Rationale: Cystic Fibrosis (CF) newborn screening (NBS) algorithms in the USA vary by state. Differences in CF NBS algorithms could potentially affect the detection rate of CF newborns and lead to disparities in CF diagnosis amongst different racial and ethnic groups. Objectives: Generate a database of CF NBS algorithms in the USA and identify processes that may potentially lead to missed diagnoses or lead to health care disparities. Methods: We sent an online survey to state and regional CF and NBS leaders about the type and threshold of immunoreactive trypsinogen (IRT) cutoff used and methods used for CFTR gene variant analysis. Follow-up by email and phone was done to ensure a response from every state, clarify responses, and resolve discordances . Results: There was wide variation in the NBS algorithms employed by different states. Approximately half the states use a floating IRT cutoff and half use a fixed IRT cutoff. CFTR variant analysis also varied widely, with 2 states analyzing only for the F508del variant and 4 states incorporating CFTR gene sequencing. The other states used CFTR variant panels ranging from 23 to 365 CFTR variants. Conclusions: CF NBS algorithms vary widely amongst the different states in the USA, which affects the ability of CF NBS to diagnose newborn infants with CF consistently and uniformly across the country and potentially may miss more infants with CF from minority populations. Our results identify an important area for quality improvement in CF NBS.

Although COP is idiopathic by definition, it is important to investigate each diagnosed case for potential causes, such as iatrogenic from radiation or known causative medications [1], connective tissue diseases, inflammatory bowel disease, malignancies, history of lung transplant or bone marrow graft. OP may present weeks to months before other signs of connective tissue disorders and therefore the patient should undergo serologic testing for these diseases. [6] In the case presented, the specific etiology was unyielding and so remains cryptogenic in nature. This patient’s symptoms quickly improved following the use of steroids and tolerated tapering off completely without relapse one year out from initial diagnosis.

Background: Whether Lung ultrasound (LUS) can be used for pathogenic diagnosis is still controversial. This was conducted to test the accuracy and reliability of ultrasound in the diagnosis of pneumonia and to clarify whether ultrasound has diagnostic value for the etiology. Methods: A total of 135 neonatal pneumonia patients with an identified pathogen and 50 newborns with normal lungs in the newborn intensive care unit of 10 tertiary hospitals in China were enrolled. The study ran from November 2020 to December 2021. The infants were divided into various groups according to pathogens, the time of infection, the gestational age, the severity of the disease. The distribution of pleural line abnormalities, pulmonary edema, and pulmonary consolidation, as well as the incidence of air bronchogram and pleural effusion based on LUS, were compared between the above groups and between the pneumonia and healthy control groups. Results: There were significant differences in pulmonary consolidation. The sensitivity and specificity of the diagnosis of severe pneumonia based on the extent of pulmonary consolidation were 83.3% and 85.2%, respectively. The area under the receiver operating characteristic curve for the identification of mild or severe pneumonia based on the distribution of pulmonary consolidation was 0.776. Conclusion: Lung ultrasound has good performance in differentiating the severity of neonatal pneumonia, but cannot be used for pathogenic diagnosis.

Introduction: E-cigarette, or vaping, product use-associated lung injury (EVALI) results from inhaling the aerosol of e-cigarettes and has similar clinical features to coronavirus disease 2019 (COVID-19). EVALI case counts since the declaration of the COVID-19 pandemic is unknown. Methods: A retrospective electronic health record chart review of adolescents hospitalized at one institution with EVALI was conducted. Clinical characteristics and hospital course of patients hospitalized during the pandemic were compared to those pre-pandemic. Results: The clinical presentation of adolescents hospitalized prior-to (n=19) and during the COVID-19 pandemic (n=22) were similar with respect to constitutional, respiratory, and gastrointestinal symptoms. All patients hospitalized during the pandemic were tested for COVID-19 at least once. Only one patient had a positive SARS-CoV-2 RT-PCR test result. 31 out of 39 patients treated with corticosteroids had clinical improvement within 24 hours (79%). Patients hospitalized during the pandemic had a shorter median length of stay (5 vs 7 days, p<0.01), and were less often discharged with home oxygen (1 vs 6 patients, p=0.04). Pulmonary function tests improved pre-to post-corticosteroid treatment and post-corticosteroid to follow-up. Conclusions: Eliciting a history of vaping in adolescents presenting with constitutional, respiratory, and gastrointestinal symptoms is important to identify EVALI cases, which have continued throughout the COVID-19 pandemic. A shorter length of stay with less need for mechanical ventilation and home oxygen in adolescents hospitalized during the pandemic may reflect increased familiarity with EVALI characteristics. Corticosteroids led to clinical and pulmonary function improvement.

