Article
Respiratory System
Qianting Lv, Leticia Gallardo-Estrella, Eleni-Rosalina Andrinopoulou, Yuxin Chen, Jean-Paul Charbonnier, Rikke Mulvad Sandvik, Daan Caudri, Kim Gjerum Nielsen, Marleen de Bruijne, Pierluigi Ciet, Harm Tiddens
Summary: An artificial intelligence algorithm was validated to assess dimensions of bronchus-artery pairs on chest CT scans from patients with CF. The automatic BA analysis could detect and monitor progression of bronchial wall thickening and bronchial widening in patients with CF.
Article
Pediatrics
Courtney Gushue, Mariah Eisner, Shasha Bai, Terri Johnson, Melissa Holtzlander, Karen McCoy, Shahid Sheikh
Summary: This study aimed to determine the impact of Elexacaftor-Tezacaftor-Ivacaftor (ETI) on clinical parameters and structural lung disease in people with cystic fibrosis (pwCF). The results showed that ETI therapy significantly improved ppFEV1 and BMI within 3 months and maintained the improvement throughout 1 year. Additionally, after 1 year of ETI therapy, there was a significant reduction in the presence of Pseudomonas aeruginosa and MRSA in pwCF's sputum. The chest CT scans did not worsen during 1 year of ETI therapy compared to baseline.
PEDIATRIC PULMONOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Valentina Fainardi, Kaltra Skenderaj, Andrea Ciuni, Gianluca Milanese, Michela Deolmi, Francesco Longo, Cinzia Spaggiari, Nicola Sverzellati, Susanna Esposito, Giovanna Pisi
Summary: The study evaluated the clinical and morphological response to Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) treatment in cystic fibrosis (CF) patients, including bronchiectasis, bronchial wall thickening, mucus plugging, abscess, and consolidations. The results showed significant improvements in respiratory function, quality of life, and lung structure after treatment with ELX/TEZ/IVA.
RESPIRATORY MEDICINE
(2023)
Article
Multidisciplinary Sciences
Maria Pino-Argumedo, Anthony J. Fischer, Brieanna M. Hilkin, Nicholas D. Gansemer, Patrick D. Allen, Eric A. Hoffman, David A. Stoltz, Michael J. Welsh, Mahmoud H. Abou Alaiwa
Summary: Defective mucociliary transport in cystic fibrosis (CF) lungs is caused by abnormally elastic mucus strands that impair clearance. Breaking these mucus strands can improve mucociliary clearance in CF.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Pediatrics
Angus McCashney, Phil Robinson
Summary: This study found that children with CF who also have ABPA exhibit more severe structural lung disease compared to CF patients without ABPA. Furthermore, those with ABPA showed a faster rate of progression in lung disease compared to control CF patients, with no significant correlation found between lung function and the degree of structural lung disease.
PEDIATRIC PULMONOLOGY
(2021)
Review
Pharmacology & Pharmacy
Alessandro Rimessi, Veronica A. M. Vitto, Simone Patergnani, Paolo Pinton
Summary: Cystic fibrosis is a genetic disorder characterized by airway issues and lung damage. Calcium signaling plays a crucial role in the pathophysiology of CF lung disease, with dysregulated calcium signals leading to impaired cell function and exacerbated inflammation.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Obstetrics & Gynecology
Alena J. Haines, Peter G. Middleton
Summary: This chapter provides expert guidance on the interactions between pregnancy and different lung diseases, covering both physiological changes and specific diseases. Due to limited randomized control trials, the literature mainly consists of observational studies and case series. Other recently published guidelines may also be helpful for readers.
