J Korean Soc Transplant.  2014 Dec;28(4):241-245. 10.4285/jkstn.2014.28.4.241.

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

• Lee KH
• Kim HS
• Yun SH
• Lee JH
• Oh JS
• Kim SM
• Sin YH
• Kim JK

• KMID: 1941320
• DOI: http://doi.org/10.4285/jkstn.2014.28.4.241

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.

Keyword

Tuberous sclerosis complex; Kidney transplantation; Sirolimus

MeSH Terms

Adolescent
Angiofibroma
Angiomyolipoma
Astrocytoma
Brain
Hamartoma
Hand
Humans
Immunosuppression
Kidney Failure, Chronic*
Kidney Transplantation
Male
Rhabdomyoma
Sirolimus*
Skin
Tuberous Sclerosis*
Sirolimus

Figure

• 

  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.

Reference

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