Introduction: Children with a history of bronchopulmonary dysplasia (BPD) may have lower physical activity levels, but evidence to date is based on self-report. This study compared physical activity levels between children born extremely preterm with and without history of BPD, and examined their associations with pulmonary magnetic resonance imaging (MRI) and pulmonary function test (PFT) indices. Methods: This multi-centre cross-sectional study included children aged 7-9 years born extremely preterm, with and without BPD. Children wore a pedometer for one week, then completed the Physical Activity Questionnaire (PAQ), pulmonary MRI, and PFT. Spearman correlations and multivariable linear regression modelling were performed. Results: Of 45 children, 28 had a history of moderate-severe BPD. There were no differences in any physical activity outcomes by BPD status. Higher average daily step count and higher average daily moderate-vigorous physical activity (MVPA) were each correlated with greater forced vital capacity (r=0.41 and 0.58), greater MRI lung proton density at full expiration (r=0.42 and 0.49), and lower lung clearance index (r=-0.50 and -0.41). After adjusting for MRI total proton density and BPD status, a 5% increase in forced expiratory volume at one second was associated with 738 (95%CI: 208, 1268) more steps per day and 0.1 (0.0, 0.2) more hours of MVPA, respectively. Conclusion: School-aged children born extremely preterm have similar physical activity levels to their peers, regardless of history of BPD. MRI and PFT measures suggestive of gas trapping and/or airflow obstruction are associated with lower physical activity levels.

Background: Infants with Bronchopulmonary Dysplasia (BPD) are often prescribed diuretics before the neonatal intensive care unit (NICU) discharge. It is unknown whether outpatient medication weaning strategies affect duration of home oxygen therapy. Methods: This was a secondary cohort analysis of infants born <32 weeks gestational age with BPD from 2015-2018 discharged from our NICU or regional NICUs, referred to our pulmonary clinic for home oxygen management. We compared three groups: those discharged with no diuretics, diuretics actively weaned (dose decreased) and diuretics passively weaned (dose not adjusted). Results: Out of 125 infants, 116 were included in the analysis. Forty-five infants were discharged without diuretics; 52 infants were discharged with diuretics that were actively weaned; 19 infants were discharged with diuretics that were passively weaned. Infants who were passively weaned spent the most time on home oxygen (median 28 weeks, IQR 16-52; p=0.011); there were no differences in home oxygen duration in infants actively weaned (median 13 weeks, IQR 10-26) versus not on diuretics (median 22 weeks, IQR 12-30, p=0.285). Multivariable adjustment for other illness characteristics associated with duration of home oxygen did not change this finding. Conclusions: Active weaning of diuretics did not prolong duration of home oxygen, in the setting of a standardized clinical guideline for weaning home oxygen in infants with BPD. These data can serve as baseline information to implement and test standardized strategies for outpatient medication management.

