Background. Median survival age in cystic fibrosis (CF) has increased in developed countries. Scarce literature exists about survival in Latin American, especially in Mexico. The aim of our study was to assess the median age of CF patients’ survival in Mexico over a 20-year period. Methods. We conducted a retrospective study, with all patients registered and followed in the CF Center in Monterrey, Mexico from 2000 to 2020. Median survival age was the primary outcome, assessed with the Kaplan-Meier analysis. Influence of clinical, biological, and demographic factors on survival were analyzed with the Cox regression model. Results. Two-hundred five patients were included. Median survival for the cohort was 21.37 years (95% CI 17.20 – 25.55). In the multivariate Cox regression model, low socioeconomic status (hazard ratio [HR] 4.21, 95% CI 2.43 – 7.27), chronic Pseudomonas aeruginosa (P. aeruginosa) infection at 6 years (HR 10.45, 95% CI 5.66 – 19.28), and pancreatic insufficiency (HR 3.13, 1.38 – 7.13) were independent risk factors for mortality. Conclusion. Median survival in Mexican patients with CF is lower than in high-income countries, and socioeconomic status plays a conspicuous role in the disparity. To increase patient survival for those residing in low-income countries, public health authorities must design policies that fully cover diagnosis and treatment strategies for the CF population.

Introduction: A number of measures were began due to coronavirus 2019 disease (Covid-19) pandemic in many countries worldwide. A lockdown was applied for aged <18 years, education was continued online, and wearing a mask became mandatory in public places, which created an unprecedented period for children. Real-life data is limited showing how children with asthma are affected due to major changes. This study reveal how asthmatic children are affected by pandemic conditions based on real-life data. Methods: Patients with asthma aged 6–18 years who were followed up in March, April, and May 2019—before the Covid-19 pandemic—were included in the study. Data from March-April-May 2020 and 2019 were compared to reveal the effects of the pandemic-related lifestyle changes on symptoms, frequency of exacerbations, and drug use in asthmatic children. Results: A total of 86 children with asthma aged 6–18 years were included in this study. Time spent inside the home was significantly higher in 2020 than in 2019. Need for rescue medications and emergency department visits were significantly lower in 2020 compared to 2019 (p<0.001). The number of well controlled patients with asthma was higher in 2020 than in 2019 (p < 0.0001). Number of patients using prophylactic drugs within the last 3 months was lower in 2020 compared to 2019 (p = 0.007). Conclusion: The present study provides valuable insights into the condition of children over the age of 6 years during the Covid-19 pandemic based on real-life data. During the pandemic period, the number of asthmatic exacerbations, rescue drug use and asthma control were positively affected in school aged children with asthma.

Introduction: Although prolonged respiratory symptoms following SARS-CoV-2 infection have been reported in adults, there is a paucity of literature describing post-acute symptoms in pediatric patients following COVID-19. In this study we describe health data and respiratory findings in pediatric patients presenting with complaints of persistent respiratory symptoms following acute COVID-19 infection. Methods: This study included patients referred to Pulmonary Clinic at the Children’s Hospital of Philadelphia between December 2020 and April 2021 (n=29). Inclusion criteria included a history of SARS-CoV-2 RNA positivity or confirmed close household contact. A retrospective chart review was performed and demographic, clinical, imaging, and functional test data were collected. Results: The mean age at presentation to clinic was 13.1 years (range: 4-19 years). Patients had persistent respiratory symptoms ranging from 1.3 to 6.7 months post-acute infection. Persistent dyspnea and/or exertional dyspnea were present in nearly all (96.6%) of the patients at the time of clinic presentation. Other reported chronic symptoms included cough (51.7%) and exercise intolerance (48.3%). Fatigue was reported in 13.7% of subjects. Many subjects were overweight or obese (62.1%) and eleven subjects had a prior history of asthma. Lung function was normal in most patients. The six-minute walk test (6MWT) revealed exercise intolerance and significant tachycardia in two-thirds of children tested. Conclusion: Exertional dyspnea, cough and exercise intolerance were the most common respiratory symptoms in children with post-acute COVID-19 respiratory symptoms seen in an outpatient pulmonary clinic. Lung function, however, was mostly normal, and exertional intolerance was frequently demonstrated using the 6MWT.

