Estrogen-Progesterone Treatment of Osier-Weber-Rendu Disease

Treatment of epistaxis in hereditary hemorrhagic telangiectasia with tranexamic acid - a double-blind placebo-controlled cross-over phase IIIB study

“…Three of them examine the efficacy of drugs related to female sex hormones. Van Cutsem et al successfully treated patients with bleeding angiodysplastic gastrointestinal lesions due to HHT and other medical reasons successfully with estrogens and progesterons [12]. Vase could not confirm an effect of estrogen on hemoglobin nor on epistaxis [13].…”

Hereditary hemorrhagic teleangiectasia

“…Das therapeutische Repertoire der Epistaxis umfasst das Einbringen von Vasokonstriktiva, mechanische Kompression der Nasenflügel, vordere/hintere Nasentamponade, Lasertherapieverfahren, die Dermoplastik nach Saunders (41), Gefäßembolisationen, Gefäßligaturen (12), die Therapie mit Östrogenen (3,47) oder als ultimo ratio einen Verschluss der Nasenhaupthöhle (11). Die Anwendung von fettenden Nasensalben (50) ist grundsätzlich zu empfehlen.…”

Gastrointestinal and Hepatic Manifestations of Hereditary Hemorrhagic Telangiectasia

“…Repeated blood transfusion with iron supplementation to replace the ongoing losses is the only wellaccepted treatment. In some small trials, hormonal therapy with estrogen-progestogen (ethinyloestridiol 0.05 mg and norethisterone 1 mg daily, given orally) combinations has been shown to decrease transfusion requirements in patients with gastrointestinal bleeding [44][45][46][47]. The exact mechanism is not known but a primary effect on coagulation, stasis in the mesenteric circulation, restoration of the endothelium of the abnormal vessels, and inhibition of prostacyclin production and release are among the proposed hypotheses [46][47][48][49][50].…”