Objectives: Universal implementation of cystic fibrosis (CF) newborn screening (NBS) has led to the diagnostic dilemma of infants with CF screen positive, inconclusive diagnosis (CFSPID), for which there is limited guidance regarding prognosis and standardized care. Rates of reclassification from CFSPID to CF vary and risk factors for reclassification are unknown. We investigated whether clinical characteristics are associated with risk of reclassification from CFSPID to a CF diagnosis. Methods: Children with a positive CF NBS were recruited from two sites in California. Retrospective, longitudinal, and cross-sectional data were collected. A subset of subjects had nasal epithelial cells collected for CFTR functional assessment. Multivariate logistic regression was used to assess the risk of CFSPID-to-CF reclassification. Results: A total of 112 children completed the study (CF=53, CFSPID=59). Phenotypic characteristics between groups showed differences in pancreatic insufficiency prevalence, immunoreactive trypsinogen (IRT) levels, and Pseudomonas aeruginosa (PSA) colonization. Spirometry measures were not different between groups. Nasal epithelial cells from 10 subjects showed 7-30% of wild type (WT)-CFTR function in those who reclassified and 27-67% of WT-CFTR function in those who retained the CFSPID designation. Modeling revealed that increasing sweat chloride concentration (sw[Cl -]) and PSA colonization were independent risk factors for reclassification to CF. Conclusion: Increasing sw[Cl -] and history of PSA colonization are associated with risk of reclassification from CFSPID to CF in a population with high IRT and two CFTR variants. Close follow-up to monitor phenotypic changes remains critical in this population. The role of CFTR functional assays in this population requires further exploration.

Background. The COVID-19 pandemic has spread across the world, leading to government measures associated with a negative impact on mental health. The aim of this study was to evaluate the impact of COVID-19 on depression, anxiety and resilience in Dutch people with cystic fibrosis (PwCF) or primary ciliary dyskinesia (PwPCD) and their caregivers during the pandemic. Methods. Adolescents (12-17 years), adults and caregivers of children (0-17 years) with CF or PCD completed questionnaires on depression (PHQ-9), anxiety (GAD-7) and resilience (BRS) between September 2020 and February 2021. The psychosocial impact of COVID-19 was measured by the Exposure and Family Impact Survey (CEFIS) Part 2. Mixed model analyses compared PHQ-9 and GAD-7 results to participants’ pre-pandemic scores. Results. 110 participants (10 PwCF, 31 PwPCD, 52 CF caregivers, 17 PCD caregivers) completed questionnaires during the pandemic. Pre-pandemic outcomes were available for 87 participants. The prevalence of symptoms of depression and anxiety (PHQ-9 or GAD-7 scores ≥5) in PwCF and PwPCD and their caregivers before and during the pandemic was high, with an increase in depression in PwCF (2.75; 95%-CI: 0.82 to 4.68) and increase in anxiety in CF caregivers (1.03; 0.09 to 1.96) during the pandemic. Resilience was within the normal range for all groups, CEFIS scores corresponded to a low to normal impact. Conclusion. PwCF and PwPCD and their caregivers were at high risk of increased depression and anxiety symptoms both before and during the pandemic, which emphasizes the importance of mental health screening and psychological care in CF and PCD.

Background and aims: We aimed to analyze the correlation of urinary with serum NT-proBNP concentrations in acute bronchiolitis and its association with the severity of the disease.  Material and Methods: A pilot observational study conducted between 1st October and 31st March 2022, including acute bronchiolitis cases who attended our institution. Serum and urinary NT-proBNP concentrations were determined using the Alere NT-proBNP assay in time-matched urine and blood samples. We explored the linear relationship between both concentrations and compared clinical outcomes indicative of severe acute bronchiolitis between groups of raised and normal urinary NT-proBNP.  Results: 17 infants (median age 68 (36-91) days) with 36 time-matched samples were included. The urinary and serum concentrations of NT-proBNP were significantly correlated with (r=0.867 & R-squared coefficient=0.751; p<0.001). The log-10-transformed urinary NT-proBNP concentrations were higher at the time of hospital admission in those infants that required PICU admission with ventilatory support compared with those without this outcome (1.85 (1.16-2.44) pg/mg vs 0.63 (0.45-0.84) pg/mg); p<0.001); and resulted positively and strongly correlated with the duration of the ventilatory support (rho=0.76; p<0.001) and the LOS hospitalization (rho=0.84; p<0.001) Conclusion: The measurement of urinary NT-proBNP concentrations could be a reliable surrogate for serum NT-proBNP levels highlighting the potential value of the urinary NT-proBNP as a non-invasive tool to assess severity in acute bronchiolitis.

