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结节性硬化症患者成功进行活体亲属肾移植一例。

Successful live related renal transplant in a case of tuberous sclerosis.

作者信息

Minz M, Sharma A, Mahawar K, Heer M, Kashyap R

机构信息

Department of Transplant Surgery, PGIMER, Chandigarh.

出版信息

J Assoc Physicians India. 2005 Jan;53:43-5.

Abstract

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.

摘要

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。

相似文献

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Cystic renal involvement in tuberous sclerosis.结节性硬化症的肾囊肿性病变
Clin Pediatr (Phila). 1985 Jan;24(1):36-9. doi: 10.1177/000992288502400108.
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Diagnosis of tuberous sclerosis in a patient who presented with polycystic kidney disease.
Nephrol Dial Transplant. 2000 Apr;15(4):547-8. doi: 10.1093/ndt/15.4.547.

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