Cachin Y, Sauvage J P, Schwaab G
Ann Otolaryngol Chir Cervicofac. 1976 Jan-Feb;93(1-2):103-8.
Rendu-Osler disease is an hereditary disease due to a predominantly antosomic heredity. The statistics given for 50 cases include 27 males 7 p. 100 of which had no familial antecedents. Clinically, Rendu-Osler disease begins in adulthood (44 p. 100) and often in late adulthood. In fact, it does not become really severe until about the age of 50 or 60. Epistaxis is the most frequent accident and proves fatal in more than 10 p. 100 of cases. Treatment of the nasal symptoms, whether this involves surgical excision followed by grafting or plesiocurietherapy using irridium wires, is very disappointing. The future alone will prove whether embolization gives more permanent results.
遗传性出血性毛细血管扩张症是一种主要由常染色体遗传引起的遗传性疾病。50例病例的统计数据显示,其中有27名男性,7%无家族病史。临床上,遗传性出血性毛细血管扩张症始于成年期(44%),且常始于成年晚期。事实上,它直到大约50岁或60岁才会变得真正严重。鼻出血是最常见的症状,超过10%的病例会因此致命。无论是手术切除后植皮还是使用铱丝进行近距离放疗来治疗鼻部症状,效果都非常令人失望。只有未来才能证明栓塞治疗是否能取得更持久的效果。