In conclusion, our data suggest that subjects with genotype 5T;TG12/VVCC likely have a very low risk of progressing to CF, as compared to those with F508del/5T;TG12. 4 This observation could lead to differentiate follow up in presence of at least one 5T;TG12. Knowing these data is crucial to offer a useful counseling for CRMS/CFSPID infants and for non‐CF adults with CBAVD alone. Anyway further data are needed to evaluate the outcomes after a longer follow up.

Introduction: Assessment of and intervention for sleep-disordered breathing and malnutrition are related to respiratory management for preventing recurrent respiratory tract infections (RRTIs) and acute respiratory failure (ARF) in children with spinal muscular atrophy (SMA). However, the specific standard has not been clarified. Purpose: The study aimed to obtain the risk factors and the predictive index for RRTIs and/or ARF in children with SMA. Methods: In this retrospective study, the differences in clinical characteristics in patients with or without RRTIs and ARF were compared, and binary logistic regression analysis was carried out. The best cutoff points of the positive predictive index were obtained. Results: Type 1 (OR = 4.12, 95% CI 1.30-13.07, P =.016) and apnea hypopnea index (AHI) (OR = 1.14, 95% CI 1.05-1.24, P =.001) were risk factors, while body mass index z score (BMIz) (OR = 0.68, 95% CI 0.49-0.94, P =.018) and mean pulse oxygen saturation (MSpO 2) (OR = 0.67, 95% CI 0.50-0.91, P =.010) were protective factors. The sensitivity and specificity of the standard of MSpO 2 < 96% and AHI > 10 events/h or BMIz < -1 with the occurrence of RRTIs and/or ARF were 0.513 and 0.957, respectively. Conclusion: SMA Type 1, BMIz, AHI and MSpO 2 should be used to estimate the risk for RRTI or ARF in children with SMA. MSpO 2 < 96%, and AHI > 10 events/h or BMIz < -1 should be used as the intervention standard.

Objective: To evaluate the functional capacity, pulmonary function and quality of life of children and adolescents with sickle cell anemia (SCA) and to test the reproducibility of functional capacity tests in this population. Method: Cross-sectional study with  volunteers with SCA genotype Hb-SS (SCAG), aged 6 to 18 years matched in age and  gender to the control group (CG). Spirometry, 5-repetition sit-to-stand test (5STS-test),  modified shuttle walk test (MSWT), and Pediatric Quality of Life Questionnaire (PedsQL) were performed. The reproducibility of 5STS-test and MSWT was evaluated:   Results: 48 volunteers of SCAG and 48 of CG were evaluated. Pulmonary function of SCAG (FVC: 92 ± 15% pred.; FEV 1 /FVC: 84 ± 8% pred.) was worse than the CG (104 ± 15% pred.; FEV 1 /FVC: 90 ± 6% pred.) p < 0.05. SCAG had worse functional capacity registered by distance walked: 576m (515-672m) and 5STS-test: 8 seconds (7.4-8.9seconds) compared to the CG who showed distance walked: 1010m (887- 1219m) and 5STS-test: 7 seconds (7.0-8.1seconds), p < 0.001. SCAG had worse quality compared to CG, p < 0.05. The reproducibility was good of MSWT (ICC 0.99 (0.98-0.99 IC-95%)) and 5STS-test (ICC 0.80 (0.69 – 0.88), p < 0.001 . Conclusion: Children and adolescents with sickle cell anemia showed worse capacity to walk or run, and to perform sit-to-stand test. Additionally, they have poor quality of life when compared with their control peers. The MSWT and 5STS-test showed reproducible to be applied in pediatric individual with SCA.

