Article
Medicine, General & Internal
Rita Padoan, Serena Quattrucci, Annalisa Amato, Vincenzo Carnovale, Donatello Salvatore, Marco Salvatore, Giuseppe Campagna
Summary: This study examined a subgroup of Italian CF patients diagnosed late, with a median age of 36.2 years at diagnosis. Among male patients, 45.9% were diagnosed due to infertility, and there was a low prevalence of pancreatic insufficiency and Pseudomonas aeruginosa infection in late diagnosis patients, suggesting a mild CF phenotype in the majority of cases.
Article
Biochemistry & Molecular Biology
Francesco Amati, Andrea Gramegna, Martina Contarini, Anna Stainer, Cristina Curcio, Stefano Aliberti, Angelo Guido Corsico, Francesco Blasi
Summary: This study investigated the prevalence of AAT deficiency in CF adults and found that AAT deficiency is not common among adults with CF.
Article
Respiratory System
Wang Chun Kwok, James Chung Man Ho, Terence Chi Chun Tam, Mary Sau Man Ip, David Chi Leung Lam
Summary: The study revealed that patients with longer duration of non-cystic fibrosis bronchiectasis and those on proton pump inhibitors (PPIs) are more likely to have Pseudomonas aeruginosa colonization, leading to more extensive lung involvement and higher risks of exacerbation requiring hospitalization.
RESPIRATORY RESEARCH
(2021)
Article
Medicine, General & Internal
Layla Diab Caceres, Ester Zamarron de Lucas
Summary: Cystic fibrosis is a genetic multisystemic disease with respiratory involvement as the main comorbidity in adulthood. The advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulators has changed the treatment paradigm by improving defective CFTR protein production and expression, thus enhancing ion exchange and reducing airflow obstruction.
Article
Medicine, General & Internal
Seung Jun Lee, Jong Hwan Jeong, Manbong Heo, Sunmi Ju, Jung-Wan Yoo, Yi Yeong Jeong, Jong Deog Lee
Summary: In patients with non-CF bronchiectasis, serum fibrinogen level is significantly associated with the severity and exacerbation of the disease, while adiponectin and angiopoietin-2 are not. High fibrinogen levels and Pseudomonas colonization are independent risk factors for future exacerbation.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Microbiology
Chun-Yu Lin, Hung-Yu Huang, Meng-Heng Hsieh, Yueh-Fu Fang, Yu-Lun Lo, Shu-Min Lin, Yu-Tung Huang, Chih-Hsin Yeh, Chun-Hua Wang, Horng-Chyuan Lin
Summary: This study investigated the impact of nontuberculous mycobacteria (NTM) in non-cystic fibrosis bronchiectasis in Taiwan. The results showed that NTM were associated with frequent exacerbations and mortality in non-cystic fibrosis bronchiectasis patients, especially when coexisting with Pseudomonas aeruginosa and fungus. Additionally, the presence of single or multiple NTM isolates was an independent risk factor for mortality.
FRONTIERS IN MICROBIOLOGY
(2022)
Article
Medicine, General & Internal
Chun-Yu Lin, Meng-heng Hsieh, Yueh-Fu Fang, Chien-Wei Peng, Jia-Shiuan Ju, Yu-Lun Lo, Shu-Min Lin, Horng-Chyuan Lin
Summary: The BSI, FACED score, and DSP showed similar predictive power for mortality in non-CF bronchiectasis patients. However, DSP did not correlate with acute exacerbations and emergency department visits.
ANNALS OF MEDICINE
(2021)
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)
Review
Medicine, General & Internal
Salony Verma, Joseph L. Mathew, Pallab Ray
Summary: Cystic fibrosis (CF) bronchiectasis and non-CF bronchiectasis may have differences in microbial communities and antibiotic susceptibility patterns, studying these differences could help improve the management of both conditions.
SYSTEMATIC REVIEWS
(2021)
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.
Article
Genetics & Heredity
Yuelin Shen, Xiaolei Tang, Qionghua Chen, Hui Xu, Hui Liu, Jinrong Liu, Haiming Yang, Huimin Li, Shunying Zhao
Summary: This study characterizes the genotypic features of cystic fibrosis (CF) in Chinese children, revealing significant differences from the Caucasian population. The research also provides the first report on the geographical distribution of the most frequent CF variants among Chinese patients.
JOURNAL OF MEDICAL GENETICS
(2023)
Article
Respiratory System
Wen-Wen Wang, Bei Mao, Yang Liu, Shu-Yi Gu, Hai-Wen Lu, Jiu-Wu Bai, Shuo Liang, Jia-Wei Yang, Jian-Xiong Li, Xiao Su, Hai-Yang Hu, Chen Wang, Jin-Fu Xu
Summary: This study utilized a multi-omics approach to identify differences in gut microbiota and metabolic profiles between bronchiectasis patients and healthy volunteers. It revealed the potential of gut microbiota as biomarkers for bronchiectasis.
RESPIRATORY RESEARCH
(2022)
Article
Pediatrics
Kang Zhu, Yingkang Jin
Summary: SLC26A4 gene mutations are closely related to Pendred syndrome and bronchiectasis. The mutations disrupt the ion transport of chloride, iodide, and bicarbonate, leading to the acidification of airway surface fluid, compromised airway defense, and increased thickness of the fluid. This study describes the first case of co-occurrence of Pendred syndrome and non-cystic fibrosis bronchiectasis, highlighting the importance of considering the possibility of bronchiectasis in patients with SLC26A4 gene mutations.
