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Pulmonary Medicine

CFTR Modulation Therapy Improves Markers of Inflammation and Lung Function in Cystic Fibrosis
 CFTR Modulation Therapy Improves Markers of Inflammation and Lung Function in Cystic Fibrosis 1024 575 Jessica Nye, PhD
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Illustration of lungs on blue silhouette of upper chest on black background

Six months of elexacaftor-tezacaftor-ivacaftor (ETI) therapy significantly reduced proinflammatory cytokines, increased immune cell composition, and improved clinical outcomes in cystic fibrosis (CF).   “Cystic fibrosis is a progressive lung disease that carries significant morbidity,” says Shahid Sheikh, MD, of the Pulmonary Medicine and Allergy/Immunology section at Nationwide Children’s Hospital and a professor at The Ohio…

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Long-Term Pulmonary Symptoms Following COVID-19 Infection in Children
 Long-Term Pulmonary Symptoms Following COVID-19 Infection in Children 1024 683 Mary Bates, PhD
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Fatigue and shortness of breath may persist, even in the absence of abnormal test results. Researchers at Nationwide Children’s recently published the largest U.S. study to date of pediatric patients with long-term pulmonary sequelae following COVID-19 infection. The authors note the persistence of symptoms such as shortness of breath and fatigue and highlight at least…

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The Importance of Body Composition in Cystic Fibrosis
 The Importance of Body Composition in Cystic Fibrosis 150 150 Mary Bates, PhD
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Lean mass deficits are common in pediatric patients with cystic fibrosis and associated with impaired lung and bone health. Now, experts are calling for increased use of body composition measurements in clinical care.  In cystic fibrosis — a multisystem disorder characterized by progressive lung disease, pancreatic insufficiency, malabsorption and malnutrition — nutrition status is correlated with…

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After Lung Transplantation for Cystic Fibrosis, Migration of Stem Cells May Lead to Chimeric Phenotype
 After Lung Transplantation for Cystic Fibrosis, Migration of Stem Cells May Lead to Chimeric Phenotype 1024 575 Jessica Nye, PhD
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Illustration of lungs on blue silhouette of upper chest on black background

Lung transplantation (LTx) for cystic fibrosis (CF) may trigger bidirectional, long-distance migration of tissue specific stem cells (TSC), causing a chimeric phenotype which could have implications for host defense capabilities. CF is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene and is characterized by progressive chronic obstructive lung disease which can lead…

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Repeated Injury to Stem Cells Could be a Significant Contributor to Chronic Lung Disease
 Repeated Injury to Stem Cells Could be a Significant Contributor to Chronic Lung Disease 1024 575 Abbie Miller
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Illustration of lungs on blue silhouette of upper chest on black background

A series of experiments shows that biological aging of tracheobronchial stem cells could drive the development of chronic lung disease. In a recent publication in STEM CELLS Translational Medicine (SCTM), researchers showed that repeated injury to epithelial cells in the airway caused biological aging of the stem cells located there. Tracheobronchial stem cells (TSCs) are…

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Featured Researcher — Rodney Britt, PhD
 Featured Researcher — Rodney Britt, PhD 150 150 Natalie Wilson
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Rodney D. Britt Jr., PhD, is a principal investigator in the Center for Perinatal Research at the Abigail Wexner Research Institute at Nationwide Children’s Hospital and an assistant professor of Pediatrics at The Ohio State University. Dr. Britt earned his bachelor’s degree in Chemistry from North Carolina A&T State University and his doctorate degree in…

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Video Game-Based Therapy Makes Cardiopulmonary Rehabilitation Program More Patient-Centric
 Video Game-Based Therapy Makes Cardiopulmonary Rehabilitation Program More Patient-Centric 1024 683 Katie Brind'Amour, PhD, MS, CHES
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Smiling boy outside

