Eller P, Rosenkranz A R, Mark W, Theurl I, Laufer J, Lhotta K
Department of Internal Medicine, Clinical Nephrology Division, Innsbruck University Hospital, Innsbruck, Austria.
Clin Nephrol. 2004 Mar;61(3):217-21. doi: 10.5414/cnp61217.
We report a patient with complete adenine phosphoribosyltransferase deficiency and urolithiasis, in whom 4 consecutive cadaveric renal transplantations were performed; 2,8-dihydroxyadenine crystal nephropathy recurred within weeks in the first and second graft when the patient was not treated with allopurinol immediately after transplantation. In the third graft, recurrence of disease could be prevented by immediate allopurinol treatment. This graft was lost due to chronic allograft nephropathy without significant crystal deposition. After a fourth transplantation, again without initial allopurinol, the disease recurred following an initial vascular rejection. Addition of allopurinol significantly improved renal function of the 2nd and 4th graft. This case indicates that outcome of renal transplantation in patients with adenine phosphoribosyltransferase deficiency critically depends on immediate postoperative pharmacotherapy with allopurinol, which is able to prevent 2,8-dihydroxyadenine nephropathy in the graft. Furthermore, rapid recurrence of disease without allopurinol seems to be triggered by delayed graft function and acute rejection.
我们报告了一例患有完全性腺嘌呤磷酸核糖转移酶缺乏症和尿路结石的患者，该患者接受了4次连续的尸体肾移植；在第一次和第二次移植后，如果患者在移植后未立即使用别嘌醇治疗，2,8 - 二羟基腺嘌呤晶体肾病会在数周内复发。在第三次移植中，通过立即使用别嘌醇治疗可预防疾病复发。该移植肾因慢性移植肾肾病而丢失，无明显晶体沉积。在第四次移植后，同样在最初未使用别嘌醇的情况下，疾病在最初的血管排斥反应后复发。添加别嘌醇显著改善了第二次和第四次移植肾的肾功能。该病例表明，腺嘌呤磷酸核糖转移酶缺乏症患者肾移植的结果严重依赖于术后立即使用别嘌醇进行药物治疗，这能够预防移植肾中的2,8 - 二羟基腺嘌呤肾病。此外，未使用别嘌醇时疾病的快速复发似乎是由移植肾功能延迟和急性排斥反应引发的。
