Department of Urology, University of California, San Francisco, San Francisco, California.
Department of Urology, University of California, Irvine, Irvine, California.
J Urol. 2018 Jan;199(1):280-286. doi: 10.1016/j.juro.2017.06.075. Epub 2017 Jun 20.
We evaluated predictive factors for compensatory hypertrophy and renal outcomes in a large cohort of patients with multicystic dysplastic kidneys.
We conducted a retrospective review from 1997 to 2016. Contralateral kidney and multicystic dysplastic kidney length were recorded from all ultrasounds as well as creatinine when available. We used generalized estimating equations to determine predictors of contralateral kidney length.
A total of 443 children with multicystic dysplastic kidneys were identified based on sonographic findings and lack of function on nuclear scan. Average followup was 3.2 years (IQR 1.5 to 5.7). Median time to involution in patients diagnosed before age 2 years was 5.5 years (95% CI 3.8-7.0). In all patients the median time to contralateral hypertrophy was 2.7 years (95% CI 2.2-3.3), and 90% of patients manifested contralateral hypertrophy by 10 years. After adjusting for age, gender, multicystic dysplastic kidney side and cohort status for each year of involution after age 2 years, the contralateral kidney grows 0.35 cm longer (95% CI 0.01-0.68, p = 0.04) compared to cases without involution. Patients with contralateral hypertrophy had greater creatinine clearance at followup (83 vs 61 ml per minute, p = 0.07), although this finding was not statistically significant due to limited data.
The majority of children with multicystic dysplastic kidneys will have contralateral hypertrophy by age 3 years. Multicystic dysplastic kidney involution predicts contralateral kidney growth rate after age 2 years. A small cohort of patients with multicystic dysplastic kidneys will not exhibit contralateral hypertrophy and may be at risk for renal insufficiency.
我们评估了一组多房性肾发育不良患者的预测因素，以评估代偿性肥大和肾脏结局。
我们进行了一项回顾性研究，时间范围为 1997 年至 2016 年。从所有超声图像中记录对侧肾脏和多囊性肾发育不良的长度，以及有条件时的肌酐值。我们使用广义估计方程来确定对侧肾脏长度的预测因素。
根据超声检查结果和核扫描无功能，共确定了 443 例多房性肾发育不良患儿。平均随访时间为 3.2 年（IQR 1.5 至 5.7）。2 岁以下诊断为多房性肾发育不良的患者，其退行性变的中位时间为 5.5 年（95%CI 3.8-7.0）。在所有患者中，对侧肥大的中位时间为 2.7 年（95%CI 2.2-3.3），90%的患者在 10 年内出现对侧肥大。调整年龄、性别、多房性肾发育不良侧和每年退行性变的队列状态后，与无退行性变的病例相比，对侧肾脏的长度增加了 0.35 厘米（95%CI 0.01-0.68，p=0.04）。有对侧肥大的患者在随访时的肌酐清除率更高（83 比 61 毫升/分钟，p=0.07），但由于数据有限，这一发现没有统计学意义。
大多数多房性肾发育不良的儿童在 3 岁时会出现对侧肥大。多房性肾发育不良的退行性变预测 2 岁后对侧肾脏的生长速度。一小部分多房性肾发育不良患者不会出现对侧肥大，可能有肾功能不全的风险。
