Department of Pediatrics, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Zdravotniku 248/7, 779 00, Olomouc, Czech Republic.
Department of Urology, University Hospital Olomouc, Olomouc, Czech Republic.
Pediatr Nephrol. 2024 Sep;39(9):2645-2654. doi: 10.1007/s00467-024-06360-2. Epub 2024 Apr 15.
Individuals with congenital solitary functioning kidney (SFK) are at an increased risk of kidney damage. According to some studies, the risk is higher in unilateral kidney agenesis (UKA) than in unilateral multicystic dysplastic kidney (UMCDK). We hypothesized that with early detection of children with UKA and UMCDK, there would be no difference in the presence of hypertension, proteinuria, and reduced glomerular filtration rate (GFR) between UKA and UMCDK.
Based on a long-term follow-up protocol, we evaluated a cohort of 160 children followed from birth for SFK (84 with UKA and 76 with UMCDK) detected by prenatal or routine neonatal ultrasound screening. Hypertension, proteinuria, and reduced GFR were monitored as markers of kidney damage. We compared the characteristics and outcomes of the subgroups of children with UKA and UMCDK.
GFR was reduced in 42 (26.2%) children, of whom 41 showed only mild reduction. Hypertension and proteinuria were found in 22 (13.8%) and 14 (8.8%) children, respectively. Combined kidney damage was present in 57 (35.6%) children. The UMCDK and UKA subgroups differed in GFR at final examination, with UMCDK patients being significantly more likely to have normal GFR compared to UKA patients (82% vs. 67%; p = 0.039).
One third of the children showed signs of SFK damage, albeit mild. Patients with UKA had reduced GFR significantly more often than those with UMCDK, but did not differ in the rates of hyperfiltration injury or congenital anomalies of the kidneys and urinary tract (CAKUT) in SFK.
先天性孤立功能肾（SFK）个体发生肾脏损伤的风险增加。根据一些研究，单侧肾发育不全（UKA）的风险高于单侧多囊性发育不良肾（UMCDK）。我们假设通过对 UKA 和 UMCDK 患儿的早期检测，UKA 和 UMCDK 之间高血压、蛋白尿和肾小球滤过率（GFR）降低的发生率没有差异。
根据长期随访方案，我们评估了一组 160 名 SFK 患儿的队列，这些患儿通过产前或常规新生儿超声筛查发现（84 名患有 UKA，76 名患有 UMCDK）。监测高血压、蛋白尿和 GFR 降低作为肾脏损伤的标志物。我们比较了 UKA 和 UMCDK 亚组患儿的特征和结局。
42 名（26.2%）儿童的 GFR 降低，其中 41 名仅表现为轻度降低。22 名（13.8%）和 14 名（8.8%）儿童分别发现高血压和蛋白尿。57 名（35.6%）儿童存在联合肾损伤。终检时，UMCDK 和 UKA 亚组的 GFR 不同，UMCDK 患者的 GFR 明显更有可能正常，而 UKA 患者的 GFR 明显更有可能异常（82%比 67%；p=0.039）。
尽管程度较轻，但三分之一的儿童出现了 SFK 损伤的迹象。与 UKA 患者相比，UMCDK 患者的 GFR 降低更为常见，但在 UKA 和 UMCDK 患者中，SFK 中高滤过损伤或肾脏和泌尿道先天性异常（CAKUT）的发生率没有差异。
