| VON HIPPEL LINDAU DISEASE(附4例报告) |
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| 引用本文： | 崔守信,张德秀. VON HIPPEL LINDAU DISEASE(附4例报告)[J]. 西安交通大学学报(医学版), 1993, 0(2) |
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| 作者姓名： | 崔守信 张德秀 |
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| 作者单位： | 西安医科大学第一附属医院眼科，西安医科大学第一附属医院眼科 西安 710061，西安 710061 |
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| 摘 要： | Von Hippel Lindau disease(VHL病)为临床罕见的常染色体显性遗传病,以引起视网膜、中枢神经系统及其他器官的血管瘤为主要表现。本文报告一家族两代4人患VHL病之病案,所有之病例均经萤光眼底血管造影检查及开颅手术探查、病理检查确诊为VHL病。笔者结合临床病案,复习文献,对VHL病的临床表现、诊断、治疗及预后进行讨论。
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| 关 键 词： | 视网膜血管瘤 脑血管瘤 萤光眼底血管造影 |
VON HIPPEL LINDAU DISEASE (ENCLOSED HEREWITH FOUR-CASE REPORT) |
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| Cui Shouxin,Zhang Dexiu The Specialty of Dphthalmology,The First Affiliated Hospital. VON HIPPEL LINDAU DISEASE (ENCLOSED HEREWITH FOUR-CASE REPORT)[J]. Journal of Xi‘an Jiaotong University:Medical Sciences, 1993, 0(2) |
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| Authors: | Cui Shouxin Zhang Dexiu The Specialty of Dphthalmology The First Affiliated Hospital |
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| Abstract: | Von Hippel Lindau disease (VHL) is a kind ofrare case of autosomal dominant inherittance dis-ease. It is demonstrated mainly by the angioma ofretina, craniul nerves and other organs. In this pa-per 4 cases of VHL are reported in a family withintwo generations. All of them have been examinedby the fluorescein fundus angiography and thecraniotomic operation, and finally confirmed thediagnosis of VHL disease. Using these VHL cas-es, and reviewing the relative references, the writ-er of the paper discussed the VHL's clinical mani-festation, diagnosis, treatment, prevention and re-covery. |
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| Keywords: | retinal angioma encephalic angioma fluorescein fundus angiography |
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