Respiratory and Neurodevelopmental Outcomes at 3 Years of Age of Neonates Diagnosed with Sleep-Disordered Breathing.

Objectives: Understanding the long-term consequences of sleep-disordered breathing (SDB) in neonates is crucial. A lack of consensus on diagnostic and treatment thresholds has resulted in limited research in this area. Our study aims to describe the trajectory of SDB in a cohort of high-risk neonates and their respiratory and neurodevelopmental outcomes at 3 years of age, and explore the relationship between SDB during early infancy and neurocognitive outcomes. Methods: A retrospectively identified cohort of neonates with moderate-severe SDB were prospectively followed at 3 years of age. Data collected included last polysomnography (PSG) parameters up to the age of 3 years and sleep physician's recommendations, duration of CPAP use, compliance with treatment, timing of SDB resolution, and neurodevelopmental outcomes. Univariate and multivariate logistic regression analyses were performed to evaluate the association between important respiratory and sleep breathing parameters with the developmental outcomes. Results: Eighty neonates were included. Respiratory and developmental outcomes were available for 58 (72.5%) and 56 (70%) patients, respectively. In most patients (47/58, 81%), SDB had resolved by 3 years of age. Survival without major developmental delay was seen in 32/56 (57%), but a significant proportion (21/56, 37.5%) demonstrated global developmental delay. Following univariate analysis, primary diagnosis, apnoea-hypopnoea index (AHI) at the time of last PSG and SDB outcome was significantly associated with developmental delay. However, these associations were not seen in multivariate analysis. Conclusions: Despite severity at baseline, SDB resolved in the majority of patients with time and treatment. Although statistically insignificant, logistic regression analysis identified some clinically important associations between neonatal SDB and neurodevelopmental outcomes.

Hereditary haemorrhagic telangiectasia: A primer for the paediatrician.

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant condition characterised by small telangiectasias and larger multisystem arteriovenous malformations (AVMs). Common sites of AVMs include in the nose, lungs, brain and liver. These lesions are prone to rupture, leading to complications including recurrent epistaxis and significant haemorrhage. Pulmonary hypertension (PH) can also occur. This review presents an update on the genetics, clinical manifestations, management options, and screening recommendations for children with HHT.

Provision of bubble continuous positive airway pressure for the stabilisation of extremely and very preterm infants after birth: A single-centre experience.

AIM: To describe the effect of resuscitation with bubble CPAP (bCPAP) versus T-piece device at birth on early clinical parameters and hospital outcomes in infants born <32 weeks gestation. METHODS: This is a single-centre pre- and post-implementation study comparing outcomes in two epochs. In epoch 1 (1 July 2013-31 December 2014), infants were managed with non-humidified gas using Neopuff® T-piece devices to support breathing after birth. In epoch 2 (1 March 2020-31 December 2021), routine application of bCPAP with humidified gas was introduced at birth. RESULTS: Three hundred fifty-seven patients were included (176 epoch 1, 181 epoch 2). The mean gestational age was 28 ± 2 weeks. The demographics of the two epochs were comparable. There were significant improvements in outcomes of infants in epoch 2 with less infants intubated at delivery (16% vs. 4%, P ≤ 0.001), improved 5 min Apgar (7 vs. 8, P ≤ 0.001), reduced need for ventilation (21% vs. 8.8%, P ≤ 0.001), duration of ventilation in the first 72 h (9.6 vs. 4.6 h) and mortality (10.8% vs. 1.7%, P ≤ 0.001). There was, increased incidence of chronic lung disease (30% vs. 55%, P = 0.02) but no increase in infants discharged on oxygen (3.8% vs. 5%, P = 0.25). Similar findings were observed in a subgroup of infants born <25 weeks' gestation with no increase in the incidence of CLD. CONCLUSION: Introducing application of bCPAP from the first breaths in infants <32 weeks' gestation was associated with better short-term outcomes and mortality, albeit with increased incidence of CLD. The subgroup of infants born <25 weeks' gestation showed similar change in outcomes, with no increase in CLD.

Comparative sensitivity of early cystic fibrosis lung disease detection tools in school aged children.

