Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern, Dallas, TX.
Head and Neck Institute, Cleveland Clinic Foundation, Cleveland, OH.
Int Forum Allergy Rhinol. 2018 Jun;8(6):713-728. doi: 10.1002/alr.22094. Epub 2018 Feb 2.
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder resulting in vascular dysplasia and formation of arteriovenous malformations. Recurrent epistaxis is a hallmark of the disease. An array of medical therapies are used in this patient population, but robust evidence-based recommendations regarding the medical treatment of epistaxis are lacking. This systematic review was performed to look at the current literature and make meaningful evidence-based recommendations.
A search of the Ovid MEDLINE, Embase, and Cochrane databases was conducted by a research librarian. Abstracts in the English language and published in a peer-review journal were reviewed for relevance and inclusion. PRISMA guidelines were followed.
Eighteen studies met the inclusion criteria. In a few small studies, thalidomide was shown to consistently improve severity and frequency of epistaxis and improve hemoglobin concentrations while decreasing the need for transfusion. Tranexamic acid appeared to only impact the epistaxis severity score and not other clinical outcomes. Selective estrogen modulators (SERMs), propranolol, rose geranium oil, and N-acetylcysteine, have demonstrated promising efficacy in small trials.
Appropriate medical therapies for epistaxis outcomes in HHT remain undefined, and there is no "gold standard." Many of the studies are small and the data reported are heterogeneous, and therefore the ability to make strong evidence-based recommendations is limited. However, many different medications appear to be promising options.
遗传性出血性毛细血管扩张症（HHT）是一种常染色体显性遗传性疾病，导致血管发育不良和动静脉畸形形成。复发性鼻出血是该疾病的一个标志。在该患者群体中使用了多种医学疗法，但缺乏针对鼻出血的强有力的循证推荐建议。进行了这项系统评价，以查看当前的文献并提出有意义的循证推荐建议。
由研究图书馆员对 Ovid MEDLINE、Embase 和 Cochrane 数据库进行了搜索。对发表在同行评审期刊上的英语摘要进行了相关性和纳入性评估。遵循 PRISMA 指南。
有 18 项研究符合纳入标准。在一些小型研究中，沙利度胺被证明可一致改善鼻出血的严重程度和频率，并提高血红蛋白浓度，同时减少输血需求。氨甲环酸似乎仅影响鼻出血严重程度评分，而不影响其他临床结果。选择性雌激素调节剂（SERMs）、普萘洛尔、玫瑰天竺葵油和 N-乙酰半胱氨酸在小型试验中表现出有希望的疗效。
HHT 患者的鼻出血结局的适当医学疗法仍未确定，也没有“金标准”。许多研究规模较小，报告的数据存在异质性，因此制定强有力的循证推荐建议的能力受到限制。然而，许多不同的药物似乎是有希望的选择。
