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What is Each of the following is true about the idiopathic primary pulmonary hypeension except and explain in detail?
Idiopathic pulmonary aerial hypeension can be sporadic or familial with autosomal dominant inheritance.Heterozygous germ-line mutations involving the gene that code for type ll bone morphogenetic protein receptor(BMPR ll),a member of the transforming growth factor beta superfamily,appear to account for most cases of familial IPAH. There is a female predominance,with most patients presenting in the fouh and fifth decades.The disease typically is diagnosed late in its course.Before current therapies,a mean survival of 2-3 years from the time of diagnosis was repoed. Ref:Harrison' s medicine-18th edition,page no:2077,2078.