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Chiari type 1 malformation (CM1) may occasionally lead to central sleep apnea (CSA). We studied, in a large clinical cohort of pediatric CM1 patients, the effect of CM1 on breathing during sleep. This is a retrospective single pediatric pulmonology center study with a systematic evaluation of pediatric CM1 patients under age 18 with polysomnography (PSG) during 2008–2020. Children with syndromes were excluded. All patients had undergone head and spine magnetic resonance imaging. We included 104 children with CM1 with a median age of 7 (interquartile range (IQR) 5–13) years. The median extent of tonsillar descent (TD) was 13 (IQR 10–18) mm. Syringomyelia was present in 19 children (18%). Of all children, 53 (51%) had normal PSG, 35 (34%) showed periodic breathing or central apnea and hypopnea index ≥5 h−1, and 16 (15%) displayed features of compensated central hypoventilation and end-tidal or transcutaneous carbon dioxide 99th percentile level above 50 mmHg. TD had the best predictive value for central breathing disorders. In a linear model, both age (61%) and TD (39%) predicted median breathing frequency (R = 0.33, p < 0.001). Although severe CSA is a rare complication of brainstem compression in pediatric patients with CM1, short arousal-triggered episodes of periodic breathing and mild compensated central hypoventilation are common. TD shows the best but still poor prediction of the presence of a central breathing disorder. This highlights the use of PSG in patient evaluation. Posterior fossa decompression surgery effectively treats central breathing disorders. • Severe central sleep apnea (CSA) is rare in Chiari type 1 malformation (CM1). • CM1 leads often leads to mild CSA and compensated central hypoventilation. • Tonsillar descent shows the best but poor prediction of central breathing disorder. • Polysomnography is valuable tool in patient evaluation in CM1. • Posterior fossa decompression effectively treats central breathing disorders in CM1. [ABSTRACT FROM AUTHOR]

Background: B-lines in lung ultrasound can be used to estimate lung liquid. B-lines are ring-down artifacts that arise from alterations to subpleural lung parenchyma. Lung ultrasound has been used to differentiate between diseases causing respiratory symptoms in neonates. B-lines are also seen in healthy infants during postnatal adaptation. Static lung compliance is a measure of the elasticity of the lungs. Objectives: Our aim was to document lung ultrasound findings, static lung compliance and their relationship during postnatal adaptation in healthy term infants. Methods: Lung ultrasound and measurement of static lung compliance were performed in 34 infants at ages of 0-4 and 24 h. B-lines in lung ultrasound were scored using a 5-step scale. Separate ultrasound scores for the upper and lower fields were also calculated. Results: A significant decrease in the abundance of B-lines and a concomitant significant improvement in static lung compliance was observed from <4 to 24 h of age. At <4 h the B-lines were significantly more abundant in the lower fields. No significant correlation existed between lung ultrasound and static lung compliance. Conclusion: The concomitant decrease in the B-lines in ultrasound and the increase in lung compliance during the first 24 h are likely to reflect clearance of lung liquid. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: Delayed postnatal removal of lung liquid may result in respiratory distress, which is more common in infants born by cesarean section. Vertical artefacts (B-lines) arising from the lung surface in lung ultrasound have been shown to correlate with the liquid content of the lungs. Objectives: We studied whether lung ultrasound could be used for the assessment of postnatal lung liquid in healthy term infants born vaginally and by cesarean section. Methods: Lung ultrasound was performed 1, 3 and 24 h after birth to 22 vaginally born infants and 20 infants born by elective cesarean section. The abundance of B-lines was scored for each infant and time point by two independent observers blinded to the mode of delivery and time point on the examination on a five-step scale. Results: In both groups, a significant decrease in abundance of B-lines, indicative of lung liquid absorption, was observed during the first 24 h. 3 h after birth cesarean section was associated with significantly higher lung liquid content than vaginal delivery. Conclusion: The noninvasive bedside ultrasound method for estimation of lung liquid is a promising tool for the early identification of infants at risk for pulmonary maladaptation. Copyright © 2013 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Cardiomyopathies (CMPs) are a heterogeneous group of diseases, frequently genetic, affecting the heart muscle. The symptoms range from asymptomatic to dyspnea, arrhythmias, syncope, and sudden cardiac death. This study is focused on MYH7 (beta-myosin heavy chain), as this gene is commonly mutated in cardiomyopathy patients. Due to the high combined prevalence of MYH7 variants and severe health outcomes, it is one of the most frequently tested genes in clinical settings. We analyzed the clinical presentation and natural history of 48 patients with MYH7- related cardiomyopathy belonging to a cohort from a tertiary center at Helsinki University Hospital, Finland. We made special reference to three age subgroups (0--1, 1--12, and >12 years). Our results characterize a clinically significant MYH7 cohort, emphasizing the high variability of the CMP phenotype depending on age. We observed a subgroup of infants (0--1 years) with MYH7 associated severe DCM phenotype. We further demonstrate that patients under the age of 12 years have a similar symptom burden compared to older patients. [ABSTRACT FROM AUTHOR]

