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该病患者平均起病年龄为26岁,65岁时外显率超过90%,平均生存年龄不足50 岁,中枢神经系统(central nervous system,CNS)血管母细胞瘤和肾细胞癌( renal cell carcinoma,RCC) 的并发症为VHL 患者最主要的死因。 VHL 综合征表型可分为两型:1 型(无嗜铬细胞瘤),2 型(有嗜铬细胞瘤)。其中 VHL 2 型又分为3 个...
该病患者平均起病年龄为26岁,65岁时外显率超过90%,平均生存年龄不足50 岁,中枢神经系统(central nervous system,CNS)血管母细胞瘤和肾细胞癌( renal cell carcinoma,RCC) 的并发症为VHL 患者最主要的死因。 VHL 综合征表型可分为两型:1 型(无嗜铬细胞瘤),2 型(有嗜铬细胞瘤)。其中 VHL 2 型又分为3 个...
Patients with haemangioblastoma as their first presenting symptom were related to a higherrisk of death from central nervous system haemangioblastoma than those with abdominal lesions (HR 8.84, 95%CI 2.04 to 38.37, P=0.004).CONCLUSIONS:This largest VHL survival analysis indicates that onset age,...
Patients with haemangioblastoma as their first presenting symptom were related to a higherrisk of death from central nervous system haemangioblastoma than those with abdominal lesions (HR 8.84, 95%CI 2.04 to 38.37, P=0.004).CONCLUSIONS:This largest VHL survival analysis indicates that onset age,...
positive family history and truncating mutation types had poorer overall and VHL-related survival. Patients with haemangioblastoma as their first presenting symptom were related to a higherrisk of death from central nervous system haemangioblastoma than those with abdominal lesions (HR 8.84, 95% ...
[摘要] 目的 探讨VHL综合征的外科治疗方法。方法 总结1999年3月至2006年10月收治的4例VHL综合征患者的资料。例1,男,56岁。主诉乏力、心悸2天。空腹血糖2.37mmol/L,CT示左肾上、下极直径分别为8 cm 、7cm 、4cm肿瘤。10年前行脑血管母细胞瘤切除术。例2,女,57岁。主诉左腰痛不适1个月。CT示左肾上腺、...
positive family history and truncating mutation types had poorer overall and VHL-related survival. Patients with haemangioblastoma as their first presenting symptom were related to a higherrisk of death from central nervous system haemangioblastoma than those with abdominal lesions (HR 8.84, 95% ...
positive family history and truncating mutation types had poorer overall and VHL-related survival. Patients with haemangioblastoma as their first presenting symptom were related to a higherrisk of death from central nervous system haemangioblastoma than those with abdominal lesions (HR 8.84, 95% ...
and possesses ubiquitin ligase E3 activity. This protein is involved in the ubiquitination and degradation of hypoxia-inducible-factor (HIF), which is a transcription factor that plays a central role in the regulation of gene expression by oxygen. RNA polymerase II subunit POLR2G/RPB7 is also re...