Pulmonology Commons^™

Current Practices And Variations Among Pediatric Severe Asthma Programs In The United States-The North American Severe Pediatric Asthma Consortium., Kristina Gaietto, Nadia Krupp, Avani V. Shah, Erhan Ararat, Samantha H. Averill, Sachin Baxi, Matejka Cernelc-Kohan, Jeffrey M. Chambliss, Heather De Keyser, Monica Federico, Bob Geng, Akilah A. Jefferson, Parisa Kaviany, Lila C. Kertz, Kirsten Kloepfer, Allyson Larkin, Sydney Leibel, Tanya Martinez-Fernandez, Samira Naime, Robert D. Pesek, Dinesh K. Pillai, Deepa Rastogi, Katherine Rivera-Spoljaric, Kristie R. Ross, Franziska J. Rosser, Tregony C. Simoneau, Jade Tam-Williams, Kelan G. Tantisira, William Anderson, Jonathan M. Gaffin, Theresa W. Guilbert, Erick Forno

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Severe and difficult to treat asthma in children is a complex condition causing significant morbidity and associated healthcare costs. While treatment guidelines exist for severe asthma, optimal treatment approaches for the pediatric population are less well established. Furthermore, best practices regarding real world management and implementation of guidelines for the severe pediatric asthma population are lacking. In order to provide care for this population, institutions around the United States have developed multidisciplinary severe pediatric asthma programs (SPAPs). In recent years, the North American Severe Pediatric Asthma Consortium (NASPAC) was established as a mechanism for SPAPs to collaborate and share …

Persistent Wheezing In An Adolescent Patient, Matilda Dervisevic, Stephen Cliff

Posters

No abstract provided.

Leappt: Leveraging Generative Artificial Intelligence-Pediatric Pulmonology Training. Curriculum And Content Development., Alvin Singh, Erin Khan, Mary Moffatt

Manuscripts, Articles, Book Chapters and Other Papers

After redesigning the curriculum of the pediatric pulmonology fellowship program at Children's Mercy-Kansas City, leadership continued to make changes based on feedback from a faculty survey concerned about the quality and structure of didactics. These changes included leveraging generative artificial intelligence to create a detailed course content outline containing specific objectives and study points for each subject related to pediatric pulmonology. Using the content outline as a foundational reference for learners, fellowship leadership continues to enhance the curriculum by creating testing questions, flipped classroom sessions and evaluations, and hope that these specific changes will help address decreased passing rates on …

Genetic Testing Utilization In The U.S. Registry For Childhood Interstitial And Diffuse Lung Diseases., Laura A. Voss, Rebekah J. Nevel, Jennifer A. Wambach, Lawrence M. Nogee, Robin R. Deterding, Alicia M. Casey, Michael G. O'Connor, Daniel I. Craven, Jane B. Taylor, Gail H. Deutsch, Jade B. Tam-Williams, Lea C. Steffes, Steven K. Brennan, Maria T. Santiago, Sara C. Sadreameli, Andrea F. Heras, Michael R. Powers, Antonia P. Popova, Manvi Bansal, Aaron Hamvas, William A. Gower, Fernando Urrego, Lisa R. Young, Child Registry Collaborative

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Childhood interstitial and diffuse lung diseases (chILD) comprise a diverse group of rare disorders. Identifying the underlying cause is crucial for treatment, prognosis, and estimating recurrence risk. The objective of this study was to assess the utilization of genetic testing for subjects enrolled in the United States National Registry for ChILD, a multicenter observational study.

METHODS: Genetic data from participating sites were reviewed and analyzed in relationship to clinical characteristics.

