局灶节段性肾小球硬化与肾移植
Transplant Proc. 2007 Apr;39(3):737-43.Focal segmental glomerulosclerosis and renal transplantation.
Crosson JT.
Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, Minneapolis, Minnesota.
Primary focal segmental glomerulosclerosis (FSGS) is a major cause of nephrotic syndrome and eventual end-stage renal disease. It is known to be due to an abnormality of the visceral epithelial cells (podocytes) of the glomerulus. The morphological hallmark of primary FSGS is diffuse effacement of podocyte foot processes. The etiology of the podocyte damage is not been clearly established. FSGS can also be a secondary process due to underlying conditions including obesity and heroin use. In the secondary processes, the mechanism appears to be a decreased ratio of podocytes to the glomerular filtration surface area. Familial forms of FSGS also exist due to alterations of several different podocyte proteins. Primary FSGS is an increasing cause of end-stage renal disease. Recurrence of severe FSGS in renal allograft recipients presents a major challenge to transplant physicians. The incidence of recurrence is generally accepted to be between 20% and 30%. Risk factors for and characteristics of recurrence include a rapid progression of the primary disease to end-stage renal failure, early onset of nephrotic range proteinuria after allografting, frequent loss of the allograft, a high frequency of recurrence in subsequent allografts, and children less than 15 years of age. Some investigators have identified a circulating factor called the FSGS factor that appears to be associated with recurrence after transplantation. This factor has been shown to be a protein between 30 and 50 kd molecular weight. Logically, the possibility of a circulating factor associated with recurrence of FSGS led investigators to treat patients with plasmapheresis. Several studies have been reported with varying success. The response of patients to plasmapheresis seems to be completely individual. Other studies have added cyclophosphamide and/or mycophenolate mofetil to the plasmapheresis protocol. Again success in these studies has been variable. However, because some patients show complete recovery with plasmapheresis, individuals who develop recurrent FSGS after transplantation usually are given a trial of plasmapheresis therapy.
PMID: 17445586 [PubMed - in process] 转自DXY
局灶性节段性肾小球硬化和肾移植
明尼苏达大学医学院实验医学和病理学系Crosson最近的研究认为血浆置换疗法治疗移植肾局灶性节段性肾小球硬化可能有助于移植肾的存活。文章发表在2007年4月《移植进程》第39卷第3期上。
局灶性节段性肾小球硬化是肾病综合征的一个主要病因和肾终末期疾病的表现，通常认为和肾小球足细胞异常有关。初期的局灶性节段性肾小球硬化的形态学标志为足细胞的足突弥散性消失，其病因学并不清楚确定；局灶性节段性肾小球硬化也可能继发于肥胖和吸食海洛因，在继发过程中，机制表现为足细胞和肾小球滤过膜表面积的比值下降。家族型局灶性节段性肾小球硬化因足细胞被不同蛋白修饰而存在。初期的局灶性节段性肾小球硬化是终末期肾病越来越多的原因。肾移植受体的再发的严重的局灶性节段性肾小球硬化对移植医生来说是一个主要的挑战。再发的局灶性节段性肾小球硬化的发病率一般认可的值为20%-30%之间，其危险因子和再发的特征包括从初期疾病到终末期肾功能衰竭的迅速进展、肾移植后早发的的肾病变范围内的蛋白尿、移植肾的快速肾失功、继续移植肾高频再发（局灶性节段性肾小球硬化）和年龄不足15岁的儿童。一些研究者已经鉴别出循环中分子量在30-50KD的称为局灶性节段性肾小球硬化因子，可能和移植肾局灶性节段性肾小球硬化再发有关，逻辑上来讲，该发现可以引导研究者通过血浆置换疗法滤过局灶性节段性肾小球硬化因子来治疗移植肾的局灶性节段性肾小球硬化。研究者已经报道单用或者联合使用环磷酰胺或/和霉酚酸酯，取得了不稳定的成功，患者对于血浆置换疗法的反应似乎完全是个体化的。因为有些患者表现了血浆置换疗法后完全治愈，肾移植术后再发性局灶性节段性肾小球硬化的患者个体通常给予血浆置换疗法的试验性治疗可能有助于移植肾的存活。
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