Article
Medicine, Research & Experimental
John T. Benjamin, Erin J. Plosa, Jennifer M. S. Sucre, Riet van der Meer, Shivangi Dave, Sergey Gutor, David S. Nichols, Peter M. Gulleman, Christopher S. Jetter, Wei Han, Matthew Xin, Peter C. Dinella, Ashley Catanzarite, Seunghyi Kook, Kalsang Dolma, Charitharth V. Lal, Amit Gaggar, J. Edwin Blalock, Dawn C. Newcomb, Bradley W. Richmond, Jonathan A. Kropski, Lisa R. Young, Susan H. Guttentag, Timothy S. Blackwell
Summary: Emerging evidence suggests that early life events may increase the risk for developing chronic obstructive pulmonary disease (COPD), with neutrophilic inflammation potentially contributing to early-life predisposition to COPD. Mechanistic studies reveal a critical role for NE in the expression of elastic fiber components.
JOURNAL OF CLINICAL INVESTIGATION
(2021)
Article
Physiology
Rebecca J. Calthorpe, Caroline Poulter, Alan R. Smyth, Don Sharkey, Jayesh Bhatt, Gisli Jenkins, Amanda L. Tatler
Summary: As survival rates for extremely preterm infants improve, there is an increase in bronchopulmonary dysplasia (BPD), one of the most significant complications of preterm birth. BPD development is multifactorial, resulting from exposure to various antenatal and postnatal stressors. BPD has short-term and long-term implications, including respiratory, cardiovascular, and neurological issues. The review focuses on the role of transforming growth factor I3 (TGF-I3) in lung development, the impact of known risk factors on the TGF-I3 signaling pathway in BPD, and the effect of current and potential medications on TGF-I3 signaling for BPD prevention and treatment.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
(2023)
Article
Pediatrics
Csaba Galambos, J. Wells Logan, Pawel Stankiewicz, Przemyslaw Szafranski, Carola Zalles, Jose Gonzales, Sfurti Nath, Shalinkumar Patel, Steven H. Abman
Summary: We present the case of a preterm infant with severe bronchopulmonary dysplasia (sBPD) who exhibited histologic features consistent with alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) but lacked genetic confirmation. We also found that sBPD is associated with decreased levels of lung FOXF1 and TMEM100, suggesting potential shared mechanistic links between ACDMPV and sBPD involving impaired FOXF1 signaling.
PEDIATRIC PULMONOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Min Liu, Cristiana Iosef, Shailaja Rao, Racquel Domingo-Gonzalez, Sha Fu, Paige Snider, Simon J. Conway, Gray S. Umbach, Sarah C. Heilshorn, Ruby E. Dewi, Mar J. Dahl, Donald M. Null, Kurt H. Albertine, Cristina M. Alvira
Summary: The study identified TGFBI as a developmentally regulated protein that promotes NF-kappa B-mediated angiogenesis during early alveolarization by enhancing nitric oxide production. Dysregulation of TGFBI expression may contribute to diseases marked by impaired alveolar and vascular growth.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
(2021)
Article
Immunology
Tracy X. Cui, Alexander E. Brady, Ying-Jian Zhang, Chase Anderson, Antonia P. Popova
Summary: Activated IL-17a+ γδ T cells and NKG2D signaling play a central role in neonatal LPS-induced lung injury.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Cell Biology
Giacomo Roessler, Jonas Labode, Yannick Regin, Thomas Salaets, Andre Gie, Jaan Toelen, Christian Muehlfeld
Summary: Bronchopulmonary dysplasia (BPD) is a developmental disorder of infants born prematurely, characterized by disrupted alveolarization and microvascular maturation. The study used a rabbit model to evaluate alveolar and vascular development under preterm birth and hyperoxia. Preterm birth had a strong effect on alveolar development, while hyperoxia had a more pronounced effect on capillary development. The findings suggest that the vascular hypothesis of BPD may be more influenced by ambient oxygen concentration than premature birth.
