached to the child's body. Children are more comfortable with respiratory polygraphic (RP) recording, which needs fewer sensors. OBJECTIVE: To compare respiratory parameters obtained by home RP with those obtained by home PSG with the device installed at the child's home by a trained sleep nurse from a national health care provider. METHODS: Data from home PSGs performed in children aged 2-19 years were retrospectively included. The obstructive apnea-hypopnea index (OAHI) was computed in PSG and then in RP after removing the sleep signals. The two indexes were compared using non-parametric paired Wilcoxon rank test, Bland-Altman analysis and sensitivity-specificity analysis. RESULTS: 44 PSGs of 44 children were included with only 34 (77%) PSGs interpretable. Median (min-max) OAHI was significantly underestimated in RP than in PSG (2.2 (0-25) vs 4.0 (0.4-28), p < 0.0001), confirmed also by the Bland-Altman diagram, the magnitude of the difference being mean ± standard deviation -1.7 ± 1.7. The sensitivity and specificity of OAHI in RP to identify an OAHI ≥2/h in PSG was 0.91 for both. CONCLUSION: Unattended ambulatory RP performed at child's house and installed under carefully controlled conditions is a useful exam for diagnosing OSA in children with or without comorbidities. However, RP must be installed in a supervised environment and interpreted with caution as it tends to underestimate OSA severity.

Neurocognitive evaluation of children with down syndrome and obstructive sleep apnea syndrome.

Obstructive sleep apnea syndrome (OSAS) treatment has been shown to improve cardiac behavioral and cognitive functions in typically developing children. Early OSAS diagnosis in children with Down syndrome (DS) would be important to prevent its complications, especially cognitive ones, but remains overlooked. The main objective of our study was to assess the cognitive function of children with DS, with and without OSAS. The second objective was to determine the impact of the therapeutic intervention on the cognitive function of children with OSAS. This study included 41 children with DS who underwent polysomnography for OSAS diagnosis and a cognitive evaluation. They were aged between 3.4 and 17.3 years and 24 (59%) were boys. Their median OAHI was 2.6 (0-31)/h of sleep, 30 (73%) were diagnosed with OSAS (15 had mild OSAS, and 15 had moderate/severe OSAS). Some scores of the Raven's colored progressive matrices were negatively correlated with the respiratory arousal index, OAHI tended to be positively correlated with Reiss behavioral problems. 24 (59%) patients received a treatment. Even if we were unable to demonstrate this formally due that only 16 children (39%) accepted a follow-up visit, some displayed improvement in their neuropsychological scores, especially those with moderate/severe OSAS after treatment. Children with DS have low intellectual abilities and more risk of developing OSAS compared to the general population, which may lead to further neurocognitive impairment. Early screening and management are important in this population to prevent any further neurocognitive delay in their development.

Long term noninvasive ventilation and continuous positive airway pressure in children with neuromuscular diseases in France.

The aim of the study was to describe the characteristics of children with neuromuscular diseases treated with long term noninvasive ventilation or continuous positive airway pressure in France. On June 1st 2019, 387 patients (63% boys, mean age 11.2 ± 5.5 years) were treated with long term noninvasive ventilation/continuous positive airway pressure. Thirty three percent of patients had spinal muscular atrophy, 30% congenital myopathy/dystrophy, 20% Duchenne muscular dystrophy, 7% Steinert myotonic dystrophy, and 9% other neuromuscular diseases. Ninety-four percent of patients were treated with long term noninvasive ventilation and 6% with continuous positive airway pressure. Treatment was initiated electively for 85% of patients, mainly on an abnormal overnight gas exchange recording (38% of patients). Noninvasive ventilation/continuous positive airway pressure was initiated during a respiratory exacerbation in 15% of patients. Mean duration of noninvasive ventilation/continuous positive airway pressure was 3.3 ± 3.1 years. Mean objective long term noninvasive ventilation/continuous positive airway pressure use was 8.0 ± 3.1 h/24. Spinal muscular atrophy, congenital myopathy/dystrophy, and Duchenne muscular dystrophy represented 83% of children with neuromuscular diseases treated with long term noninvasive ventilation in France. Screening for nocturnal hypoventilation was satisfactory as noninvasive ventilation /continuous positive airway pressure was predominantly initiated electively.

Respiratory management of spinal muscular atrophy type 1 patients treated with Nusinersen.