Childhood interstitial lung disease (chILD) is an umbrella concept covering a wide range of rare lung diseases, many of which are unique to childhood. The diagnosis is based on clinical presentation, multidetector computed tomography (MDCT), genetic testing, lung-function testing, and lung biopsy. Because knowledge of the usefulness of MDCT pattern recognition in ChILD is at present limited, we examined the occurrence of MDCT patterns in children with histologically confirmed interstitial lung disease. We searched the biopsy, MDCT, and clinical information database of a single national paediatric referral hospital for 2004–2020. Data were from affected children under age 18. MDCT images we reanalysed while blinded to the identity and referral information. We included 90 patients, of whom 63 (70 %) were male. The median age at biopsy was 1.3 years (interquartile range 0.1–16.8). Biopsy findings fell into 26 histological classes covering all nine chILD classification categories. We recognized six distinct MDCT patterns: neuroendocrine cell hyperplasia of infancy (23), organizing pneumonia (5), non-specific interstitial pneumonia (4), bronchiolitis obliterans (3), pulmonary alveolar proteinosis (2), and bronchopulmonary dysplasia (n = 2). Of the total 90, in 51 (57 %) children, none of these six MDCT patterns appeared. Of those 39 children with a recognizable MDCT pattern, in 34 (87 %), that pattern predicted their final diagnosis. Among cases of chILD, we identified a specific predefined MDCT pattern in only 43 %. However, when such a recognizable pattern occurred, it was predictive of the final chILD diagnosis. [ABSTRACT FROM AUTHOR]

The 38 infants with a clinical presentation of NEHI were divided into 11 with NEHI, with an NEC count of 10% or more, 11 with NEHI-like disease, with an NEC count of less than 10% and 16 with NEHI syndrome, where no lung biopsy had been taken (Figure 1). All infants with NEHI and NEHI-like disease had normal lung histology and we ruled out ongoing interstitial lung disease with normal follow-up, high-resolution lung CTs and an extensive 114 gene pulmonology panel. Abbreviations CT computerised tomography NEC neuroendocrine cell NEHI neuroendocrine cell hyperplasia of infancy Neuroendocrine cell hyperplasia of infancy (NEHI) is the most common interstitial childhood lung disease. [Extracted from the article]

Measurement of dynamic lung compliance during breathing requires measurement of esophageal pressure, whereas static respiratory system compliance (Crs) method requires several airway occlusions. Despite their precision these compliance methods are cumbersome and not suitable for evaluation of pulmonary system in intensive care. The current ventilators display dynamic Crs, which, however, is seldom utilized in clinical practice. We studied the feasibility of ventilator-derived dynamic Crs measurement in pulmonary evaluation after congenital cardiac surgery in children. In 50 children static Crs was measured by double-occlusion technique, and compared with simultaneous ventilator-derived dynamic Crs values. The early postoperative dynamic and static Crs showed a correlation (r = 0.57, p < 0.0001), but static Crs was 48% higher than dynamic (p < 0.0001). Dynamic Crs measurement showed no correlation with radiographic lung edema findings, whereas the static Crs showed a negative correlation with radiographic lung edema scoring (r = −0.50, p = 0.0002). Thus ventilator-derived dynamic Crs seems less reliable in postoperative pulmonary evaluation than static Crs. [ABSTRACT FROM AUTHOR]