Patients can benefit from traditional therapeutic services as well as engaging, updated approaches to improve lung capacity and fitness.  The Cardiopulmonary Rehabilitation Program at Nationwide Children’s Hospital, launched in 2005 after a year-long pilot, has long offered services for patients to improve their ability to engage in exercise, school and activities of daily living. As…

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EVALI and MIS-C: Overlapping Diagnoses
 EVALI and MIS-C: Overlapping Diagnoses 1024 575 Mary Bates, PhD
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Taking a thorough history can differentiate these similarly presenting conditions. In a new report, researchers from Nationwide Children’s Hospital describe six patients admitted to the hospital with concerns for MIS-C who were later diagnosed with EVALI. The researchers discuss the factors that led them to differentiate between the two disorders and arrive at the correct…

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Even Severe Asthma Can Improve if Guidelines Are Followed
 Even Severe Asthma Can Improve if Guidelines Are Followed 1024 683 Mary Bates, PhD
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Patients treated with adequate medications and asthma family education can significantly improve irrespective of asthma severity. Severe asthma accounts for 5-8% of patients with asthma, but this group is more challenging to treat and is responsible for up to 40% of total asthma-care expenses. The majority of patients with severe asthma have difficult-to-treat asthma (in…

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Increasing the Use of the Teach-Back Method in Asthma Education
 Increasing the Use of the Teach-Back Method in Asthma Education 1024 575 JoAnna Pendergrass, DVM
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Lisa Ulrich standing in hospital hallway

A standardized approach to asthma education using the teach-back method helps respiratory therapists effectively address common issues with asthma management in pediatric patients. For the 8% of children and adults in the United States who have asthma, effective asthma education is essential to adequate disease management. Unfortunately, written asthma action plans (AAP) alone may be…

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Polysomnography in Infants: How Much Total Sleep Time is Really Needed?
 Polysomnography in Infants: How Much Total Sleep Time is Really Needed? 1024 575 Abbie Miller
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small baby with nose canula

Study identifies factors affecting total sleep time and suggests shorter sleep times can be effective for accurate interpretation and clinical decision making. In a recent study published in American Journal of Perinatology, experts from Nationwide Children’s Hospital investigated factors that affect total sleep time(TST) during infant polysomnography (PSG) in an effort to determine how much TST is…

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How Have Respiratory Outcomes Changed With New Spinal Muscular Atrophy Treatments?
 How Have Respiratory Outcomes Changed With New Spinal Muscular Atrophy Treatments? 1024 575 Mary Bates, PhD
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Illustration of lungs on blue silhouette of upper chest on black background

Three new therapies are changing the prognosis for spinal muscular atrophy, but long-term monitoring of respiratory outcomes is still necessary.

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Measuring the Progression of CF in School-Aged Children
 Measuring the Progression of CF in School-Aged Children 1024 575 Abbie Miller
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Illustration of lungs on blue silhouette of upper chest on black background

A multicenter study utilized chest computed tomography and lung function tests taken repeatedly over two years in school-aged children with mild cystic fibrosis. Understanding disease progression in chronic illnesses is important to managing patients and improving outcomes. For children with cystic fibrosis, it can be difficult to predict how long it will take for negative…

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Collaborative Study Seeks to Understand Gene Expression Changes During Acute Events in Patients With SCD
 Collaborative Study Seeks to Understand Gene Expression Changes During Acute Events in Patients With SCD 1024 575 Natalie Wilson
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Researchers explore gene responses among SCD patients hospitalized for acute complications to inform understandings of the under-studied disease. Sickle cell disease (SCD) is a chronic, genetic disorder characterized by structural changes in circulating red blood cells. According to the CDC, SCD affects approximately 100,000 individuals in the United States alone and accounts for 3,000 births…

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Exploring the Link Between Cystic Fibrosis and Celiac Disease
 Exploring the Link Between Cystic Fibrosis and Celiac Disease 1024 575 Lauren Dembeck
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Overlapping symptoms can cause delayed diagnosis of celiac disease in patients with cystic fibrosis. Physician scientists at Nationwide Children’s Hospital have recently identified three cases of patients with cystic fibrosis, all of whom had the same genotype, were treated with the CFTR modulator, ivacaftor, and were diagnosed with celiac disease. This adds to a novel body…

