VEGFA and Hermansky-Pudlak syndrome: Our findings indicate the presence of elevated VEGF in PEFs from severe and moderate HPS cases that are 4–10 fold above VEGF levels in patients with mild HPS and similar to PEF VEGF levels from patients with hydrostatic edema (median 799 pg/mL) or acute lung injury (median 501 pg/mL) [39, 40, 52, 62].