RESULTS: Of 609 children enrolled from 22 centers, genetic testing was performed for 55.5% (n = 338). Genetic testing results were positive (diagnostic) for 22.8% (n = 77), negative …

Navigating Diagnostic And Treatment Challenges Of Pulmonary Hypertension In Infants With Bronchopulmonary Dysplasia., Nidhy P. Varghese, Gabriel Altit, Megan M. Gubichuk, Roopa Siddaiah

Manuscripts, Articles, Book Chapters and Other Papers

Advances in perinatal intensive care have significantly enhanced the survival rates of extremely low gestation-al-age neonates but with continued high rates of bronchopulmonary dysplasia (BPD). Nevertheless, as the survival of these infants improves, there is a growing awareness of associated abnormalities in pulmonary vascular development and hemodynamics within the pulmonary circulation. Premature infants, now born as early as 22 weeks, face heightened risks of adverse development in both pulmonary arterial and venous systems. This risk is compounded by parenchymal and airway abnormalities, as well as factors such as inflammation, fibrosis, and adverse growth trajectory. The presence of pulmonary hypertension in …

Safety And Efficacy Of Ivacaftor In Infants Aged 1 To Less Than 4 Months With Cystic Fibrosis., Paul Mcnally, Alvin Singh, Susanna A. Mccolley, Jane C. Davies, Mark Higgins, Meng Liu, Jennifer Lu, Violeta Rodriguez-Romero, Judy L. Shih, Margaret Rosenfeld, Vx15-770-124 Study Group

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Ivacaftor (IVA) has been shown to be safe and efficacious in children aged ≥4 months with cystic fibrosis (CF) and CFTR gating variants. We evaluated safety, pharmacokinetics (PK), and efficacy of IVA in a small cohort of infants aged 1 toCF.

METHODS: In this phase 3, open-label study, infants 1 toinfants, doses were adjusted at day 15 to better match median adult exposures based on individual PK measurements taken on day 4. Primary endpoints were safety and PK measurements.

RESULTS: Seven infants (residual function CFTR variants [n=5]; minimal function CFTR variants [n=2]) received ≥1 dose of IVA. Six infants …

Vitamin D Oral Replacement In Children With Obesity Related Asthma: Vdora1 Randomized Clinical Trial., Brian O'Sullivan, Song Ounpraseuth, Laura James, Marc Majure, Jason Lang, Zhuopei Hu, Alan Simon, Scott Bickel, Brian Ely, L E Faricy, Maryam Garza, Melody Greer, Daniel Hsia, Akilah Jefferson, Lisa Knight, Jeannette Lee, Deborah Liptzin, Mehtap Haktanir Abul, Tamara T. Perry, Fred Prior, Christine Sangiovanni, Jade B. Tam-Williams, Brian Wu, Jessica Snowden, Echo Idea States Pediatric Clinical Trial Network

Manuscripts, Articles, Book Chapters and Other Papers

Children with asthma and obesity are more likely to have lower vitamin D levels, but the optimal replacement dose is unknown in this population. The objective of this study is identifying a vitamin D dose in children with obesity-related asthma that safely achieves serum vitamin D levels of ≥ 40 ng/mL. This prospective multisite randomized controlled trial recruited children/adolescents with asthma and body mass index ≥ 85% for age/sex. Part 1 (dose finding), evaluated 4 oral vitamin D regimens for 16 weeks to identify a replacement dose that achieved serum vitamin D levels ≥ 40 ng/mL. Part 2 compared the …

Increasing Annual Eye Exam Completion For Children With Cystic Fibrosis Receiving Cftr Modulators, Paula Capel, Claire Elson, Stephanie Duehlmeyer, Jessica Haynes, Amanda Conway, Christopher M. Oermann

Posters

Background: CFTR modulators are life-changing therapy for people with CF. During preclinical studies of ivacaftor, cataracts were identified in juvenile rat pups. Subsequently, noncongenital cataracts have been reported in children receiving CFTR modulator therapy [1], so baseline and annual slit lamp eye examinations for cataract development are recommended for children with CF (aged <18). At Children’s Mercy Kansas City (CMKC), our standard practice is to conduct baseline and annual ophthalmologic examinations to assess for cataracts in children with CF prescribed CFTR modulator therapy, but there was no formal process for tracking adherence to this standard and ensuring that examinations were completed. A quality improvement project was undertaken in May 2022 to determine the rate of completion of annual eye exams and increase adherence to our annual monitoring standard.