Background Cystic Fibrosis (CF) and autism spectrum disorder (ASD) are life-long conditions with intense treatment burdens for patients and families. Patients with a concurrent diagnosis (CF-ASD) experience unique challenges to CF care. This study describes the experiences of our multidisciplinary CF team in caring for patients with CF-ASD and provides insight into provider and parental perspectives on clinical management. Methods This is a three-part IRB-approved study involving 1) retrospective chart review of patients with CF-ASD, 2) qualitative interviews with multi-disciplinary care teams, and 3) qualitative interviews with caregivers of patients with CF-ASD. Challenges in clinical management of this specific cohort were compiled using data from chart review and care team interviews. Strategies to address these challenges were identified and rated by individual families based on relevance and practicality. Results Within our CF center, 12 patients have an official diagnosis of ASD. Median age of patients with CF-ASD was 8.5 years (range 3-20 years), 75% were male, and 83% were on highly effective modulator therapy. Clinical challenges included sensory processing issues, environmental overstimulation, intolerance to procedures and disrupted routines. Potentially impactful strategies include patient-specific coping plans, guided behavioral interventions, parental advocacy, and improved communication between the family and multidisciplinary team. Conclusions Children with CF-ASD face extraordinary challenges beyond the experience of neurotypical children with CF. Increased awareness of this complex dual diagnosis will help providers be sensitive to the unique needs of these patients, to help build consistent and trustworthy relationships with families, and to provide effective clinical care despite limitations.

Background: Asthma is a leading cause of pediatric hospitalization in the United States. Children hospitalized with asthma are often cared for in different care settings during a single hospitalization. Our objective was to study the reliability and safety of a new pediatric hospital-wide asthma severity score (HASS) across different care units within a single tertiary-care pediatric center. Methods. 150 patients between the ages of 2 and 18 years hospitalized with a principal diagnosis of status asthmaticus were included. Study patients were followed from initial triage in the emergency department until the time of medical readiness for discharge. Rates of medical errors, early transfers to a higher level of care and medically indicated hospital length of stay (LOS) were compared between 75 patients prior to and 75 patients after implementation of the HASS using retrospective chart review. Inter-rater reliability was determined by collecting independent HASS scores from blinded staff members after tandem or simultaneous patient assessment. Results. Inter-rater reliability among untrained staff members using the HASS was high. Rates of preventable adverse events and medical errors were low and not significantly different before and after implementation of the HASS. LOS was shorter after implementation of the HASS but without statistical significance. Rates of early transfer to a higher level of care were unchanged between study years. Conclusion. The HASS is a reliable asthma severity tool that can be used throughout hospitalization and also among multiple clinical providers to trend clinical progress and optimize communication, particularly during times of care handoffs.

Noninvasive ventilation (NIV) use was initially reported in cystic fibrosis (CF) in 1991 as a bridge to lung transplantation, and over the decades the use of NIV has increased in the CF population. Individuals with CF are prone to various physiologic changes as lung function worsens, and they may benefit from NIV for advanced lung disease. As life expectancy in CF has been increasing due to advances such as highly effective modulator therapy, people with CF are now subject to the same co-morbidities that may benefit from NIV as their age matched cohorts. NIV can improve gas exchange, quality of sleep, exercise tolerance and augment airway clearance in CF, and it is important that CF providers are comfortable with this therapeutic modality. In this review, we will summarize the physiologic basis for NIV use in CF, describe indications for initiation of NIV, and postulate a practical approach for CF clinicians to take fin monitoring patients on NIV. We will discuss aspects unique to people with CF and the use of NIV. We hope that this serves as a resource for CF providers, especially those of us who do not have dedicated training in sleep medicine, as we continue to care for our patient population.

