Lupi A, Casado B, Soli M, Bertazzoni M, Annovazzi L, Viglio S, Cetta G, Iadarola P
Department of Biochemistry A.Castellani, University of Pavia, Via Taramelli 3/B, I-27100 Pavia, Italy.
Br J Dermatol. 2002 Dec;147(6):1237-40. doi: 10.1046/j.1365-2133.2002.04998.x.
Prolidase deficiency is a rare genetic disorder for which a cure has not yet been found.
To assess the effectiveness of apheresis exchange as a new therapeutic approach.
Apheresis exchanges were repeated monthly for four consecutive months, in parallel, on two patients, replacing prolidase-deficient red blood cells with normal filtered cells. Prolidase activity and urinary dipeptides were determined at regular intervals.
The constant presence of active prolidase inside cells allowed a continuous, although partial, degradation of imidodipeptides, with a concomitant improvement of skin ulceration.
Apheresis exchange could be a reasonable way of obtaining a clinical improvement in these patients.
氨肽酶缺乏症是一种罕见的遗传性疾病，目前尚未找到治愈方法。
评估血液成分单采置换作为一种新的治疗方法的有效性。
连续四个月每月对两名患者并行进行血液成分单采置换，用正常过滤后的细胞替代缺乏氨肽酶的红细胞。定期测定氨肽酶活性和尿二肽。
细胞内持续存在活性氨肽酶使得亚氨基二肽能够持续（尽管是部分）降解，同时皮肤溃疡有所改善。
血液成分单采置换可能是使这些患者临床症状改善的一种合理方法。
