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Olschewski, H.
Pulmonary veno-occlusive disease
PNEUMOLOGE. 2016; 13(1): 20-25. Doi: 10.1007/s10405-015-0013-2
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Leading authors Med Uni Graz: Olschewski Horst
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Abstract:: Pulmonary veno-occlusive disease (PVOD) is an important differential diagnosis of idiopathic (IPAH) and hereditary pulmonary arterial hypertension (HPAH), where the primary obstruction of the pulmonary blood is located in the small pulmonary venules. The obstruction is due to fibrotic material which is located in the intima of the veins and the interlobular septae where the veins are located. In addition thickened interalveolar pulmonary capillaries form a patchy capillary hemangiomatosis. Mutations in the eukaryotic translation initiation factor 2 alpha kinase 4 protein (EIF2AK4) gene have recently been identified as the cause of most cases of hereditary PVOD and some 25% of sporadic PVOD cases. Another set of PVOD patients develop the disease after therapy with mitomycin C or alkylating substances. Therapy is very difficult. Targeted IPAH therapy is prone to adverse effects caused by lung edema. Lung transplantation should be considered early after diagnosis.
Find related publications in this database (Keywords): EIF2AK4 protein, human; Mutation; Alkylating agents; Mitomycin C; Capillary hemangioma