FRONTIERS IN PEDIATRICS
(2023)
Review
Critical Care Medicine
Melanie Chin, Amanda L. Brennan, Scott C. Bell
Summary: Improved treatments for CF-related lung disease have led to increased longevity for patients, but also increased prevalence of extrapulmonary complications. Recognition of mild CF phenotypes has also changed the landscape of the disease.
Review
Nutrition & Dietetics
Nirajan Shrestha, Alexandra McCarron, Nathan Rout-Pitt, Martin Donnelley, David W. Parsons, Deanne H. Hryciw
Summary: Adequate intake of essential fatty acids (EFA) is critical in cystic fibrosis (CF) patients. EFA deficiency is closely associated with the risk of pulmonary infection, a significant pathology in CF. Although some progress has been made in understanding the dysfunctional metabolic pathways in CF, further research is needed, particularly in investigating EFA metabolism in the liver. Adequate EFA concentrations are important for normal membrane structure and function, and investigating the effects of genotype and sex on fatty acid metabolism may support personalized nutritional therapies for CF management.
Editorial Material
Critical Care Medicine
Don B. Sanders, Dave P. Nichols
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2019)
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
Biochemistry & Molecular Biology
Brian J. Day, Preston E. Bratcher, Joshua D. Chandler, Matthew B. Kilgore, Elysia Min, John J. LiPuma, Robert J. Hondal, David P. Nichols
FREE RADICAL BIOLOGY AND MEDICINE
(2020)
Article
Respiratory System
Alex H. Gifford, Nicole Mayer-Hamblett, Kelsie Pearson, David P. Nichols
JOURNAL OF CYSTIC FIBROSIS
(2020)
Editorial Material
Critical Care Medicine
Dave P. Nichols, Richard B. Moss
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2020)
Review
Pharmacology & Pharmacy
Ranjani Somayaji, Dave P. Nichols, Scott C. Bell
EXPERT OPINION ON INVESTIGATIONAL DRUGS
(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
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)
Review
Respiratory System
Dave P. Nichols, Scott H. Donaldson, Carla A. Frederick, Steven D. Freedman, Daniel Gelfond, Lucas R. Hoffman, Andrea Kelly, Michael R. Narkewicz, Jessica E. Pittman, Felix Ratjen, Scott D. Sagel, Margaret Rosenfeld, Sarah Jane Schwarzenberg, Pradeep K. Singh, George M. Solomon, Michael S. Stalvey, Shannon Kirby, Jill M. VanDalfsen, John P. Clancy, Steven M. Rowe
Summary: Highly effective CFTR modulator drug therapy is increasingly available for cystic fibrosis patients, with multiple observational research studies underway to better understand its impacts. The PROMISE study focuses on the broad impacts of starting elexacaftor/tezacaftor/ivacaftor in the US population, aiming to address important health outcomes, clinical care priorities, and research needs.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Article
Respiratory System
Eden J. VanDevanter, Sonya L. Heltshe, Michelle Skalland, Noah Lechtzin, Dave Nichols, Christopher H. Goss
Summary: The study findings suggest that in this cohort, PO antimicrobial treatment of CF PEx were less effective than IVs at improving ppFEV1 during treatment.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Article
Respiratory System
Amalia S. Magaret, Jack Salerno, Jason F. Deen, Margaret Kloster, Nicole Mayer-Hamblett, Bonnie W. Ramsey, Dave P. Nichols
Summary: The study showed that chronic Azithromycin use in children with CF was not associated with an increased risk of QT prolongation.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Article
Respiratory System
Nicole Mayer-Hamblett, David P. Nichols, Katherine Odem-Davis, Kristin A. Riekert, Greg S. Sawicki, Scott H. Donaldson, Felix Ratjen, Michael W. Konstan, Noah Simon, Daniel B. Rosenbluth, George Retsch-Bogart, John P. Clancy, Jill M. VanDalfsen, Rachael Buckingham, Alex H. Gifford
Summary: The study aims to evaluate whether the daily treatment burden can be reduced for individuals with cystic fibrosis receiving new triple-combination therapy, by comparing the effects of discontinuing versus continuing two commonly used chronic therapies.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2021)
Article
Respiratory System
Jonathan D. Cogen, Anna Faino, Frankline Onchiri, Ronald L. Gibson, Lucas R. Hoffman, Matthew P. Kronman, Margaret Rosenfeld, David P. Nichols
Summary: This study aimed to determine whether concomitant use of azithromycin (AZM) with intravenous tobramycin for pulmonary exacerbation (PEx) treatment is associated with worse clinical outcomes. The results suggested that concomitant use of AZM and intravenous tobramycin for in-hospital PEx treatment was associated with poorer clinical outcomes than treatment with intravenous tobramycin without AZM.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(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)
Letter
Respiratory System
Jason S. Debley, Kaitlyn A. Barrow, Lucille M. Rich, Pradeep Singh, Edward F. McKone, David P. Nichols
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2020)