PT -期刊文章AU -亨伯特,Marc AU - Guignabert, Christophe AU - Bonnet, Sébastien AU - Dorfmüller, Peter AU - Klinger, James R. AU - Nicolls, Mark R. AU - Olschewski, Andrea J. AU - Pullamsetti, Soni S. AU - Schermuly, Ralph T. AU - Stenmark, Kurt R. AU - Rabinovitch, Marlene TI - Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives AID - 10.1183/13993003.01887-2018 DP - 2019 Jan 01 TA - European Respiratory Journal PG - 1801887 VI - 53 IP - 1 4099 - //www.qdcxjkg.com/content/53/1/1801887.short 4100 - //www.qdcxjkg.com/content/53/1/1801887.full SO - Eur Respir J2019 Jan 01; 53 AB - Clinical and translational research has played a major role in advancing our understanding of pulmonary hypertension (PH), including pulmonary arterial hypertension and other forms of PH with severe vascular remodelling (e.g. chronic thromboembolic PH and pulmonary veno-occlusive disease). However, PH remains an incurable condition with a high mortality rate, underscoring the need for a better transfer of novel scientific knowledge into healthcare interventions. Herein, we review recent findings in pathology (with the questioning of the strict morphological categorisation of various forms of PH into pre- or post-capillary involvement of pulmonary vessels) and cellular mechanisms contributing to the onset and progression of pulmonary vascular remodelling associated with various forms of PH. We also discuss ways to improve management and to support and optimise drug development in this research field.State of the art and research perspectives in the cellular and molecular basis and pathology of pulmonary vascular remodelling associated with various forms of pulmonary hypertension http://ow.ly/cjwp30mgzmH