青少年型肾髓质囊性病肾移植受者的长期预后。

Long-Term Outcomes of Kidney Transplant Recipients With Juvenile Nephronophthisis.

机构信息

From the Department of Pediatric Nephrology, Baskent University Faculty of Medicine, Ankara, Turkey.

出版信息

Exp Clin Transplant. 2022 May;20(Suppl 3):122-125. doi: 10.6002/ect.PediatricSymp2022.O39.

DOI:10.6002/ect.PediatricSymp2022.O39

PMID:35570616

Abstract

OBJECTIVES

Nephronophthisis is the most common genetic cause of kidney failure in childhood. Treatment for nephronophthisis is symptomatic, and kidney transplant is a good treatment option when kidney failure has developed. We reported the outcomes of kidney transplant recipients with primary diagnosis of juvenile nephronophthisis who were followed-up in our center.

MATERIALS AND METHODS

We retrospectively examined medical records of 17 kidney transplant patients with a primary diagnosis of juvenile nephronophthisis. We compared this group of 17 patients with kidney transplant recipients who had other etiologies of kidney failure in terms of transplant age, donor type, immunosuppressive treatment, acute rejection, graft loss rates, and glomerular filtration rates at 1 and 5 years posttransplant (N = 180 total analyzed).

RESULTS

Among 180 kidney transplant recipients, the 17 patients (9.4%) with nephronophthisis had a mean age of 12.6 ± 4.3 years and mean follow-up time posttransplant of 79.5 ± 41.9 months. Five of 17 patients received a kidney transplant from a deceased donor (29.4%), and the remaining 12 patients (70.6%) received transplants from living related donors. Preemptive kidney transplant was performed in 4 patients (23.5%). There was a statistically significant difference (P < .05) in terms of acute rejection between patients with nephronophthisis (17.6%) versus patients with other primary diagnoses (34%). However, the patients with nephronophthisis versus those with other primary diagnoses were similar (P > .05) in terms of transplant age (12.6 ± 4.3 vs 13.8 ± 6.7 years, respectively) and follow-up time (79.5 ± 41.9 vs 59.1 ± 38.8 months, respectively). Donor type, immunosuppressive treatment, and 1-year (96.7 ± 23.2 vs 97.6 ± 28.4 mL/min/1.73 m2) and 5-year (84.7 ± 31.1 vs 86.7 ± 21.7 mL/min/1.73 m2) glomerular filtration rates were also similar (P > .05) between groups.

CONCLUSIONS

Posttransplant prognosis was good among kidney transplant recipients with juvenile nephronophthisis.

摘要

目的

肾髓质囊性病变是儿童肾衰竭的最常见遗传原因。肾髓质囊性病变的治疗是对症治疗，当肾衰竭发生时，肾移植是一种很好的治疗选择。我们报告了在我们中心接受治疗的原发性青少年肾髓质囊性病变的肾移植受者的结果。

材料和方法

我们回顾性地检查了 17 名原发性青少年肾髓质囊性病变的肾移植患者的病历。我们将这 17 名患者与其他病因导致肾衰竭的肾移植受者（共 180 名分析）在移植年龄、供体类型、免疫抑制治疗、急性排斥反应、移植物丢失率以及移植后 1 年和 5 年的肾小球滤过率方面进行了比较。

结果

在 180 名肾移植受者中，17 名（9.4%）肾髓质囊性病变患者的平均年龄为 12.6 ± 4.3 岁，平均移植后随访时间为 79.5 ± 41.9 个月。5 名（29.4%）患者接受了来自已故供体的肾移植，其余 12 名（70.6%）患者接受了活体亲属供体的肾移植。4 名（23.5%）患者接受了预防性肾移植。肾髓质囊性病变患者（17.6%）与其他原发性诊断患者（34%）之间的急性排斥反应存在显著差异（P<.05）。然而，肾髓质囊性病变患者与其他原发性诊断患者在移植年龄（分别为 12.6 ± 4.3 岁和 13.8 ± 6.7 岁）和随访时间（分别为 79.5 ± 41.9 个月和 59.1 ± 38.8 个月）方面相似（P>.05）。供体类型、免疫抑制治疗以及 1 年（96.7 ± 23.2 比 97.6 ± 28.4 mL/min/1.73 m2）和 5 年（84.7 ± 31.1 比 86.7 ± 21.7 mL/min/1.73 m2）肾小球滤过率也相似（P>.05）。