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常染色体显性多囊肾病患者肾移植的结局:基于35年随访的经验

Outcomes of Kidney Transplantation in Patients with Autosomal Dominant Polycystic Kidney Disease: Our Experience Based on 35-Years Follow-Up.

作者信息

Tsai Tsung-Yin, Chen Cheng-Hsu, Wu Ming-Ju, Tsai Shang-Feng

机构信息

Division of Nephrology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung 40705, Taiwan.

Department of Life Science, Tunghai University, Taichung 40704, Taiwan.

出版信息

Diagnostics (Basel). 2022 May 8;12(5):1174. doi: 10.3390/diagnostics12051174.

Abstract

Background and objectives: For patients with end-stage renal disease (ESRD), the best replacement therapy is renal transplant (RTx) to ensure life with good quality. Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder and a common cause of ESRD. Different from ESRD of other causes, ADPKD patients need careful pre-RTx evaluations like detecting the presence of intracranial aneurisms, cardiac manifestations, and complications of liver and renal cysts. Materials: We retrieved a total of 1327 RTx patients receiving 1382 times RTx (two recipients with three times, 48 recipients with two times) over the last 35 years. Only 41 of these patients were diagnosed with ADPKD. Results: At the first RTx, patients’ ages were 42.9 ± 12.6 (mean ± SD) years. Ages of the ADPKD group (52.5 ± 10.1 years) were older than the non-ADPKD group (42.7 ± 12.7 years, p = 0.001). We found more cell mediated and antibody mediated rejection (29.3% vs. 26.0%, and 22.0% vs. 7.0%; both p < 0.001), new onset diabetes after transplant (NODAT) (21, 51.2% vs. 326, 25.3%; p = 0.005), and worse graft survival (p < 0.001) in the ADPKD group, and with the development of more malignancies (18; 43.9% vs. 360; 28.0%; p = 0.041). The long-term patient survivals were poorer in the ADPKD group (38.9% vs. 70.3%; p = 0.018). ADPKD was found as an independent risk factor for long-term patient survival (HR = 2.64, 95% CI 1.03−6.76, p = 0.04). Conclusions: Patients with ADPKD-related ESRD developed more NODAT, and also more malignancies if not aggressively surveyed before surgery. Due to poor long-term graft and patient survivals, regular careful examinations for NODAT and malignancies, even in the absence of related symptoms and signs, are highly recommended in the follow-ups.

摘要

背景与目的

对于终末期肾病(ESRD)患者,最佳的替代治疗方法是肾移植(RTx),以确保高质量生活。常染色体显性多囊肾病(ADPKD)是一种遗传性疾病,也是ESRD的常见病因。与其他病因导致的ESRD不同,ADPKD患者在肾移植前需要进行仔细评估,如检测颅内动脉瘤的存在、心脏表现以及肝肾囊肿的并发症。

材料

我们检索了过去35年中总共1327例接受1382次肾移植的患者(2例接受了3次移植,48例接受了2次移植)。其中只有41例患者被诊断为ADPKD。

结果

首次肾移植时,患者年龄为42.9±12.6(均值±标准差)岁。ADPKD组患者年龄(52.5±10.1岁)大于非ADPKD组(42.7±12.7岁,p = 0.001)。我们发现ADPKD组细胞介导性排斥反应和抗体介导性排斥反应更多(分别为29.3%对26.0%,22.0%对7.0%;p均<0.001),移植后新发糖尿病(NODAT)更多(21例,51.2%对326例,25.3%;p = 0.005),移植肾存活率更差(p < 0.001),并且发生更多恶性肿瘤(18例;43.9%对360例;28.0%;p = 0.041)。ADPKD组患者长期生存率更低(38.9%对70.3%;p = 0.018)。ADPKD被发现是患者长期生存的独立危险因素(HR = 2.64,95%CI 1.03 - 6.76,p = 0.04)。

结论

与ADPKD相关的ESRD患者发生更多NODAT,并且如果术前未进行积极检查,也会发生更多恶性肿瘤。由于移植肾和患者长期存活率较低,因此在随访中强烈建议定期仔细检查是否存在NODAT和恶性肿瘤,即使没有相关症状和体征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d038/9139921/2e4cffd1e345/diagnostics-12-01174-g001.jpg

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