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KMID : 0361120140280040241
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Korean Journal of Transplantation
2014 Volume.28 No. 4 p.241 ~ p.245
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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
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Lee Kang-Hun
Kim Han-Sae
Yun Sung-Han
Lee Jin-Ho
Oh Joon-Seok
Kim Sung-Min
Shin Yong-Hun
Kim Joong-Kyung
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Abstract
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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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KEYWORD
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Tuberous sclerosis complex, Kidney transplantation, Sirolimus
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