Journal List > J Korean Soc Transplant > v.28(4) > 1034440

Lee, Kim, Yun, Lee, Oh, Kim, Sin, and Kim: Using Sirolimus for Posttransplant Management and Reduction of Tuberous Sclerosis Complex Progression in a Patient with End Stage Renal Disease Caused by Tuberous Sclerosis Complex

Abstract

Tuberous sclerosis complex (TSC) is a neurocutaneous disease characterized by the formation of hamartomas in multiple organs. TSC can show lesions including facial angiofibroma, shagreen patch on the skin, cortical tuber, subependymal nodule, astrocytoma in the brain, cardiac rhabdomyoma, and renal angiomyolipoma. In particular, renal angiomyolipoma may be a cause of end-stage renal disease (ESRD). On the other hand, sirolimus has regulatory effects on cellular growth and proliferation via its inhibitory effect on a protein, mammalian target of rapamycin. We report on a case of an 18-year-old male who underwent renal transplantation due to ESRD induced by TSC. Sirolimus played a role in successful treatment of TSC and effective immunosuppression for transplantation.

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Fig. 1.
(A) Facial angiofibromas. Multiple erythematous papules with telangiectasia were located on his nose and cheeks on physical examination. (B) Shagreen patch on the left back. Flesh-colored soft plaques were seen on the left back. (C) Ungual fibroma on left third finger. Fibrous growth arising from nail bed was noted in patient's left third finger.
jkstn-28-241f1.tif
Fig. 2.
Contrast enhanced abdominal computed tomography (CT) image. Abdominal CT scan shows huge, well-marginated masses composed of both fat and soft tissue density in both kidneys.
jkstn-28-241f2.tif
Fig. 3.
(A, B) Subependymal nodules. Brain magnetic resonance imaging show multiple nodules (subependymal nodules) in walls of both lateral ventricle.
jkstn-28-241f3.tif
Fig. 4.
(A, B) Gross specimen of both kidneys. Removed both kidneys were enlarged with multiple cysts and fatty tissue between the cysts.
jkstn-28-241f4.tif
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