Methods: To improve adherence to annual monitoring standards, a quality improvement taskforce including CF care team nurses and pharmacists was developed. A database was used to document baseline and follow-up eye examinations. A standardized communication process was developed and deployed that included an eye exam brochure (Figure …

A Rare Case Of Suspected Generalized Arterial Calcification Of Infancy (Gaci) In An Infant Presenting With Respiratory Failure In An Infant Presenting With Respiratory Failure And Arterial Calcification, Lilah Melzer, Catharine Kral, Bonnie Sullivan, Eric T. Rush, Erin Khan

Posters

No abstract provided.

Recurrent Primary Spontaneous Pneumothorax Masquerading As A Congenital Pulmonary Airway Malformation In A Young Female, Chandra Swanson, Justin Sobrino, Tolulope A. Oyetunji Md Mph, Erin Khan

Posters

Introduction: Spontaneous pneumothoraxes in children are uncommon, may be idiopathic or associated with underlying pulmonary disease, and can present management challenges. We present a 12-year-old female with recurrent right sided spontaneous pneumothorax in the setting of an asymptomatic SARS-Co-V2 (COVID) infection and imaging concerning for congenital lobar overinflation (CLO) versus congenital pulmonary airway malformation (CPAM), prompting surgical intervention. Case: A 12-year-old pre-menstrual female with remote history of eczema, asthma, and environmental allergies presented from an outside facility with four-days of progressive chest pain and dyspnea on exertion and diagnosis of right-sided spontaneous pneumothorax, improving after pigtail chest tube placement. Physical …

Obstructive Sleep Apnea In Neonates., Indira Chandrasekar, Mary Anne Tablizo, Manisha Witmans, Jose Cruz, Marcus Cummins, Wendy Estrellado-Cruz

Manuscripts, Articles, Book Chapters and Other Papers

Neonates have distinctive anatomic and physiologic features that predispose them to obstructive sleep apnea (OSA). The overall prevalence of neonatal OSA is unknown, although an increase in prevalence has been reported in neonates with craniofacial malformations, neurological disorders, and airway malformations. If remained unrecognized and untreated, neonatal OSA can lead to impaired growth and development, cardiovascular morbidity, and can even be life threatening. Polysomnography and direct visualization of the airway are essential diagnostic modalities in neonatal OSA. Treatment of neonatal OSA is based on the severity of OSA and associated co-morbidities. This may include medical and surgical interventions individualized for …

Covid-19 Vaccination In Individuals With Cystic Fibrosis At A Pediatric Cystic Fibrosis Center, Christopher M. Oermann, Claire Elson, Ellen Meier, Paula Capel, Jessica Haynes, Michelle Fischer, Stephanie Duehlmeyer

Posters

Background: Observational data suggest that most people with cystic fibrosis (PwCF) who contract COVID-19 have outcomes similar to those of the general population, although PwCF who are older or have CF-related diabetes, poor lung function, or a history of lung transplantation may be at greater risk for more severe disease. Therefore, the CF Foundation advocates for PwCF to discuss vaccination with care teams. At present, the FDA has authorized emergency use of 3 COVID-19 vaccines. ACIP/CDC guidance allows each state to determine vaccine distribution based on an individual’s exposure and risk for severe disease. This study describes the attitudes of …

Optimizing Oral Glucose Tolerance Test Completion At A Pediatric Cystic Fibrosis Care Center: A 10-Year Continuing Quality Improvement Effort, Christopher M. Oermann, Paula Capel, Jessica Haynes, Michelle Fischer, Jill Kohmetscher

Posters

Background: Cystic fibrosis–related diabetes (CFRD) is a common comorbidity among people with CF (PwCF). It is associated with weight loss, protein catabolism, lung function decline, and increased mortality. Nutritional status and pulmonary function begin to decline in PwCF several years before the diagnosis of CFRD. Early CFRD detection and aggressive insulin therapy have been shown to reduce the mortality gap between PwCF who have CFRD and those who do not. The Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes recommend annual screening for people with CF starting at age 10 [1]. Methods: In 2011, team members at Children’s Mercy Kansas City …

