Review
Critical Care Medicine
Gemma E. Stanford, Kavita Dave, Nicholas J. Simmonds
Summary: Pulmonary exacerbations in cystic fibrosis patients are significant events associated with declining lung function, reduced quality of life, hospitalizations, and decreased survival. With the increasing adult CF population globally, the management of PExs needs to evolve to reflect changing demographics and improve outcomes. Studies are underway to address unmet needs and optimize treatment strategies for PExs in adults with CF.
Letter
Pediatrics
Shreya Patel, Misty D. Thompson, James E. Slaven, Don B. Sanders, Clement L. Ren
Summary: The restrictions on social interaction during the COVID-19 pandemic were associated with a lower number of cystic fibrosis pulmonary exacerbation events at a pediatric CF Center, indicating a reduction in exposure to other respiratory viral infections in children with CF.
PEDIATRIC PULMONOLOGY
(2021)
Article
Medicine, General & Internal
Alejandro A. Diaz, Wei Wang, Jose L. Orejas, Rim Elalami, Wojciech R. Dolliver, Pietro Nardelli, Ruben San Jose Estepar, Bina Choi, Carrie L. Pistenmaa, James C. Ross, Diego J. Maselli, Andrew Yen, Kendra A. Young, Gregory L. Kinney, Michael H. Cho, Raul San Jose Estepar
Summary: This study found that suspected bronchiectasis is associated with an increased risk of mortality in adults with normal and obstructive spirometry. When using computed tomography (CT) to identify bronchiectasis, the difference in mortality risk was attenuated in participants with normal spirometry.
ANNALS OF INTERNAL MEDICINE
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Layla Diab-Caceres, Rosa Maria Giron-Moreno, Elena Garcia-Castillo, Maria Teresa Pastor-Sanz, Casilda Olveira, Marta Maria Garcia-Clemente, Rosa Nieto-Royo, Concha Prados-Sanchez, Paloma Caballero-Sanchez, Maria Jose Olivera-Serrano, Alicia Padilla-Galo, Encarnacion Nava-Tomas, Amparo Esteban-Peris, Maria Fernandez-Velilla, Maribel Torres, Rosa Mar Gomez-Punter, Julio Ancochea
Summary: The study found that the modified Bhalla score can predict future PEx in CF patients and is well correlated with pulmonary function test results. A statistical model based on the overall Bhalla score was constructed to predict the number of PEx.
EUROPEAN RADIOLOGY
(2021)
Article
Immunology
Clemence Martin, Theo Dhote, Maha Zohra Ladjemi, Muriel Andrieu, Souganya Many, Vaarany Karunanithy, Frederic Pene, Jennifer Da Silva, Pierre-Regis Burgel, Veronique Witko-Sarsat
Summary: This study compared different subsets of neutrophils in clinically stable adults with CF and CF adults during pulmonary exacerbations, as well as healthy donors. The results showed an increase in expression of CD11b in neutrophils from CF patients during exacerbations, indicating a circulating activation state due to infection. Additionally, an increase in the CD16(high)/CD62L(low) subset was observed in all CF patients, while the CD16(low)/CD62L(high) subset increased only in CF patients during exacerbations. Unsupervised analysis identified a PD-L1(high)/CD114(high) population present in stable CF patients and during exacerbations.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Frederic Schlemmer, Agnes Hamzaoui, Sonia Zebachi, Aurelie Le Thuaut, Gilles Mangiapan, Isabelle Monnet, Amel Boudjema, Laurence Jabot, Bruno Housset, Sylvie Bastuji-Garin, Laurence Bassinet, Bernard Maitre
Summary: This study aimed to develop a clinical score to identify adult patients with bronchiectasis who are at higher risk of having cystic fibrosis or primary ciliary dyskinesia. Through diagnostic work-ups and logistic-regression analyses, specific factors associated with CF/PCD diagnosis were identified and a score was validated in a second cohort. The results showed that age at symptom onset, chronic ENT symptoms, digestive symptoms, and specific microbial isolates from sputum were significantly associated with CF or PCD diagnosis, with the developed score demonstrating good performance in identifying high-risk patients.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Respiratory System
Kang Dong, Sung Moon Huh, Grace Y. Lam, Jiah Jang, Alessandro N. Franciosi, Pearce G. Wilcox, Bradley S. Quon
Summary: This study identified three distinct systemic inflammatory phenotypes in adults with cystic fibrosis during pulmonary exacerbations. One phenotype, characterized by low levels of inflammatory proteins, showed poorer clinical response to intravenous antibiotic treatment compared to the other two phenotypes.
JOURNAL OF CYSTIC FIBROSIS
(2023)
Review
Pediatrics
Brigitte Fauroux, Karen Waters, Joanna E. MacLean
Summary: Significant progress has been made in the management of respiratory diseases associated with cystic fibrosis (CF). Recent studies have shown that reduced sleep duration and poor sleep quality are common in CF patients, even those with better lung function. These sleep problems may be caused by various factors such as chronic pain, cough, frequent stools, and medication use. They have been associated with negative outcomes such as depression, poor academic performance, and decreased quality of life. Sleep evaluation and interventions to improve sleep quality are urgently needed in CF patients.
