Article
Multidisciplinary Sciences
Christopher S. King, Emily White, Shambhu Aryal, Oksana A. Shlobin, Anju Singhal, Whitney Brown, Christopher Thomas, Vikramjit Khangoora, Alan Nyquist, Kevin R. Flaherty, Steven D. Nathan, Joshua J. Mooney
Summary: This study aimed to determine the proportion of lung transplant listing in patients with idiopathic pulmonary fibrosis (IPF) and the characteristics associated with transplantation. The study found that only a small proportion of IPF patients were listed for lung transplant, and advanced age was the primary factor associated with failure to be listed. Further research is needed to determine the exact reasons for low rates of listing.
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
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.
Article
Medicine, General & Internal
Anna Engell Holm, Hans Henrik Lawaetz Schultz, Helle Krogh Johansen, Tania Pressler, Thomas Kromann Lund, Martin Iversen, Michael Perch
Summary: This study monitored the time of bacterial re-colonization in lung transplant patients with cystic fibrosis and found that 70% of patients were re-colonized by bacteria after 2 years, with no observed impact on survival rates from re-colonization or different bacterial species.
JOURNAL OF CLINICAL MEDICINE
(2021)
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
Medicine, General & Internal
Katarzyna Walicka-Serzysko, Magdalena Postek, Justyna Milczewska, Dorota Sands
Summary: Pulmonary exacerbation (PEx) significantly impacts the quality of life and life expectancy of patients with cystic fibrosis (CF). In our study, we found that LCI increased by 65% and FEV1 decreased by ≥10% in 40% of CF patients during PEx. After PEx treatment, FEV1 increased by 11.05% on average, while LCI decreased by 1.21 units on average, representing a 9.42% decrease compared to the beginning of PEx.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Respiratory System
Christina Thornton, Ranjani Somayaji, Angel Chu, Michael D. Parkins
Summary: Human papillomavirus (HPV) is a major risk factor for cervical cancer, and transplant recipients have a higher risk of HPV complications. A retrospective study on adult female cystic fibrosis (CF) lung transplant recipients found that 35.3% had abnormal pap smear results, including refractory anogenital warts, vulvectomy, and cervical cancer. The study also revealed that lung transplant recipients had a higher likelihood of cervical dysplasia compared to the control group, emphasizing the importance of HPV vaccination to prevent future morbidity and mortality.
Article
Medicine, General & Internal
Katarzyna Walicka-Serzysko, Magdalena Postek, Urszula Borawska-Kowalczyk, Justyna Milczewska, Dorota Sands
Summary: This prospective study aimed to assess the course of early lung disease in children with cystic fibrosis (CF) using multiple breath nitrogen washout (MBNW), impulse oscillometry (IOS), and conventional techniques. The results showed that MBNW and IOS are more helpful tools than conventional techniques in evaluating early lung disease in CF. The lung clearance index (LCI) is a more useful parameter than forced expiratory volume in 1 second (FEV1) for detecting functional abnormalities in school-age children.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Francesca Pennati, Irene Borzani, Laura Moroni, Maria Chiara Russo, Nadia Faelli, Andrea Aliverti, Carla Colombo
Summary: This study suggests that nonenhanced multivolume MRI may serve as a feasible tool for regionally mapping early pulmonary alterations in CF lung disease, and is correlated with spirometric measures.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2021)
Article
Cardiac & Cardiovascular Systems
Angela Koutsokera, Jenna Sykes, M. Math, Olga Theou, Kenneth Rockwood, Carolin Steinack, Marie-France Derkenne, Christian Benden, Thorsten Krueger, Cecilia Chaparro, John-David Aubert, Paola Soccal Gasche, Christophe Von Garnier, Elizabeth Tullis, Anne L. Stephenson, Lianne G. Singer
Summary: This study developed a CF-specific frailty index and found that it was significantly associated with wait-list and post-LT outcomes in CF patients listed for lung transplantation.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2022)
Article
Respiratory System
Maryam Hassanzad, Arda Kiani, Atefeh Abedini, Hoseinali Ghaffaripour, Habib Emami, Niloufar Alizadeh, Ghazal Zoghi, Saeed Hashemi, Ali Akbar Velayati
Summary: Lung ultrasound (LUS) shows better diagnostic performance in evaluating cystic fibrosis (CF) pulmonary exacerbation, especially in detecting air bronchogram and consolidation. LUS exhibits high specificity for diagnosing pleural effusion and atelectasis.
