Is Pulmonary Arterial Hypertension in Neurofibromatosis Type 1 Secondary to a Plexogenic Arteriopathy?

Autor: 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.
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Zdroj: CHEST; Sep2007, Vol. 132 Issue 3, p798-808, 11p, 1 Color Photograph, 4 Charts
Abstrakt: The article presents a study which examines the correlation of pulmonary arterial hypertension (PAH) with neurofibromatosis type 1 (NF1). It has found out that after the genetic testing was performed, patients with NF1-PAH have a generally poor long-term prognosis. It has observed the complex plexiform lesions in the one available autopsy with several severe types PAH. These results have implied that NF1 should be considered as being associated with PAH.
Databáze: Complementary Index