Roozbeh Jamshid, Malekmakan Leila, Harifi Mohammad Mostafa, Tadayon Taraneh
From the Department of Internal Medicine, Shiraz Nephro-Urology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Exp Clin Transplant. 2018 Dec;16(6):676-681. doi: 10.6002/ect.2016.0163. Epub 2016 Oct 14.
Autosomal dominant polycystic kidney disease is the most common hereditary disorder resulting in end-stage renal disease that can affect other organs besides kidneys. Extrarenal involvement may increase mortality and morbidity. Approximately 50% of patients with this disorder require renal transplant. Posttransplant complications have been reported to be equal in these patients versus other recipients. We conducted this study to determine and compare characteristics and outcomes of transplanted patients with this disease versus other recipients at the only transplant center in southern Iran.
Detailed data of patients with autosomal dominant polycystic kidney disease were collected from medical records from the Shiraz Organ Transplantation Center. We also gathered data on a matched control group of transplant recipients who had end-stage renal disease due to other reasons, including other congenital kidney diseases and type 2 diabetes mellitus. Medical records of patients before and after transplant were reviewed. Statistical analyses were performed with SPSS software version 19 (Chicago, IL, USA).
We collected data from 2002 to 2012 on 101 patients with autosomal dominant polycystic kidney disease and 100 control patients, including 74 female (36.8%) and 127 male patients (63.2%). Average duration of pretransplant dialysis was 14.9 ± 10.3 months in study patients and 12.8 ± 8.1 months in control patients. Rejection rate was statistically greater in recipients with this disease (30 patients; 68%) versus controls (14 patients; 32%) (P = .010).
Short- and long-term survival rates of patients with autosomal dominant polycystic kidney disease were not statistically different from control patients. However, graft survival was significantly lower. In short- and long-term follow-up of autosomal dominant polycystic kidney disease patients, hemoglobin and hematocrit levels were slightly higher than those of the control group. Autosomal dominant polycystic kidney disease is not a risk factor for posttransplant diabetes mellitus.
常染色体显性多囊肾病是导致终末期肾病的最常见遗传性疾病,除肾脏外还可影响其他器官。肾外受累可能会增加死亡率和发病率。约50%的该疾病患者需要肾移植。据报道,这些患者移植后并发症与其他受者相当。我们开展这项研究以确定并比较伊朗南部唯一一家移植中心中患有该疾病的移植患者与其他受者的特征及结局。
从设拉子器官移植中心的医疗记录中收集常染色体显性多囊肾病患者的详细数据。我们还收集了一组匹配的对照移植受者的数据,这些受者因其他原因导致终末期肾病,包括其他先天性肾病和2型糖尿病。对患者移植前后的医疗记录进行回顾。使用SPSS软件19版(美国伊利诺伊州芝加哥)进行统计分析。
我们收集了2002年至2012年期间101例常染色体显性多囊肾病患者和100例对照患者的数据,其中包括74名女性(36.8%)和127名男性患者(63.2%)。研究患者移植前透析的平均时长为14.9±10.3个月,对照患者为12.8±8.1个月。该疾病受者的排斥率在统计学上高于对照组(30例患者;68%对比14例患者;32%)(P = 0.010)。
常染色体显性多囊肾病患者的短期和长期生存率与对照患者在统计学上无差异。然而,移植物存活率显著更低。在常染色体显性多囊肾病患者的短期和长期随访中,血红蛋白和血细胞比容水平略高于对照组。常染色体显性多囊肾病不是移植后糖尿病的危险因素。