Cheng L H, Tsai W J
Department of Otolaryngology, Sin-Lau Christian Hospital, Tainan, Taiwan, Republic of China.
Gaoxiong Yi Xue Ke Xue Za Zhi. 1992 Sep;8(9):495-502.
A Taiwanese family, with at least nine members in six generations has been affected with Osler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia. It is an autosomal dominant familial disease which involves systemic subepithelial fibrovascular dysplasia and manifests with telangiectasia or angiomas of the face, the palms, or the mucosa of the aerodigestive tract (such as the nose, lips, oral cavity, or stomach, etc.). The most common clinical symptom is recurrent epistaxis with/without upper gastrointestinal bleeding which may be resistant to the otolaryngologist's best efforts for control. Repeated bleeding marks the typical course of the disease. Epistaxis from hereditary hemorrhagic telangiectasias often requires a combination of therapeutic approaches. Carbon dioxide laser surgery was highly successful in decreasing postoperative morbidity and epistaxis in the four patients we treated.
一个台湾家庭，六代人中至少有九名成员患有奥斯勒-韦伯-伦杜病，又称遗传性出血性毛细血管扩张症。这是一种常染色体显性遗传病，涉及全身性上皮下纤维血管发育异常，表现为面部、手掌或气消化道黏膜（如鼻子、嘴唇、口腔或胃等）的毛细血管扩张或血管瘤。最常见的临床症状是反复鼻出血伴或不伴上消化道出血，耳鼻喉科医生尽最大努力控制出血可能也难以奏效。反复出血是该病的典型病程。遗传性出血性毛细血管扩张症引起的鼻出血通常需要多种治疗方法联合使用。我们治疗的四名患者接受二氧化碳激光手术，在降低术后发病率和鼻出血方面取得了显著成功。