BEST PRACTICE & RESEARCH CLINICAL OBSTETRICS & GYNAECOLOGY
(2022)
Article
Multidisciplinary Sciences
Wenjie Yu, Thomas O. Moninger, Andrew L. Thurman, Yuliang Xie, Akansha Jain, Keyan Zarei, Linda S. Powers, Alejandro A. Pezzulo, David A. Stoltz, Michael J. Welsh
Summary: Submucosal glands (SMGs) play a crucial role in protecting the lungs but can also contribute to disease. In cystic fibrosis (CF), SMGs produce abnormal mucus that disrupts mucociliary transport. This study used single-cell RNA sequencing, immunohistochemistry, and in situ hybridization to investigate the expression and distribution of ion transporters and cell types in SMGs. The results suggest that loss of epithelial anion secretion, rather than intrinsic cell defects, causes CF mucus abnormalities. The study also revealed different functions of acinar mucous cells and acinar serous cells in producing mucins and antimicrobials, respectively.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
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
Eva Steinke, Olaf Sommerburg, Simon Y. Graeber, Cornelia Joachim, Christiane Labitzke, Gyde Nissen, Isabell Ricklefs, Isa Rudolf, Matthias V. Kopp, Anna-Maria Dittrich, Marcus A. Mall, Mirjam Stahl
Summary: This study is a prospective, longitudinal cohort study aimed at determining influencing factors of early cystic fibrosis (CF) lung disease through the combined analysis of clinical information and biomaterials. The primary endpoints are the lung clearance index and magnetic resonance imaging scores, while secondary endpoints include pulmonary exacerbations, infection with pro-inflammatory pathogens, and anthropometric data.
FRONTIERS IN MEDICINE
(2023)
Article
Cell Biology
Jeremy Charriot, Mathilde Volpato, Aurelie Petit, Isabelle Vachier, Arnaud Bourdin
Summary: Obstructive lung diseases share common pathophysiological features related to mucus production and regulation. Understanding the composition, behavior, and regulatory mechanisms of mucus in patients is crucial for therapeutic interventions. This article provides a comprehensive overview of sample collection, analytical techniques, and potential integration with treatment options.
Article
Cell Biology
Rosy Ghanem, Philippe Roquefort, Sophie Ramel, Veronique Laurent, Tanguy Haute, Tony Le Gall, Thierry Aubry, Tristan Montier
Summary: The mucus in CF patients' airways is a yield stress fluid with unique rheological properties. Rheological analyses can provide biophysical markers for disease management. Treatment with rhDNAse appears to lower the apparent yield stress of CF sputum, which could be a predictive biomarker for prognosis and aid in the development of new mucolytic agents.
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
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
Radiology, Nuclear Medicine & Medical Imaging
Mathieu David, Ilyes Benlala, Stephanie Bui, Thomas Benkert, Patrick Berger, Francois Laurent, Julie Macey, Gael Dournes
Summary: The study found that ETI has a significant impact on lung structural changes in CF patients, particularly in the reversibility of bronchiectasis. Furthermore, UTE-MRI is a reproducible tool for longitudinal follow-up of CF patients, allowing quantification of response to ETI and demonstrating the reversibility of certain structural alterations in a portion of the study population.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2023)
Article
Pediatrics
Stefanie Theuring, Mascha Kern, Franziska Hommes, Marcus A. A. Mall, Joachim Seybold, Frank P. P. Mockenhaupt, Toivo Glatz, Tobias Kurth
Summary: During the COVID-19 pandemic, children and adolescents worldwide have been disproportionately affected in their psychological health and wellbeing. A cohort study in Germany found that anxiety symptoms among school children decreased from June to August 2021, but increased again in September 2021. Factors such as gender, school type, household education and income level, and COVID-19 vaccination were significantly associated with reporting anxiety symptoms.
CHILD AND ADOLESCENT PSYCHIATRY AND MENTAL HEALTH
(2023)
Article
Pediatrics
Oliver Weinheimer, Philip Konietzke, Willi L. Wagner, Dorothea Weber, Beverly Newman, Craig J. Galban, Hans-Ulrich Kauczor, Marcus A. Mall, Terry E. Robinson, Mark O. Wielpuetz
Summary: This study aimed to quantify airway dimensions and air trapping on chest CT scans of school-age children with mild CF-lung disease over two years, and found that there were regional interdependencies between wall thickening and air trapping, as well as short-term regional interdependencies between air trapping and an increase in bronchiectasis.
FRONTIERS IN PEDIATRICS
(2023)
Article
Respiratory System
Patricia Leutz-Schmidt, Daiva-Elzbieta Optazaite, Olaf Sommerburg, Monika Eichinger, Sabine Wege, Eva Steinke, Simon Y. Graeber, Michael U. Puderbach, Jens-Peter Schenk, Abdulsattar Alrajab, Simon M. F. Triphan, Hans-Ulrich Kauczor, Mirjam Stahl, Marcus A. Mall, Mark O. Wielputz
Summary: This study evaluated the occurrence of bronchial artery dilatation (BAD) in patients with cystic fibrosis (CF) and its association with disease severity using magnetic resonance imaging (MRI). The results showed that the onset of BAD was associated with disease severity, lung function, and chronic Pseudomonas aeruginosa infection. This finding is important for assessing the severity of CF.
