Korean Journal of Pediatrics 2007;50(2):178-181.
Published online February 15, 2007.
Renal manifestations in tuberous sclerosis complex
Il Cheon Jeong, Ji Tae Kim, You Sik Hwang, Jung A Kim, Jae Seung Lee
Department of Pediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine Seoul, Korea
결절성 경화증 환자에서의 신장 발현
정일천, 김지태, 황유식, 김정아, 이재승
연세대학교 의과대학 소아과학교실, 신장질환 연구소
Jae Seung Lee, Email: isyonsei@yumc.yonsei.ac.kr
: The renal manifestations of tuberous sclerosis complex (TSC) are remarkably diverse, including polycystic kidney disease, simple renal cysts, renal cell carcinomas, and angiomyolipomas. All of these occur in children as well as adults in TSC. Angiomyolipomas, which can cause spontaneous life-threatening hemorrhages, are by far the most prevalent and the greatest source of morbidity. Here, we will address our experience, adding to the literature on pediatric patients with TSC requiring evaluation and treatment for renal manifestations.
: A retrospective analysis was made on 19 patients in whom TSC was diagnosed between May 2001 and Oct. 2005 at Severance Hospital. All patients had clinical diagnoses of TSC as defined by the 1998 tuberous sclerosis complex consensus conference.
: The patients consisted of 13 boys and 6 girls with a mean age of 7.3 years (range 1 to 22). The renal disease associated with TSC included angiomyolipoma in nine patients (47.4 percent), renal simple cyst in one (5.3 percent), hydronephrosis in one (5.3 percent) patient. Eight patients (42.1 percent) presented with normal kidney contours at abdominal ultrasonography. One patient underwent renal replacement therapy due to chronic renal insufficiency after nephrectomy. Hemorrhage from angiomyolipoma was not detected.
: In our review of 19 cases of TSC, renal manifestations are reported in 57.9 percent of patients. Asymptomatic angiomyolipoma associated with TSC grow gradually, although severe hemorrhages are rare. So patients with TSC should be followed up with serial computerized tomography or abdominal ultrasonography. And also, renal function should be monitored conservatively.
Key Words: Tuberous sclerosis , Angiomyolipoma , Hemorrhage , Chronic renal failure , Ultrasonography

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