INTRODUCTION: The recent development of disease-modifying treatments in spinal muscular atrophy (SMA) type 1 shifted these patients' management from palliative to proactive. The aim of this study was to assess patients' nocturnal gas exchanges before noninvasive ventilation (NIV) initiation and their clinical evolution to determine if capnia is a good criterion to decide when to introduce respiratory support. PATIENTS AND METHODS: This multicentric retrospective study reports the respiratory management and evolution of 17 SMA type 1 children (10 females) for whom treatment with Nusinersen was initiated between 2016 and 2018. RESULTS: Median [interquartile range-IQR] age at diagnosis and at first Nusinersen injection was of 4 [3;8] and 4 [3;9] months, respectively. Patients were followed during 38 [24;44] months. Thirteen (76%) patients were started on NIV at a median [IQR] age of 12 [9;18] months. Repeated hospitalizations and intensive care unit admissions were needed for 11 of them. Blood gas and nocturnal gas exchange recordings performed before NIV initiation were always normal. 9/13 X-ray performed before NIV showed atelectasis and/or acute lower respiratory tract infections. There was a significant decrease in the total number of hospital admissions between the first and second year of treatment (p = 0.04). CONCLUSION: This study shows that patients do not present with nocturnal hypoventilation before respiratory decompensations and NIV initiation, and suggests that a delay in NIV initiation might result in respiratory complications. There is a need for disease-centered guidelines for the respiratory management of these patients, including NIV indications.

Pulse transit time as a diagnostic test for OSA in children with Down syndrome.

STUDY OBJECTIVES: Children with Down syndrome (DS) are at risk of obstructive sleep apnea (OSA), but the access to sleep lab polysomnography (PSG) is limited. Simplified techniques are needed, such as polygraphy coupled with pulse transit time (PTT-PG) that detects respiratory events and the total autonomic arousals index (PTTAI). Our objective was to assess the ability of PTT-PG compared with PSG to diagnose OSA in children with DS. METHODS: In this prospective multicenter study, patients with DS underwent a full-night PSG coupled with PTT. Sleep questionnaires (Sleep Disturbance Scale for Children and Pediatric Sleep Questionnaire) were filled by parents. PSG and PTT-PG results were compared to test their sensibility and specificity to diagnose OSA. RESULTS: A total of 53 patients with DS were included; their median age was 9.3 years. An obstructive apnea-hypopnea index (OAHI) by PSG > 1 event/h was found in 36 (68%) patients, OAHI was > 1 and < 5 events/h in 18 patients (34%), ≥ 5 and < 10 events/h in 11 patients (21%), and ≥ 10 events/h in 7 patients (13%). OAHI was larger on PSG than on PTT-PG (P = .0005). For OSA diagnosis, the sensitivity was excellent for OAHI by PTT-PG if the added total PTTAI was > 1 event/h (1.0) and the specificity was high for the Pediatric Sleep Questionnaire (0.88) and OAHI > 1 event/h on PTT-PG (1.0). CONCLUSIONS: More than two-thirds of children with DS referred for screening by a genetics specialist had OSA diagnosed by PSG. With its excellent sensitivity and specificity, PTT-PG could be a good and simplified alternative to PSG to diagnose OSA in children with DS. CITATION: Ioan I, Weick D, Sevin F, et al. Pulse transit time as a diagnostic test for OSA in children with Down syndrome. J Clin Sleep Med. 2022;18(1):119-128.

Paediatric long term continuous positive airway pressure and noninvasive ventilation in France: A cross-sectional study.

OBJECTIVE: To describe the characteristics of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in France. DESIGN: Cross-sectional national survey. SETTING: Paediatric CPAP/NIV teams of 28 tertiary university hospitals in France. PATIENTS: Children aged <20 years treated with CPAP/NIV since at least 3 months on June 1st, 2019. INTERVENTION: An anonymous questionnaire was filled in for every patient. RESULTS: The data of 1447 patients (60% boys), mean age 9.8 ± 5.8 years were analysed. The most frequent underlying disorders were: upper airway obstruction (46%), neuromuscular disease (28%), disorder of the central nervous system (13%), cardiorespiratory disorder (7%), and congenital bone disease (4%). Forty-five percent of the patients were treated with CPAP and 55% with NIV. Treatment was initiated electively for 92% of children, while 8% started during an acute illness. A poly(somno)graphy (P(S)G) was performed prior to treatment initiation in 26%, 36% had a P(S)G with transcutaneous carbon dioxide monitoring (PtcCO2), while 23% had only a pulse oximetry (SpO2) with PtcCO2 recording. The decision of CPAP/NIV initiation during an elective setting was based on the apnea-hypopnea index (AHI) in 41% of patients, SpO2 and PtcCO2 in 25% of patients, and AHI with PtcCO2 in 25% of patients. Objective adherence was excellent with a mean use of 7.6 ± 3.2 h/night. Duration of CPAP/NIV was 2.7 ± 2.9 years at the time of the survey. CONCLUSION: This survey shows the large number of children treated with long term CPAP/NIV in France with numerous children having disorders other than neuromuscular diseases.