An Unusual Case of Necrotizing Pneumonia Presenting with Acute Kidney InjuryUgur Berkay Balkanci, MDSchool of Public Health, University of Minnesota, Minneapolis, MNDavid J. Sas, DODivision of Pediatric Nephrology and Hypertension, Department of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, MinnesotaNadir Demirel, MDDivision of Pediatric Pulmonology, Department of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, MinnesotaCorresponding Author:Nadir Demirel, MDDivision of Pediatric Pulmonology200 First Street SWRochester, MN 55906Tel. No.: 5075380754Fax No.: [email protected] words: postinfectious glomerulonephritis, pneumothorax, complications, complicated pneumoniaFinancial Disclosure: The authors have indicated they have no financial relationships relevant to this article to disclose.Funding: No external funding.Short title: “An unusual case of necrotizing pneumonia”To the Editor:Lower respiratory tract infections are the most common reason for hospitalization in the pediatric age group in the United States. Although pneumonia is prevalent, complicated pneumonia such as empyema, lung abscess and necrotizing pneumonia (NP) is uncommon in children1. The prevalence of complicated pneumococcal pneumonia decreased significantly after the introduction of the thirteen-valent pneumococcal vaccine in 20101. NP in the pediatric population is a severe disease characterized by extensive destruction and liquefaction of the lung tissue resulting in loss of the pulmonary parenchymal architecture, cavitation of the lung, and pleural involvement. Renal complications of complicated pneumonia are rare and mostly reported as atypical hemolytic uremic syndrome (HUS)2. Post-infectious glomerulonephritis (PIGN) is an unexpected complication of bacterial pneumonia3.We report a six-year-old otherwise healthy fully vaccinated girl with a 4-day history of fever, abdominal pain, vomiting, non-bloody diarrhea, and poor oral intake. Parents reported decreased urine output and dark-colored urine on the day of admission. Initial evaluation revealed serum creatinine of 5.01 mg/dL and blood urea nitrogen of 86 mg/dL, elevated acute phase reactants suggesting acute kidney injury (AKI) in the setting of an undiagnosed acute infectious process. The patient was admitted with decreased effective circulatory volume. Urinalysis revealed hematuria with <25% dysmorphic red blood cells (RBCs), proteinuria, pyuria, and RBC casts and granular casts, suggestive of acute glomerulonephritis.She was started on intermittent hemodialysis at day 2 of admission to address uremia, fluid overload, and hyperphosphatemia. A renal biopsy revealed diffuse exudative glomerulonephritis, consistent with infection-related glomerulonephritis. ASO, Anti-DNase B were negative; C3, C4 levels were low. She was treated with pulse IV methylprednisolone 10mg/kg/day for three days. The first 5 days in the hospital, the patient remained afebrile and her lung exam was normal without respiratory symptoms.On day six of admission, she developed acute right-sided chest pain and shortness of breath during hemodialysis. Chest x-ray (CXR) revealed a large right-sided tension pneumothorax, prompting therapeutic chest tube placement. Repeat CXR revealed reexpansion of the right lung and a significant right upper lobe consolidation with an ovoid hyperlucency and an air-fluid level. A chest CT scan confirmed the diagnosis of NP with multiple cavities (Image).Flexible bronchoscopy was performed with bronchoalveolar lavage revealing 42% neutrophils and negative cultures. She was treated with broad spectrum intravenous antibiotics.During admission, she developed hypertension, well-controlled with scheduled enalapril and amlodipine, as well as isradipine as needed. On day 14 of admission, hemodialysis was discontinued as kidney function improved, and chest tube was removed. She was discharged at day 26 of admission on intravenous ceftriaxone and oral metronidazole to complete 30 days of treatment. A repeat chest CT at end of treatment showed complete resolution of NP. Renal functions and blood pressure normalized on follow up.NP is characterized by persistent high fevers and prolonged hospitalizations even with appropriate antibiotic treatment1. Most often, NP affects immunocompetent children with no underlying risk factors4. The pathophysiology of this complication is acute liquefactive necrosis of the lung parenchyma which results in the development of pneumatoceles4. The most common pathogen causing NP is Streptococcus pneumoniae followed by Staphylococcus aureus and Streptococcus pyogenes. Other rarer bacterial and viral pathogens are Mycoplasma pneumonia, Influenza, and Adenovirus1. Identifying the microbiologic pathogen can be challenging and is only made in 50% of cases1. In our case, we did not isolate the causative microorganism. NP typically resolves without residual morbidity, even after a protracted course1,4.Pleural involvement is almost universal in NP, and the course of pleural disease often determines duration and outcome, particularly as it relates to the complication of bronchopleural fistula (BPF)1. BPF is most likely due to the necrotic development of a connection between bronchial space and pleural space4. BPF formation is associated with a significantly longer hospital stay in children with NP4. Yet, most cases heal without surgical intervention4. Tension pneumothorax has been observed as a rare complication of NP1.Renal involvement in complicated pneumonia is rare. Atypical HUS has been reported as a complication of pneumonia, particularly associated with empyema. (most commonly due to invasive Streptococcus pneumoniae)2. In a case series of 37 cases of atypical HUS, 34 patients (92%) had pneumonia with 10 patients (29%) with NP5. Less commonly, pneumonia can be associated with PIGN. PIGN is the most common glomerulonephritis in children worldwide. Pneumonia-associated PIGN is rare. In a case series from the US, PIGN accounted for 0.15% of admissions for pneumonia and 0.39% of admissions for glomerulonephritis6. Pneumonia-associated PIGN is known to be caused by various bacterial pathogens including Streptococcus pneumoniae, Staphylococcus aureus, Mycoplasma pneumoniae, Chlamydia pneumoniae, Nocardia, and Coxiella burnetii3. Different from the usual presentation of the PIGN (in which the time interval between a pharyngeal group A Streptococcal infection and PIGN is 6 to 10 days), pneumonia-associated PIGN is usually concomitant with the pulmonary disease3,6.Our case is unusual in several ways: pneumonia-associated PIGN typically presents with respiratory symptoms first, and acute kidney injury developing during the course of pneumonia3. More surprisingly, the patient developed NP which is characterized by even more severe respiratory symptoms1. Yet, our patient presented without respiratory complaints and pneumonia became apparent only after the development of pneumothorax. We could only identify 2 cases of pneumonia-associated PIGN who presented with renal involvement before pulmonary complaints6,7. Also, previous cases in the literature of pneumonia-associated PIGN report mostly a non-complicated course of pulmonary disease3,6. In a case series of 11 children with pneumonia-associated PIGN, only one case developed a small empyema6. Similarly, the majority of the reported cases of pneumonia-associated PIGN describe a benign course of renal disease3,6. Our patient’s kidney failure progressed rapidly, and she required 2 weeks of intermittent hemodialysis and a three-day course of pulse steroid therapy. At present, systemic corticosteroids are not recommended for patients with complicated pneumonia. A Cochrane review including 17 randomized controlled trials, of which four were conducted on children, found that corticosteroid therapy reduced mortality and morbidity in adults with severe CAP, and morbidity, but not mortality, in adults and children with non-severe CAP1. We speculate that pulse steroid treatment may have modified the course of NP in our patient.This case suggests an atypical presentation of NP with predominant renal complications is possible. Pediatricians should be aware of renal complications of respiratory diseases. Systemic steroids should be considered in the treatment of NP.References:1. de Benedictis FM, Kerem E, Chang AB, Colin AA, Zar HJ, Bush A. Complicated pneumonia in children. Lancet 2020;396:786-798.2. Spinale JM, Ruebner RL, Kaplan BS, Copelovitch L. Update on Streptococcus pneumoniae associated hemolytic uremic syndrome. Curr Opin Pediatr 2013;25:203-208.3. Carceller Lechón F, de la Torre Espí M, Porto Abal R, Écija Peiró JL. Acute glomerulonephritis associated with pneumonia: a review of three cases. Pediatr Nephrol 2010;25:161-164.4. Sawicki GS, Lu FL, Valim C, Cleveland RH, Colin AA. Necrotising pneumonia is an increasingly detected complication of pneumonia in children. Eur Respir J 2008;31:1285-1291.5. Banerjee R, Hersh AL, Newland J, Beekmann SE, Polgreen PM, Bender J, Shaw J, Copelovitch L, Kaplan BS, Shah SS. Streptococcus pneumoniae-associated Hemolytic Uremic Syndrome Among Children in North America. Pediatr Infect Dis J 2011;30:736-739.6. Srivastava T, Warady BA, Alon US. Pneumonia-associated acute glomerulonephritis. Clin Nephrol 2002;57:175-182.7. Schachter J, Pomeranz A, Berger I, Wolach B. Acute glomerulonephritis secondary to lobar pneumonia. Int J Pediatr Nephrol 1987;8:211-214.

