Article
Pulmonary veno-occlusive disease caused by an inherited mutation in bone morphogenetic protein receptor II.
American journal of respiratory and critical care medicine - 15 Mar 2003
Runo James R, Vnencak-Jones Cindy L, Prince Melissa, Loyd James E, Wheeler Lisa, Robbins Ivan M, Lane Kirk B, Newman John H, Johnson Joyce, Nichols William C, Phillips John A
Abstract excerpt
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension in which the vascular changes originate in the small pulmonary veins and venules. The pathogenesis is unknown and any link with primary pulmonary hypertension (PPH) has been speculative. Mutations in the bone morphogenetic protein receptor II (BMPR2) gene have been identified in at least 50% of familial cases and in 25% of sporadic...
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