Pulmonology Commons^™

Global Respiratory Syncytial Virus-Related Infant Community Deaths, Natalie I. Mazur, Yvette N. Löwensteyn, Joukje E. Willemsen, Christopher J. Gill, Leah Forman, Lawrence M. Mwananyanda, Dianna M. Blau, Robert F. Breiman, Shabir A. Madhi, Abdul Momin Kazi

Department of Paediatrics and Child Health

Background: Respiratory syncytial virus (RSV) is a leading cause of pediatric death, with >99% of mortality occurring in low- and lower middle-income countries. At least half of RSV-related deaths are estimated to occur in the community, but clinical characteristics of this group of children remain poorly characterized.
Methods: The RSV Global Online Mortality Database (RSV GOLD), a global registry of under-5 children who have died with RSV-related illness, describes clinical characteristics of children dying of RSV through global data sharing. RSV GOLD acts as a collaborative platform for global deaths, including community mortality studies described in this supplement. We aimed …

Challenges Of Assessing Community Mortality Due To Respiratory Viruses In Children Aged Less Than 5 Years, Mauricio T. Caballero, Ashish Satav, Christopher J. Gill, Saad B. Omer, Rachel C. Pieciak, Abdul Momin Kazi, Eric Af Simões, Fernando P. Polack

Department of Paediatrics and Child Health

Background: Estimating the real impact of respiratory syncytial virus (RSV) disease is key for the development of vaccines and treatments. Ascertaining the burden of community mortality due to RSV is challenging due to the lack of primary data. Therefore, conducting observational studies to determine the factors associated with community mortality due to the virus in developing countries is important.
Objective: Our aim in this study was to describe the obstacles, gaps, and challenges that investigators face in low-income, vulnerable regions in 4 developing countries on 3 continents.
Results: The main obstacles and challenges of ascertaining community mortality due to RSV …

Risk Factors Leading To Pulmonary Exacerbation In Patients With Cystic Fibrosis: A Systematic Review, Danish Abdul Aziz, Syeda Khadija Fatima, Hasan Nawaz Tahir

Department of Paediatrics and Child Health

Objective: To ascertain major risk factors associated with pulmonary exacerbation and pulmonary function decline in cystic fibrosis.
Methods: The systematic review was conducted at Aga Khan University, Karachi, in September 2018, and comprised electronic search of PubMed, Ovid, Science Direct and Cumulative Index of Nursing and Allied Health Literature databases of studies conducted from January 1990 to September 2018 which were categorised into 3 sets; 1990-98, 1999-2007 and 2008-18. Studies included for review focussed on articles with pulmonary exacerbation as the health outcome indicator, and had diagnosis of cystic fibrosis as the inclusion criteria, while risk factors were the exposure …

Occlusive Pulmonary Artery Thrombosis In A Healthy Neonate With No Identifiable Risk Factors, Cynthia Laviolette, Jason Turner, Latasha Lewis, Song-Gui Yang, Tim Pettitt, Kurt D Piggott

Faculty, Staff and Student Publications

Pulmonary artery thrombosis is reported in neonates with risk factors for hypercoagulability. No consensus exists regarding standard therapy for this condition. We present a neonate, with no risk factors for thrombosis, who was admitted after birth to the Pediatric Cardiac Intensive Care Unit with an occlusive left pulmonary artery thrombus.

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 …

Serial Lung Ultrasounds In Pediatric Pneumonia In Mozambique And Pakistan, Amy Sarah Ginsburg, Muhammad Imran Nisar, Lola Madrid, Jennifer L. Lenahan, Benazir Baloch, Pio Vitorino, Jun Hwang, Alessandro Lamort, Nee Kanth, Fyezah Jehan

Department of Paediatrics and Child Health

Lung ultrasound (LUS) is a promising point-of-care imaging technology for diagnosing and managing pneumonia. We sought to explore serial LUS examinations in children with chest-indrawing pneumonia in resource-constrained settings and compare their clinical and LUS imaging courses longitudinally. We conducted a prospective, observational study among children aged 2 through 23 months with World Health Organization Integrated Management of Childhood Illness chest-indrawing pneumonia and among children without fast breathing, chest indrawing or fever (no pneumonia cohort) at 2 district hospitals in Mozambique and Pakistan. We assessed serial LUS at enrollment, 2, 6, and 14 days, and performed a secondary analysis of …

