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

J Korean Soc Transplant. 2014 Dec;28(4):241-245. Korean.
Published online December 31, 2014.  https://doi.org/10.4285/jkstn.2014.28.4.241
Copyright © 2014 The Korean Society for Transplantation
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
Kang Hun Lee, M.D., Han Sae Kim, M.D., Sung Han Yun, M.D., Jin Ho Lee, M.D., Joon Seok Oh, M.D., Seung Min Kim, M.D., Yong Hun Sin, M.D. and Joong Kyung Kim, M.D.
Division of Nephrology, Department of Internal Medicine, Bong Seng Memorial Hospital, Busan, Korea.

Corresponding author: Joon Seok Oh. Division of Nephrology, Department of Internal Medicine, Bong Seng Memorial Hospital, 401 Jungang-daero, Dong-gu, Busan 601-723, Korea. Tel: 82-51-664-4224, Fax: 82-51-664-4229, Email: j-seok@hanmail.net
Received October 06, 2014; Revised November 24, 2014; Accepted December 05, 2014.

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.

Keywords: Tuberous sclerosis complex; Kidney transplantation; Sirolimus

Figures


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.
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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.
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Fig. 3
(A, B) Subependymal nodules. Brain magnetic resonance imaging show multiple nodules (subependymal nodules) in walls of both lateral ventricle.
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Fig. 4
(A, B) Gross specimen of both kidneys. Removed both kidneys were enlarged with multiple cysts and fatty tissue between the cysts.
Click for larger image

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