Pulmonology Commons^™

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 …

Congenital Abnormalities Of The Lower Airway 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 (CALA). Explain the classification of CALAL. Compare the clinical characteristic, pathology and management of common CALAL. Discuss the long term prognosis of CALAL.

Implementation Of A Guideline-Based Nontuberculous Mycobacteria Management Algorithm, Claire Elson, Ellen Meier, Douglas Swanson, Christopher M. Oermann

Posters

Nontuberculous mycobacterial (NTM) disease is a challenge to manage in patients with cystic fibrosis (CF). Diagnosis of NTM pulmonary disease is complex. Effective treatment requires long term, multi-drug therapy delivered by several routes. Consensus recommendations published in 2016 were developed to guide CF providers in NTM screening, diagnosis and management. Primary Outcomes. Children’s Mercy-Kansas City (CMKC) developed an NTM working group to facilitate implementation of standardized NTM management. Methods. NTM working group: Pulmonologist (CF Center Director), Infectious Diseases specialist, nurse practitioner (CF Center Coordinator), and pharmacist. Sought expertise from the Director of Microbiology Laboratory regarding susceptibility testing. Developed NTM Management …

Sustainability And Outcomes Of A Standardized Aminoglycoside Induced Ototoxicity Monitoring Algorithm, Claire Elson, Christopher M. Oermann, Michelle Weltman, Ellen Meier

Posters

No abstract provided.

Improving Pneumococcal Polysaccharide Vaccination In Children With Cystic Fibrosis, Adam Van Mason, Wendy Estrellado-Cruz, Kristi Williams, Ellen Meier, Elizabeth Elson, Stephanie Duehlmeyer, Paula Capel, Jessica Banks, Christopher M. Oermann

Posters

No abstract provided.

Implementation Of A Multi-Institution, Multistate Cystic Fibrosis Transition Program, Christopher M. Oermann, D Polineni, A Andrews, A Moog, Paula Capel, T Bomstedt, Ellen Meier, J Mermis

Posters

Background: Children’s Mercy-Kansas City (CMKC) is an independent children’s hospital in Kansas City, MO. Its CF Care Center (CFCC) provides care for 250 patients. The University of Kansas Medical Center (KUMC) is an academic medical center in Kansas City, KS. Its CFCC includes adult/pediatric programs (230/50 patients). Interactions between CMKC and KUMC were minimal prior to 2013. CMKC provided care through adulthood despite CF Foundation mandates requiring transition of patients aged 18-21 years. Transition only occurred per patient request. Methods: The CMKC CFCC underwent restructuring and staff expansion in 2013-2014. KUMC underwent staff expansion in 2012, adding a nurse, respiratory …

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 airway 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.

Molecular Evolution And Intraclade Recombination Of Enterovirus D68 During The 2014 Outbreak In The United States., Yi Tan, Ferdaus Hassan, Jennifer E. Schuster, Ari Simenauer, Rangaraj Selvarangan, Rebecca A. Halpin, Xudong Lin, Nadia Fedorova, Timothy B. Stockwell, Tommy Tsan-Yuk Lam, James D. Chappell, Tina V. Hartert, Edward C. Holmes, Suman R. Das

Manuscripts, Articles, Book Chapters and Other Papers

In August 2014, an outbreak of enterovirus D68 (EV-D68) occurred in North America, causing severe respiratory disease in children. Due to a lack of complete genome sequence data, there is only a limited understanding of the molecular evolution and epidemiology of EV-D68 during this outbreak, and it is uncertain whether the differing clinical manifestations of EV-D68 infection are associated with specific viral lineages. We developed a high-throughput complete genome sequencing pipeline for EV-D68 that produced a total of 59 complete genomes from respiratory samples with a 95% success rate, including 57 genomes from Kansas City, MO, collected during the 2014 …

Randomized Trial Of Late Surfactant Treatment In Ventilated Preterm Infants Receiving Inhaled Nitric Oxide., Roberta A. Ballard, Roberta L. Keller, Dennis M. Black, Philip L. Ballard, Jeffrey D. Merrill, Eric C. Eichenwald, William E Truog, Mark C. Mammel, Robin H. Steinhorn, Elizabeth E. Rogers, Rita M. Ryan, David J. Durand, Jeanette M. Asselin, Catherine M. Bendel, Ellen M. Bendel-Stenzel, Sherry E. Courtney, Ramasubbareddy Dhanireddy, Mark L. Hudak, Frances R. Koch, Dennis E. Mayock, Victor J. Mckay, T Michael O'Shea, Nicolas F. Porta, Rajan Wadhawan, Lisa Palermo, Tolsurf Study Group

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: To assess whether late surfactant treatment in extremely low gestational age (GA) newborn infants requiring ventilation at 7-14 days, who often have surfactant deficiency and dysfunction, safely improves survival without bronchopulmonary dysplasia (BPD).

STUDY DESIGN: Extremely low GA newborn infants (GA ≤28 0/7 weeks) who required mechanical ventilation at 7-14 days were enrolled in a randomized, masked controlled trial at 25 US centers. All infants received inhaled nitric oxide and either surfactant (calfactant/Infasurf) or sham instillation every 1-3 days to a maximum of 5 doses while intubated. The primary outcome was survival at 36 weeks postmenstrual age (PMA) without …

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Allergen Immunotherapy For Allergic Asthma: Protocol For A Systematic Review., Sangeeta Dhami, Ulugbek Nurmatov, Ioana Agache, Susanne Lau, Antonella Muraro, Marek Jutel, Graham Roberts, Cezmi Akdis, Matteo Bonini, Moises Calderon, Thomas Casale, Ozlem Cavkaytar, Linda Cox, Pascal Demoly, Breda Flood, Eckard Hamelmann, Kenji Izuhara, Ömer Kalayci, Jörg Kleine-Tebbe, Antonio Nieto, Nikolaos Papadopoulos, Oliver Pfaar, Lanny Rosenwasser, Dermot Ryan, Carsten Schmidt-Weber, Stan Szefler, Ulrich Wahn, Roy-Gerth Van Wijk, Jamie Wilkinson, Aziz Sheikh

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The European Academy of Allergy and Clinical Immunology (EAACI) is in the process of developing the EAACI Guidelines for Allergen Immunotherapy (AIT) for Allergic Asthma. We seek to critically assess the effectiveness, cost-effectiveness and safety of AIT in the management of allergic asthma.

METHODS: We will undertake a systematic review, which will involve searching international biomedical databases for published, in progress and unpublished evidence. Studies will be independently screened against pre-defined eligibility criteria and critically appraised using established instruments. Data will be descriptively and, if possible and appropriate, quantitatively synthesised.

DISCUSSION: The findings from this review will be used …

Exercise-Induced Asthma: Critical Analysis Of The Protective Role Of Montelukast., Terrence W. Carver

Manuscripts, Articles, Book Chapters and Other Papers

Exercise-induced asthma/exercise-induced bronchospasm (EIA/EIB) is a prevalent and clinically important disease affecting young children through older adulthood. These terms are often used interchangeably and the differences are not clearly defined in the literature. The pathogenesis of EIA/EIB may be different in those with persistent asthma compared to those with exercise-induced symptoms only. The natural history of EIA is unclear and may be different for elite athletes. Leukotriene biology has helped the understanding of EIB. The type and intensity of exercise are important factors for EIB. Exercise participation is necessary for proper development and control of EIA is recommended. Symptoms of …