Minz M, Sharma A, Mahawar K, Heer M, Kashyap R
Department of Transplant Surgery, PGIMER, Chandigarh.
J Assoc Physicians India. 2005 Jan;53:43-5.
Tuberous sclerosis (TS) is an autosomal dominant multisystemic disease involving primarily the skin, the brain and the kidneys. Inspite of the kidney being involved in 40-80% of patients with this disease, the incidence of end stage renal disease is only about 1%. There are only 34 reported cases of successful renal transplantation in tuberous sclerosis patients with end stage renal disease. We report a case of successful renal transplantation in a patient of tuberous sclerosis with bilateral polycystic kidneys presenting with renal failure who also underwent bilateral native nephrectomies on follow up.
结节性硬化症（TS）是一种常染色体显性遗传的多系统疾病，主要累及皮肤、大脑和肾脏。尽管40%-80%的该疾病患者肾脏受累，但终末期肾病的发病率仅约为1%。据报道，结节性硬化症合并终末期肾病患者成功进行肾移植的病例仅有34例。我们报告一例结节性硬化症患者成功进行肾移植的病例，该患者双侧多囊肾伴肾衰竭，随访期间还接受了双侧肾切除术。
