De Paepe J P, Michel L, Pirson Y, Squifflet J P, Alexandre G
Service de Transplantation Rénale, Cliniques Universitaires St-Luc, Bruxelles.
Acta Chir Belg. 1987 Nov-Dec;87(6):376-81.
A kidney transplantation was performed in July 1981 on a 29 year old woman who presented the signs of tuberous sclerosis and suffered from chronic renal failure. The frequency and the genetic transmission of Bourneville's disease are explained. The signs of the disease are exposed with special emphasis on the renal lesions. Only the patients with minor neurological symptoms can survive. These patients are able to develop chronic renal failure. This occurs either when the kidneys are destructed by renal cysts or tumors, or when a bilateral nephrectomy must be performed for bleeding or tumoral compression. A kidney transplantation can give them an opportunity to live almost normally. When nephrectomy is not performed, a regularly follow-up is necessary because the unknown future of the renal lesions in place.
1981年7月，对一名患有结节性硬化症并伴有慢性肾衰竭的29岁女性进行了肾移植手术。文中解释了结节性硬化症的发病频率和遗传传递方式。阐述了该疾病的症状，并特别强调了肾脏病变。只有神经症状较轻的患者能够存活。这些患者可能会发展为慢性肾衰竭，这要么是由于肾囊肿或肿瘤破坏了肾脏，要么是因为必须进行双侧肾切除术以治疗出血或肿瘤压迫。肾移植可以为他们提供几乎正常生活的机会。当未进行肾切除术时，由于肾脏病变的未来情况不明，定期随访是必要的。
