Pulmonary Vascular Pathology

Synchrotron-Based Phase-Contrast Micro-CT Combined With Histology to Decipher Differences Between Hereditary and Sporadic Pediatric Pulmonary Veno-Occlusive Disease

Ida Jeremiasen, Niccolò Peruzzi, Elna Lampei, Sofie Meyer, Levent M. Akyürek, Erik Gebre‐Medhin, Ceren Mutgan, Peter Dorfmüller, Lavinia Neubert, Danny Jonigk, Csaba Galambos, Karin Tran‐LundmarkDepartment of Experimental Medical Science and Wallenberg Center for Molecular Medicine Lund University. Skåne University Hospital. Sahlgrenska Academy Hospital. Ludwig Boltzmann Institute for Lung Vascular Research. Universities of Giessen and Marburg […]

Synchrotron-Based Phase-Contrast Micro-CT Combined With Histology to Decipher Differences Between Hereditary and Sporadic Pediatric Pulmonary Veno-Occlusive Disease Read More »

Pulmonary venous infarction

Warren A. Williamson, Bruce S. Tronic, Nathan Levitan, David G. Webb-Johnson, David M. Shahian, F. Henry Ellis, Jr.Lahey Clinic Medical Center.United States ChestChest 1992; 102: 937-940DOI: 10.1378/chest.102.3.937 AbstractPulmonary venous infarction, although rare, can develop in patients with the various pathologic conditions outlined. The triad of cough, dyspnea, and hemoptysis should raise clinical suspicion. The venous phase

Pulmonary venous infarction Read More »

SOX17 – Associated Pulmonary Hypertension in Children: A Distinct Developmental and Clinical Syndrome

Mary P. Mullen, D. Dunbar Ivy, Nidhy P. Varghese, Abbey J. Winant, Nahir Cortes-Santiago, Sara O. Vargas, Diego Porres, Nicola Maschietto, Paul J. Critser, Russel Hirsch, Catherine M. Avitabile, Rachel K. Hopper, Benkamin S. Frank, Ryan D. Coleman, Pankaj B. Agrawal, Jill A. Madden, Amy E. Roberts, Shane L. Collins, J. Usha Raj, Eric D.

SOX17 – Associated Pulmonary Hypertension in Children: A Distinct Developmental and Clinical Syndrome Read More »

A bovine model of hypoxia-induced pulmonary hypertension reveals a gradient of immune and matrisome response with a complement signature found in circulation

Jason Williams, Franklyn N. Iheagwam, Sean P. Maroney, Lauren R. Schmitt, R. Dale Brown, Greta M. Krafsur, Maria G. Frid, Maxwell C. McCabe, Aneta Gandjeva, Kurt J. Williams, James P. Luyendyk, . Anthony J. Saviola, Rubin M. Tuder, Kurt Stenmark, Kirk C. HansenUniversity of Colorado Denver and University of Colorado Anschutz Medical Campus. Michigan State

A bovine model of hypoxia-induced pulmonary hypertension reveals a gradient of immune and matrisome response with a complement signature found in circulation Read More »

Arterial-Lymphatic-Like Endothelial Cells Appear in Hereditary Hemorrhagic Telangiectasia 2 and Contribute to Vascular Leakage and Arteriovenous Malformations

Yang Yang, Xiuju Wu, Yan Zhao, Daoqin Zhang, Li Zhang, Xinjiang Cai, Jaden Ji, Zheng Jing, Kristina I. Boström, Yucheng YaoDavid Geffen School of Medicine and University of California. Stanford University.United States CirculationCirculation 2024; DOI: 10.1161/CIRCULATIONAHA.124.070925 AbstractBackground: Arteriovenous malformations (AVMs) are characteristic of hereditary hemorrhagic telangiectasia. Loss-of-function mutations in the activin receptor-like kinase 1 (Alk1) are linked

Arterial-Lymphatic-Like Endothelial Cells Appear in Hereditary Hemorrhagic Telangiectasia 2 and Contribute to Vascular Leakage and Arteriovenous Malformations Read More »

ASXL1-related Bohring-Optiz Syndrome complicated by Persistent Neonatal Pulmonary Hypertension and Abnormal Alveoli Formation

