UCSF Benioff Children's Hospital, Department of Urology, Division of Pediatric Urology, San Francisco, CA.
UCSF Benioff Children's Hospital, Department of Radiology and Biomedical Imaging, San Francisco, CA.
Urology. 2019 Jul;129:188-193. doi: 10.1016/j.urology.2019.04.003. Epub 2019 Apr 12.
To evaluate the incidence and durability of compensatory hypertrophy with solitary kidneys in the setting of those with multicystic dysplastic kidney (MCDK) or Wilms tumor (WT) status postnephrectomy.
We conducted a retrospective cohort study of patients with MCDK and WT. MCDK patients were verified by sonographic findings prenatally. WT patients entered our study at time of nephrectomy. We compared the natural history of hypertrophy between the 2 cohorts via renal length measurement. We performed linear regression to predict creatinine clearance from renal length after adjusting for age and cohort status (MCDK versus WT).
Fifty-two patients (56%) were diagnosed with WT, and 71 patients (44%) with MCDK patients met study criteria with a median age of postnatal ultrasound at 1.6 months (interquartile range 0.5-3.6). The median (IQR) follow-up time was 7.5 years. At the time of nephrectomy, 25/52 (48%) of Wilms patients had contralateral hypertrophy, while at diagnosis 22/71 (31%) of MCDK patients had contralateral hypertrophy, P = .03. Contralateral hypertrophy was a consistent finding throughout follow-up. As renal length increases by 1 cm, glomerular filtration rate increased by 7.8 mL/min/m (95% confidence interval 1.8-13.8, P = .01).
Contralateral hypertrophy appears to be a compensatory mechanism for unilateral kidney pathology as demonstrated by MCDK and WT patients. Hypertrophy often occurs at the time of diagnosis and appears to be a permanent finding as children reach their teenage years. Additionally, in nonhydronephrotic kidneys, increases in renal length correlate with improvement in glomerular filtration rate. Overall, the majority of children with solitary kidneys demonstrate compensatory hypertrophy.
评估在肾切除术后患有多囊性发育不良肾（MCDK）或Wilms 瘤（WT）的患者中，单肾发生代偿性肥大的发生率和耐久性。
我们对患有 MCDK 和 WT 的患者进行了回顾性队列研究。MCDK 患者通过产前超声检查结果得到证实。WT 患者在肾切除时进入我们的研究。我们通过测量肾脏长度来比较两组的肥大自然史。我们进行了线性回归，以调整年龄和队列状态（MCDK 与 WT）后，从肾脏长度预测肌酐清除率。
52 例（56%）患者被诊断为 WT，71 例（44%）MCDK 患者符合研究标准，其产后超声中位年龄为 1.6 个月（四分位距 0.5-3.6）。中位（IQR）随访时间为 7.5 年。在肾切除时，25/52（48%）WT 患者对侧肾脏肥大，而在 MCDK 患者诊断时，22/71（31%）对侧肾脏肥大，P=0.03。对侧肥大是整个随访过程中的一个一致发现。肾脏长度增加 1 厘米，肾小球滤过率增加 7.8 mL/min/m（95%置信区间 1.8-13.8，P=0.01）。
正如 MCDK 和 WT 患者所证明的那样，对侧肥大似乎是单侧肾脏病变的代偿机制。肥大通常在诊断时发生，并且随着儿童进入青少年时期，似乎是一种永久性发现。此外，在非积水性肾脏中，肾脏长度的增加与肾小球滤过率的改善相关。总体而言，大多数单肾儿童都表现出代偿性肥大。
