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结节性硬化症复合体:肾脏影像学表现

Tuberous sclerosis complex: renal imaging findings.

作者信息

Casper Keith A, Donnelly Lane F, Chen Bin, Bissler John J

机构信息

Department of Radiology, Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229-3039, USA.

出版信息

Radiology. 2002 Nov;225(2):451-6. doi: 10.1148/radiol.2252011584.

Abstract

PURPOSE

To review the renal imaging findings and changes over time in a large series of young patients with tuberous sclerosis complex (TSC).

MATERIALS AND METHODS

One hundred thirty-nine renal imaging studies (113 ultrasonographic scans, 15 computed tomographic scans, and 11 magnetic resonance images) were identified in 59 patients with TSC (mean age, 11.4 years; age range, 3 days to 36 years). There were 31 male and 28 female patients. Angiomyolipomas and cysts were identified and characterized according to the following features: number, unilateral versus bilateral, largest diameter, and change over time. In patients whose initial examination results were normal, the age at onset of lesions was noted. The chi(2) test was used to determine whether cysts and angiomyolipomas occurred in different subpopulations.

RESULTS

Angiomyolipomas were identified in 47 (80%) patients and were too numerous to count in 36 (76%), focal in 38 (81%), and bilateral in 42 (89%). The mean largest diameter was 21 mm. Cysts were identified in 28 (47%) patients, 18 (64%) of whom had fewer than five cysts. Cysts were bilateral in 17 (61%) patients. The mean largest diameter was 20 mm. In patients with initially normal examination results, the mean age at presentation was 9.0 years for those with cysts and 9.2 years for those with angiomyolipomas. In 80 follow-up examinations, size and/or number increased in 32 (40%) angiomyolipomas and 21 (26%) cysts. Cysts and angiomyolipomas did not occur in significantly different subpopulations (P =.13).

CONCLUSION

Both angiomyolipomas and cysts occur commonly in pediatric patients with TSC and tend to increase in size and number with increasing age. Angiomyolipomas are more common than cysts and tend to be numerous.

摘要

目的

回顾大量结节性硬化症(TSC)年轻患者的肾脏影像学表现及随时间的变化。

材料与方法

在59例TSC患者(平均年龄11.4岁;年龄范围3天至36岁)中确定了139项肾脏影像学检查(113次超声扫描、15次计算机断层扫描和11次磁共振成像)。其中男性31例,女性28例。根据以下特征对肾血管平滑肌脂肪瘤和囊肿进行识别和特征描述:数量、单侧与双侧、最大直径以及随时间的变化。对于初始检查结果正常的患者,记录病变出现的年龄。采用卡方检验确定囊肿和肾血管平滑肌脂肪瘤是否在不同亚组中出现。

结果

47例(80%)患者发现肾血管平滑肌脂肪瘤,其中36例(76%)数量太多无法计数,38例(81%)为局灶性,42例(89%)为双侧性。平均最大直径为21毫米。28例(47%)患者发现囊肿,其中18例(64%)囊肿少于5个。17例(61%)患者囊肿为双侧性。平均最大直径为20毫米。初始检查结果正常的患者中,出现囊肿的患者平均就诊年龄为9.0岁,出现肾血管平滑肌脂肪瘤的患者平均就诊年龄为9.2岁。在80次随访检查中,32例(40%)肾血管平滑肌脂肪瘤和21例(26%)囊肿的大小和/或数量增加。囊肿和肾血管平滑肌脂肪瘤在不同亚组中的出现无显著差异(P = 0.13)。

结论

肾血管平滑肌脂肪瘤和囊肿在TSC儿科患者中均常见,且往往随年龄增长而增大和增多。肾血管平滑肌脂肪瘤比囊肿更常见,且往往数量较多。

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