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Von Hippel-Lindau病一例报告并文献复习

Von Hippel-Lindau Disease:a Case Report and Review of Literatures
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摘要 目的探讨Von Hippel-Lindau(VHL)病的发病机制、临床特点及治疗方法。方法1例脊髓血管母细胞瘤伴有小脑多发肿瘤和左肾上腺肿瘤患者,脊髓血管母细胞瘤行椎管探查肿瘤切除术,小脑多发性肿瘤行伽玛刀治疗,左肾上腺肿瘤待二期手术。结果随访9个月,双下肢及鞍区感觉障碍明显恢复,双下肢肌力恢复正常。复查头部及脊髓MRI均提示:脊髓肿瘤完全消失,小脑多发性肿瘤未见明显变化。结论手术切除加伽玛刀辅助治疗VHL病是较为有效的治疗方法。 Objective To investigate the pathogenesis,clinical characteristics and treatment ot Von Hippel-Lindau(VHL) disease. Methods The treatment procedure of one patient with spinal cord hemangioblastoma and cerebellar multiple tumors and left adrenal gland tumor was reviewed. The spinal cord hemangioblastoma was resected via intravertebral exporatory, and cere-bellar multiple tumors were treated by gamma knife therapy, while the left adrenal gland tumor waited for the secondary operation. Results Follow-up was done nine months after operation. Sensory disturbance in saddle area and lower extremities was significantly recovered,myodynamia of double lower extremities was recovered to normal. The cranial and spinal cord MRI detection showed that spinal cord tumor was completely disappeared and cerebellar multiple tumors had no obvious change. Conclusion Surgical resection combined with gamma knife therapy are effective treatment for VHL disease.
出处 《江西医学院学报》 2009年第8期48-49,52,共3页 Acta Academiae Medicinae Jiangxi
关键词 Von HIPPEL Lindau病 伽玛刀治疗 外科手术 Von Hippel-Lindau disease gamma knife therapy surgery
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参考文献6

  • 1Butman J A,Linehan W M,Lonser R R. Neurologic Manifestations of Von Hippel-Lindau Disease[J]. JAMA, 2008, 300 (11) :1334-1342.
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  • 3Puri S,Cano D, Hebrok M. A Role for Von-Hippel Lindau Protein in Pancreatic β-Cell Function[J].Diabetes, 2009,58 (2) : 433-441.
  • 4Courcoutsakis N A, Prassopoulos P K, Patronas N J. Aggressive Leptomeningeal Hemangioblastomatosis of the Central Nervous System in a Patient with Von Hippel-Lindau Disease[J].AJNR,2009,30(4) :758-760.
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