Background: Multisystem Inflammatory Syndrome in Children (MISC) is a phenomenon that appeared in children infected with or exposed to SARS-CoV-2. The typical onset of MISC is 4-6 weeks following SARS-CoV-2 infection and is formulated to be due to an immune response. Methods: Our study retrospectively analyzed data from a tertiary center in UAE of MISC patients who were admitted to either general pediatric wards or pediatric intensive care (PICU) or who came exclusively for follow-up (post PICU admission) from May 2020 to August 2021.   Results: The total sample size is 50 patients and the study included a comparison of PICU admissions with none PICU admissions. The PICU sample size was 18 patients, 50% females, with mean age of 8.3 years all were previously healthy. PICU patients had deranged blood counts with a lower hemoglobin count, a more pronounced lymphopenia and thrombocytopenia along with hypoalbuminemia. PICU patients presented with relatively higher inflammatory markers: CRP, PCT, ferritin and D-dimer. Immunological studies were significantly higher for IL-6 levels in PICU patients. On echocardiography, higher myocardial dysfunction was more notable in patients admitted to PICU. Children admitted in PICU were provided with more extensive therapy. As part of our study course, we re-evaluated our PICU patients twice, once at 48 hours post PICU admission and again 4-6 weeks after discharge from the hospital. No deaths have been recorded in the cohort. Conclusion: This study evaluated risk factors of MISC and potential severity features. Follow up of patients on discharge showed improvement across all domains.