Rationale: As a result of the SARS-CoV-2 pandemic, all pediatric pulmonary fellowship programs conducted virtual interviews for the first time in the Fall of 2020. This study aimed to understand the accuracy of virtual-interview derived-impressions of fellowship programs, as well as applicant preference for future fellowship interview cycles. Methods: A group of pediatric pulmonary fellows and Program Directors designed a REDCap survey. The survey was distributed to all first-year pediatric pulmonary fellows who participated in the 2020-2021 virtual interview season. Results: 23/52 (44%) of first-year pediatric pulmonary fellows completed the survey. 96% were able to form general impressions about fellowship programs during their virtual interviews. 96% reported that generally their fellowship experience matched their virtual-interview derived-impressions. 17 of 19 factors applicants use to rank programs had no statistically significant change (p > 0.05) in impression from virtual interview to fellowship experience. The two factors with a statistically significant (p < 0.05) change in impression were patient care related – volume of ‘bread and butter’ pediatric pulmonary patients and volume of tertiary care pediatric pulmonary patients. 87% prefer some form of in-person interview option in future application cycles. A tiered interview format in which applicants are first invited to a virtual interview day followed by an optional in-person second look day was the most popular preference for future interview cycles (48%). Conclusions: Virtual interviews may provide accurate representations of pediatric pulmonary fellowship programs and applicants prefer some type of in-person interview option in future application cycles.

Introduction: Acute bronchiolitis (AB) is the main cause of hospitalization in children under two years of age, with a regular seasonality, mostly due to the respiratory syncytial virus (RSV).Objectives: To describe the epidemiology of bronchiolitis hospitalizations in our centre in the last twelve years, and analyse the changes in clinical characteristics, microbiology, and adverse outcomes during the SARS-CoV2 pandemic.Methods: Observational study including patients admitted for bronchiolitis between April 2010 and December 2021 in a Spanish tertiary paediatric hospital. Relevant demographic, clinical, microbiological, and adverse outcome variables were collected in an anonymized database. The pandemic period (April 2020 to December 2021) was compared to 2010-2015 seasons using appropriate statistical tests.Results: There were 2138 bronchiolitis admissions, with a mean of 195.6 per year between 2010-2019 and a 2–4-month peak between November and March. In the expected season of 2020, there was a 94.4% reduction of bronchiolitis hospitalizations, with only eleven cases admitted in the first year of the pandemic. Bronchiolitis cases increased from the summer of 2021 during a six-month long peak, reaching a total of 171 cases. Length of stay was significantly shorter during the pandemic, but no differences were found in clinical and microbiological characteristics or other adverse outcomes.Conclusions: The SARS-CoV2 pandemic has modified the seasonality of bronchiolitis hospitalizations, with a dramatic decrease in cases during the expected season of 2020-2021, and an extemporaneous summer-autumn peak in 2021 with longer duration but similar patient characteristics and risk factors.

This case illustrates another promising example of the recent advances within pediatric interventional bronchoscopy. As innovative medical therapies continue to make their way into the pediatric realm (e.g. a 1.1-mm flexible cryoprobe has been recently developed by Erbe), opportunities for novel approaches and techniques will continue to present themselves.

Objective: This systematic review aimed to systematize different designs of exercise-based pulmonary rehabilitation (PR) for children with asthma and explore which designs are optimal. Methods: PubMed, EMBASE, Cochrane Library, Web of Science Core Collection and MEDLINE were searched up until April 01, 2021, which was conducted for any relevant randomized controlled trials (RCTs) of exercise-based PR in childhood asthma. Language is limited to English. Network meta-analyses and standard meta-analyses were performed using STATA (version 16.0), quality analyses were performed using RevMan (version 5.3). Results: A total of 24 RCTs involving 1031 patients were included. 14 studies were endurance training, which was the most commonly used form of exercise, and 7 studies rehabilitation sites were conducted in hospitals. A network meta-analysis showed that compared with other forms of exercise, interval training significantly improved the PAQLQ (Pediatric Asthma Quality of Life Questionnaire), including activity scores [MD=3.02, 95% CI (1.74,4.30)], symptom scores [MD=2.68, 95% CI (2.04,3.32)], emotional scores [MD=2.47, 95% CI (0.91,4.03)], and total scores [MD=2.68, 95% CI (1.79,3.57)]. Interval training [MD=188.97, 95% CI (-59.27, 437.21)] also had a more significant effect on the 6MWT (6-minute walk test). No adverse events were found in this study. Exercise training had no significant effect on FEV 1(the forced expiratory volume at 1s to predicted value ratio) [WMD=0.59, 95% CI (-2.00, 3.19)], however, the combined of endurance training and respiratory training was found to significantly improve both FVC (the forced vital capacity to predicted value ratio) [MD=5.37, 95% CI (0.07,10.67)] and FEF25-75% (the forced expiratory flow between 25% and 75% of vital capacity ratio) [WMD=11.31, 95% CI (2.13, 20.48)]. Conclusions: Exercise-based PR is a safe and effective for childhood asthma. Interval training may be a core component of improving quality of life and exercise capacity in childhood asthma, the combination of respiratory training and endurance training has significant effects on lung function.This result should be viewed with caution, and high-quality RCTs are still needed to confirm its clinical efficacy,

