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 ch aracterized 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-sta ge 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 o n a case of an 18-year-old male who underwent renal trans-plantation due to ESRD induced by TSC. Sirolimus played a role in successful treatment of TSC and effective immunosuppression for transplantation.Key Words: Tuberous sclerosis complex, Kidney transplantation, Sirolimus중심 단어: 결절성 경화증, 신장이식, 시로리무스