Article
Critical Care Medicine
Christopher H. Goss, Sonya L. Heltshe, Natalie E. West, Michelle Skalland, Don B. Sanders, Raksha Jain, Tara L. Barto, Barbra Fogarty, Bruce C. Marshall, Donald R. VanDevanter, Patrick A. Flume
Summary: This study investigated different durations of intravenous antimicrobial treatment for CF exacerbations, revealing that for patients with early improvement, 10 days is as effective as 14 days, while for those with slower progress, 21 days does not show superiority over 14 days.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(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
Suzanne C. Carter, Alessandro N. Franciosi, Kate M. O'Shea, Orla M. O'Carroll, Ashutosh Sharma, Aoife Bell, Brian Keogan, Paul O'Reilly, Suzie Coughlan, Sheonagh M. Law, Robert D. Gray, Katherine B. Hisert, Pradeep K. Singh, Gordon Cooke, Brenda Grogan, Cillian F. De Gascun, Charles G. Gallagher, Trevor T. Nicholson, Bradley S. Quon, Edward F. McKone
Summary: This study reveals that there are different subphenotypes of CF pulmonary exacerbations, characterized by differences in biomarker profiles, clinical presentation, and outcomes. Viral infection status and C-reactive protein levels can be used to classify these subphenotypes, providing potential guidance for predicting prognosis.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(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)
Review
Critical Care Medicine
Christina M. Mingora, Patrick A. Flume
Summary: Cystic fibrosis is an autosomal recessive genetic condition with significant manifestations in the respiratory system. Despite advancements in treatment, pulmonary complications like pneumothorax and respiratory failure still occur. Palliative care and CF transmembrane conductance regulator therapies play evolving roles in managing the care of CF patients.
Article
Respiratory System
Jonathan D. Cogen, Don B. Sanders, James E. Slaven, Anna V. Faino, Ranjani Somayaji, Ron L. Gibson, Lucas R. Hoffman, Clement L. Ren
Summary: This retrospective study found that changing antibiotics during pulmonary exacerbation treatment in children with cystic fibrosis was common, but not associated with improved clinical outcomes.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2023)
Article
Immunology
Shivanthan Shanthikumar, Sarath C. Ranganathan, Richard Saffery, Melanie R. Neeland
Summary: By using high parameter flow cytometry and inflammatory cytokine profiling, the study investigated the immune response in preschool children with CF and healthy controls, revealing pulmonary infiltration of CD66b(+) granulocytes and elevated levels of inflammatory cytokines in CF children. Moreover, the proportions of systemic neutrophils in CF children were positively correlated with age, while CD4 T cells and B cells showed an inverse association with age. This work emphasizes the importance of multiplex immune profiling and advanced analytical pipelines in understanding lung disease mechanisms in childhood.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Cardiac & Cardiovascular Systems
Frederick W. Woodley, Emrah Gecili, Rhonda D. Szczesniak, Chandra L. Shrestha, Christopher J. Nemastil, Benjamin T. Kopp, Don Hayes
Summary: This study characterized the sweat metabolomes of people with cystic fibrosis (PWCF) before and after intravenous (IV) antibiotics to treat pulmonary exacerbation (PEx). The results showed significant changes in sweat metabolites before and after IV antibiotic treatment for PEx, suggesting the potential of sweat metabolomics as a diagnostic and research tool for PEx in PWCF.
RESPIRATORY MEDICINE
(2022)
Article
Respiratory System
Carolina Silva Barboza de Aquino, Joaquim Carlos Rodrigues, Luiz Vicente Ribeiro Ferreira da Silva-Filho
Summary: This study suggests that routine spirometry can increase the diagnosis and treatment rates of PExs, potentially affecting long-term pulmonary function.
JORNAL BRASILEIRO DE PNEUMOLOGIA
(2022)
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
Critical Care Medicine
Tijana Milinic, Oliver J. McElvaney, Christopher H. Goss
Summary: With improved survival of CF patients and effective CFTR therapy, the clinical spectrum of CF continues to evolve. Acute PEx is a crucial event in the disease course and ongoing studies provide important insights into its pathophysiology, outcome, and treatment. This review aims to summarize the current knowledge on CF PEx and the impact of highly effective modulator therapy.
