Webb D W, Kabala J, Osborne J P
Bath Unit for Research into Paediatrics, Royal United Hospital, UK.
Br J Urol. 1994 Aug;74(2):151-4. doi: 10.1111/j.1464-410x.1994.tb16577.x.
To establish the prevalence of renal disease, asymptomatic renal lesions and possible renal symptoms in a geographically defined population of individuals with tuberous sclerosis.
The study involved 131 patients (64 men, 67 women) with tuberous sclerosis who were resident in nine of the districts within the Wessex Region and three Bristol Health Districts and who had been identified by a prevalence study [1]. The patients' mean age was 22 years (range 6 months-74 years). Established renal disease was identified by history. Where possible individuals were seen and were examined. Specific enquiry was made for flank pain and macroscopic haematuria. Renal ultrasound, blood pressure measurement and urine analysis was offered to all individuals with tuberous sclerosis who were resident in the Bath Health District.
Eight patients (6%) had a history of either renal polycystic kidney disease (two patients) or haemorrhage from renal angiomyolipomas (five female patients, one male patient). A further 21% of female and 3% of male patients had a history of severe flank pain or haematuria. Renal ultrasound screening revealed abnormalities in 10 of 21 individuals; angiomyolipomas > 1 cm were found in seven and were twice as common in female patients.
Regular clinical review of individuals with tuberous sclerosis should include enquiry for renal symptoms and abdominal examination. Lesions > 4 cm are most likely to be symptomatic but longitudinal studies are needed before renal ultrasound screening of adolescents or adults can be recommended.
确定结节性硬化症患者在特定地理区域人群中的肾脏疾病、无症状性肾脏病变及可能的肾脏症状的患病率。
本研究纳入了131例结节性硬化症患者（64例男性，67例女性），他们居住在韦塞克斯地区的9个区和布里斯托尔的3个健康区，这些患者是通过一项患病率研究确定的[1]。患者的平均年龄为22岁（范围6个月至74岁）。通过病史确定已患有的肾脏疾病。尽可能对患者进行检查。专门询问了侧腹痛和肉眼血尿情况。为居住在巴斯健康区的所有结节性硬化症患者提供了肾脏超声、血压测量和尿液分析检查。
8例患者（6%）有肾多囊性肾病（2例）或肾血管平滑肌脂肪瘤出血（5例女性患者，1例男性患者）病史。另有21%的女性患者和3%的男性患者有严重侧腹痛或血尿病史。肾脏超声筛查显示21例中有10例存在异常；发现7例血管平滑肌脂肪瘤直径>1 cm，在女性患者中更为常见，是男性患者的两倍。
对结节性硬化症患者进行定期临床检查应包括询问肾脏症状和腹部检查。直径>4 cm的病变最有可能出现症状，但在推荐对青少年或成年人进行肾脏超声筛查之前，还需要进行纵向研究。
