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The genetic epidemiology and genotype-phenotype correlations among Chinese children with idiopathic and heritable pulmonary arterial hypertension

Yuan He, Qiangqiang Li, Chen Zhang, Bradley B. Keller, Yiping Shen, Hong GuBeijing Anzhen Hospital and Capital Medical University. Cincinnati Children’s Hospital Medical Center. Boston Children’s Hospital and Harvard Medical School.China and United States Respiratory ResearchRespir Res 2025; 26: DOI: 10.1186/s12931-025-03249-y AbstractObjective: This study aims to analyze the genetic characteristics, genotype-phenotype correlation and long-term prognosis of children […]

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Transcription factor EB improves hypoxic pulmonary hypertension in fetal rats by suppressing NLRP3 inflammasome activation via induction of mitophagy

Chaohong Chen, Zaoye Xie, DangAo, Yinhui Chen, Ling Liu, Chengyan LiAffiliated Hospital of Guangdong Medical University. China Scientific ReportsSci Rep 2025; 15: DOI: 10.1038/s41598-025-07068-5 AbstractPersistent pulmonary hypertension of the newborn (PPHN) represents a life-threatening cardiopulmonary condition characterized by hypoxia-driven pulmonary vascular remodeling. While transcription factor EB (TFEB), a master regulator of cellular adaptation to hypoxia, has

Transcription factor EB improves hypoxic pulmonary hypertension in fetal rats by suppressing NLRP3 inflammasome activation via induction of mitophagy Read More »

Co-development of mesoderm and endoderm enables organotypic vascularization in lung and gut organoids

Yifei Miao, Nicole M. Pek, Cheng Tan, Cheng Jiang, Zhiyun Yu, Kentaro Iwasawa, Min Shi, Daniel O. Kechele, Nambirajan Sundaram, Victor Pastrana-Gomez, Debora I. Sinner, Xingchen Liu, Ko Chih Lin, Cheng-Lun Na, Keishi Kishimoto, Min-Chi Yang, Sushila Maharjan, Jason Tchieu, Jeffrey A. Whitsett, Yu Shrike Zhang, Kyle W. McCracken, Robbert J. Rottier, Darrell N. Kotton,

Co-development of mesoderm and endoderm enables organotypic vascularization in lung and gut organoids Read More »

Transferrin Purification, Biophysical Characterization, and Lung Biodistribution in Sickle Cell Disease Mice

Shuwei Lu, Mohd A. Khan, Saini Setua, Quintin O’Boyle, Kiruphagaran Thangaraju, Pedro Cabrales, Delaney C. Swindle, David C. Irwin, Paul W. Buehler, Andre F. PalmerOhio State University. University of Maryland School of Medicine. University of California San Diego. University of Colorado, Anschutz Medical Campus. United States Biotechnology and BioengineeringBiotechnol Bioeng 2025; DOI: 10.1002/bit.70012 AbstractPlasma transferrin (Tf)

Transferrin Purification, Biophysical Characterization, and Lung Biodistribution in Sickle Cell Disease Mice Read More »

Development of late pulmonary hypertension after antenatal inflammation in experimental bronchopulmonary dysplasia

Paula Dias Maia, Gregory Seedorf, Tania Gonzalez, Elisa Bye, Benjamin S. Frank, Erica W. Mandell, Steven H. AbmanUniversity of Colorado Anschutz School of Medicine and Children’s Hospital Colorado. Unoted States Pediatric ResearchPediatr Res 2025; DOI: 10.1038/s41390-025-04223-6 AbstractBackground: Antenatal inflammation due to chorioamnionitis is strongly associated with the development of bronchopulmonary dysplasia (BPD) and BPD-associated pulmonary hypertension (BPD-PH)

Development of late pulmonary hypertension after antenatal inflammation in experimental bronchopulmonary dysplasia Read More »

Procedural Variation May Contribute to 6-Minute Walk Distance Variability in Real-World Pediatric Pulmonary Arterial Hypertension Study

Cynthia Kleppinger, Dunbar Ivy, Norman Stockbridge, Angela Bates, Stephanie Handler, Usha S. Krishnan, Mary P. Mullen, Delphine Yung, Rachel K. Hopper, Nidhy P. Varghese, Jef Fineman, Eric D. Austin, Catherine M. Avitabile, Grace Freire, Jennifer Clark, Haihao SunUnited States Food and Drug Administration. University of Colorado. Stollery Children’s Hospital. Medical College of Wisconsin. Columbia University

Procedural Variation May Contribute to 6-Minute Walk Distance Variability in Real-World Pediatric Pulmonary Arterial Hypertension Study Read More »

Echo-Derived Right Ventricular Strain Identifies Pulmonary Hypertension in Pediatric Ventricular Assist Device Patients

Ryan S. Bishop, Tam T. Doan, Claudia Lara, Lindsay F. Eilers, Hari P. Tunuguntla, Joseph A. Spinner, Ricardo H. Pignatelli, Anitha Parthiban, James C. WilkinsonTexas Children’s Hospital and Baylor College of Medicine.United States American Society for Artificial Internal OrgansASAIO 2025; DOI: 10.1097/MAT.0000000000002489 AbstractIdentifying pulmonary hypertension in pediatric patients with continuous-flow ventricular assist device (cf-VAD) remains challenging,

Echo-Derived Right Ventricular Strain Identifies Pulmonary Hypertension in Pediatric Ventricular Assist Device Patients Read More »

TNF drives aberrant BMP signaling to induce endothelial and mesenchymal dysregulation in pulmonary hypertension

M. L. Garcia-Hernandez, Javier Rangel-Moreno, Qingfu Xu, Ye Jin Jeong, Soumyaroop Bhattacharya, Ravi Misra, Stacey Duemmel, Ke Yuan, Benjamin D. KormanUniversity of Rochester Medical Center. Boston Children’s Hospital.United States Journal of Clinical Investigation InsightJCI Insight 2025; DOI: 10.1172/jci.insight.174456 AbstractThe pathobiology of pulmonary hypertension (PH) is complex and multiple cell types contribute to disease pathogenesis. We sought

TNF drives aberrant BMP signaling to induce endothelial and mesenchymal dysregulation in pulmonary hypertension Read More »

Anxiety and Depression Screening of Youth in Pediatric Pulmonary Hypertension Clinic: A Multi-Center, Cross-Sectional Study

Claire Parker, Elise Whalen, Michael A. Smith, Jasmine Becerra, Leah Stevens, Catherine M. Avitabile, Anna Brown, Michelle Cash, Emma Olson Jackson, Julia McSweeney, Kathleen Miller‐Reed, Janette T. Reyes, Cathy Sheppard, Mary P. Mullen, Pulmonary Hypertension Network (PPHNet)University of California, San Francisco and Benioff Children’s Hospital. Texas Children’s Hospital. University of Pennsylvania Perelman School of Medicine

Anxiety and Depression Screening of Youth in Pediatric Pulmonary Hypertension Clinic: A Multi-Center, Cross-Sectional Study Read More »

The Use of Vasodilator Therapy in Fontan Patients: A Single-Centre Experience

Alessia Faccini, Martina Avesani, Roberta Biffanti, Elettra Pomiato, Domenico Sirico, Alice Pozza, Alessia Cerutti, Elena Reffo, Biagio Castaldi, Giovanni Di SalvoUniversity Hospital Padua.Italy ChildrenChildren 2025; 12:DOI: 10.3390/children12060751 AbstractBackground: The aim of this study was to describe our centre experience in the use of pulmonary vasodilator therapy in Fontan patients.Methods: We retrospectively enrolled patients that underwent Fontan operation between

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