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目的总结VonRecklinghausen综合征的临床表现,尤其是眼部特征和临床转归。方法回顾性分析6例7眼被确诊为VonRecklinghausen综合征患者的皮肤、眼部、全身、检查及家系结果。结果所有患者均有不同程度皮肤散在、大小不一、棕褐色斑、颜面肿大。本病累及除晶状体、泪器外所有眼及眼眶组织,并可伴发青光眼症状。结论本病是致盲及造成身体各器官严重障碍并危及生命的疾病。必须杜绝近亲婚配,采取强制性绝育措施,减少人群致残率,利国利民。
Objective To summarize the clinical manifestations of Von Recklinghausen syndrome, especially the ocular characteristics and clinical outcome. Methods The skin, eye, body, examinations and pedigrees of 6 patients diagnosed as Von Recklinghausen syndrome were retrospectively analyzed. Results All patients had varying degrees of skin scattered, size, brown spots, face enlargement. In addition to the disease involving the lens, tear outside all eyes and orbital tissues, and may be associated with glaucoma symptoms. Conclusion This disease is a disease that causes blindness and causes serious obstacles to various organs of the body and endanger life. Progeny marriage must be put an end to mandatory sterilization measures to reduce the disability rate of the population, the country benefit the people.