Our case was a pediatric patient with very extensive lung involvement who also had involvement in other organs. We believe our case demonstrates clinical features that are complementary for the case series by Mfingwana L and colleagues and thus, will be beneficial for the readers.

Background: Inhaler technique (IT) knowledge among healthcare providers is poor. The aim was to improve PED healthcare providers’ IT technique by carrying out an education intervention, and sustain it for 6 months. Methods: open-label, quasi-experimental, prospective and unicentric study. Healthcare professionals working at the Pediatric Emergency Department (PED) were enrolled. The study was developed in three phases: baseline evaluation and education intervention (P1) and reevaluation 1 month (P2) and 6 months (P3) after the education intervention. Participants fulfilled an eight-question theoretical test. Practical skills were evaluated by demonstrating IT in all three phases. The education intervention consisted in a verbal explanation of IT followed by a demonstration of IT with metered-dose inhaler using a mannequin. Results: 84 healthcare providers (medical residents, nurses and nursing assistants) were involved. In the theoretical questionnaire, the mean score at baseline was 4.4/8 (SD 1.7) improving to 6.3/8 (SD 1.2) in P2 and 6.47/8 (SD 1.1) in P3. In the IT evaluation for children <7 years old, the score improved from 5.7/7 (SD1.3) to 6.5/7 in P2 and 6.7/7 in P3 (p<0.001). For children >7 years old, the mean score of IT at baseline was 3.1/10 (SD 4), which improved to 7.4/10 (SD3) and 8.2/10 in P2 and P3 respectively (p<0.001). Only laboral category influenced results at baseline. Conclusion: Healthcare providers’ theoretical knowledge and practical skills on IT are low. The education intervention performed is a useful strategy to ameliorate IT among healthcare providers.

Pleural drainage was differently performed in two similar neighboring hospitals (32.0 % vs. 58.2 %, p < 0.001), but the length of stay was shorter in the hospital using a more conservative approach (median 12 days vs. 18 days, p < 0.001). This result seemed unrelated to severity but associated with the shorter duration of intravenous treatment. This study adds to previous reports suggesting that pleural drainage is unnecessary in many cases; controlled studies are needed to determine which patients may actually benefit from its use.

Background and Objective: With improved survival in neonates with meconium aspiration syndrome (MAS), the focus is currently on mitigating the morbidities. The objective of this study was to predict factors determining prolonged hospital stay in neonates with MAS. Materials and methods: It was a retrospective cohort from five centres of south India between 2018 and 2020. Neonates ≥35 weeks of gestation admitted to NICU with the diagnosis of MAS and requiring oxygen beyond 24 hours of life were included in the study. The morbidities in the neonates with stay ≤7 days (short stay) were compared with >7 days (prolonged stay). Logistic regression by the backward stepwise method was used for predictive score creation. Results: Out of 347 neonates with MAS discharged home, 103 (29%) had a short stay and 244 (71%) had prolonged stay. The primary support beyond O2 (CPAP/MV) (42% vs 83%, p<0.001), FiO2 at 1hr>30% (45% vs 87%, p<0.001), HIE stage 2 or 3 (2% vs 27%, p<0.001), moderate-severe PPHN (3% vs 31%, p<0.001) were independent factors associated with prolonged stay on logistic regression. A prediction model was devised using weighted scores of these four associated morbidities. The clinical score thus developed had 83% sensitivity, 68% specificity for the prediction of prolonged stay [AUC- 82, 95% CI (78-87), p<0.001]. Conclusion: More than two-thirds of neonates with MAS had prolonged stay. The primary support beyond oxygen, Fio2 requirement >30%, Moderate to severe PPHN, HIE stage 2 or 3 were predictive of prolonged stay in neonates with MAS.

