[Hemostatic system factors and endothelial function in primary pulmonary hypertension].
Primary pulmonary hypertension (PPH) is a rare disorder, usually fatal. Although the cause of the disease is unknown, the vascular endothelium seems to play a key role. It has been proposed that a vascular endothelial dysfunction would provoke pulmonary vasoconstriction, platelet activation and thrombin formation; some of these events have already been proven. The presence of thrombosis in PPH patients has been demonstrated, and it seems to have a relationship with a vascular endothelium-dependent coagulation abnormality. Substances of endothelial cell origin capable of modifying the coagulation mechanisms are: heparan-sulfate, thrombomodulin, protein S, tissue factor pathway inhibitor (TFPI), tissue factor, von Willebrand factor, prostacyclin and endothelial-derived relaxing factor; functional and multimeric-pattern alterations in von Willebrand factor have already been reported in PPH patients.
Laboratorio de Hematología, Instituto Nacional de Cardiología Ignacio Chávez, México, D.F.
M T
MT
Collados Larumbe
J R
JR
Borbolla Escoboza
D004730:Endothelium, Vascular; D006487:Hemostasis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000209:etiology*
Factores del sistema hemostático y función endotelial en la hipertensión pulmonar primaria.