Objective and design: Our prospective observational study is the first study that evaluates the LUS findings of cardiopulmonary interactions in acutely ill children with elevated pro-BNP levels,with the aim of establishing the specific LUS pattern in this category of patients without primary lung diseases.Methodology:We prospectively analyzed epidemiological, clinical, laboratory, instrumental and lung ultrasound parameters in acutely ill children aged 1 month to 18 years admitted to the Department of Pediatrics between March 2020 to August 2020.Among the acutely ill patients evaluated, only patients with pro-BNP> 300 pg / ml and who underwent LUS before the start of any treatment were included. They were stratified into three sub-categories based on the diagnosis A) cardiac disease, B) systemic inflammatory disease / sepsis without functional and / or organic alterations of the myocardium and C) systemic inflammatory disease / sepsis and cardiac disease, and were classified into two groups based on the level of pro-BNP.We also enrolled patients belonging to two other categories (patients with primary infectious lung disease and completely healthy patients) analyzing their epidemiological, clinical, laboratory, instrumental parameters and lung ultrasound findings and comparing them with those of acutely ill children.Results and Conclusion: We found that LUS findings in these acutely ill children are different from the ultrasound pattern of other categories of children and in particular 1) children with acute lower respiratory tract infections and 2) healthy infants.The finding in a child of a sonographic interstitial syndrome with multiple, bright, long, separate and non-confluent B-lines / long vertical artefacts deriving from a normal and regular pleural line, in the absence of subpleural consolidations, is strongly predictive of cardiogenic pulmonary edema or pulmonary congestion in the course of systemic inflammatory disease / sepsis.

Thrombotic complications of SARS-CoV-2 have been increasingly recognized as an important component of COVID-19 in adults; however, they have been less evident in children. We report a case of a teenager with positive SARS‐CoV‐2 RT–PCR and underlying prothrombotic risk factors, including aromatase inhibitor therapy, who developed deep vein thrombosis resulting in pulmonary embolism. Laboratory tests revealed deranged coagulation parameters (high D-dimers and Factor VIII and low antithrombin). The patient required intensive care and was managed with anticoagulants, dexamethasone and antithrombin concentrate. Clinical condition and hemostatic profile gradually improved. A review of the available literature for similar cases is presented.

Acute respiratory distress syndrome (ARDS) is a disabling and potentially lethal syndrome requiring prompt recognition and urgent interventions to prevent morbidity and mortality[1]. Although constipation is not generally recognized as a cause for ARDS or usually listed within the differential diagnosis, there have been case reports describing such an association[2,3]. We present the case of a patient with history of intermittent constipation presenting with progressive abdominal pain and an acute abdomen that required emergent surgical fecal decompaction. This was followed by hypoxemic respiratory distress leading to respiratory failure in the setting of severe constipation and aspirated feculent material. To our knowledge, this is the first published case report describing aspirated feculent material in a child with respiratory failure due to ARDS.

