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[遗传性出血性毛细血管扩张症患者的鼻出血]

[Epistaxis in patients with hereditary hemorrhagic teleangiectasia].

作者信息

Jovancević Ljiljana, Mitrović Slobodan M

机构信息

Klinicki centar "Novi Sad", Novi Sad, Klinika za bolesti uva, grla i nosa.

出版信息

Med Pregl. 2006 Sep-Oct;59(9-10):443-9. doi: 10.2298/mpns0610443j.

DOI:10.2298/mpns0610443j
PMID:17345820
Abstract

INTRODUCTION

Spontaneous recurrent epistaxis is the most common clinical manifestation of hereditary hemorrhagic teleangiectasia (HHT). It occurs in more than 90% of HHT patients and is the most distressing symptom. Nasal teleangiectasias tend to increase with age both in size and number, so epistaxis is heavier and more frequent. For patients with mild to moderate disease, there are many adequate treatment options. For those with severe disease, most treatments offer just a hemorrhage-free interval. Experienced otorhinolaryngologists who treat epistaxis in these patients often use the adage "to do as little as possible for as long as possible".

MANAGEMENT OF ACUTE EPISTAXIS

The recommendations for the management of acute epistaxis include: compression, use of topical antifibrinolytics, laser therapy, argon plasma coagulation therapy, fibrin sealant spray or gelatin sponge soaked in adrenaline. In cases of heavy acute epistaxis, an epistaxis balloon combined with artery ligation and/or embolization is the most effective treatment. Nasal packing and electrocauterisation should be avoided to prevent further trauma to the blood vessels.

TREATMENT OF RECURRENT EPISTAXIS

Management of recurrent epistaxis includes topical application of laser energy (argon, Nd:YAG, KTP/532 and diode, not CO2), argon plasma coagulation in combination with 0.1% estriol ointment, caustics, antifibrinolytics, bleomycin and sclerosing substances. Systemic estrogen-progesterone at doses used for oral contraception may eliminate bleeding in women with heavy epistaxis. Systemic antifibrinolitics (used with extreme precaution) and septal dermoplasty give good results. The only method which successfully and permanently solves the problem of severe refractory epistaxis in hereditary hemorrhagic teleangiectasia is closure of the nasal cavities.

摘要

引言

自发性反复鼻出血是遗传性出血性毛细血管扩张症(HHT)最常见的临床表现。超过90%的HHT患者会出现鼻出血,这也是最令人困扰的症状。鼻毛细血管扩张症往往会随着年龄增长在大小和数量上增加,因此鼻出血会更严重且更频繁。对于轻至中度病情的患者,有许多合适的治疗选择。对于重症患者,大多数治疗方法只能提供无出血间隔期。治疗这些患者鼻出血的经验丰富的耳鼻喉科医生常使用这句格言:“尽可能少做,尽可能久做”。

急性鼻出血的处理

急性鼻出血的处理建议包括:压迫、使用局部抗纤溶药物、激光治疗、氩等离子体凝固治疗、纤维蛋白密封剂喷雾或浸泡在肾上腺素中的明胶海绵。在严重急性鼻出血的情况下,鼻出血球囊联合动脉结扎和/或栓塞是最有效的治疗方法。应避免鼻腔填塞和电灼,以防止对血管造成进一步损伤。

反复鼻出血的治疗

反复鼻出血的治疗包括局部应用激光能量(氩、钕:钇铝石榴石、磷酸钛钾/532和二极管,而非二氧化碳)、氩等离子体凝固联合0.1%雌三醇软膏、腐蚀剂、抗纤溶药物、博来霉素和硬化剂。用于口服避孕的剂量的全身性雌激素 - 孕激素可能使鼻出血严重的女性止血。全身性抗纤溶药物(极其谨慎使用)和鼻中隔真皮成形术效果良好。在遗传性出血性毛细血管扩张症中成功且永久解决严重难治性鼻出血问题的唯一方法是封闭鼻腔。

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