Feasibility, Usability And Acceptability Of Paediatric Lung Ultrasound Among Healthcare Providers And Caregivers For The Diagnosis Of Childhood Pneumonia In Resource-Constrained Settings: A Qualitative Study, Atif Riaz, Olga Cambaco, Laura Elizabeth Ellington, Jennifer L. Lenahan, Khatia Munguambe, Usma Mehmood, Sana Qaisar, Benazir Baloch, Muhammad Imran Nisar, Fyezah Jehan

Department of Paediatrics and Child Health

Objectives: Paediatric pneumonia burden and mortality are highest in low-income and middle-income countries (LMIC). Paediatric lung ultrasound (LUS) has emerged as a promising diagnostic tool for pneumonia in LMIC. Despite a growing evidence base for LUS use in paediatric pneumonia diagnosis, little is known about its potential for successful implementation in LMIC. Our objectives were to evaluate the feasibility, usability and acceptability of LUS in the diagnosis of paediatric pneumonia.
Design: Prospective qualitative study using semistructured interviews SETTING: Two referral hospitals in Mozambique and Pakistan PARTICIPANTS: A total of 21 healthcare providers (HCPs) and 20 caregivers were enrolled.
Results: HCPs …

Cardio-Pulmonary Sequelae In Recovered Covid-19 Patients: Considerations For Primary Care, Zouina Sarfraz, Azza Sarfraz, Alanna Barrios, Radhika Garimella, Asimina Dominari, Manish Kc, Krunal Pandav, Juan C. Pantoja, Varadha Retnakumar, Ivan Cherrez-Ojeda

Department of Paediatrics and Child Health

Background: Current literature lacks characterization of the post-recovery sequelae among COVID-19 patients. This review characterizes the course of clinical, laboratory, radiological findings during the primary infection period, and the complications post-recovery. Primary care findings are presented for long-COVID care.
Methods: Adhering to PRISMA guidelines, 4 databases were searched (PubMed, Embase, CINAHL Plus, Scopus) through December 5, 2020, using the keywords "COVID-19 and/or recovered and/or cardiovascular and/or long-term and/or sequelae and/or sub-acute and/or complication." We included published peer-reviewed case reports, case series, and cross-sectional studies providing the clinical course of COVID-19 infection, and cardiopulmonary complications of patients who recovered from COVID-19, …

Global Burden Of Acute Lower Respiratory Infection Associated With Human Metapneumovirus In Children Under 5 Years In 2018: A Systematic Review And Modelling Study, Xin Wang, You Li, Maria Deloria-Knoll, Shabir A. Madhi, Cheryl Cohen, Syed Asad Ali, Sudha Basne, Quique Bassat, W Abdullah Brooks, Malinee Chittaganpitch

Department of Paediatrics and Child Health

Background: Human metapneumovirus is a common virus associated with acute lower respiratory infections (ALRIs) in children. No global burden estimates are available for ALRIs associated with human metapneumovirus in children, and no licensed vaccines or drugs exist for human metapneumovirus infections. We aimed to estimate the age-stratified human metapneumovirus-associated ALRI global incidence, hospital admissions, and mortality burden in children younger than 5 years.
Methods: We estimated the global burden of human metapneumovirus-associated ALRIs in children younger than 5 years from a systematic review of 119 studies published between Jan 1, 2001, and Dec 31, 2019, and a further 40 high …

Performance Of Lung Ultrasound In The Diagnosis Of Pediatric Pneumonia In Mozambique And Pakistan, Amy Sarah Ginsburg, Jennifer L. Lenahan, Fyezah Jehan, Rubao Bila, Alessandro Lamorte, Jun Hwang, Lola Madrid, Muhammad Imran Nisar, Benazir Baloch, Naila Nadeem