Context: In adults, permissive hypercapnia reduces mortality and ventilation duration. However, in preterm infants, findings from past research regarding the efficacy and safety of permissive hypercapnia are controversial. Objective: To evaluate the efficacy and safety of permissive hypercapnia versus normocapnia in preterm infants on mechanical ventilation. Data Sources: MEDLINE, EMBASE, CENTRAL, and CINAHL Study Selection: Published randomized controlled trials (RCTs), non-RCTs, interrupted time series, cohort studies, case-control studies, and controlled before-and-after studies were included. Data Extraction: Two reviewers independently screened the title and abstract and full text, extracted data, assessed the risk of bias, and evaluated certainty of evidence (CoE) according to the Grading of Recommendations Assessment, Development and Evaluation approach. A meta-analysis of RCTs was performed using the random-effects model. Results: Four RCTs (693 infants) and one cohort study (371 infants) were included. No significant differences existed between the permissive hypercapnia and normocapnia groups for bronchopulmonary dysplasia (BPD) (risk ratio [RR] 0.94; 95% confidence interval [CI] 0.74-1.18; very low CoE) and a composite outcome of death or BPD (RR 1.05; 95% CI 0.90-1.23; very low CoE). Permissive hypercapnia may increase necrotizing enterocolitis (RR 1.69; 95% CI 0.98-2.91; very low CoE), although the null or trivial effect cannot be excluded. No significant differences existed between the two groups for any other outcome assessed (very low-to-low CoE). Limitations: The sample sizes were less than the optimal sizes for all outcomes assessed, indicating the need for further trials. Conclusions: Permissive hypercapnia did not have any significant benefit or harm in preterm infants.

Background: Newborn screening (NBS) for cystic fibrosis (CF) has been underway universally in the USA for more than a decade, as well in most European countries, and algorithms have been evolving throughout this period with quality improvement projects as immunoreactive trypsinogen determinations alone have been transformed to a 2-tier strategy with DNA analyses. Objective: To apply next generation sequencing (NGS) as a method for expanding the DNA tier for identifying variants in the cystic fibrosis transmembrane conductance regulator ( CFTR) gene with minimization of unintended outcomes. Design: Sequential quality improvement project in three phases using plan coupled to statewide follow up and analysis of screening outcomes in comparison to other NBS programs that use CFTR sequencing. Results: After demonstrating feasibility in the first phase, we studied an IRT/NGS algorithm that included CFTR Variants with Varying Clinical Consequences (VVCCs). This revealed a high identification of CF patients with 2-variants detected through screening, but for every CF case there were 1.4 with cystic fibrosis metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID). This led us to a third phase of quality improvement in which the VVCCs were eliminated except for R117H, resulting in 94% 2-variant detection of patients and 0.44:1 ratio of CRMS/CFSPID to CF. Conclusion: NGS can be used with IRT as an effective method of identifying infants at risk for CF without an appreciable increase in detection of either carriers or CRMS/CFSPID cases.

Objective(s): To determine the impact of Clostridioides difficile Infection (CDI) among pediatric Cystic Fibrosis (CF) hospitalizations using a large nationally representative pediatric hospital database . Study design: We identified Cystic Fibrosis-related hospitalizations during the years 1997 to 2016 in the Kids’ Inpatient Database [KID] and compared in-hospital mortality, Length of Stay [LOS], and hospital charges among hospitalizations with and without a coexisting diagnosis of C. difficile using logistic regression models for mortality and general linear models with gamma distribution and logarithmic transformation for LOS and hospital charges. We also evaluated temporal trends in the proportion of CF hospitalizations with concomitant CDI using data published triennially Results: We analyzed 21, 616 pediatric CF hospitalizations between the years 1997 to 2016 and found a total of 240 (1.1%) hospitalizations with concurrent CDI diagnosis. Adjusted analyses demonstrated an association of CDI with increased mortality (OR 5.2, 95% 95% CI 2.5-10.7), longer LOS (46.5% increment, 95% CI 36.0-57.1), and higher charges (65.8% increment, 95% CI 53.5-78.1) for all comparisons. The proportion of CF hospitalizations with CDI increased over time from 0.64% in 1997 to 1.73% in 2016 (p<0.001). Conclusion(s): As CDI is associated with excess mortality, LOS, and cost in children hospitalized for CF, efforts to reduce infection rates and aggressive diagnosis and treatment of active infections should be prioritized to improve hospital outcomes among children with CF.