JOURNAL OF HISTOCHEMISTRY & CYTOCHEMISTRY
(2023)
Review
Obstetrics & Gynecology
Bernard Thebaud
Summary: Lung diseases are a major cause of mortality and morbidity in neonates. Cell-based therapies, such as mesenchymal stromal cells and amnion epithelial cells, have shown potential for preventing and repairing lung injury in preterm infants. This review summarizes the current evidence and challenges in the clinical translation of cell-based therapies for lung diseases in neonates.
SEMINARS IN PERINATOLOGY
(2023)
Article
Pediatrics
Yoshie Fukasawa, Hidenori Yamamoto, Miharu Ito, Akiko Saito, Kiyotaka Go, Yoshihito Morimoto, Kazushi Yasuda, Yoshiaki Sato, Masahiro Hayakawa, Taichi Kato
Summary: Administration of specific pulmonary vasodilators can improve the long-term prognosis of pediatric PH patients with developmental lung disease, and high-dose epoprostenol therapy may be one of the therapeutic options.
FRONTIERS IN PEDIATRICS
(2023)
Article
Biochemistry & Molecular Biology
Laura G. Sherlock, William C. McCarthy, Maya R. Grayck, Mack Solar, Andres Hernandez, Lijun Zheng, Cassidy Delaney, Trent E. Tipple, Clyde J. Wright, Eva S. Nozik
Summary: Exposure to selenium deficiency during pregnancy and lactation can impair weight gain and lung growth in offspring. Selenium deficiency reduces the levels and activity of multiple selenoproteins in the neonatal lung, affecting oxidative stress and cell signaling.
Article
Critical Care Medicine
Alia Dani, Don Hayes, Amalia Guzman-Gomez, Md Monir Hossain, Jason C. Woods, David L. S. Morales, Russel Hirsch, Farhan Zafar, Erik B. Hysinger
Summary: Patients with bronchopulmonary dysplasia (BPD) may benefit from lung transplant (LTx) as their post-LTx outcomes are similar to those with other diagnoses.
Article
Critical Care Medicine
Arun Pradhan, Lixiao Che, Vladimir Ustiyan, Abid A. Reza, Nicole M. Pek, Yufang Zhang, Andrea B. Alber, Timothy R. Kalin, Jennifer A. Wambach, Mingxia Gu, Darrell N. Kotton, Matthew E. Siefert, Assem G. Ziady, Tanya Kalin, Vladimir V. Kalinichenko
Summary: This study identified a small molecule compound called TanFe that can activate FOXF1 signaling, which is associated with Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV). TanFe was found to stabilize the FOXF1 protein and showed potential therapeutic effects in mouse models and human vascular organoids. This discovery provides a new treatment candidate for ACDMPV and other neonatal pulmonary vascular diseases.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Pediatrics
Mehmet Aslan, Ismail Kursat Gokce, Hatice Turgut, Suat Tekin, Asli Cetin Taslidere, Mehmet Fatih Deveci, Huseyin Kaya, Kevser Tanbek, Cemile Ceren Gul, Ramazan Ozdemir
Summary: This study evaluated the effectiveness of Molsidomine in treating hyperoxic lung injury. The results showed that Molsidomine significantly reduced oxidative stress markers, restored antioxidant enzyme activities, and decreased inflammatory cytokine levels. It may provide a new and promising therapy for bronchopulmonary dysplasia in the future.
PEDIATRIC RESEARCH
(2023)
Article
Physiology
Shailaja Rao, Min Liu, Cristiana Iosef, Carsten Knutsen, Cristina M. Alvira
Summary: This study demonstrates the crucial role of the IKKb signaling pathway in endothelial cells for alveolarization and vascular development in the lung. Loss of IKKb leads to disorganized lung vasculature and decreased alveolar counts. Understanding this pathway may provide new targets for enhancing beneficial angiogenic signaling in lung development and disease.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
(2023)
Article
Multidisciplinary Sciences
Paris Clarice Papagianis, Siavash Ahmadi-Noorbakhsh, Rebecca Lim, Euan Wallace, Graeme Polglase, J. Jane Pillow, Timothy J. Moss
Summary: The study showed that postnatal administration of a single dose of human amnion epithelial cells (hAECs) stimulated a pulmonary immune response in preterm lambs born after intrauterine inflammation, reducing lung tissue injury without affecting ventilator requirements.