SEMINARS IN RESPIRATORY AND CRITICAL CARE 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
Respiratory System
Donald R. VanDevanter, Sonya L. Heltshe, Jay B. Hilliard, Michael W. Konstan
Summary: The study investigated the relationships between Pseudomonas aeruginosa (Pa) isolate AST results, antipseudomonal PEx treatments, and treatment responses. The findings suggest that complete Pa coverage did not show superior responses compared to lesser coverage treatments, indicating that AST may have limited utility in selecting antimicrobials for CF PEx treatment.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Review
Pharmacology & Pharmacy
Akshay Parihar, Bhupendra G. Prajapati, Himanshu Paliwal, Maheka Shukla, Dignesh Khunt, Sumedh Devrao Bahadure, Sathish Dyawanapelly, Vijayabhaskarreddy Junnuthula
Summary: Cystic fibrosis (CF) is a fatal genetic disease that causes thick, sticky mucus. It also leads to pancreatic dysfunction, bacterial infection, and excessive salt loss. Current treatments can improve the quality of life for patients. Nanotechnology-assisted drug delivery has been explored to change the pharmacokinetics and toxicity of drugs. This review summarizes different formulations and focuses on advanced formulations delivered through the lungs for CF treatment. There is significant interest in advanced drug delivery formulations to address the challenges of CF. However, translating these formulations for clinical use may still require considerable effort.
DRUG DISCOVERY TODAY
(2023)
Article
Pharmacology & Pharmacy
Amin Rakhshan, Nazanin Farahbakhsh, Ghamartaj Khanbabaee, Seyed Ahmad Tabatabaii, Saeed Sadr, Maryam Hassanzad, Mohammad Sistanizad, Farzaneh Dastan, Mahmoud Hajipour, Amir Reza Bahadori, Bahador Mirrahimi
Summary: This study evaluated the concurrent use of inhaled and intravenous aminoglycoside antibiotics for treating pulmonary exacerbation caused by Pseudomonas aeruginosa in cystic fibrosis patients. The results showed that this treatment method was safe and effective, and it improved patients' forced expiratory volume and treatment outcomes.
FRONTIERS IN PHARMACOLOGY
(2023)
Review
Critical Care Medicine
Scott C. Bell, Marcus A. Mall, Hector Gutierrez, Milan Macek, Susan Madge, Jane C. Davies, Pierre-Regis Burgel, Elizabeth Tullis, Claudio Castaos, Carlo Castellani, Catherine A. Byrnes, Fiona Cathcart, Sanjay H. Chotirmall, Rebecca Cosgriff, Irmgard Eichler, Isabelle Fajac, Christopher H. Goss, Pavel Drevinek, Philip M. Farrell, Anna M. Gravelle, Trudy Havermans, Nicole Mayer-Hamblett, Nataliya Kashirskaya, Eitan Kerem, Joseph L. Mathew, Edward F. McKone, Lutz Naehrlich, Samya Z. Nasr, Gabriela R. Oates, Ciaran O'Neill, Ulrike Pypops, Karen S. Raraigh, Steven M. Rowe, Kevin W. Southern, Sheila Sivam, Anne L. Stephenson, Marco Zampoli, Felix Ratjen
LANCET RESPIRATORY MEDICINE
(2020)
Article
Health Care Sciences & Services
Laura Liliana Obregon, Kwonho Jeong, Zachariah P. Hoydich, Jonathan Yabes, Joseph Pilewski, Connie Richless, Laura T. Moreines, Elisabeth P. Dellon, Christopher H. Goss, Robert M. Arnold, Dio Kavalieratos
Summary: Adults living with cystic fibrosis (CF) have significant unmet supportive care needs, particularly related to anxiety, sadness, pain, and uncertainty about the future. These needs are associated with demographic characteristics, with insufficient income being a significant factor. This highlights the importance of developing treatment approaches that consider patient demographics when addressing unmet supportive care needs in adults with CF.
