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Title

Review of Pharmacological Strategies with Repurposed Drugs for Hereditary Hemorrhagic Telangiectasia Related Bleeding

AuthorsAlbiñana, Virginia ; Cuesta, Ángel M. ; Rojas-P, Isabel de; Gallardo-Vara; Recio-Poveda, Lucía ; Bernabéu, Carmelo ; Botella, Luisa María
KeywordsHHT
ALK1
Endoglin
Raloxifene
Bazedoxifene
Tranexamic acid
Propranolol
FK506
Etamsylate
N-acetylcysteine
Issue Date2020
PublisherMultidisciplinary Digital Publishing Institute
CitationJournal of Clinical Medicine 9(6): 1766 (2020)
AbstractThe diagnosis of hereditary hemorrhagic telangiectasia (HHT) is based on the Curaçao criteria: epistaxis, telangiectases, arteriovenous malformations in internal organs, and family history. Genetically speaking, more than 90% of HHT patients show mutations in ENG or ACVRL1/ALK1 genes, both belonging to the TGF-β/BMP9 signaling pathway. Despite clear knowledge of the symptoms and genes of the disease, we still lack a definite cure for HHT, having just palliative measures and pharmacological trials. Among the former, two strategies are: intervention at “ground zero” to minimize by iron and blood transfusions in order to counteract anemia. Among the later, along the last 15 years, three different strategies have been tested: (1) To favor coagulation with antifibrinolytic agents (tranexamic acid); (2) to increase transcription of ENG and ALK1 with specific estrogen-receptor modulators (bazedoxifene or raloxifene), antioxidants (N-acetylcysteine, resveratrol), or immunosuppressants (tacrolimus); and (3) to impair the abnormal angiogenic process with antibodies (bevacizumab) or blocking drugs like etamsylate, and propranolol. This manuscript reviews the main strategies and sums up the clinical trials developed with drugs alleviating HHT.
Description© 2020 by the authors.
Publisher version (URL)https://doi.org/10.3390/jcm9061766
URIhttp://hdl.handle.net/10261/215680
DOIhttp://dx.doi.org/10.3390/jcm9061766
E-ISSN2077-0383
Appears in Collections:(CIB) Artículos
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