Vancomycin Auc Monitoring In Individuals With Cystic Fibrosis At A Pediatric Institution, Christopher M. Oermann, Stephanie Duehlmeyer, Ellen Meier, Claire Elson

Posters

Vancomycin AUC monitoring in individuals with cystic fibrosis at a pediatric institution S. Duehlmeyer1, C. Oermann1, E. Meier1, E. Elson1. 1Pulmonology, Children’s Mercy Kansas City, Kansas City, USA Background: Antibiotic therapy is essential for the treatment of cystic fibrosis (CF) lung infections. Methicillin-resistant Staphylococcus aureus (MRSA) infects 20% to 25% of people with CF (PwCF) and is associated with increased morbidity. Treatment of pulmonary exacerbations (PEs) often requires hospitalization including respiratory treatments and intravenous (IV) antimicrobials. IV vancomycin, which is commonly used for MRSA infections, requires serum concentration monitoring to ensure efficacy and minimize toxicity. Previous guidelines recommended trough concentrations …

Congenital Abnormalities Of The Lower Airways And Lungs, Christopher M. Oermann

Presentations

Goals and Objectives. Participants will be able to: Describe the 5 stages of fetal lung development. Summarize the prenatal identification and management of congenital abnormalities of the lower airways and lung (CALAL). Explain the classification of CALAL. Compare the clinical characteristics, pathology, and management of common CALAL. Discuss the long-term prognosis of CALAL.

Outcomes Of Infants With Severe Rop At Risk Of Treatment With Avastin Compared To Laser Surgery, Stephen Doxey

Research Days

Background: Retinopathy of prematurity (ROP) is a well-known complication occurring in the most premature infants leading to visual impairment and in the most severe cases associated with retinal detachment and childhood blindness. Factors contributing the development of ROP include chronic hypoxia and disordered vascular genesis primarily through the vascular endothelial growth factor (VEGF) signaling pathway. Infants at risk of developing ROP are screened with serial eye exam and treated with either laser surgery or intraocular injects of bevacizumab (Avastin) to prevent progression of ROP and preserve vision. Bevacizumab, an inhibitor of VEGF signaling, may have positive effects on preventing ROP …

Effect Of Intrauterine Smoke Exposure On Microrna-15a Expression In Human Lung Development And Subsequent Asthma Risk., Sunita Sharma, Alvin T. Kho, Divya Chhabra, Kathleen Haley, Carrie A. Vyhlidal, R Gaedigk, J Steven Leeder, Kelan G. Tantisira, Benjamin Raby, Scott T. Weiss

Manuscripts, Articles, Book Chapters and Other Papers

Background: In utero smoke (IUS) exposure is associated with asthma susceptibility.

Objective: We sought to test the hypothesis that changes in miRNA expression by IUS exposure during human lung development is associated with asthma susceptibility.

Methods: Gene expression was profiled from 53 IUS unexposed and 51 IUS exposed human fetal lung tissues. We tested for the differential expression of miRNAs across post-conception age and by IUS using linear models with covariate adjustment. We tested the IUS-associated miRNAs for association with their gene expression targets using pair-wise inverse correlation. Using our mouse model, we investigated the persistence of the IUS-associated miRNA …

Development And Interim Analysis Of A Cystic Fibrosis-Specific Antibiogram, Claire Elson, Ellen Meier, Douglas Swanson, Rangaraj Selvarangan, Megan Gripka, Christopher M. Oermann

Posters

Antimicrobial therapy is essential to treat cystic fibrosis (CF) lung infections. Empiric antimicrobial selection is generally based on previous culture information and, if available, an institution-specific antibiogram (ABGM). Most institutions antibiograms exclude cultures from individuals with cystic fibrosis, imposing challenges with empiric antimicrobial selection and monitoring susceptibility patterns over time. A cystic fibrosis-specific antibiogram may help drive population-specific antimicrobial selection and improve antimicrobial stewardship.