BMJ SUPPORTIVE & PALLIATIVE CARE
(2022)
Article
Critical Care Medicine
Dave P. Nichols, Katherine Odem-Davis, Jonathan D. Cogen, Christopher H. Goss, Clement L. Ren, Michelle Skalland, Ranjani Somayaji, Sonya L. Heltshe
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2020)
Article
Critical Care Medicine
Kathleen J. Ramos, Siddhartha G. Kapnadak, Miranda C. Bradford, Ranjani Somayaji, Eric D. Morrell, Joseph M. Pilewski, Erika D. Lease, Michael S. Mulligan, Moira L. Aitken, Cynthia J. Gries, Christopher H. Goss
Article
Respiratory System
Susanna Kosamo, Katherine B. Hisert, Victoria Dmyterko, Catherine Nguyen, R. Anthony Black, Tarah D. Holden, Frank Radella, Pauline A. Cotten, Christopher H. Goss, Moira L. Aitken, Mark M. Wurfel
JOURNAL OF CYSTIC FIBROSIS
(2020)
Article
Health Care Sciences & Services
Elisabeth P. Dellon, Melissa Basile, Mara R. Hobler, Anna M. Georgiopoulos, Elaine Chen, Jessica Goggin, Christopher H. Goss, Sarah E. Hempstead, Albert Faro, Dio Kavalieratos
JOURNAL OF PALLIATIVE MEDICINE
(2020)
Article
Respiratory System
Rebecca Cosgriff, Susannah Ahern, Scott C. Bell, Keith Brownlee, Pierre-Regis Burgel, Cass Byrnes, Harriet Corvol, Stephanie Y. Cheng, Alexander Elbert, Albert Faro, Christopher H. Goss, Vincent Gulmans, Bruce C. Marshall, Edward McKone, Peter G. Middleton, Rasa Ruseckaite, Anne L. Stephenson, Siobhan B. Carr
JOURNAL OF CYSTIC FIBROSIS
(2020)
Article
Pediatrics
Elizabeth T. Trandel, Dio Kavalieratos, Melissa Basile, Mara R. Hobler, Anna M. Georgiopoulos, Elaine Chen, Jessica L. Goggin, Christopher H. Goss, Sarah E. Hempstead, Albert Faro, Elisabeth P. Dellon
PEDIATRIC PULMONOLOGY
(2020)
Review
Respiratory System
N. Mayer-Hamblett, S. Van Koningsbruggen-Rietschel, D. P. Nichols, D. R. VanDevanter, J. C. Davies, T. Lee, A. G. Durmowicz, F. Ratjen, M. W. Konstan, K. Pearson, S. C. Bell, J. P. Clancy, J. L. Taylor-Cousar, K. De Boeck, S. H. Donaldson, D. G. Downey, P. A. Flume, P. Drevinek, C. H. Goss, I Fajac, A. S. Magaret, B. S. Quon, S. M. Singleton, J. M. VanDalfsen, G. Z. Retsch-Bogart
JOURNAL OF CYSTIC FIBROSIS
(2020)
Article
Respiratory System
D. R. VanDevanter, S. L. Heltshe, D. B. Sanders, N. E. West, M. Skalland, P. A. Flume, C. H. Goss
Summary: This study evaluates the changes in CRISS during intravenous antimicrobial exacerbation treatments and finds that CRISS reduction is associated with higher baseline CRISS and younger age of subjects. CRISS may serve as an effective endpoint for PEx interventional trials.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Article
Gastroenterology & Hepatology
Molly Bozic, Christopher H. Goss, Rabindra M. Tirouvanziam, Arthur Baines, Margaret Kloster, Liebe Antoine, Drucy Borowitz, Sarah Jane Schwarzenberg
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2020)
Article
Respiratory System
Kelsie Pearson, Nicole Mayer-Hamblett, Christopher H. Goss, George Z. Retsch-Bogart, Jill M. VanDalfsen, Patricia Burks, Daniel Rosenbluth, John Paul Clancy, Amy Hoffman, David P. Nichols
Summary: The global pandemic of SARS-CoV-2 significantly impacted CF clinical research, but over time, research activities gradually recovered and new opportunities for CF research emerged.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Article
Respiratory System
Robert Bain, Rebecca Cosgriff, Marco Zampoli, Alexander Elbert, Pierre-Regis Burgel, Siobhan B. Carr, Claudio Castanos, Carla Colombo, Harriet Corvol, Albert Faro, Christopher H. Goss, Hector Gutierrez, Andreas Jung, Nataliya Kashirskaya, Bruce C. Marshall, Joel Melo, Pedro Mondejar-Lopez, Isabelle de Monestrol, Lutz Naehrlich, Rita Padoan, Maria Dolores Pastor-Vivero, Samar Rizvi, Marco Salvatore, Luiz Vicente Ribeiro Ferreira da Silva Filho, Keith G. Brownlee, Iram J. Haq, Malcolm Brodlie
Summary: Data on 105 children with cystic fibrosis and SARS-CoV-2 infection were collected and analyzed, showing that most cases were managed in the community, with a small percentage requiring hospitalization, and one child unfortunately passed away.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Article
Cardiac & Cardiovascular Systems
Anne L. Stephenson, Kathleen J. Ramos, Jenna Sykes, Xiayi Ma, Sanja Stanojevic, Bradley S. Quon, Bruce C. Marshall, Kristofer Petren, Joshua S. Ostrenga, Aliza K. Fink, Albert Faro, Alexander Elbert, Cecilia Chaparro, Christopher H. Goss
Summary: Discrepancies in post-transplant survival and waitlist mortality between CF patients in the US and Canada can explain up to a third of the observed survival gap, highlighting the potential impact of transplantation on reducing this disparity.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2021)
Article
Immunology
Ranjani Somayaji, Moni B. Neradilek, Adam A. Szpiro, Kathryn H. Lofy, Michael L. Jackson, Christopher H. Goss, Jeffrey S. Duchin, Kathleen M. Neuzil, Justin R. Ortiz
EMERGING INFECTIOUS DISEASES
(2020)