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Eosinophilic Pneumonia Brought on by Vaping
 Eosinophilic Pneumonia Brought on by Vaping 1024 680 Mary Bates, PhD
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A case study emphasizes the importance of a detailed history in differentiating between eosinophilic and infectious pneumonia. Eosinophilic pneumonia is a rare and potentially life-threatening condition. It is often seen in previously healthy individuals and can be difficult to distinguish from infectious pneumonia, leading to delays in the appropriate treatment. According to a new case…

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THRIVING After Severe Bronchopulmonary Dysplasia
 THRIVING After Severe Bronchopulmonary Dysplasia 1024 731 Abbie Miller
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Meet Willow. She was born via emergency C-section at just 22 weeks. Doctors at the delivering hospital told Willow’s mom Cortney that her baby’s chances of survival were low. But after a long journey through the newborn intensive care unit (NICU) at Nationwide Children’s Hospital, Willow is a vivacious 4-year-old looking forward to starting kindergarten…

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Lung Transplant Index Enhances Identification of Preventable Harms in Adolescents and Young Adults
 Lung Transplant Index Enhances Identification of Preventable Harms in Adolescents and Young Adults 150 150 Lauren Dembeck
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A recent Quality Improvement (QI) Initiative at Nationwide Children’s Hospital has reduced the percent of missed opportunities to address preventable harms in a population of adolescent and young adult patients who underwent lung transplant at the hospital. The study is published in Pediatric Quality and Safety. “We tried to focus on all issues, all body systems,…

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A Major Milestone in Cystic Fibrosis Treatment
 A Major Milestone in Cystic Fibrosis Treatment 1024 575 Mary Bates, PhD
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Illustration of lungs on blue silhouette of upper chest on black background

A triple medication combination could be life-changing for the majority of patients with cystic fibrosis. A Phase III clinical trial shows that elexacaftor added to ivacaftor and tezacaftor improves lung function and quality of life in cystic fibrosis patients with the most common genetic mutation, F508del. The triple therapy, known as Trikafta, could effectively treat 90%…

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Health Care Leaders Offer Interim Guidelines on Vaping and EVALI Care
 Health Care Leaders Offer Interim Guidelines on Vaping and EVALI Care 150 150 Mary Bates, PhD
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While investigations are ongoing, an interim report offers clinical guidance regarding vaping-associated lung injuries. Centers for Disease Control (CDC), the Food and Drug Administration (FDA), state and local health departments, and public health and clinical partners are looking into a multistate outbreak of lung injury associated with the use of electronic cigarette (e-cigarette), or vaping,…

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Gene Expression Changes With CFTR Modulator Treatment
 Gene Expression Changes With CFTR Modulator Treatment 150 150 Mary Bates, PhD
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Patients with cystic fibrosis show transcriptomic changes after starting treatment with lumacaftor/ivacaftor. In a new study, researchers from Nationwide Children’s analyzed the gene expression profiles of cystic fibrosis patients before and after treatment with the drug lumacaftor/ivacaftor. The findings have implications for the evaluation of existing medications as well as the development of new treatments. Care…

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Long-Term Follow Up of Patients Receiving Novel Gene Therapy for SMA Type I
 Long-Term Follow Up of Patients Receiving Novel Gene Therapy for SMA Type I 150 150 Abbie Miller
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Spinal muscular atrophy type 1 (SMA1) is a rare neuromuscular disease in which 75 percent of affected children die or require permanent ventilation by 13.6 months. Researchers recently published the long-term outcomes of patients who received the investigational drug AVXS-101 – an adeno-associated virus serotype 9 mediated gene replacement therapy. Twelve children aged 1 to…