Congenital Abnormalities Of The Lower Airways And Lungs, Christopher M. Oermann

Presentations

Goals and Objectives. Participants will be able to: Describe the 5 stages of fetal lung development. Summarize the prenatal identification and management of congenital abnormalities of the lower airways and lung (CALAL). Explain the classification of CALAL. Compare the clinical characteristics, pathology, and management of common CALAL. Discuss the long term prognosis of CALAL

A Multidisciplinary Chronic Lung Disease Team In A Neonatal Intensive Care Unit Is Associated With Increased Survival To Discharge Of Infants With Tracheostomy, Taylor Hansen

Research Days

No abstract provided.

Development Of Drive-Through And Mail-In Systems For Obtaining Surveillance Respiratory Specimens In A Pediatric Cystic Fibrosis Center, Ellen Meier, Paula Capel, Christopher M. Oermann

Posters

Routine surveillance cultures are an essential part of health maintenance for people with cystic fibrosis (CF). The Cystic Fibrosis Foundations (CFF) recommends that respiratory cultures be obtained every three months. The CF Care Center at Children’s Mercy Kansas City (CMKC) also obtains respiratory cultures when there is an acute change in respiratory symptoms. The COVID-29 pandemic resulted in ambulatory clinic closures and an inability to obtain surveillance respiratory cultures. A creative solution for obtaining respiratory cultures was needed during the pandemic.

Pneumothorax: What’S A Pediatric Pulmonologist To Do?, Terrence W. Carver

Manuscripts, Articles, Book Chapters and Other Papers

A pneumothorax is a collection of air in the pleural space commonly associated with respiratory symptoms. Patients may be relatively asymptomatic but may present with life-threatening distress. Confirmation of a clinically suspected pneumothorax is most often by chest x-ray when patients have been stabilized. Pneumothoraces are first categorized as primary, secondary, or iatrogenic and informs the evaluation and treatment plan. However, despite management guidelines in the literature, their applicability for pediatric patients is limited. Understanding of the historical risk factors including the acute clinical course and how radiographs, both chest X-ray and CT chest illuminate which patients are at highest …

Standardizing Aminoglycoside Induced Ototoxicity Monitoring, Claire Elson, Christopher M. Oermann, Stephanie Duehlmeyer, Ellen Meier

Posters

Aminoglycoside (AG) antibiotics are essential for the treatment of cystic fibrosis (CF) lung infections. Pseudomonas aeruginosa. Nontuberculous mycobacteria. Monitoring is critical secondary to potential nephrotoxicity and ototoxicity. Children’s Mercy Kansas City (CMKC), standardized nephrotoxicity monitoring in 2016. Observed variable ototoxicity monitoring practices. Prevalence of ototoxicity, 2016 CFF Patient Registry, 1.1% in pediatric patients (less than or equal to 18 years). 2.2% in pediatric and adult population. National Institute of Deafness and Other Communication Disorders, 13% in US population equal to or less than 12 years old. A standardized AG induced ototoxicity monitoring algorithm (AIOA) was developed and implemented at CMKC …

A Multifaceted Approach To Improve Quarterly Visit Rates At A Pediatric Cystic Fibrosis Care Center, Paula Capel, Jessica Banks, Micaela Mckenna, Ashley Andrews, Christopher M. Oermann

Posters

Standard quality improvement methodology was used to improve quarterly visit rates among cystic fibrosis patients at Children's Mercy Kansas City Cystic Fibrosis Center.

• A family-centered, team-based approach was adopted
• A change in culture led to sustained improvement

Improved quarterly visit rates should drive improvement in outcomes including pulmonary function and nutritional status.

Improving Pneumococcal Vaccination Rates In High Risk Patients Across Multiple Specialty Divisions, Rachel Moran, Julia G. Harris, Claire Olsen, Rana El Feghaly, Liset Olarte, Douglas Blowey, Luke A. Harris

Posters

Pediatric patients with deficient immune systems or certain chronic medical conditions have an increased risk of acquiring invasive pneumococcal disease.