Department of Paediatrics and Child Health

Introduction: Improved pneumonia diagnostics are needed in low-resource settings (LRS); lung ultrasound (LUS) is a promising diagnostic technology for pneumonia. The objective was to compare LUS versus chest radiograph (CXR), and among LUS interpreters, to compare expert versus limited training with respect to interrater reliability.
Methods: We conducted a prospective, observational study among children with World Health Organization (WHO) Integrated Management of Childhood Illness (IMCI) chest-indrawing pneumonia at two district hospitals in Mozambique and Pakistan, and assessed LUS and CXR examinations. The primary endpoint was interrater reliability between LUS and CXR interpreters for pneumonia diagnosis among children with WHO IMCI …

Simulator-Based Ultrasound Training For Identification Of Endotracheal Tube Placement In A Neonatal Intensive Care Unit Using Point Of Care Ultrasound, Khushboo Qaim Ali, Sajid Bashir Soofi, Ali Shabbir Hussain, Uzair Ansari, Shaun Morris, Mark Oliver Tessaro, Shabina Ariff, Hasan Merali

Department of Paediatrics and Child Health

Background: Simulators are an extensively utilized teaching tool in clinical settings. Simulation enables learners to practice and improve their skills in a safe and controlled environment before using these skills on patients. We evaluated the effect of a training session utilizing a novel intubation ultrasound simulator on the accuracy of provider detection of tracheal versus esophageal neonatal endotracheal tube (ETT) placement using point-of-care ultrasound (POCUS). We also investigated whether the time to POCUS image interpretation decreased with repeated simulator attempts.
Methods: Sixty neonatal health care providers participated in a three-hour simulator-based training session in the neonatal intensive care unit (NICU) …

Ace2 Mouse Models: A Toolbox For Cardiovascular And Pulmonary Research, Hongpeng Jia, Xinping Yue, Eric Lazartigues

School of Medicine Faculty Publications

Angiotensin-converting enzyme 2 (ACE2) has been identified as the host entry receptor for the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) responsible for the COVID-19 pandemic. ACE2 is a regulatory enzyme of the renin-angiotensin system and has protective functions in many cardiovascular, pulmonary and metabolic diseases. This review summarizes available murine models with systemic or organ-specific deletion of ACE2, or with overexpression of murine or human ACE2. The purpose of this review is to provide researchers with the genetic tools available for further understanding of ACE2 biology and for the investigation of ACE2 in the pathogenesis and treatment of COVID-19.

Respiratory Failure Due To A Large Mediastinal Mass In A 4-Year-Old Female With Blast Cell Crisis: A Case Report., Christian I Wade, Cody J Couperus-Mashewske, Mia E Geurts, Nicholas Derfler, John Ngo, Kyle S Couperus

Articles, Abstracts, and Reports

INTRODUCTION: Symptomatic leukostasis is an exceptionally atypical presentation of blast crisis; and when coupled with an enlarged neoplastic mediastinal mass in a four-year-old female, an extremely rare and challenging pediatric emergency arises.

CASE REPORT: We present a unique case of a four-year-old female who arrived via emergency medical services in cardiopulmonary arrest with clinical and radiographic evidence suggestive of bilateral pneumothoraces, prompting bilateral chest tube placement. Further evaluation revealed a large mediastinal mass and a concurrent white blood cell count of 428,400 per milliliter (/mL) (4,400-12,900/mL), with a 96% blast differential, consistent with complications of T-cell acute lymphoblastic leukemia.

CONCLUSION: …

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

Idiopathic Pulmonary Hemosiderosis - A Rare Cause Of Chronic Anemia, Ayesha Butt, Rashida Ahmed, Muhammad Dawood Amir Sheikh, Omar Khan, Nousheen Iqbal, Arshalooz Rahman, Javaid Khan

Medical College Documents

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease marked by alveolar bleeding and accumulation of hemosiderin in the lungs. Here we present three cases of IPH. The first case is of a 26-year-old male with anemia, hemoptysis and dyspnea. Bronchoscopy confirmed diffuse alveolar hemorrhage (DAH). A diagnosis of IPH was made after ruling out other causes of DAH and observing good response to steroids. The patient's condition improved with prednisolone and azathioprine. The second case is of 26-year-old female with severe anemia. Imaging suggested IPH and lung biopsy confirmed it. She died shortly afterwards. The third case is of a …

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.