Feasibility of parent-attended ambulatory polysomnography in children with suspected obstructive sleep apnea.

STUDY OBJECTIVES: Due to a limited number of pediatric sleep centers, the aim was to test the feasibility of ambulatory polysomnography (PSG-home) in a group of French children suspected of OSA. METHODS: Children undergoing one-night PSG-home, with the device installed at the pediatric sleep physician's office, were prospectively included. General failure was considered when PSG-home recording captured < 5 h of artifact-free sleep or when ≥ 1 channel (nasal flow, thoraco-abdominal belts, oximetry) presented artifacts > 75% of the recording time. No-OSA was defined as an obstructive apnea-hypopnia index (OAHI) < 1 event/h and respiratory-related arousals index (RAI) < 1 event/h. OSA was defined as upper airways resistance syndrome (UARS) with OAHI < 1 event/h with RAI ≥ 1 event/h, or mild OSA (OAHI ≥ 1 event/h-5 events/h), moderate OSA (OAHI ≥ 5 events/h-10 events/h), or severe OSA (OAHI ≥ 10 events/h). Parents completed a severity hierarchy score questionnaire, Conners Parent Rating Scale, and an adapted Epworth Sleepiness Scale. RESULTS: Fifty-seven children aged 3 through 16 years were included. PSG-home was technically acceptable in 46 (81%). Failure due to nasal cannula was observed in 11% (n = 6), oximetry in 7% (n = 4), and both in 2% (n = 1) of cases. No difference in feasibility was found according to age, sex, OSA severity, or comorbidities. There were 14 (25%) children categorized as no-OSA, 43 (75%) as OSA, 4 (7%) as UARS, 26 (46%) as mild, 6 (10%) as moderate, and 7 (12%) as severe OSA. Neither questionnaires nor clinical and physical examination predicted OSA diagnosis. CONCLUSIONS: When equipment is installed at the professional's office and a parent monitors the child, PSG-home is feasible and technically acceptable in children aged 3 through 16 years old. The short delay and feasibility provided by PSG-home could improve the management of children suspected of OSA.

Diaphragmatic paralysis in young children: A literature review.

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.

[Sleep-related breathing disorders in children and teenagers: diagnosis, consequences and comorbidities]. / Troubles respiratoires obstructifs du sommeil chez l'enfant et l'adolescent: diagnostic, conséquences et comorbidités.

Obstructive sleep apnoea syndrome (OSAS) is a frequent sleep-related breathing disorder in children with a prevalence of approximately 3% between the ages of 3 and 8. Its origin is multifactorial (hypertrophy of pharyngeal soft tissues, narrowing of the bone airways, damage to the neuromuscular tone). The symptoms and clinical signs to look for during the day and at night are directly related to upper airway (UA) obstruction or poor sleep quality. After a complete anamnestic and clinical evaluation, including nasofibroscopy, the ENT specialist will refer to an adeno-amygdalectomy or night recording by polysomnography or polygraph to confirm the diagnosis in the child. Among adolescents, the prevalence of OSAS is reported to be between 0.5 and 6%. The main risk factors are obesity, male sex and a history of tonsillectomy. In addition to the classic symptoms of childhood OSAS, this syndrome may, in adolescents, manifest itself as a misleading semiology of dyssomnia, excessive daytime sleepiness and/or mood disorders. Differential diagnoses with risk behaviours, phase delay or narcolepsy should be systematically discussed. It is essential to record breathing during sleep. Even if the obstructive apnea/hypopnea index is low, it must be considered. In both children and adolescents, multidisciplinary management (ENT, orthodontist, maxillofacial physiotherapist, pulmonologist) should be early in order to avoid neurocognitive, behavioural, cardiovascular and metabolic complications. Maxillofacial surgery can be discussed from the age of 15. It is important not to forget to pay attention to the rules of healthy living and sleep as well as the management of obesity.