PAEDIATRIC RESPIRATORY REVIEWS
(2023)
Article
Critical Care Medicine
Heather D. Green, Andrew M. Jones
Summary: Cystic fibrosis is a disease characterized by chronic airway infection and progressive decline in respiratory function. Recent changes in the landscape of airway infection in CF have resulted in an increased prevalence of atypical gram-negative bacterial infections. The clinical relevance and treatment strategies for many of these lower prevalence organisms are still not well understood.
Review
Respiratory System
Lucy Perrem, Isaac Martin, Felix Ratjen
Summary: Pulmonary exacerbations in children with cystic fibrosis are common and even mild clinical events contribute to the progression of lung disease. Prompt diagnosis and management are necessary to preserve lung function.
EXPERT REVIEW OF RESPIRATORY MEDICINE
(2023)
Article
Pediatrics
Mordechai Pollak, Michelle Shaw, David Wilson, Melinda Solomon, Felix Ratjen, Hartmut Grasemann
Summary: Significant bronchodilator response in CF patients treated for pulmonary exacerbations is rare, shows poor correlation with baseline pulmonary function, and does not correlate with the recovery of FEV1 with treatment. These data suggest that routine testing for bronchodilator response is not indicated during pulmonary exacerbations.
PEDIATRIC PULMONOLOGY
(2021)
Article
Respiratory System
Patrick A. Flume, Joseph Feliciano, Matthew Lucci, Jasmanda Wu, Sebastian Fucile, Mariam Hassan, Anjan Chatterjee
Summary: This study investigated the frequency of exacerbations and all-cause hospitalisation in patients with bronchiectasis. It found that frequent exacerbations increased the likelihood of future exacerbations over a two-year follow-up period, leading to increased hospitalisation rates over time.
Article
Pediatrics
Drake C. Bouzek, Clement L. Ren, Misty Thompson, James E. Slaven, Don B. Sanders
Summary: This study aims to explore the association between FEV1 decline and PEx diagnosis in CF patients, as well as the correlation between respiratory symptoms, FEV1 decline, and antibiotic treatment. The results indicate that FEV1 decline is associated with an increased likelihood of cough and sputum being diagnosed as PEx.
PEDIATRIC PULMONOLOGY
(2022)
Article
Pediatrics
Noel Gonzalez-Rosales, Ajay S. S. Kasi, Courtney E. E. McCracken, George L. L. Silva, Miah Starks, Arlene Stecenko, Lokesh Guglani
Summary: In pediatric patients with cystic fibrosis, one-third of pulmonary exacerbations requiring hospitalization were associated with viral infections, with rhinovirus/enterovirus being the most common. Viral-positive pulmonary exacerbations did not lead to a greater decline or delayed recovery of lung function, increased risk for Pseudomonas aeruginosa acquisition, or higher frequency of subsequent exacerbations.
PEDIATRIC PULMONOLOGY
(2023)
Article
Medicine, General & Internal
Irena Wojsyk-Banaszak, Zuzanna Stachowiak, Barbara Wieckowska, Marta Andrzejewska, Katarzyna Tapolska-Jozwiak, Aleksandra Szczepankiewicz, Paulina Sobkowiak, Anna Breborowicz
Summary: This study compared the predictive values of lung clearance index (LCI) in cystic fibrosis pediatric patients using different versions of software and found that although there were significant differences in LCI values measured with different software versions in stable condition, there was no significant difference during pulmonary exacerbations. Therefore, using recalculated LCI values does not affect the diagnostic accuracy of this parameter in cystic fibrosis pulmonary exacerbations.
Article
Respiratory System
Elizabeth Juarez-Colunga, Margaret Rosenfeld, Edith T. Zemanick, Brandie Wagner
JOURNAL OF CYSTIC FIBROSIS
(2020)
Article
Respiratory System
Don B. Sanders, Joshua S. Ostrenga, Margaret Rosenfeld, Aliza K. Fink, Michael S. Schechter, Gregory S. Sawicki, Patrick A. Flume, Wayne J. Morgan
JOURNAL OF CYSTIC FIBROSIS
(2020)
Review
Critical Care Medicine
Demet Toprak, Chelsea Davis, Margaret Rosenfeld
SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE
(2019)
Article
Pediatrics
Demet Toprak, Laura Nay, Sharon McNamara, Abby R. Rosenberg, Margaret Rosenfeld, Joyce P. Yi-Frazier
PEDIATRIC PULMONOLOGY
(2020)
Article
Dentistry, Oral Surgery & Medicine
Jordan T. Banks, Margaret Rosenfeld, Lloyd Mancl, Donald L. Chi
Summary: The study assessed patient-level agreement for medication use between self-reported survey and electronic health record (EHR) in children with cystic fibrosis (CF), finding considerable heterogeneity in level of agreement between the two methods and highlighting the need for standardized approaches to improve medication data accuracy.