Article
Critical Care Medicine
Minzhe Guo, Kathryn A. Wikenheiser-Brokamp, Joseph A. Kitzmiller, Cheng Jiang, Guolun Wang, Allen Wang, Sebastian Preissl, Xiaomeng Hou, Justin Buchanan, Justyna A. Karolak, Yifei Miao, David B. Frank, William J. Zacharias, Xin Sun, Yan Xu, Mingxia Gu, Pawel Stankiewicz, Vladimir V. Kalinichenko, Jennifer A. Wambach, Jeffrey A. Whitsett
Summary: This study reveals the cellular and molecular mechanisms underlying the pathogenesis of Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV), a lethal disorder of lung development. The deficiency of FOXF1 transcription factor disrupts lung formation, leading to changes in cell types, gene expression, and cell-cell interactions in ACDMPV lungs. These findings provide potential targets for the development of therapies for ACDMPV and other diffuse lung diseases of infancy.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Physiology
Michal Mastalerz, Elisabeth Dick, Ashesh Chakraborty, Elisabeth Hennen, Andrea C. Schamberger, Andreas Schroppel, Michael Lindner, Rudolf Hatz, Jurgen Behr, Anne Hilgendorff, Otmar Schmid, Claudia A. Staab-Weijnitz
Summary: This study investigated the effects of different cigarette smoke extract (CSE) exposure protocols on human bronchial epithelial cells and compared them with whole cigarette smoke (wCS) exposure. The results showed that different exposure protocols had differing effects on gene expression, with chronic CSE exposure, acute submerged CSE exposure, and wCS exposure of differentiated phBECs resulting in significant upregulation of smoke-induced genes. These findings provide guidance for the design of in vitro cigarette smoke exposure models in lung research.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
(2022)
Article
Pediatrics
Alida S. D. Kindt, Kai M. Foerster, Suzan C. M. Cochius-den Otter, Andreas W. Flemmer, Stefanie M. Hauck, Andrew Flatley, Juliette Kamphuis, Stefan Karrasch, Juergen Behr, Axel Franz, Christoph Haertel, Jan Krumsiek, Dick Tibboel, Anne Hilgendorff
Summary: This study aims to improve the current risk stratification for BPD and validate the effectiveness of plasma protein markers. The research identified three biomarkers, BCAM, SIGLEC-14, and ANGPTL-3, that can predict the need for mechanical ventilation, oxygen supplementation, and length of neonatal intensive care stay in premature infants at risk for BPD.
PEDIATRIC RESEARCH
(2023)
Article
Respiratory System
Motaharehsadat Heydarian, Prajakta Oak, Xin Zhang, Nona Kamgari, Alida Kindt, Markus Koschlig, Tina Pritzke, Erika Gonzalez-Rodriguez, Kai Foerster, Rory E. Morty, Friederike Haefner, Christoph Huebener, Andreas W. Flemmer, Ali Oender Yildirim, Deepti Sudheendra, Xuefei Tian, Agnese Petrera, Holger Kirsten, Peter Ahnert, Nick Morrell, Tushar J. Desai, Jennifer Sucre, Edda Spiekerkoetter, Anne Hilgendorff
Summary: We identified impaired BMP signaling as a hallmark of early vascular disease in the injured neonatal lung and outlined its promising potential as a future biomarker or therapeutic target.