More Relaxation by Deep Breath on Methacholine- Than on Exercise-Induced bronchoconstriction during the Routine Testing of Asthmatic Children.

Deep inspiration (DI) dilates normal airway precontracted with methacholine. The fact that this effect is diminished or absent in asthma could be explained by the presence of bronchial inflammation. The hypothesis was tested that DI induces more relaxation in methacholine induced bronchoconstriction-solely determined by the smooth muscle contraction-than in exercise induced bronchoconstriction, which is contributed to by both smooth muscle contraction and airway wall inflammation. The respiratory conductance (Grs) response to DI was monitored in asthmatic children presenting a moderately positive airway response to challenge by methacholine (n = 36) or exercise (n = 37), and expressed as the post- to pre-DI Grs ratio (GrsDI). Both groups showed similar change in FEV1 after challenge and performed a DI of similar amplitude. GrsDI however was significantly larger in methacholine than in exercise induced bronchoconstriction (p < 0.02). The bronchodilatory effect of DI is thus less during exercise- than methacholine-induced bronchoconstriction. The observation is consistent with airway wall inflammation-that characterizes exercise induced bronchoconstriction-rendering the airways less responsive to DI. More generally, it is surmised that less relief of bronchoconstriction by DI is to be expected during indirect than direct airway challenge. The current suggestion that airway smooth muscle constriction and airway wall inflammation may result in opposing effects on the bronchomotor action of DI opens important perspective to the routine testing of asthmatic children. New crossover research protocols comparing the mechanical consequences of the DI maneuver are warranted during direct and indirect bronchial challenges.

Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis.

The combination of lumacaftor and ivacaftor (LUM/IVA) has been reported to induce a mean acute absolute drop of -4.1% predicted forced expiratory volume in 1s (FEV1) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV1 changes after the first dose of LUM/IVA in CF patients. A total of 32 pediatric patients were included. Respiratory manifestations occurred in only 3 patients (9.4%), but FEV1 consistently decreased (-10.4±4.6%, range: -1.5; -21.8%). FEV1 only partially resumed after salbutamol inhalation. Patients with previously known significant reversible airway obstruction and low FEV1 were more at risk of FEV1 decrease.

Palivizumab prophylaxis in infants with cystic fibrosis does not delay first isolation of Pseudomonas aeruginosa or Staphylococcus aureus.

Respiratory syncytial virus (RSV) infections may worsen cystic fibrosis (CF) lung disease and favor Pseudomonas aeruginosa (Pa) or Staphylococcus aureus (Sa) acquisition, which is of particular importance in the youngest patients. We aimed to determine the effectiveness of PVZ on microbiological outcomes in young children with CF. We conducted a retrospective case-control study to compare these outcomes in children who systematically received PVZ (PVZ+; n = 40) or not (PVZ-; n = 140). One case was matched with at least three same-gender controls born the same year and month. Median (range) age at first Pa isolation was not statistically different between PVZ- (12.3 [3.8-32.6] months) and PVZ+ (10.4 [1.2-33.0] months; p = 0.953) patients. A similar trend was found for Sa (PVZ+: 6.4 [2.0-59.0] months; PVZ-: 3.8 [0.1-74.1] months; p = 0.191). The proportion of Pa isolations by 3 years of age did not differ between groups (PVZ+ 40% vs. PVZ- 41.4%), but this proportion was higher for Sa in the PVZ+ group (97%) than in the PVZ- group (85%; p = 0.001). Healthcare consumption and growth outcomes did not significantly differ between groups. CONCLUSION: Systematic PVZ use did not delay key pathogen acquisition in young children with CF. What is known: • Palivizumab is the only available monoclonal antibody against respiratory syncytial virus infection. • Whether or not it is useful in infants with cystic fibrosis remains controversial. What is new: • Palivizumab does not delay key pathogens (Pseudomonas aeruginosa, Staphylococcus aureus) first isolation in young children with cystic fibrosis. • Palivizumab does not reduce healthcare consumption or improve growth during the first 3 years of life of young children with cystic fibrosis.

Breathing-related changes of respiratory resistance in vocal cord dysfunction.

Vocal cord dysfunction induced by exercise in children with uncontrolled asthma was identified by laryngoscopy. The paradoxical adduction of the vocal cords was also indicated by the breathing-related changes of the forced oscillation respiratory resistance showing prominent increase during inspiration and a large positive difference between inspiration and expiration. The breathing-related changes of respiratory resistance offer thus a useful first-line technique to diagnose vocal cord dysfunction.