Article
Idiopathic pulmonary hypertension: what did we learn from genes?
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG - 1 Dec 2005
Sztrymf Benjamin, Yaïci Azzedine, Jaïs Xavier, Sitbon Olivier, Simonneau Gerald, Humbert Marc
Abstract excerpt
Pulmonary arterial hypertension (PAH) is an uncommon disorder. PAH can be idiopathic, associated with other conditions or clustered in families. Indeed, at least 6% of individuals diagnosed with so-called "primary" pulmonary hypertension have a family history of the disorder. Familial PAH segregates as an autosomal dominant trait but with markedly reduced penetrance. Defects within bone morphogenetic protein...
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