Temporal Gene Expression Of Mesenchymal Cells In The Pediatric Lung, Quinlen F. Marshall, Soumyaroop Bhattacharya, Gautam Bandyopadhyay, Ravi Misra, Thomas Mariani, Gloria Pryhuber

Chemistry Student Work

INTRODUCTION: The newborn lung undergoes vast biochemical and physiological changes during adaptation from the intrauterine to the extrauterine environment. Lung morphogenesis continues from birth into early childhood, mediated by dynamic gene expression and a diversity of pulmonary cell types (Whitsett, JA. et al. Physiol. Rev, 2019). Murine models demonstrate that pulmonary mesenchymal cells exhibit remarkable heterogeneity in function and morphology during development, however, confirmation of their role is lacking in human neonates and early childhood (Guo, M. et al. Nat. Comm, 2019). In addition, many current human genomic studies of lung maturation suffer from limited sample size, limiting …

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 …

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.

Quickcough: An Instrumentational Proximal Airway Clearance Technique (Act) For Select Patients With Neuromuscular Disease (Nmd), Sean X. Sullivan, Russell K. Catania, Madison K. Plaster, Sydney E. Kandray, Mariah Costa

Williams Honors College, Honors Research Projects

Patients with neuromuscular disease (NMD) requiring tracheostomy and mechanical ventilation secondary to respiratory failure encounter increased difficulty in removing pulmonary secretions from the airways. To combat issues associated with current treatment modalities for insufficient cough efficacy (cost, poor mobility, discomfort, lack of evidence), we have developed an instrumentational proximal airway clearance technique (ACT) which augments a manual proximal ACT developed by a client with NMD. QuickCough is a 3D-printed PLA attachment to the tracheostomy apparatus which has demonstrated its ability to facilitate pressure changes necessary to increase patient’s peak cough flow (PCF) by providing a stronger exsufflation for the patient. …

Bronchopulmonary Dysplasia: Comparison Between The Two Most Used Diagnostic Criteria, Enrique Gomez Pomar, Vanessa A. Concina, Aaron Samide, Philip M. Westgate, Henrietta S. Bada

Pediatrics Faculty Publications

Objectives: To compare the Shennan's and the consensus definition of Bronchopulmonary Dysplasia (BPD) from the National Institutes of Health (NIH) workshop and analyze specific risk factors associated with each definition.

Study design: Retrospective analysis of records of 274 infants admitted to a level IV intensive care unit. Infants were classified as having BPD or no BPD by both definitions. Differences in incidence and risk factors were analyzed. Statistical methods included descriptive statistics, comparative tests, and marginal logistic regression modeling.

Results: The estimated difference in prevalence was 32% [95% CI: (26%, 37%), (p < 0.0001)] between both criteria. The prevalence of BPD was 80% higher based on the NIH criteria [RR = 1.80; 95% CI: (1.58, 2.06)]. Infants with no BPD by the Shennan definition were breathing room air with or without positive or continuous pressure support and were most likely to be discharged home on oxygen [OR = 4.47, 95% CI: (1.20, 16.61), p = 0.03]. Gestational age, birth weight, …

A Multicenter Study To Evaluate Pulmonary Function In Osteogenesis Imperfecta., Allison Tam, Shan Chen, Evan Schauer, Ingo Grafe, Venkata Bandi, Jay R. Shapiro, Robert D. Steiner, Peter A. Smith, Michael B. Bober, Tracy Hart, David Cuthbertson, Jeffrey Krischer, Mary Mullins, Peter H. Byers, Robert A. Sandhaus, Michaela Durigova, Francis H. Glorieux, Frank Rauch, Vernon Reid Sutton, Brendan Lee, Members Of The Brittle Bone Disorders Consortium, Eric T. Rush, Sandesh C S Nagamani

Manuscripts, Articles, Book Chapters and Other Papers

Pulmonary complications are a significant cause for morbidity and mortality in osteogenesis imperfecta (OI). However, to date, there have been few studies that have systematically evaluated pulmonary function in individuals with OI. We analyzed spirometry measurements, including forced vital capacity (FVC) and forced expiratory volume in the first second (FEV₁ ), in a large cohort of individuals with OI (n = 217) enrolled in a multicenter, observational study. We show that individuals with the more severe form of the disease, OI type III, have significantly reduced FVC and FEV₁ which do not follow the expected trends of the …