Pulmonary arterial hypertension: pathogenesis and clinical management

T Thenappan, ML Ormiston, JJ Ryan, SL Archer - Bmj, 2018 - bmj.com
Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm
Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type of …

Genetics and genomics of pulmonary arterial hypertension

NW Morrell, MA Aldred, WK Chung… - European …, 2019 - Eur Respiratory Soc
Since 2000 there have been major advances in our understanding of the genetic and
genomics of pulmonary arterial hypertension (PAH), although there remains much to …

Whole-genome sequencing of patients with rare diseases in a national health system

E Turro, WJ Astle, K Megy, S Gräf, D Greene… - Nature, 2020 - nature.com
Most patients with rare diseases do not receive a molecular diagnosis and the aetiological
variants and causative genes for more than half such disorders remain to be discovered …

Epidemiology and treatment of pulmonary arterial hypertension

EMT Lau, E Giannoulatou, DS Celermajer… - Nature Reviews …, 2017 - nature.com
In the past 2 decades, major changes have occurred in the epidemiological and treatment
landscape of pulmonary arterial hypertension (PAH). Previously regarded as a disease of …

[HTML][HTML] Haemodynamic definitions and updated clinical classification of pulmonary hypertension

G Simonneau, D Montani… - European respiratory …, 2019 - Eur Respiratory Soc
Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary
hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure …

Pulmonary arterial hypertension: diagnosis, treatment, and novel advances

BA Maron, SH Abman, CG Elliott, RP Frantz… - American journal of …, 2021 - atsjournals.org
The diagnosis and management of pulmonary arterial hypertension (PAH) includes several
advances, such as a broader recognition of extrapulmonary vascular organ system …

Context-dependent TGFβ family signalling in cell fate regulation

L Richardson, SG Wilcockson, L Guglielmi… - … Reviews Molecular Cell …, 2023 - nature.com
The transforming growth factor-β (TGFβ) family are a large group of evolutionarily conserved
cytokines whose signalling modulates cell fate decision-making across varying cellular …

[HTML][HTML] The quiescent endothelium: signalling pathways regulating organ-specific endothelial normalcy

N Ricard, S Bailly, C Guignabert… - Nature Reviews Cardiology, 2021 - nature.com
Endothelial cells are at the interface between circulating blood and tissues. This position
confers on them a crucial role in controlling oxygen and nutrient exchange and cellular …

The latest in animal models of pulmonary hypertension and right ventricular failure

O Boucherat, V Agrawal, A Lawrie, S Bonnet - Circulation research, 2022 - Am Heart Assoc
Pulmonary hypertension (PH) describes heterogeneous population of patients with a mean
pulmonary arterial pressure> 20 mm Hg. Rarely, PH presents as a primary disorder but is …

Bone morphogenetic protein receptor signal transduction in human disease

MC Gomez‐Puerto, PV Iyengar… - The journal of …, 2019 - Wiley Online Library
Bone morphogenetic proteins (BMPs) are secreted cytokines that were initially discovered
on the basis of their ability to induce bone. Several decades of research have now …