Background: During COVID-19 pandemic, a metered-dose inhaler (MDI) with a valved holding chamber (VHC) is a preferred route of bronchodilator delivery. We have developed a new homemade VHC, made of a paper coffee cup and a drinking water bottle. This study was conducted to compare the bronchodilator response in children with airway hyperresponsiveness after the use of our homemade VHC and that of a standard commercial one. Methods: In a randomized, two-period, two-sequence crossover design, we recruited 20 children, aged 6-15 years, who had greater than 12% increase in FEV1 after inhaled salbutamol. They were randomized into Group A and B. Group A used our VHC on the first day and Aerochamber® on the second day. Group B used the same VHCs but in alternate sequence. Spirometries were performed before and after 400 microgram of salbutamol MDI was administered via those VHCs. Results: Baseline demographic data and spirometric values did not have statistically significant differences between group A and B and between the first and second day (P > 0.05). After giving salbutamol MDI, both VHCs produced significant increases in FVC, FEV1 and FEF25-75% (P < 0.005). The improvement in FEV1 did not significantly differ between our homemade VHC and Aerochamber® (P > 0.05). Conclusion: Our homemade VHC is effective for an MDI bronchodilator delivery. Since it is very cheap and easy to make, it may be used as a disposable device to minimize airborne transmission especially when commercial VHC are not available.

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.

Background: Children should be weaned from the ventilator once their clinical condition improves. Extubation failure is associated with poorer clinical outcomes in children. Predictive indicators of successful extubation are needed. This study aims to evaluate the predictive value of ultrasonographic diaphragm imaging could help predict weaning success. Methods: In this prospective, observational study conducted between March and December 2021, children between 1 month and 10 years of age who were mechanically ventilated for more than 48 hours were included. Diaphragm ultrasound (DUS) examinations were performed at the end of 2-hour extubation readiness test (ERT). The end-inspiratory thickness, end-expiratory thickness, diaphragmatic thickening fraction, diaphragmatic excursion, inspiratory slope and expiratory slope were evaluated. Results: Twenty-four (60%) patients were successfully extubated, while 16 (40%) required invasive or non-invasive mechanical ventilation support which were classified as failed extubation group. Three of the sixteen patients in the failed extubation group required re-intubation. Diaphragm thickening fraction was significantly greater in the successful weaning group (55,05 ± 23,75% vs. 30,9 ± 10,38%) (p<0,001). Diaphragm excursion was significantly greater in the SW group (14 ± 4,4 mm vs 11,05 ± 3,25 mm) (p<0,001). DTF and DE were found to have a sensitivity and specificity of 91.67 %, 87.50 %, and 83.33 %, 81.25 %, respectively. Conclusion: Diaphragm ultrasound is a feasible and promising tool to guide physicians during weaning from IMV. Among all DUS measurements, the DE and DTF indexes showed better performance in extubation failure than other diaphragmatic parameters.

Introduction: Point of care ultrasound (POCUS) is a useful tool to determine endotracheal tube placement; however, few studies have compared it with standard methods of confirmation. We evaluated the diagnostic accuracy of POCUS and time-to-interpretation for correct identification of tracheal versus esophageal intubations compared to a composite of standard-of-care methods in neonates. Methods: A cross-sectional study was conducted in the Neonatal Intensive Care Unit (NICU) at Aga Khan University Hospital Karachi Pakistan. All required intubations were performed as per NICU guidelines. The ETT placement was determined using standard-of-care methods (auscultation, colorimetric capnography, and chest X-ray) by a clinical team, and simultaneously by POCUS. Timings were recorded for each method by independent study staff. Results: A total of 348 neonates were enrolled in the study. More than half (58%) of intubations were in an emergency scenario. Using an expert as the reference standard, POCUS user interpretation showed 100% sensitivity and 94% specificity. We found a 99.4% agreement (Kappa: 0.96; p<0.001) between the POCUS user and expert. Diagnostic accuracy of POCUS compared with at least two standard-of-care methods demonstrated 99.7% sensitivity, 91% specificity, and 98.9% agreement (Kappa:0.93; p<0.001). The median time required for POCUS interpretation was 3.0 (IQR 3.0 -4.0) seconds for tracheal intubation. The time recorded for auscultation and capnography was 6.0 (IQR 5.0 -7.0) and 3.0 (IQR 3.0-4.0) respectively. Conclusion: POCUS is a rapid and reliable method of identifying ETT placement in neonates. Early and correct identification of airway management is critical to save lives and prevent mortality and morbidity.

