Mojahedi Mohammad Javad, Hekmat Reza, Ahmadnia Hassan
Division of Nephrology, Department of Internal Medicine, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
Urol J. 2007 Fall;4(4):234-7.
The aim of this study was to evaluate the results of kidney transplantation in patients with Alport syndrome.
A total of 15 patients with Alport syndrome underwent kidney transplantation and the result of their transplantation was compared with the results in patients without Alport Syndrome. Rejection episodes and the presence of antiglomerular basement membrane (anti-GBM) nephritis were assessed in these patients.
Fifteen patients with Alport syndrome were compared with a control group including 212 kidney allograft recipients. One patient with Alport syndrome (6.7%) and 30 controls (14.2%) experienced delayed graft function. Renal artery thrombosis was reported in 1 patient (6.7%) with Alport syndrome and 10 (4.7%) in the control group, which led to nephrectomy in all cases. Acute rejection was confirmed in 2 patients (13.3%) by kidney biopsy and classic treatment yielded relative response. However, they lost their grafts 35 and 44 months after the transplantation. On pathologic examination, no specific finding of anti-GBM nephritis was found. In the control group, 43 cases of acute rejection (20.3%) were reported and 12 patients (5.7%) returned to dialysis. The 1-, 3-, and 5-year graft survival rates were 100%, 92%, and 84% in the patients with Alport syndrome, which was not different from those in the control group (P = .53).
In spite of the risk of anti-GBM nephritis in the patients with Alport Syndrome, it seems that kidney transplantation can yield favorable results and anti-GBM nephritis is not a common etiology of rejection.
本研究旨在评估Alport综合征患者肾移植的结果。
共有15例Alport综合征患者接受了肾移植，并将其移植结果与非Alport综合征患者的结果进行比较。评估了这些患者的排斥反应发作情况和抗肾小球基底膜（anti-GBM）肾炎的存在情况。
将15例Alport综合征患者与包括212例肾移植受者的对照组进行比较。1例Alport综合征患者（6.7%）和30例对照组患者（14.2%）出现移植肾功能延迟。1例Alport综合征患者（6.7%）和对照组10例患者（4.7%）报告发生肾动脉血栓形成，所有病例均导致肾切除。通过肾活检确诊2例患者（13.3%）发生急性排斥反应，经典治疗产生了相对反应。然而，他们在移植后35个月和44个月失去了移植肾。病理检查未发现抗GBM肾炎的特异性表现。对照组报告43例急性排斥反应（20.3%），12例患者（5.7%）恢复透析。Alport综合征患者的1年、3年和5年移植肾存活率分别为100%、92%和84%，与对照组无差异（P = 0.53）。
尽管Alport综合征患者存在抗GBM肾炎的风险，但肾移植似乎可以产生良好的结果，抗GBM肾炎不是排斥反应的常见病因。
