Article
Pulmonary veno-occlusive disease.
The European respiratory journal - 1 May 2016
Montani David, Lau Edmund M, Dorfmüller Peter, Girerd Barbara, Jaïs Xavier, Savale Laurent, Perros Frederic, Nossent Esther, Garcia Gilles, Parent Florence, Fadel Elie, Soubrier Florent, Sitbon Olivier, Simonneau Gérald, Humbert Marc
Abstract excerpt
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterised by preferential remodelling of the pulmonary venules. In the current PH classification, PVOD and pulmonary capillary haemangiomatosis (PCH) are considered to be a common entity and represent varied expressions of the same disease. The recent discovery of biallelic mutations in the EIF2AK4 gene as the cause of...
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