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Hypoxic pulmonary endothelial cells release epidermal growth factor leading to vascular smooth muscle cell arginase-2 expression and proliferation

Bernadette Chen, Yi Jin, Caitlyn M. Pool, Yusen Liu, Leif D. NelinNationwide Children’s Hospital and Ohio State University.United States Physiological ReportsPhysiol Rep 2022; DOI: 10.14814/phy2.15342 AbstractThe hallmark of pulmonary hypertension (PH) is vascular remodeling. We have previously shown that human pulmonary microvascular endothelial cells (hPMVEC) respond to hypoxia with epidermal growth factor (EGF) mediated activation of the receptor tyrosine kinase, EGF […]

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Neuroblastoma Suppressor of Tumorigenicity 1 Mediates Endothelial-to-Mesenchymal Transition in Pulmonary Arterial Hypertension Related to Congenital Heart Disease

Bin Wen, Rui Peng, Pengxu Kong, Zefu Li, Yao Liu, Wenbin Ouyang, Yongquan Xie, Xiaopeng Hu, Qiang Wang, Xiangbin PanNational Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College. Beijing Chao-Yang Hospital. Beijing Friendship Hospital. Beijing Anzhen Hospital and Capital Medical University.China American Journal of Respiratory Cell and

Neuroblastoma Suppressor of Tumorigenicity 1 Mediates Endothelial-to-Mesenchymal Transition in Pulmonary Arterial Hypertension Related to Congenital Heart Disease Read More »

Fetal pulmonary hypertension: dysregulated microRNA-34c-Notch1 axis contributes to impaired angiogenesis in an ovine model

Devashis Mukherjee, Ujala Rana, Alison J. Kriegel, Pengyuan Liu, Teresa Michalkiewicz, Girija Ganesh KonduriRainbow Babies and Children’s Hospital and Case Western Reserve University School of Medicine. Medical College of Wisconsin and Children’s Wisconsin.United States Pediatric ResearchPediatr Res 2022; 93: 551-558DOI: 10.1038/s41390-022-02151-3 AbstractBackground: Persistent pulmonary hypertension of the newborn (PPHN) occurs when pulmonary vascular resistance (PVR) fails to

Fetal pulmonary hypertension: dysregulated microRNA-34c-Notch1 axis contributes to impaired angiogenesis in an ovine model Read More »

Persistence of persistent pulmonary hypertension of the newborn: A case of de novo TBX4 variant

Stephanie M. Tsoi, Kirk Jones, Elizabeth Colglazier, Claire Parker, Hythem Nawaytou, David Teitel, Jeffrey R. Fineman, Roberta L. KellerUniversity of California San Francisco.United States Pulmonary CirculationPul Circ 2022; 12: DOI: 10.1002/pul2.12108 AbstractWe present a case of a late preterm infant placed on extracorporeal life support in the first day of life for persistent pulmonary hypertension of

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A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report

Ludger Sieverding, Michael Hofbeck, Jörg Michel, Andreas Hornung, Christian Scheckenbach, Gerd Grözinger, Ekkehard Sturm, Steven W. Warmann, Anja HanserUniversity Children’s Hospital, University Hospital of Tübingen and University of Tübingen. Germany BioMed Central GastroenterologyBMC Gastroenterol 2022; 22: DOI: 10.1186/s12876-022-02123-1 AbstractBackground: Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic

A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report Read More »

Multimodality Deep Phenotyping Methods to Assess Mechanisms of Poor Right Ventricular-Pulmonary Artery Coupling

Farhan Raza, Callyn Kozitza, Chris Lechuga, Daniel Seiter, Philip Corrado, Mohammed Merchant, Naga Dharmavaram, Claudia Korcarz, Marlowe Eldridge, Christopher Francois, Oliver Wieben, Naomi CheslerUniversity of Wisconsin, Madison. University of California, Irvine. Mayo Clinic. United States FunctionFunction 2022; 3: DOI: 10.1093/function/zqac022 AbstractDeep phenotyping of pulmonary hypertension (PH) with multimodal diagnostic exercise interventions can lead to early focused

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Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH)

Ivonne Wieland, Franziska Diekmann, Julia Carlens, Laura Hinze, Katharina Lambeck, Thomas Jack, Georg HansmannHannover Medical School.Germany Frontiers in PediatricsFront Pediat 2022; 10: DOI: 10.3389/fped.2022.1012738 AbstractBackground and objectives: Emerging evidence suggests that increased degradation of von Willebrand factor and decrease in high molecular weight multimers occurs in patients with pulmonary hypertension (PH). However, the link between acquired von

Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH) Read More »

OLA1 Phosphorylation Governs the Mitochondrial Bioenergetic Function of Pulmonary Vascular Cells

Paul Sidlowski, Amanda Czerwinski, Yong Liu, Pengyuan Liu, Ru-Jeng Teng, Suresh Kumar, Clive Wells, Kirkwood Pritchard Jr, Girija G. Konduri, Adeleye J. AfolayanMedical College of Wisconsin. United States American Journal of Respiratory Cell and Molecular BiologyAm J Respir Cell Mol Biol 2022; DOI: 10.1165/rcmb.2022-0186OC AbstractMitochondrial function and metabolic homeostasis are integral to cardiovascular function and influence

OLA1 Phosphorylation Governs the Mitochondrial Bioenergetic Function of Pulmonary Vascular Cells Read More »

Heterogenous Disease Course and Long-Term Outcome of Children’s Interstitial Lung Disease Related to Filamin A Gene Variants

Julia Carlens, K. Taneille Johnson, Andrew Bush, Diane Renz, Ute Hehr, Florian Laenger, Claire Hogg, Martin Wetzke, Nicolaus Schwerk, Jonathan H. RaymentImperial College London and Royal Brompton Hospital. University of Regensburg. Hannover Medical School. University of British Columbia and British Columbia Children’s Hospital Research Institute.United Kingdom, Germany and Canada Annals of the American Thoracic SocietyAnn

Heterogenous Disease Course and Long-Term Outcome of Children’s Interstitial Lung Disease Related to Filamin A Gene Variants Read More »

DDAH1 SNP rs480414 that protects against the development of pulmonary hypertension in bronchopulmonary dysplasia results in lower nitric oxide production in neonatal cord blood-derived lymphoblastoid cell lines

Avante D. Milton, Hanadi Almazrouea, Yi Jina, Gloria Zenderb, Jennifer K. TrittmannaAbigail Wexner Research Institute at Nationwide Children’s Hospital. Ohio State University College of Medicine. United States Journal of Neonatal and Perinatal MedicineJ Neonatal Perinatal Med 2022; 15: 113-121DOI: 10.3233/NPM-210710 AbstractBackground: Bronchopulmonary dysplasia (BPD) is chronic lung disease of prematurity and pulmonary hypertension (PH) is a major

DDAH1 SNP rs480414 that protects against the development of pulmonary hypertension in bronchopulmonary dysplasia results in lower nitric oxide production in neonatal cord blood-derived lymphoblastoid cell lines Read More »

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