Article
Is pulmonary arterial hypertension in neurofibromatosis type 1 secondary to a plexogenic arteriopathy?
Chest - 1 Sept 2007
Stewart Douglas R, Cogan Joy D, Kramer Mordechai R, Miller Wallace T, Christiansen Leif E, Pauciulo Michael W, Messiaen Ludwine M, Tu George S, Thompson William H, Pyeritz Reed E, Ryu Jay H, Nichols William C, Kodama Makoto, Meyrick Barbara O, Ross David J
Abstract excerpt
BACKGROUND: Neurofibromatosis type 1 (NF1) is a common disorder of dysregulated tissue growth secondary to mutations in the tumor suppressor gene NF1. Pulmonary arterial hypertension (PAH) in patients with NF1 is hypothesized to be secondary to an underlying vasculopathy. METHODS: We describe the entity we term NF1-associated PAH (NF1-PAH) in four new patients and update the data on four previously published...
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