BMC PULMONARY MEDICINE
(2021)
Article
Respiratory System
Dario L. Frey, Sebastien Boutin, Susanne A. Dittrich, Simon Y. Graeber, Mirjam Stahl, Sabine Wege, Felix J. F. Herth, Olaf Sommerburg, Carsten Schultz, Marcus A. Mall, Alexander H. Dalpke
Summary: This study found that the diversity of sputum microbiota is negatively correlated with airway inflammation markers and positively correlated with FEV1% predicted in CF patients. CF patients could be classified into 7 clusters based on microbiota structure, with the most diverse cluster showing lower inflammatory markers and higher FEV1% predicted compared to other clusters.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Article
Cardiac & Cardiovascular Systems
Pali Shah, Erin Lowery, Cecilia Chaparro, Gary Visner, Sarah E. Hempstead, James Abraham, Zubin Bhakta, Maggie Carroll, Lillian Christon, Lara Danziger-Isakov, Joshua M. Diamond, Erika Lease, Jessica Leonard, Marina Litvin, Ray Poole, Fanny Vlahos, Chelsey Werchan, Michelle A. Murray, Erin Tallarico, Albert Faro, Joseph M. Pilewski, Ramsey R. Hachem
Summary: Cystic Fibrosis Lung Transplant Recipients have good long-term outcomes but require specialized care. This document provides recommendations for the management of perioperative and underlying comorbidities of CFLTRs.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2021)
Article
Critical Care Medicine
Kathleen J. Ramos, Jenna Sykes, Sanja Stanojevic, Xiayi Ma, Joshua S. Ostrenga, Aliza Fink, Bradley S. Quon, Bruce C. Marshall, Albert Faro, Kristofer Petren, Alexander Elbert, Christopher H. Goss, Anne L. Stephenson
Summary: The study revealed that among patients with FEV1 < 40% predicted, there are lower rates of lung transplant and an increased risk of death without lung transplant for US patients compared with Canadian patients. This difference is more significant among US patients with Medicaid/Medicare health insurance and nonwhite patients, suggesting potential disparities in access to lung transplants among vulnerable populations.
Review
Health Care Sciences & Services
Guillermo Garcia-Perez-de-Sevilla, Thomas Yvert, Angela Blanco, Alicia Irene Sosa Pedreschi, Israel J. Thuissard, Margarita Perez-Ruiz
Summary: Physical exercise interventions in patients with cystic fibrosis have positive effects on muscle strength, cardiovascular capacity, and respiratory muscle function. However, they do not improve lung function significantly. Strength training and high-intensity interval training have been shown to be the most effective types of exercise for cystic fibrosis patients.
Article
Respiratory System
Kaivon Assani, Chandra L. Shrestha, Hannah Rinehardt, Shuzhong Zhang, Frank Robledo-Avila, Jack Wellmerling, Santiago Partida-Sanchez, Estelle Cormet-Boyaka, Susan D. Reynolds, Larry S. Schlesinger, Benjamin T. Kopp
JOURNAL OF CYSTIC FIBROSIS
(2019)
Article
Respiratory System
B. T. Kopp, E. Joseloff, D. Goetz, B. Ingram, S. L. Heltshe, D. H. Leung, B. W. Ramsey, K. McCoy, D. Borowitz
JOURNAL OF CYSTIC FIBROSIS
(2019)
Article
Pediatrics
Danielle Goetz, Benjamin T. Kopp, Ann Salvator, Melissa Moore-Clingenpeel, Karen McCoy, Daniel H. Leung, Margaret Kloster, Bonnie R. Ramsey, Sonya H. Heltshe, Drucy Borowitz
PEDIATRIC PULMONOLOGY
(2019)
Correction
Multidisciplinary Sciences
Kaivon Assani, Mia F. Tazi, Amal O. Amer, Benjamin T. Kopp
Article
Respiratory System
Benjamin T. Kopp, James Fitch, Lisa Jaramillo, Chandra L. Shrestha, Frank Robledo-Avila, Shuzhong Zhang, Sabrina Palacios, Fred Woodley, Don Hayes, Santiago Partida-Sanchez, Octavio Ramilo, Peter White, Asuncion Mejias
JOURNAL OF CYSTIC FIBROSIS
(2020)
Article
Respiratory System
Benjamin L. Wisniewski, Chandra L. Shrestha, Shuzhong Zhang, Rohan Thompson, Myron Gross, Judith A. Groner, Karan Uppal, Octavio Ramilo, Asuncion Mejias, Benjamin T. Kopp