Background: Observational studies suggest that asthma/wheezing improve after adenotonsillectomy (AT). However, there is a paucity of RCT specifically studying the effects of AT in asthma/wheezing. Therefore, we conducted a post-hoc analysis of the Childhood Adenotonsillectomy Trial, the largest RCT of AT in children with obstructive sleep apnea (OSA) to test the hypothesis that AT would result in fewer wheezing episodes. Methods: In the CHAT study, 464 children with OSA, aged 5 to 9 years, were randomized to early AT (n=226) or watchful waiting with supportive care (WWSC) (n= 227). For this post-hoc analysis children were categorized as having “any wheezing” vs. “no wheezing” at baseline and at 7 months of follow-up. A multivariate analysis was conducted to evaluate the association between “any wheezing” at follow-up and treatment group after controlling for several potential confounders. Results: Children in the “any wheezing” group were predominantly black, had more allergic rhinitis, eczema, second-hand smoke exposure, more siblings and siblings with asthma, lower maternal education and family income than those in the “no wheezing group”. At baseline, wheezing characteristics were similar between AT and WWSC arms. At follow-up (at 7 months of the intervention) those in the AT arm had significatively less wheezing than those in the WWSC (22.4% vs. 43.8%, p=0.00001). However, the multivariate analysis of “any wheezing” vs. “no wheezing” at follow-up showed that the treatment arm was not associated with wheezing. Conclusion: This study demonstrated that AT has not effect on wheezing at 7 months of follow-up.

This study aimed to investigate epidemiological, clinical, and laboratory features of children with COVID-19 to identify predictors for pulmonary involvement. We conducted a retrospective, single-center study of pediatric COVID-19 at a tertiary care hospital in Turkey between December 2020 and June 2021. A total of 126 children (70 males, 55.6%) were examined during the study period. Their mean age was 74.73 ± 81.11 months (range, 1–216 months). The most frequent COVID-19 symptoms were fever (65.9%), cough (52.4%), and shortness of breath (18.3%). Ten patients required noninvasive mechanical ventilation. Sixty-nine patients (54.8%) had pneumonia. Longer duration of fever and the presence of cough were significantly associated with pulmonary involvement. In children with pneumonia, the C-reactive protein (CRP), procalcitonin levels, erythrocyte sedimentation rate (ESR), and viral load were significantly higher and lymphocyte and thrombocyte counts were significantly lower than in children without pneumonia. The cutoff viral load, CRP, and procalcitonin values for predicting pulmonary involvement were 26.5 cycle threshold (Ct; 95% confidence interval [CI], 0.54–0.74; sensitivity, 0.65; specificity, 0.56; area under curve [AUC]: 0.647, p = 0.005), 7.85 mg/L (95% CI, 0.56–0.75; sensitivity, 0.66; specificity, 0.64; AUC = 0.656; p = 0.003) and 0.105 ng/mL (95% CI, 0.52–0.72; sensitivity, 0.55; specificity, 0.58; AUC = 0.626; p = 0.02), respectively. High CRP, procalcitonin levels, ESR, and viral load and low lymphocyte and thrombocyte counts can predict pulmonary involvement in children with COVID-19, so better management may be provided for good prognosis.