無水エタノールによる硬化療法および鼻粘膜癒着処置を施行したオスラー病による難治性鼻出血の1例

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1 : 0 1cc : : : : : :WBC3 640/μl RBC /μl PLT /μl Hb 7 9g/dl PT APTT : : : 1ml 30G absolute ethanol Et ml 2mm

2 mg/day Et 0 3ml Al Deen endoglin ALK1 1 3 a b 2 : :

3 a b 4 : : 5 6 Aethoxysklerol AS Ethanolamine oleate EO Et in vivo 0 1 1ml Et AS EO ml Et Et AS

4 I : intensity F : frequency T: transfusion Al Dean 7 Et

5 Al Deen S, Bachmann Harildstad G : A grading scale for epistaxis in hereditary haemorrhagic teleangectasia. Rhinology 2008 ; 46 : Dakeishi M, Shioya T, Wada Y, et al : Genetic epidemiology of hereditary hemorrhagic telangiectasia in a local community in the northern part of Japan. Hum Mutat 2002 ; 19 : :. JOHNS 2005 ; 21 : Mahoney EJ, Shapshay SM : Nd YAG laser photocoagulation for epistaxis associated with hereditary hemorrhagic telangiectasia. Laryngoscope 2005 ; 115 : ,,, :Osler NBI. ; :.,,, : :. ; :. :. Vasc Lab 2007 ; 4 : :. ; :. :. ; :. Morais D, Millás T, Zarrabeitia R, et al : Local sclerotherapy with polydocanol Aethoxysklerol for the treatment of Epistaxis in Rendu Osler Weber or Hereditary Hemorrhagic Telangiectasia HHT : 15 years of experience. Rhinology 2012 ; 50 : ,, :. ; :.,, :. ; :.,, :. ; :.,, :. JOHNS 2006 ; 22 : ,, :. ; :.,, :. ; :.,, :Dieulafoy. ; :. : ethanolamine oleate, Aethoxysklerol. ; :.,,, :. ; :.,,, :Osler. ; :. :. ; :., :. ; :. Fernandez LA, Garrido Martin EM, Sanz Rodriguez F, et al : Therapeutic action of tranexamic acid in hereditary haemorrhagic telangiectasia HHT : regulation of ALK 1/endoglin pathway in endothelial cells. Thromb Haemost 2007 ; 97 : Geisthoff UW, Seyfert UT, Kübler M, et al : Treatment of epistaxis in hereditary hemorrhagic telangiectasia with tranexamic acid a double blind placebo controlled cross over phase IIIB study. Thromb Res 2014 ; 134 :

6 A Case of Absolute Ethanol Sclerotherapy and an Adhesion Maneuver for Refractory Epistaxis Associated with Osler s Disease Tetsuro Uchida, M.D. and Hiroyuki Nagai, M.D. Department of Otorhinolaryngology, Matsunami General Hospital Case Report : We report herein on the case of a 75 years old woman with refractory epistaxis associated with Osler s disease. Anemia and recurrent epistaxis occurred 10 years previously, and anemia was treated by a physician near the patient s home. Because she had gradually become unable to control her epistaxis, she visited our hospital. We diagnosed Osler s disease based on her family history, past history, and dilatory changes in the peripheral and nasal mucosa vessels. A skin graft failed and ultrasonic coagulation was not so effective. The refractory epistaxis was due to a connected arteriovenous fistula of the right upper nasal valve. Following ethanol injection sclerotherapy and an adhesion maneuver, the epistaxis was well controlled. Keywords : Osler s disease, refractory epistaxis, absolute ethanol, adhesion maneuver Nippon Jibiinkoka Gakkai Kaiho Tokyo 119 : , 2016

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