Makoto Arioka, Shinji Nakamura, Katsufumi Nishioka, Kota Inoue, Yasuhiro Nakao, Yumi Miyai, Hirosuke Morita, Kosuke Koyano, Toshiki Takenouchi, Saneyuki Yasuda, Yoichi Chiba, Takashi Iwase, Masaki Ueno, Takashi KusakaKagawa University Hospital and Kagawa University. Keio University School of Medicine.Japan European Journal of Medical GeneticsEur J Med Genet 2024; DOI: 10.1016/j.ejmg.2024.104978 AbstractBohring-Opitz syndrome (BOS) is a rare disease

ASXL1-related Bohring-Optiz Syndrome complicated by Persistent Neonatal Pulmonary Hypertension and Abnormal Alveoli Formation Read More »

Nintedanib preserves lung growth and prevents pulmonary hypertension in a hyperoxia-induced lung injury model

Kathy L. Ding, Caroline Smith, Gregory Seedorf, Steven H. AbmanUniversity of Colorado School of Medicine.United States Pediatric ResearchPediatr Res 2024; DOI: 10.1038/s41390-024-03562-0 AbstractBackground: Bronchopulmonary dysplasia (BPD), the chronic lung disease associated with prematurity, is characterized by poor alveolar and vascular growth, interstitial fibrosis, and pulmonary hypertension (PH). Although multifactorial in origin, the pathophysiology of BPD is partly

Nintedanib preserves lung growth and prevents pulmonary hypertension in a hyperoxia-induced lung injury model Read More »

GCN2 kinase activation mediates pulmonary vascular remodeling and pulmonary arterial hypertension

Maggie M. Zhu, Jingbo Dai, Zhiyu Dai, Yi Peng, You-Yang ZhaoNorthwestern University Feinberg School of Medicine.United States Journal of Clinical Investigation InsightJCI Insight 2024; DOI: 10.1172/jci.insight.177926 AbstractPulmonary arterial hypertension (PAH) is characterized by progressive increase of pulmonary vascular resistance and remodeling that result in right heart failure. Recessive mutations of EIF2AK4 gene (encoding GCN2, General control

GCN2 kinase activation mediates pulmonary vascular remodeling and pulmonary arterial hypertension Read More »

G6pdN126D Variant Increases the Risk of Developing VEGFR (Vascular Endothelial Growth Factor Receptor) Blocker-Induced Pulmonary Vascular Disease

Christina Signoretti, Shun Matsumura, Samuel Fatehi, Melinee D’Silva, Rajamma Mathew, Francesca Cendali, Angelo D’Alessandro, S. M. Shafiqul Alam, Victor Garcia, Joseph M. Miano, Sachin A. GupteNew York Medical College. University of Colorado Anschutz Medical Campus. Medical College of Georgia at Augusta University. United States Journal of the American Medical AssociationJ Am Heart Assoc 2024; DOI: 10.1161/JAHA.123.035174 AbstractBackground: G6PD (glucose-6-phosphate-dehydrogenase) is a key enzyme in the glycolytic pathway and has been implicated in the pathogenesis of cancer

G6pdN126D Variant Increases the Risk of Developing VEGFR (Vascular Endothelial Growth Factor Receptor) Blocker-Induced Pulmonary Vascular Disease Read More »

Unraveling the Mfn2-Warburg effect nexus: a therapeutic strategy to combat pulmonary arterial hypertension arising from catch-up growth after IUGR

Lingling Yan, Xiaofei Luo, Chengcheng Hang, YuWang, Ziming Zhang, Shanshan Xu, Lizhong DuChildren’s Hospital and First Affiliated Hospital of Zhejiang University School of Medicine. China Respiratory ResearchRespir Res 2024; 25: DOI: 10.1186/s12931-024-02957-1 AbstractBackground: The interplay between intrauterine and early postnatal environments has been associated with an increased risk of cardiovascular diseases in adulthood, including pulmonary arterial hypertension

Unraveling the Mfn2-Warburg effect nexus: a therapeutic strategy to combat pulmonary arterial hypertension arising from catch-up growth after IUGR Read More »

Scroll to Top