BACKGROUND: Effective detection of early lung disease in cystic fibrosis (CF) is critical to understanding early pathogenesis and evaluating early intervention strategies. We aimed to compare ability of several proposed sensitive functional tools to detect early CF lung disease as defined by CT structural disease in school aged children. METHODS: 50 CF subjects (mean±SD 11.2 ± 3.5y, range 5-18y) with early lung disease (FEV1≥70 % predicted: 95.7 ± 11.8 %) performed spirometry, Multiple breath washout (MBW, including trapped gas assessment), oscillometry, cardiopulmonary exercise testing (CPET) and simultaneous spirometer-directed low-dose CT imaging. CT data were analysed using well-evaluated fully quantitative software for bronchiectasis and air trapping (AT). RESULTS: CT bronchiectasis and AT occurred in 24 % and 58 % of patients, respectively. Of the functional tools, MBW detected the highest rates of abnormality: Scond 82 %, MBWTG RV 78 %, LCI 74 %, MBWTG IC 68 % and Sacin 51 %. CPET VO2peak detected slightly higher rates of abnormality (9 %) than spirometry-based FEV1 (2 %). For oscillometry AX (14 %) performed better than Rrs (2 %) whereas Xrs and R5-19 failed to detect any abnormality. LCI and Scond correlated with bronchiectasis (r = 0.55-0.64, p < 0.001) and AT (r = 0.73-0.74, p < 0.001). MBW-assessed trapped gas was detectable in 92 % of subjects and concordant with CT-assessed AT in 74 %. CONCLUSIONS: Significant structural and functional deficits occur in early CF lung disease, as detected by CT and MBW. For MBW, additional utility, beyond that offered by LCI, was suggested for Scond and MBW-assessed gas trapping. Our study reinforces the complementary nature of these tools and the limited utility of conventional oscillometry and CPET in this setting.

Clinical characteristics, associated comorbidities and hospital outcomes of neonates with sleep disordered breathing: a retrospective cohort study.

OBJECTIVE: Awareness of the need for early identification and treatment of sleep disordered breathing (SDB) in neonates is increasing but is challenging. Unrecognised SDB can have negative neurodevelopmental consequences. Our study aims to describe the clinical profile, risk factors, diagnostic modalities and interventions that can be used to manage neonates with SDB to facilitate early recognition and improved management. METHODS: A single-centre retrospective study of neonates referred for assessment of suspected SDB to a tertiary newborn intensive care unit in New South Wales Australia over a 2-year period. Electronic records were reviewed. Outcome measures included demographic data, clinical characteristics, comorbidities, reason for referral, polysomnography (PSG) data, interventions targeted to treat SDB and hospital outcome. Descriptive analysis was performed and reported. RESULTS: Eighty neonates were included. Increased work of breathing, or apnoea with oxygen desaturation being the most common reasons (46% and 31%, respectively) for referral. Most neonates had significant comorbidities requiring involvement of multiple specialists (mean 3.3) in management. The majority had moderate to severe SDB based on PSG parameters of very high mean apnoea-hypopnoea index (62.5/hour) with a mean obstructive apnoea index (38.7/hour). Ten per cent of patients required airway surgery. The majority of neonates (70%) were discharged home on non-invasive ventilation. CONCLUSION: SDB is a serious problem in high-risk neonates and it is associated with significant multisystem comorbidities necessitating a multidisciplinary team approach to optimise management. This study shows that PSG is useful in neonates to diagnose and guide management of SDB.

OSA type-III and neurocognitive function.

Obstructive sleep apnea (OSA) due to a hypertrophy of the adenoids and/or the tonsils in otherwise healthy children is associated with neurocognitive dysfunction and behavioural disorders with various degrees of hyperactivity, aggressiveness, sometimes evolving to a label of attention-deficit hyperactivity disorder. Children with anatomical and/or functional abnormalities of the upper airways represent a very specific population which is at high risk of OSA (also called complex OSA or OSA type III). Surprisingly, the neurocognitive consequences of OSA have been poorly studied in these children, despite the fact that OSA is more common and more severe than in their healthy counterparts. This may be explained by that fact that screening for OSA and sleep-disordered breathing is not systematically performed, the performance of sleep studies and neurocognitive tests may be challenging, and the respective role of the underlining disease, OSA, but also poor sleep quality, is complex. However, the few studies that have been performed in these children, and mainly children with Down syndrome, tend to show that OSA, but even more disruption of sleep architecture and poor sleep quality, aggravate the neurocognitive impairment and abnormal behaviour in these patients, underlining the need for a systematic and early in life assessment of sleep and neurocognitive function and behaviour in children with OSA type III.

Assessment of obstructive sleep apnoea in children: What are the challenges we face?

There is an increasing demand for the assessment of sleep-disordered breathing in children of all ages to prevent the deleterious neurocognitive and behaviour consequences of the under-diagnosis and under-treatment of obstructive sleep apnoea [OSA]. OSA can be considered in three broad categories based on predominating contributory features: OSA type 1 [enlarged tonsils and adenoids], type II [Obesity] and type III [craniofacial abnormalities, syndromal, storage diseases and neuromuscular conditions]. The reality is that sleep questionnaires or calculations of body mass index in isolation are poorly predictive of OSA in individuals. Globally, the access to testing in tertiary referral centres is comprehensively overwhelmed by the demand and financial cost. This has prompted the need for better awareness and focussed history taking, matched with simpler tools with acceptable accuracy used in the setting of likely OSA. Consequently, we present key indications for polysomnography and present scalable, existing alternatives for assessment of OSA in the hospital or home setting, using polygraphy, oximetry or contactless sleep monitoring.