Article
Critical Care Medicine
Jennifer L. Goralski, Jordana E. Hoppe, Marcus A. Mall, Susanna A. McColley, Edward McKone, Bonnie Ramsey, Jonathan H. Rayment, Phil Robinson, Florian Stehling, Jennifer L. Taylor-Cousar, Elizabeth Tullis, Neil Ahluwalia, Anna Chin, Chenghao Chu, Mengdi Lu, Tao Niu, Tanya Weinstock, Felix Ratjen, Margaret Rosenfeld
Summary: This study evaluated the safety, pharmacokinetics, pharmacodynamics, and efficacy of Elexacaftor/tezacaftor/ivacaftor in children aged 2-5 years. The results showed that the treatment was generally safe and well-tolerated in this age group and led to reductions in sweat chloride concentration and lung clearance index.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Critical Care Medicine
Nicole Mayer-Hamblett, John Paul Clancy, Raksha Jain, Scott H. Donaldson, Isabelle Fajac, Christopher H. Goss, Deepika Polineni, Felix Ratjen, Bradley S. Quon, Edith Zemanick, Scott C. Bell, Jane C. Davies, Manu Jain, Michael W. Konstan, Natanya R. Kerper, Tre LaRosa, Marcus A. Mall, Edward McKone, Kelsie Pearson, Joseph M. Pilewski, Lynne Quittell, Jonathan H. Rayment, Steven M. Rowe, Jennifer L. Taylor-Cousar, George Retsch-Bogart, Damian Downey
Summary: The use of modulator therapies for cystic fibrosis has changed clinical trial strategies, dividing the patient population based on eligibility. The development of nucleic acid-based therapies faces challenges due to limited resources and the inability to conduct placebo-controlled trials. Advancing symptomatic therapies is also difficult in a population already receiving modulator treatment.
LANCET RESPIRATORY MEDICINE
(2023)
Article
Cell Biology
Sanja Blaskovic, Pinelopi Anagnostopoulou, Elena Borisova, Dominik Schittny, Yves Donati, David Haberthur, Zhe Zhou-Suckow, Marcus A. Mall, Christian M. Schleputz, Marco Stampanoni, Constance Barazzone-Argiroffo, Johannes C. Schittny
Summary: This study developed a new pipeline for analyzing all airspaces of the entire lung. The results demonstrated irregular widening of parenchymal airspaces in mice with cystic fibrosis-like lung disease and elastase-induced emphysema. The pipeline showed applicability to different imaging modalities and is suitable for studying structural alterations in chronic obstructive lung diseases.
Article
Radiology, Nuclear Medicine & Medical Imaging
Sabine Weckbach, Mark O. Wielpuetz, Oyunbileg von Stackelberg
Summary: With the increased use of high-resolution radiological imaging, the prevalence of incidental findings (IFs) has been on the rise. While IFs can lead to early treatment of potential health-threatening diseases, many of them have low risk and little impact on health. However, their discovery often triggers additional investigations, and the handling of IFs and the associated ethical challenges are subjects of regular discussions. The well-being of the patient and society should be the focus when managing IFs, and it is important for radiologists and clinicians to be familiar with guidelines and be available for discussions and advice.