Introduction Children with Down Syndrome (DS) are at high risk of sleep disordered breathing (SDB). We aimed to examine the burden of SDB in infants with DS referred to tertiary sleep center. Methods Infants (≤12 months old) with DS who underwent consecutive polysomnography (PSG) at a single academic sleep center over a 6-year period were included. OSA (obstructive apnea hypopnea index [oAHI]>1/hr), central sleep apnea (central apnea index>5/hr) and the presence of hypoventilation (% time spent with CO2 > 50 mmHg either by end-tidal or transcutaneous> 25% of total sleep time) and hypoxemia (time spent with O2 saturation <88% >5 min) were ascertained. For infants who underwent adenotonsillectomy (AT), we compared the SDB metrics before and after the AT. Results A total of 40 infants were included (Mean age 6.6 months, male 66%). PSGs consisted of diagnostic (n=13) and split night (n=27, 68%) studies. All met criteria for OSA with mean oAHI 34.6 (32.3). Central sleep apnea was present in 11 (27.5%) of infants. A total of 11 (27.5%) had hypoxemia. Hypoventilation was present in 10 (25%) infants. There was a trend of association between hypothyroidism and hypoventilation (OR: 5.5 [0.96-34.4], p=0.056). Among 13 infants who underwent AT and had a follow up PSG, severity of OSA markedly reduced after AT (oAHI difference: 34/hr [29], p=0.0002). Conclusion This study highlights the high prevalence of SDB in infants with DS and supports early PSG assessment in this patient population.

Vaping associated lung injury (EVALI) has increased in prevalence after first being noted in an outbreak among teenagers in 2019. Vaping involves the use of a heating device to aerosolize a product, typically nicotine or more recently, cannabinoids. Products that contain cannabinoids, such as CBD oil, are being used more as these products are easy to obtain and are typically less expensive. The clinical manifestations of EVALI are widespread and include respiratory symptoms as well as cardiogenic, gastrointestinal, and constitutive symptoms. EVALI rarely presents with hemoptysis as one of the main presenting symptoms, especially in an adolescent. This case will discuss EVALI with associated hemoptysis in a teenager secondary to vaping cannabinoid oil.

Rationale: Animal models suggest pre-eclampsia (Pre-E) affects alveolar development, but data from humans are lacking. Objective: Assess the impact of Pre-E on airway function, diffusion capacity, and respiratory morbidity in preterm and term infants born from mothers with Pre-E. Methods: Infants born from mothers with and without Pre-E were recruited for this study; term and preterm infants were included in both cohorts. Respiratory morbidity in the first 12 months of life was assessed through monthly phone surveys. Raised volume rapid thoracoabdominal compression and measurement of diffusion capacity of the lung to carbon monoxide (DLCO)) were performed at 6 months corrected age. Results: There were 146 infants in the Pre-E cohort and 143 in the control cohort. The Pre-E cohort was further divided into non-severe (N=41) and severe (N=105) groups. There was no significant difference in DCLO and DLCO/aveolar volume amongst the three groups. Forced vital capacity was similar amongst the three groups, but the non-severe Pre-E group had significantly higher forced expiratory flows that the other two. After adjusting for multiple covariates including prematurity, the severe Pre-E group had a lower risk for wheezing in the first year of life compared to the other two. Conclusions: Pre-E is not associated with reduced DLCO, lower forced expiratory flows, or increased wheezing in the first year of life. These results differ from animal models and highlight the the complex relationships between Pre-E and lung function and respiratory morbidity in human infants.

Pneumothorax as a sequalae of vaping is a relatively recent complication being described in the literature. Smoking has classically been associated with increased risk of pneumothorax, and emerging evidence is showing that electronic cigarettes (e-cigarettes) likely carry some of the same risks. Since electronic cigarettes increased in popularity, especially among the adolescent population, there has been reported increased incidence of lung injury, including pneumothorax. We present a case of a 15-year-old female with a history of e-cigarette use admitted for recurrent pneumothorax with failure to re-expand requiring surgical intervention.