Background:  Heterozygote carriers of potentially pathogenic variants in the cystic fibrosis transmembrane conductance regulator ( CFTR) gene have increased asthma risk. However, the frequency and impact of CFTR variation among individuals with asthma is unknown. Objective: To determine whether potentially pathogenic  CFTR variants associate with disease severity and whether individuals with two potentially pathogenic variants exist in a severe asthma-enriched cohort . Methods: We analyzed sequencing data spanning a 190.5Kb region of  CFTR in participants from the Severe Asthma Research Program (SARP1-3). Potentially pathogenic, rare  CFTR variants (frequency<0.05) were classified as CF-causing or of varying clinical consequences (VVCC) (CFTR2.org). Regression-based models tested for association between  CFTR genotypes (0-2 potentially pathogenic variants) and severity outcomes. Results: Of 1401 participants, 9.5% (134) had one potentially pathogenic variant, occurring more frequently in non-Hispanic white (NHW, 10.1% [84 of 831]) compared to African American individuals (AA, 5.2% [22 of 426]). We found ≥2 potentially pathogenic  CFTR variants in 1.4% (19); 0.5% (4) of NHW and 2.8% (12) of AA. Potentially pathogenic  CFTR variant genotypes (≥1 or ≥2 variants) were not cumulatively associated with lung function or exacerbations. In NHW, we found three F508del compound heterozygotes with F508del and a VVCC (two 5T;TG12[c.1210-11T>G] and one Arg1070Trp) and a homozygote for the VVCC, 5T;TG12. Conclusions: We found potentially pathogenic  CFTR variants within a severe asthma-enriched cohort , including three compound heterozygote genotypes variably associated with CF in NHW individuals. These findings provide the rationale for  CFTR sequencing and phenotyping of CF-related traits in individuals with severe asthma.

AIM: To report on the clinical, laboratory and radiological findings of adolescents who presented during the SARS-CoV-2 surge with symptoms of COVID-19, did not test positive for the infection and were diagnosed with e-cigarette and vaping product use associated lung injury (EVALI). Methods: A retrospective review of 12 cases of EVALI admitted to the Bristol Meyers Squibb Children’s Hospital between February 2020 and June 2020 was conducted. Results: The ages of the patients ranged from 14 to 19 years. There were 6 males and 6 females. Three patients had a past history of anxiety, depression or other psychiatric/mental health disorder, nine had prolonged coagulation profile (PT,PTT and/or INR) and eleven had elevated inflammatory markers. Eleven needed respiratory support. All 12 were negative for SARS-CoV-2 PCR. Four were tested for IgG Antibodies and were negative. As these cases were admitted to rule out COVID infection, initial treatment included hydroxychloroquine. Steroids were started only after SARS-CoV-2 PCR was shown to be negative. Urine THC was positive in all cases. CXR and CT findings showed ground glass opacities. CONCLUSIONS: Clinical and radiological features are similar in both EVALI and SARS-CoV-2 infection. Inflammatory markers are elevated in both conditions. A detailed social and substance use history in patients presenting with ‘typical’ COVID pneumonia like illness is important. EVALI should be ruled in early to start the appropriate treatment. Given the ongoing pandemic, pediatricians and other health care providers need to be aware of other conditions that can masquerade as SARS-CoV-2.

Introduction: Bronchopulmonary dysplasia (BPD) is characterized by lung injury with varying degrees of disrupted alveolarization, vascular remodeling, inflammatory cell proliferation, and pulmonary edema. Diuretics are often used to ameliorate the symptoms or progression of BPD. Our primary objective was to use lung ultrasound (LUS) to determine if diuretics decrease pulmonary edema in infants with BPD. The secondary objective was to assess changes in respiratory support during the first week after initiation of diuretics. Methods: Premature infants requiring non-invasive respiratory support and starting diuretic therapy for evolving BPD were compared with a similar group of infants not receiving diuretics (control). For the diuretic group, LUS exams were performed before and on days 1, 3 and 6 after initiation of treatment. For the control group, LUS was performed at equivalent time points. A composite pulmonary edema severity (PES) score of 0 to 5 was calculated based on the total number of B-lines in 6 scanned areas. Respiratory support parameters (FiO2, nasal cannula flow or CPAP) were also recorded. Results: Infants in the diuretic (n=28) and control (n=23) groups were recruited at median corrected gestational ages of 34.2 (33.3-35.9) and 34.0 (33.4-36.3) weeks, respectively ( p=0.82). PES scores, FiO2, and respiratory flow support decreased significantly from day 0 to 6 ( p<.0001, p=0.001, and p=0.01, respectively) in the diuretic group, but not in the control group. Conclusion: Diuretic use is associated with decreased pulmonary edema and improved oxygenation in infants with BPD during the first week of treatment.