INTERNATIONAL JOURNAL OF PAEDIATRIC DENTISTRY
(2021)
Article
Pediatrics
N. Beydon, L. Gochicoa, M. J. Jones, L. C. Lands, E. Lombardi, M. Rosenfeld, P. D. Sly, D. J. Weiner, O. Yilmaz
PAEDIATRIC RESPIRATORY REVIEWS
(2020)
Article
Pediatrics
Jordana E. Hoppe, Daniel M. Hinds, Adrianne Colborg, Brandie D. Wagner, Wayne J. Morgan, Margaret Rosenfeld, Edith T. Zemanick, Don B. Sanders
PEDIATRIC PULMONOLOGY
(2020)
Article
Pediatrics
Jane B. Taylor, Christopher M. Oermann, Robin R. Deterding, Gregory Redding, Stephanie D. Davis, Joseph Piccione, Paul E. Moore, Oren Kupfer, Maria Teresa Santiago, Margaret Rosenfeld, David G. Ingram, Kristie Ross, Emily M. DeBoer
Summary: The COVID-19 pandemic presents unprecedented challenges for medical practice, but also showcases the collaborative nature of pediatric pulmonology and sleep medicine. Through the use of video conferencing technology and multicenter collaborative efforts, we have laid the groundwork for future challenges.
PEDIATRIC PULMONOLOGY
(2021)
Article
Respiratory System
Margaret Rosenfeld, Anna Faino, Frankline Onchiri, Melis A. Aksit, Scott M. Blackman, Elizabeth E. Blue, Joseph M. Collaco, William W. Gordon, Rhonda G. Pace, Karen S. Raraigh, Yi-Hui Zhou, Garry R. Cutting, Michael R. Knowles, Michael J. Bamshad, Ronald L. Gibson
Summary: Chronic Pseudomonas aeruginosa (Pa) infection is associated with increased morbidity and mortality in people with cystic fibrosis (CF). This study compares different definitions of chronic Pa infection and finds that an annualized definition requiring at least 1 positive culture in 3 of 4 consecutive years produces similar results to established encounter-based criteria. The findings suggest that this annualized definition is valuable for longitudinal analyses in cohorts with limited culture frequency.
JOURNAL OF CYSTIC FIBROSIS
(2022)
Article
Respiratory System
Anna V. Faino, Lucas R. Hoffman, Ronald L. Gibson, Matthew P. Kronman, David P. Nichols, Margaret Rosenfeld, Jonathan D. Cogen
Summary: This study aimed to describe the number of polymicrobial in-hospital treated pulmonary exacerbations (PEx), determine the proportion of polymicrobial PEx that received antibiotics with activity against all bacteria detected, and identify factors associated with complete antibiotic coverage.
JOURNAL OF CYSTIC FIBROSIS
(2023)
Article
Endocrinology & Metabolism
Lina Merjaneh, Demet Toprak, Sharon McNamara, Laura Nay, Erin Sullivan, Margaret Rosenfeld
Summary: The study found that hyperglycaemia is prevalent during paediatric CF exacerbations, and it appears to improve with exacerbation treatment but worsen later in association with decreased insulin secretion.
ENDOCRINOLOGY DIABETES & METABOLISM
(2021)
Article
Respiratory System
Jonathan D. Cogen, Anna Faino, Frankline Onchiri, Lucas R. Hoffman, Matthew P. Kronman, Maria Nelson, David P. Nichols, Margaret Rosenfeld, Donald R. VanDevanter, Ronald L. Gibson
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2020)
Article
Respiratory System
Kevin C. Wilson, David A. Kaminsky, Gaetane Michaud, Sunil Sharma, Linda Nici, Rodney J. Folz, Igor Barjaktarevic, Nirav R. Bhakta, George Cheng, Geoffrey L. Chupp, Adam Cole, Anne E. Dixon, James H. Finigan, Brian Graham, Teal S. Hallstrand, Jeffrey Haynes, John Hankinson, Neil MacIntyre, Jess Mandel, Kevin McCarthy, Meredith McCormack, Susheel P. Patil, Margaret Rosenfeld, Michal Senitko, Sonali Sethi, Erik R. Swenson, Sanja Stanojevic, Mihaela Teodorescu, Daniel J. Weiner, Renda Soylemez Wiener, Charles A. Powell
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2020)
Article
Respiratory System
BreAnna Kinghorn, Sharon McNamara, Alan Genatossio, Erin Sullivan, Molly Siegel, Irma Bauer, Charles Clem, Robin C. Johnson, Miriam Davis, Anne Griffiths, William Wheeler, Katherine Johnson, Stephanie D. Davis, Margaret W. Leigh, Margaret Rosenfeld, Jessica Pittman
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2020)
Review
Medicine, General & Internal
Margaret Rosenfeld, Oli Rayner, Alan R. Smyth
COCHRANE DATABASE OF SYSTEMATIC REVIEWS
(2020)