A cross sectional study of renal involvement in tuberous sclerosis
A cross sectional study of renal involvement in tuberous sclerosis
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DOI：
10.1136/jmg.33.6.480
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发表时间：
1996-06-01
影响因子：
4
通讯作者：
Sampson, J
中科院分区：
文献类型：
--
作者：
Cook, JA;Oliver, K;Sampson, J
肾脏疾病是结节性硬化症 (TSC) 的常见表现，但对其真实患病率或自然史知之甚少。我们回顾了 139 名 TSC 患者的记录，这些患者没有出现肾脏症状，但接受了肾脏超声检查。检索有关肾脏受累的频率、类型和症状的信息。肾脏受累的患病率为 61%。 49% 检出血管平滑肌脂肪瘤，32% 检出肾囊肿，2.2% 检出肾癌。血管平滑肌脂肪瘤的患病率与年龄呈正相关，符合二次发病的病因学。肾囊肿是幼儿中常见的病变，其患病率似乎与年龄无关。对 TSC 患者的肾脏检查不一致且有限。我们建议对 TSC 患者进行肾脏检查的指南。
Renal disease is a frequent manifestation of tuberous sclerosis (TSC) and yet little is known about its true prevalence or natural history. We reviewed the notes of 139 people with TSC, who had presented without renal symptoms, but who had been investigated by renal ultrasound. Information on the frequency, type, and symptomatology of renal involvement was retrieved.The prevalence of renal involvement was 61%. Angiomyolipomas were detected in 49%, renal cysts in 32%, and renal carcinoma in 2.2%. The prevalence of angiomyolipoma was positively correlated with age, compatible with a two hit aetiology. Renal cysts were the commoner lesion in young children, and their prevalence did not appear to be age related.Renal investigation in people with TSC had been inconsistent and limited. We suggest guidelines for renal investigation in those with TSC.