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结节性硬化症相关肾血管平滑肌脂肪瘤的诊断和治疗进展 被引量:3

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摘要 血管平滑肌脂肪瘤(angiomyoli.poma,AML)是肾脏常见的良性肿瘤,由血管、平滑肌和脂肪组织组成。AML可以是散发性的,也可以是结节性硬化症(tuberoussclerosiscomplex,TSC)的肾脏表现。TSC是一组影响全身多器官系统的常染色体遗传性疾病,由TSC1和TSC2基因失活性突变所致。常见临床表现包括智力障碍、癫痫、皮脂腺瘤,不同患者的临床表现有很大差异。TSC在肾脏的表现包括AML、良性囊肿和肾细胞癌,是TSC患者主要的致病和死亡原因。
作者 何志嵩
出处 《中华泌尿外科杂志》 CAS CSCD 北大核心 2013年第11期874-875,共2页 Chinese Journal of Urology
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