Article
Biochemistry & Molecular Biology
Alina Bauer, Elisabeth Pachl, Johannes C. Hellmuth, Nikolaus Kneidinger, Motaharehsadat Heydarian, Marion Frankenberger, Hans C. Stubbe, Bernhard Ryffel, Agnese Petrera, Stefanie M. Hauck, Juerge Behr, Rainer Kaiser, Clemens Scherer, Li Deng, Daniel Teupser, Narges Ahmidi, Maximilian Muenchhoff, Benjamin Schubert, Anne Hilgendorff
Summary: This study conducted quantitative proteomic analysis on COVID-19 patients, including both severe and mild cases, and found significant differences in the protein profiles among different disease stages. The study also identified specific phenomena and protein regulations associated with severe disease, providing novel information for biomarker and therapy development.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2023)
Article
Immunology
Emilia J. Berthold, Yue Ma-Lauer, Ashesh Chakraborty, Brigitte von Brunn, Anne Hilgendorff, Rudolf Hatz, Juergen Behr, Felix Hausch, Claudia A. Staab-Weijnitz, Albrecht von Brunn
Summary: Research shows that immunophilin inhibitors CsA and ALV have strong antiviral properties against coronaviruses, while FK506 has cell-type specific effects.
FRONTIERS IN CELLULAR AND INFECTION MICROBIOLOGY
(2022)
Article
Physiology
Kai Foerster, Hanna Marchi, Sophia Stoecklen, Olaf Dietrich, Harald Ehrhardt, Mark O. Welipultz, Andreas W. Flemmer, Benjamin Schubert, A. Marcus Mall, Birgit Ertl-Wagner, Anne Hillgendroff
Summary: This study developed a novel scoring system using MRI to assess lung structural changes in neonatal chronic lung disease. It showed high reproducibility and disease specificity, and has the potential to be used as a quantifiable endpoint in clinical trials and disease monitoring.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
(2023)
Article
Microbiology
Misako Nakayama, Hannah Marchi, Anna M. Dmitrieva, Ashesh Chakraborty, Juliane Merl-Pham, Elisabeth Hennen, Ronan Le Gleut, Clemens Ruppert, Andreas Guenther, Kathrin Kahnert, Juergen Behr, Anne Hilgendorff, Stefanie M. Hauck, Heiko Adler, Claudia A. Staab-Weijnitz
Summary: This study compared the susceptibility and response of airway epithelium in patients with chronic obstructive pulmonary disease (COPD) and healthy individuals to influenza virus infection. Quantitative proteomics was used to assess protein changes during influenza virus infection. The results showed no significant differences between COPD and control airway epithelial cells in terms of cell type composition, susceptibility to influenza virus infection, and proteomes pre- and post-infection. Additionally, several novel influenza A virus-regulated proteins were identified in bronchial epithelial cells, which might serve as potential targets for modulating the pathogenicity of infection and acute exacerbations.
FRONTIERS IN MICROBIOLOGY
(2023)
Article
Immunology
Miriam T. Kastlmeier, Erika Gonzalez-Rodriguez, Phoebe Cabanis, Eva M. Guenther, Ann-Christine Koenig, Lianyong Han, Stefanie M. Hauck, Fenja See, Sara Asgharpour, Christina Bukas, Gerald Burgstaller, Marie Piraud, Mareike Lehmann, Rudolf A. Hatz, Juergen Behr, Tobias Stoeger, Anne Hilgendorff, Carola Voss
Summary: Interstitial lung disease (ILD) is a diverse group of lung disorders characterized by persistent architectural distortion of the lung parenchyma. In this study, a 3D organoid co-culture model was developed to investigate the mesenchymal-epithelial crosstalk in ILD. The results showed that ILD fibroblasts increased metabolic activity and altered stem cell function in alveolar organoids.