The 23-valent pneumococcal (PPSV23) vaccine provides protection against 23 pneumococcal serotypes and is recommended for patients aged 2 years or older who are high-risk for invasive pneumococcal disease.

Unfortunately, many high-risk patients are not properly vaccinated due to lack of provider knowledge or understanding of accountability between primary care and specialty providers.

The goal of this project was to improve PPSV23 vaccination rates by 10-20% across multiple Children's Mercy Kansas City specialty divisions.

Congenital Abnormalities Of The Lower Airways And Lungs, Christopher M. Oermann

Presentations

Participants will be able to: Describe the 5 stages of fetal lung development. Summarize the prenatal identification and management of congenital abnormalities of the lower airways and lung (CALA). Explain the classification of CALAL. Compare the clinical characteristics, pathology, and management of common CALAL. Discuss the long term prognosis of CALAL.

Dehydrated Hereditary Stomatocytosis Causing Sever Ascites Leading To Pulmonary Hypoplasia And Respiratory Insufficiency In A Neonate, Michael D. Mcdowell Jr, Christopher M. Oermann

Posters

Care Presentation: EM is a 32.5-week EGA infant who was born with sever congenital ascites (following repeated prenatal paracentesis) identified on prenatal ultrasound. Initial chest-abdomen imaging is shown in Figure 1. He required CPAP at delivery but was weaned of high flow nasal cannula with intermittent episodes of prolonged intubation during repeat paracentesis. Paracentesis demonstrated simple ascites rather than chylous fluid. Lymphangiograms demonstrated transient lymphatic leakage with resolution. Genetic evaluation revealed a pathogenic mutation in the PIEZO 1 gene for autosomal dominant DHS. This disorder had reduced penetrance and variable expressivity and symptoms range for absence of clinical features to …

Direct Lung Sampling Indicates That Established Pathogens Dominate Early Infections In Children With Cystic Fibrosis., Peter Jorth, Zarmina Ehsan Md, Amir Rezayat, Ellen Caldwell, Christopher Pope, John J. Brewington, Christopher H. Goss, Dan Benscoter, John P. Clancy, Pradeep K. Singh

Manuscripts, Articles, Book Chapters and Other Papers

Culture and sequencing have produced divergent hypotheses about cystic fibrosis (CF) lung infections. Culturing suggests that CF lungs are uninfected before colonization by a limited group of CF pathogens. Sequencing suggests diverse communities of mostly oral bacteria inhabit lungs early on and diversity decreases as disease progresses. We studied the lung microbiota of CF children using bronchoscopy and sequencing, with measures to reduce contamination. We found no evidence for oral bacterial communities in lung lavages that lacked CF pathogens. Lavage microbial diversity varied widely, but decreases in diversity appeared to be driven by increased CF pathogen abundance, which reduced the …

Aztreonam Lysine Inhalation Solution In Cystic Fibrosis., Claire Elson, Joel Mermis, Deepika Polineni, Christopher M. Oermann

Manuscripts, Articles, Book Chapters and Other Papers

Patients with cystic fibrosis (CF) develop pulmonary disease secondary to airway infection and dysregulated inflammation. Therapeutic innovations such as nebulized antimicrobial therapy targeting specific pathogens have resulted in improvements in quality of life and life expectancy. Aztreonam lysine for inhalation (AZLI) solution was initially approved to improve respiratory symptoms in CF patients with Pseudomonas aeruginosa (PA) in 2010 by the Food and Drug Administration. Since then, research broadening labeling and clinical application has been developed. In this review, we analyze published and ongoing research regarding AZLI therapy in CF. A search of the Cochrane Database of Systematic Reviews and the …

Age Appropriate Assisted Airway Clearance Techniques For Children, Christopher M. Oermann

Presentations

Describe normal airway clearance and the disease processes for which assisted airway clearance therapy is indicated. Explain the physiologic rational for and data supporting use of various assisted airway clearance techniques used among children. Develop an age and diagnosis appropriate airway clearance plan for a child with impaired airway clearance.