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Toward a Cell-based Therapy for Cystic Fibrosis Lung Disease
 Toward a Cell-based Therapy for Cystic Fibrosis Lung Disease 150 150 Jeb Phillips
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Because cystic fibrosis (CF) is a genetic disease caused by mutations in the CFTR gene, the pulmonary disease could be reversed if CF airway epithelial cells were replaced with basal cells expressing CFTR without mutations. That is one of the ideas underpinning the Cystic Fibrosis Foundation’s Epithelial Stem Cell Consortium, but there are a number of…

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New Guidelines for Home Oxygen Therapy in Pediatric Patients
 New Guidelines for Home Oxygen Therapy in Pediatric Patients 150 150 Abbie Miller
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Researchers Identify Proteins Triggering Imbalance of Cells in Chronic Lung Disease
 Researchers Identify Proteins Triggering Imbalance of Cells in Chronic Lung Disease 1024 575 Kevin Mayhood
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Illustration of lungs on blue silhouette of upper chest on black background

A protein that triggers an imbalance of mucous and ciliated cells in patients with chronic lung disease could be a target for treatments to restore airways. Many chronic lung diseases in children and adults have one thing in common: the airway lining that normally traps and sweeps out bacteria, viruses and diesel particulates stops functioning…

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Opening the Door to Adult Medicine
 Opening the Door to Adult Medicine 1024 575 Katie Brind'Amour, PhD, MS, CHES
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Care transition plans aren’t just for kids with congenital conditions anymore. Could your practice benefit from a proactive transition plan for all patients? In the shift from pediatric to adult care, young patients have the responsibility to adjust to a new life of self-management. But health care providers also play an important role in this…

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Could Earlier Spirometry Get CF Inpatients Home Sooner?
 Could Earlier Spirometry Get CF Inpatients Home Sooner? 150 150 Katie Brind'Amour, PhD, MS, CHES
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A retrospective chart review suggests early spirometry is associated with shorter length of stay. As multicenter studies examine whether length of hospital stay and duration of treatment can be safely shortened for cystic fibrosis-related pulmonary exacerbations, physician-researchers at Nationwide Children’s Hospital have been exploring another unresolved angle related to these admissions: when to do pulmonary…

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Sweat Chloride Should Be the First-Line CF Test, but Often Isn’t
 Sweat Chloride Should Be the First-Line CF Test, but Often Isn’t 150 150 Jeb Phillips
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A study finds that CFTR mutation analysis is being ordered before (or without) sweat chloride in some cases, potentially leading to missed diagnoses, increased patient anxiety and unnecessary costs. The Cystic Fibrosis Foundation is clear in its diagnostic guidelines: sweat chloride needs to be the first test ordered after a positive newborn screening for cystic…

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Teens With Asthma: A High School Diploma Does Not Equal Readiness for Self-Management
 Teens With Asthma: A High School Diploma Does Not Equal Readiness for Self-Management 150 150 David Stukus, MD
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Poorly controlled asthma takes a toll on numerous aspects of health and life. Asthma is the leading pediatric chronic health condition, affecting approximately 10 percent of children and adolescents. Uncontrolled asthma can lead to acute exacerbations and need for emergency department visits or hospitalizations. A frequently unrecognized toll of poorly controlled asthma is the impact…

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New Drug Advances Cystic Fibrosis Care
 New Drug Advances Cystic Fibrosis Care 150 150 Kevin Mayhood
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Orkambi helps reduce infections and slows loss of lung function in some patients. Matt Hennessey knew he was on the experimental medicine and not a placebo just days after he began participating in a drug trial. He felt better. The addition of Orkambi pills to his daily routine was just another part of his maintenance…

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The End of “Under 12″
 The End of “Under 12″ 150 150 Kelli Whitlock Burton
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UNOS board eliminates age classification for child lung transplant candidates and alters rules for pediatric heart transplants. Many in the pediatric field were surprised last year when a federal judge ordered that a 10-year-old Pennsylvania girl with cystic fibrosis be added to the national adult waiting list for a double-lung transplant. She had spent 18 months…

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