FRONTIERS IN IMMUNOLOGY
(2023)
Correction
Immunology
Anita C. Windhorst, Motaharehsadat Heydarian, Maren Schwarz, Prajakta Oak, Kai Foerster, Marion Frankenberger, Erika Gonzalez Rodriguez, Xin Zhang, Harald Ehrhardt, Christoph Huebener, Andreas W. Flemmer, Hamid Hossain, Tobias Stoeger, Christian Schulz, Anne Hilgendorff
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Pediatrics
Motaharehsadat Heydarian, Christian Schulz, Tobias Stoeger, Anne Hilgendorff
Summary: Exposure to prenatal and early postnatal risk factors in the neonatal lung leads to the development of bronchopulmonary dysplasia (BPD), a chronic disease. This review focuses on the relationship between immaturity and prenatal conditions, as well as postnatal exposure to mechanical ventilation and oxygen toxicity, and the imbalance of pro- and anti-inflammatory regulatory networks. It discusses the role of myeloid innate immune cells, such as neutrophils and monocyte/macrophages, in the pathologic processes contributing to lung injury in BPD patients and animal models. The differences between the infant and adult immune system are also discussed as a basis for novel therapeutic strategies.
MOLECULAR AND CELLULAR PEDIATRICS
(2022)
Meeting Abstract
Respiratory System
A. Chakraborty, M. Mastalerz, R. Hatz, J. Behr, M. Lindner, A. Hilgendorff, C. A. Staab-Weijnitz
EUROPEAN RESPIRATORY JOURNAL
(2022)
Meeting Abstract
Respiratory System
Y. Chen, J. Schniering, P. Albanese, M. Ansari, C. H. Mayr, P. Dorfmuller, M. Stoleriu, R. Hatz, C. P. Schneider, J. Behr, A. Hilgendorff, R. Scheltema, H. B. Schiller
EUROPEAN RESPIRATORY JOURNAL
(2022)
Meeting Abstract
Respiratory System
M. Gerckens, K. Schorpp, F. Pelizza, M. Woegrath, K. Reichau, H. Ma, A. Dworsky, A. Sengupta, M. G. Stoleriu, K. Heinzelmann, J. Merl-Pham, M. Irmler, H. N. Alsafadi, E. Trenkenschuh, L. Sarnova, M. Jirouskova, W. Friess, S. M. Hauck, J. Beckers, N. Kneidinger, J. Behr, A. Hilgendorff, K. Hadian, M. Lindner, M. Koenigshoff, O. Eickelberg, M. Gregor, O. Plettenburg, A. O. Yildirim, G. Burgstaller
EUROPEAN RESPIRATORY JOURNAL
(2022)
Article
Cardiac & Cardiovascular Systems
Raphael W. Majeed, Martin R. Wilkins, Luke Howard, Paul M. Hassoun, Anastasia Anthi, Hector R. Cajigas, John Cannon, Stephen Y. Chan, Victoria Damonte, Jean Elwing, Kai Foerster, Robert Frantz, Stefano Ghio, Imad Al Ghouleh, Anne Hilgendorff, Arun Jose, Ernesto Juaneda, David G. Kiely, Allan Lawrie, Stylianos E. Orfanos, Antonella Pepe, Joanna Pepke-Zaba, Yuriy Sirenko, Andrew J. Swett, Olena Torbas, Roham T. Zamanian, Kurt Marquardt, Achim Michel-Backofen, Tobiah Antoine, Jochen Wilhelm, Stephanie Barwick, Phillipp Krieb, Meike Fuenderich, Patrick Fischer, Henning Gall, Hossein-Ardeschir Ghofrani, Friedrich Grimminger, Khodr Tello, Manuel J. Richter, Werner Seeger
Summary: The Pulmonary Vascular Research Institute GoDeep meta-registry is a collaboration of pulmonary hypertension reference centers worldwide, aiming to provide in-depth epidemiological and etiological understanding of PH and its various groups/subgroups on a global scale, offering insights for improved management.
PULMONARY CIRCULATION
(2022)
Meeting Abstract
Critical Care Medicine
M. Melo Narvaez, M. Stoleriu, A. Hilgendorff, M. Koenigshoff, M. Lehmann
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)