JOURNAL OF CYSTIC FIBROSIS
(2020)
Article
Pediatrics
Brett R. Loman, Chandra L. Shrestha, Rohan Thompson, Judith A. Groner, Asuncion Mejias, Kathryn L. Ruoff, George A. O'Toole, Michael T. Bailey, Benjamin T. Kopp
PEDIATRIC PULMONOLOGY
(2020)
Letter
Hematology
Brett R. Loman, Chandra L. Shrestha, Kavitha Kotha, Abena Minta, Shuzhong Zhang, Asuncion Mejias, Michael T. Bailey, Susan Creary, Benjamin T. Kopp
AMERICAN JOURNAL OF HEMATOLOGY
(2020)
Review
Biochemistry & Molecular Biology
Devi Jaganathan, Emanuela M. Bruscia, Benjamin T. Kopp
Summary: Cystic fibrosis is a disease caused by mutations in the CFTR gene, resulting in chronic inflammation and lung function decline. Macrophages play a crucial role in CF and the mutant CFTR affects phagocytosis, which is linked to disease progression. A better understanding of the interaction between CFTR and macrophages during phagosome formation could contribute to the development of new therapeutic strategies.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Pediatrics
Sabrina Palacios, Katelyn Krivchenia, Mariah Eisner, Bailey Young, Octavio Ramilo, Asuncion Mejias, Simon Lee, Benjamin T. Kopp
Summary: This study is the largest to date on long-term pulmonary sequelae in pediatric patients after COVID-19. Identified clinical phenotypes and risk factors require further study and treatment.
PEDIATRIC PULMONOLOGY
(2022)
Letter
Pediatrics
Eric S. Mull, Sarah Cohen, Ashish George, Katelyn Krivchenia, Stephen Druhan, Peter B. Baker, Benjamin Kopp
PEDIATRIC PULMONOLOGY
(2023)
Article
Hematology
Rachel N. Zeno, Joseph Stanek, Courtney Pugh, Michelle Gillespie, Benjamin T. Kopp, Susan Creary
Summary: An interdisciplinary clinic providing pulmonary care for people with sickle cell disease (pwSCD) was created in 2014 to address health care access barriers. The study found that pwSCD had significantly fewer hospitalizations for acute chest syndrome, asthma, and vaso-occlusive episodes in the 2 years after their initial SCD-pulmonary clinic visit compared with the 2 years before. This suggests that implementing a multidisciplinary SCD-pulmonary clinic can improve patient management and usage of acute care.
Letter
Respiratory System
Theresa A. Laguna, Benjamin T. Kopp, D'Ann Brown-Janowiak, Nancy Guerrero, Liliana Rose, Brandie Wagner, Paul E. Moore
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2023)
Article
Respiratory System
Devika R. Rao, Benjamin Kopp, Rory John Kamerman-Kretzmer, Folashade Afolabi, Deborah R. Liptzin, Vivek Balasubramaniam, S. Christy Sadreameli
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2021)
Article
Respiratory System
A. Parker Ruhl, S. Christy Sadreameli, Julian L. Allen, Debra P. Bennett, Andrew D. Campbell, Thomas D. Coates, Dapa A. Diallo, Joshua J. Field, Elizabeth K. Fiorino, Mark T. Gladwin, Jeffrey A. Glassberg, Victor R. Gordeuk, Leroy M. Graham, Anne Greenough, Jo Howard, Gregory J. Kato, Jennifer Knight-Madden, Benjamin T. Kopp, Anastassios C. Koumbourlis, Sophie M. Lanzkron, Robert Liem, Roberto F. Machado, Alem Mehari, Claudia R. Morris, Folasade O. Ogunlesi, Carol L. Rosen, Kim Smith-Whitley, Danna Tauber, Nancy Terry, Swee Lay Thein, Elliott Vichinsky, Nargues A. Weir, Robyn T. Cohen, Elizabeth S. Klings
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2019)
Article
Cardiac & Cardiovascular Systems
Soh Hosoba, Toshiaki Ito, Makoto Mori, Riku Kato, Koh Kajiyama, Shogo Maeda, Yuji Nakai, Yoshihiro Morishita
Summary: This study describes the approach and perioperative outcomes of totally endoscopic isolated and concomitant surgical aortic valve replacement (AVR) using various valve types. The results demonstrate that endoscopic AVR can safely address concomitant valve diseases.
ANNALS OF THORACIC SURGERY
(2024)