Article
Respiratory System
Lena Wucherpfennig, Felix Wuennemann, Monika Eichinger, Niclas Schmitt, Angelika Seitz, Ingo Baumann, Mirjam Stahl, Simon Y. Graeber, Jaehi Chung, Jens-Peter Schenk, Abdulsattar Alrajab, Hans-Ulrich Kauczor, Marcus A. Mall, Olaf Sommerburg, Mark O. Wielputz
Summary: This study longitudinally evaluated chronic rhinosinusitis (CRS) in children with cystic fibrosis (CF) using paranasal sinus magnetic resonance imaging (MRI). The results showed that CRS starts early in infancy and progresses in severity until school age. The study also found that lumacaftor/ivacaftor therapy can improve CRS in children with CF.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2023)
Article
Respiratory System
Cori L. Daines, Elizabeth Tullis, Stefano Costa, Rachel W. Linnemann, Marcus A. Mall, Edward F. McKone, Deepika Polineni, Bradley S. Quon, Felix C. Ringshausen, Steven M. Rowe, Hiran Selvadurai, Jennifer L. Taylor-Cousar, Nicholas J. Withers, Neil Ahluwalia, Samuel M. Moskowitz, Valentin Prieto-Centurion, Yaoyuan Vincent Tan, Simon Tian, Tanya Weinstock, Fengjuan Xuan, Yaohua Zhang, Bonnie Ramsey, Matthias Griese
Summary: This study assessed the long-term safety and efficacy of ELX/TEZ/IVA in patients with cystic fibrosis (CF) who have at least one F508del allele. The results showed that ELX/TEZ/IVA treatment was generally safe and well tolerated, with sustained improvements in lung function, respiratory symptoms, CF transmembrane conductance regulator function, pulmonary exacerbation rates, and nutritional status. These findings support the favorable safety profile and durable clinical benefits of ELX/TEZ/IVA.
EUROPEAN RESPIRATORY JOURNAL
(2023)
Article
Respiratory System
Lena Wucherpfennig, Simon M. F. Triphan, Sabine Wege, Hans-Ulrich Kauczor, Claus P. Heussel, Olaf Sommerburg, Mirjam Stahl, Marcus A. Mall, Monika Eichinger, Mark O. Wielpuetz
Summary: This study found that bronchial artery dilatation (BAD) may be partially reversible under elexacaftor/tezacaftor/ivacaftor therapy in adult patients with cystic fibrosis (CF) who have established disease.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Alan A. Peters, Justin B. Solomon, Oyunbileg von Stackelberg, Ehsan Samei, Njood Alsaihati, Waldo Valenzuela, Manuel Debic, Christian Heidt, Adrian T. Huber, Andreas Christe, Johannes T. Heverhagen, Hans-Ulrich Kauczor, Claus P. Heussel, Lukas Ebner, Mark O. Wielpuetz
Summary: This study aimed to determine the influence of dose reduction on a commercially available lung cancer prediction convolutional neuronal network (LCP-CNN). The results showed that CT dose reduction may affect the classification of pulmonary malignancies and potentially alter pulmonary nodule management.
EUROPEAN RADIOLOGY
(2023)
Article
Pediatrics
Lena Maria Roewer, Karl Ludger Radke, Janina Hussmann, Halima Malik, Monika Eichinger, Dirk Voit, Mark Oliver Wielpuetz, Jens Frahm, Dirk Klee, Frank Pillekamp
Summary: In pediatric patients with CHD and chronic RV volume overload, real-time cardiac MRI during free breathing reveals increased RV end-diastolic volume and stroke volume during inspiration, with a lower increase in ejection fraction and a reduced Frank-Starling relationship of the RV compared to controls.
PEDIATRIC RADIOLOGY
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Qian Li, Lin Zhu, Oyunbileg von Stackelberg, Simon M. F. Triphan, Juergen Biederer, Oliver Weinheimer, Monika Eichinger, Claus F. Vogelmeier, Rudolf A. Joerres, Hans -Ulrich Kauczor, Claus P. Heussel, Bertram J. Jobst, Mark O. Wielpuetz
Summary: This study investigated the use of morphofunctional chest MRI for the detection and management of incidental pulmonary nodules in patients with chronic obstructive pulmonary disease (COPD). The results showed that the sensitivity of this technique in detecting pulmonary nodules was moderate, but it had high agreement with LDCT in terms of Lung-RADS classification.
RADIOLOGY-CARDIOTHORACIC IMAGING
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Alan A. Peters, Oliver Weinheimer, Oyunbileg von Stackelberg, Jonas Kroschke, Lars Piskorski, Manuel Debic, Kai Schlamp, Linn Welzel, Moritz Pohl, Andreas Christe, Lukas Ebner, Hans-Ulrich Kauczor, Claus Peter Heussel, Mark O. Wielpuetz
Summary: Quantitative computed tomography (QCT) of the whole lung and nodule-bearing lobe can be valuable in assessing the malignancy risk of pulmonary nodules. The study shows that nodules in subjects with higher emphysema levels and less fibrosis in the lungs are more likely to be malignant.
EUROPEAN RADIOLOGY
(2023)