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Insights from precision-cut lung slices-investigating mechanisms and therapeutics for pulmonary hypertension

William R. Studley, Emma Lamanna, Claudia A. Nold‑Petry, Cheng Xue Qin, Jane E. BourkeMonash University. Hudson Institute of Medical Research. Baker Heart and Diabetes Institute. Australia. Respiratory ResearchRespir Res 2025; 26: DOI: 10.1186/s12931-025-03290-x AbstractPrecision-cut lung slices (PCLS) are gaining traction as a versatile ex vivo tool to study mechanisms and treatments for lung diseases. This preparation, […]

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Highly Variable Expressivity of a CNV Deletion Involving TBX4 in Three Deceased Siblings With Lung Developmental Disorder and Their Mildly Affected Mother and Grandfather

Przemyslaw Szafranski, Tomasz Gambin Michal Kadlof, Michal Denkiewicz, Dariusz Plewczynski, Hyun Jeong Kim, Gail Deutsch, Nahir Cortes-Santiago, Salmo Raskin, Pawel StankiewiczBaylor College of Medicine and Texas Children’s Hospital. Warsaw University of Technology. University of Warsaw. University of Washington School of Medicine. Federal University of Paraná.United States, Poland and Brazil Clinical GeneticsClin Genet 2025; DOI: 10.1111/cge.70010 AbstractSingle

Highly Variable Expressivity of a CNV Deletion Involving TBX4 in Three Deceased Siblings With Lung Developmental Disorder and Their Mildly Affected Mother and Grandfather Read More »

Postnatally induced TBX4 insufficiency confers pulmonary hypertension and impairs lung development in infant mice

Caroline F. Smith, Kathy L. Ding, Gregory J. Seedorf, Csaba Galambos, Steven H. AbmanUniversity of Colorado School of Medicine. United States Pediatric ResearchPediatr Res 2025; DOI: 10.1038/s41390-025-04127-5 AbstractBackground: Genetic variants in T-box transcription factor 4 (TBX4) cause pulmonary hypertension (PH); however, there are diverse phenotypes with respect to the timing and severity of disease. Previous mouse studies

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The Impact of Cardiac Output Methods on the Classification of Pulmonary Hypertension

Léon Genecand, Gaëtan Simian, Mona Lichtblau, Jean‐Marc Fellrath, Julian Klug, Hugues Turbé, Christian Lovis, Stéphane Noble, Julie Wacker, Julian Müller, Roberto Desponds, Maurice Beghetti, Benoit Lechartier, David Montani, Olivier Sitbon, Silvia Ulrich, Frédéric LadorGeneva University Hospitals and University of Geneva. University Hospital of Zurich and University of Zurich. Hopital Pourtales, Reseau Hospitalier Neuchâtelois. Lausanne University

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Isolated pulmonary valve endocarditis in a 7-year-old Nigerian girl: a case report

Ayobami Oyetunji Alabi, Bukola Adetutu Sayomi, Olanike Taye Oladibu, Mayowa Mary Adetoye, Nicholas Aderinto, Adeseye Abiodun AkintundeLadoke Akintola University of Technology (LAUTECH) Teaching Hospital. Nigeria Journal of Medical Case ReportsJ Med Case Rep 2025; 19: DOI: 10.1186/s13256-025-05241-y AbstractBackground: Right-sided infective endocarditis is a rare clinical entity, with isolated pulmonary valve infective endocarditis being extremely uncommon. Infective endocarditis

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Deciphering epigenetic control of Notch signaling in persistent pulmonary hypertension of the newborn

Matthew D. Durbin, David G. Tingay, Kok Lim KuaIndiana University School of Medicine. Murdoch Children’s Research Institute and University of Melbourne.United States and Australia Pediatric ResearchPediatr Res 2025; DOI: 10.1038/s41390-025-04234-3 AbstractAbstract Not Available CategoryClass I. Persistent Pulmonary Hypertension of the NewbornAnimal Models of Pulmonary Vascular Disease and TherapyVascular Cell Biology and Mechanisms of Pulmonary Vascular DiseaseGenetic

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Clinical Study to Evaluate the Possible Efficacy and Safety of L-Arginine in Children with Sickle Cell Disease and Increased Tricuspid Regurgitant Jet Velocity: a Randomized Controlled Trial

Dalia A. Gomaa, Sahar M. El‑Haggar, Mohamed R. El‑Shanshory, Osama El‑Razaky, Dalia R. El‑AffyTanta University. Egypt Paediatric DrugsPaediatr Drugs 2025; DOI: 10.1007/s40272-025-00701-w AbstractBackground: Pulmonary hypertension (PH) is a common chronic complication of sickle cell disease (SCD), and patients at risk for PH can be identified by measuring tricuspid regurgitant jet velocity (TRJV). We looked for the possible

Clinical Study to Evaluate the Possible Efficacy and Safety of L-Arginine in Children with Sickle Cell Disease and Increased Tricuspid Regurgitant Jet Velocity: a Randomized Controlled Trial Read More »

Fetal therapy for congenital diaphragmatic hernia: past, present and future

Nimrah Abbasi, Tim Van Mieghem, Greg RyanMount Sinai Hospital and University of Toronto.Canada World Journal of Pediatric SurgeryWorld J Pediatr Surg 2025;DOI: 10.1136/wjps-2024-000835 AbstractCongenital diaphragmatic hernia (CDH) affects 1/2500-5000 infants and is associated with significant neonatal morbidity and mortality related to pulmonary hypoplasia and pulmonary hypertension. Current estimates of perinatal mortality are between 30-40%. With advances

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Usefulness of exercise stress echocardiography in a patient with unilateral pulmonary branch stenosis

Ryohei Yokoyama, Yoshihiko Kodama, Kazunari Takamura, Masako Takahashi, Miyo Tanaka, Nozomi Watanabe, Hiroshi MoritakeUniversity of Miyazaki.Japan Journal of Cardiology CasesJ Cardiol Cases 2025; 31: 155-157DOI: 10.1016/j.jccase.2025.02.003 AbstractExercise stress echocardiography (ESE) is a feasible and valuable tool for evaluating subclinical pulmonary hypertension (PH). However, its utility in patients with unilateral pulmonary branch stenosis remains unclear. We present

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Whole exome sequencing unravels genetic architecture and its clinical implications in pediatric pulmonary arterial hypertension

Dai-Ji Jiang, Yi-Jia Yang, Yu-Zhen Wang, Xu Zhang, Wen-Xiu, Chan, Ting-Ting Yu, Hao Chen, Hao Zhang, Yi Yan, Li-Jun FuShanghai Children’s Medical Center, National Children’s Medical Center and Shanghai Jiao Tong University School of Medicine.China International Journal of CardiologyInt J Cardiol 2025; DOI: 10.1016/j.ijcard.2025.133515 AbstractBackground: Pulmonary arterial hypertension (PAH) is a severe disease with significant genetic predisposition.

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