Asthma 17q21 Polymorphism Associates with COVID-19 in Children

Introduction: Lower socioeconomic status is associated with significantly poorer outcomes in weight, lung function, and pulmonary exacerbation rates in People with CF (PwCF). Global aim: We aim to reduce health disparities and inequities faced by PwCF by screening for and addressing unmet social needs. Specific aims: We aimed to increase routine Social Determinants of Health (SDoH) screening of eligible PwCF from 0% to 95% and follow-up within two weeks for those PwCF who screened positive and requested assistance from 0% to 95% by December 31, 2021. Methods: The Model for Improvement methodology was employed. A process map and a simplified failure mode effects analysis chart were created for the screening and SDoH follow-up process. Those who screened positive for SDoH and requested assistance, a follow-up contact was made to offer intervention. Intervention: Adult PwCF who had at least one UVA Clinic encounter in 2021 were screened for SDoH. The SDoH screening tool included eight domains: housing, food, transportation, utilities, health-care access, medication access, income/employment and education. Follow-up was completed with all PwCF who screened positive for SDoH. Results: A total of 132 of 142, (93.0%) PwCF eligible for screening completed the SDoH screening. Of the PwCF who completed screening, 56 (42.4%) screened positive for SDoH. A follow-up rate of 100% was achieved in June 2021 and maintained through December 2021. Conclusion: Implementing screening for SDOH and follow-up to mitigate social difficulties in adult PwCF at UVA was successful and could be reproduced at other CF care center.

An eight-year-old girl with cystic fibrosis (CF) developed a left upper lobe collapse failing to resolve with initial conventional antibiotic treatment, mucolytics and intensified physiotherapy. Mycobacterium abscessus was isolated from her sputum. Bronchoscopy revealed thick viscous mucus plugging of the left upper lobe bronchus with complete obliteration. Three bronchoscopies with saline lavage and Dornase alfa, a rhDNase, at the end of each procedure resulted in removal of this mucus plug and the re-inflation of the affected lobe, with clinical and radiological resolution. The use of flexible bronchoscopy as a ‘secondary’ treatment with 0.9% saline lavage and instillation of rhDNase is described sparsely in the literature. This is the first reported successful therapeutic resolution of a lung collapse in a CF patient with Mycobacterium abscessus, with sequential therapeutic bronchoscopies with instillation of Dornase alfa. This should be considered for lobar collapse in CF not responding to the standard therapeutic regime.

Rationale The lung clearance index (LCI) is increasingly being used in the clinical surveillance of patients with cystic fibrosis (CF). However, there are limited data on long-term variability and clinically relevant changes in LCI during routine clinical surveillance. Objectives To evaluate long-term variability of LCI and propose a threshold for a clinically relevant change. Methods Children with CF aged 4-18 years performed LCI measurements every three months as part of routine clinical surveillance during 2011-2020 in two centers. The variability of LCI during periods of clinical stability was assessed using mixed-effects models and was used to identify thresholds for clinically relevant changes. Results Repeated LCI measurements of acceptable quality (N= 858) were available in 100 patients with CF. Variability of repeated LCI measurements over time expressed as coefficient of variation (CV%) was 7.4%. The upper limit of normal (ULN) for relative changes in LCI between visits was 19%. Conclusion We report the variability of LCI in children and adolescents with CF during routine clinical surveillance. According to our data, a change in LCI beyond 19% may be considered clinically relevant. These findings will help guide clinical decisions according to LCI changes.

We read with great interest recent research by Anderson et al.1 on how NICU respiratory support affects infant feeding, and by Behnke et al.2 on the potential interference of infant feeding with successful respiratory stabilization of the preterm infant. Since both preclinical and human studies have established that nutrition plays a key role in preterm lung growth and development, a crucial aspect of any research undertaken in the field of neonatal respiratory outcomes should be the breastfeeding history. It is now becoming well established that human milk feeding actively contributes in limiting lung injury among vulnerable neonates.

People with cystic fibrosis (CF) have an amazing outlook with the treatment availability of highly effective modulators. Unfortunately, not all PwCF are eligible for modulators leading to continued pulmonary exacerbations and advanced lung disease. Additionally, optimizing diagnosis and evaluation for CF in the newborn period continues to be an area of focus for research. This review article will work to cover articles published in 2021 with high clinical relevance related to the above topics, however due to the extensive body of research published, this review will not be comprehensive.

A Pneumothorax in the Setting of COVID-19 Associated Pneumatoceles in a Pediatric PatientEsther Blondeau-Lecomte, BA1, Nancy Joseph, MBBS11Department of Pediatrics, University of Florida, Gainesville, FL, USA