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耳鼻咽喉科・頭頸部外科87巻12号

2015年11月発行

文献概要

特集 これだけは知っておこう—鼻出血への対応法 《疾患》

遺伝性出血性毛細血管拡張症関連鼻出血

著者: 井之口豪1 髙原慎一1 藤尾久美1 丹生健一1

所属機関: 1神戸大学大学院医学系研究科耳鼻咽喉科頭頸部外科

ページ範囲:P.996 - P.1002

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POINT

●常染色体優性遺伝,頻度は1/5,000〜8,000人とされ,家族歴の聴取が重要である。

●臨床的な診断基準と重症度分類があり,2015年7月から厚生労働省の指定難病に認定された。

●動静脈奇形を合併することが多く,自覚症状がなくても肺と脳のスクリーニングを勧めるべきである。

●鼻出血は難治性再発性であり,重症度別に治療法を検討する。

●電気凝固を繰り返すと鼻中隔穿孔を生じ,さらに止血が困難になることが多い。

参考文献

1)小宮山雅樹:遺伝性出血性毛細血管拡張症.脳卒中の外科43:193-200,2015
2)Dakeishi M, et al:Genetic epidemiology of hereditary haemorrhagic telangiectasia in a local community in the northern part of Japan. Hum Mutat 19:140-148, 2002
3)Silva BM, et al:Lifestyle and dietary influences on nosebleed severity in hereditary hemorrhagic telangiectasia. Laryngoscope 123:1092-1099, 2013
4)Merlo CA, et al:The effects of epistaxis on health-related quality of life in patients with hereditary hemorrhagic telangiectasia. Int Forum Allergy Rhinol 11:921-925, 2014
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8)Begbie ME, et al:Hereditary haemorrhagic telangiectasia(Osler-Weber-Rendu syndrome):a view from the 21st century. Postgrad Med J 79:18-24, 2003
9)Kwaan HC, et al:Fibrinolytic activity in lesions of hereditary hemorrhagic telangiectasia. Arch Dermatol 107:571-573, 1973
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11)Gaillard S, et al:Tranexamic acid for epistaxis in hereditary hemorrhagic telangiectasia patients:a European cross-over controlled trial in a rare disease. J Thromb Haemost 12:1494-1502, 2014
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13)Sautter NB, et al:Hereditary hemorrhagic telangiectasia-related epistaxis:innovations in understanding and management. Int Forum Allergy Rhinol 2:422-431, 2012
14)Richer SL, et al:The Young's procedure for severe epistaxis from hereditary hemorrhagic telangiectasia. Am J Rhinol Allergy 26:401-404, 2012
15)Ting JY, et al:Management of severe epistaxis after Young's procedure:a case report. Int Forum Allergy Rhinol 3:334-337, 2013
16)Al Kadah B, et al:First experiences with an individual nasal olive in patients with hereditary haemorrhagic telangiectasia(HHT). Eur Arch Otorhinolaryngol 272:117-122, 2015
17)Jameson JJ, et al:Hormonal and antihormonal therapy for epistaxis in hereditary hemorrhagic telangiectasia. Laryngoscope 114:705-709, 2004
18)Eivazi B, et al:Lack of significant estrogen and progesterone receptor expression in nasal telangiectasias in hereditary hemorrhagic telangiectasia:an immunohistochemical analysis. Acta Otolaryngol 132:86-89, 2012
19)Sadick H, et al:Estriol induced squamous metaplasia on the nasal mucosa in patients with hereditary hemorrhagic telangiectasia. Arch Med Res 36:468-473, 2005
20)南 和彦・他:エストリオール軟膏を局所使用したオスラー病例.日鼻誌52:1-7,2013
21)Brinkerhoff BT, et al:Intravenous and topical intranasal bevacizumab(Avastin)in hereditary hemorrhagic telangiectasia. Am J Otolaryngol 33:349-351, 2012
22)Karnezis TT, et al:Treatment of hereditary hemorrhagic telangiectasia with submucosal and topical bevacizumab therapy. Laryngoscope 122:495-497, 2012
23)Boyer H, Fernandes P, Le C, et al Prospective randomized trial of sclerotherapy vs standard treatment for epistaxis due to hereditary hemorrhagic telangiectasia. Int Forum Allergy Rhinol 5:435-440, 2015
24)遠藤芳彦:難治性鼻出血例に対するエタノール硬化療法.耳鼻臨床106:699-704,2013
)for the treatment of Epistaxis in Rendu-Osler-Weber or Hereditary Hemorrhagic Telangiectasia(HHT):15 years of experience. Rhinology 50:80-86, 2012

掲載誌情報

出版社:株式会社医学書院

電子版ISSN:1882-1316

印刷版ISSN:0914-3491

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