Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Pediatria, Disciplina de Nefrologia, São Paulo, SP, Brasil.
Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Medicina, Disciplina de Nefrologia, São Paulo, SP, Brasil.
J Bras Nefrol. 2021 Jul-Sep;43(3):303-310. doi: 10.1590/2175-8239-JBN-2020-0174.
Sickle cell nephropathy begins in childhood and presents early increases in glomerular filtration, which, over the long term, can lead to chronic renal failure. Several diseases have increased circulating and urinary angiotensin-converting enzyme (ACE) activity, but there is little information about changes in ACEs activity in children with sickle cell disease (SCD).
We examined circulating and urinary ACE 1 activity in children with SCD.
This cross-sectional study compared children who were carriers of SCD with children who comprised a control group (CG). Serum and urinary activities of ACE were evaluated, as were biochemical factors, urinary album/creatinine rates, and estimated glomerular filtration rate.
Urinary ACE activity was significantly higher in patients with SCD than in healthy children (median 0.01; range 0.00-0.07 vs median 0.00; range 0.00-0.01 mU/mL·creatinine, p < 0.001. No significant difference in serum ACE activities between the SCD and CG groups was observed (median 32.25; range 16.2-59.3 vs median 40.9; range 18.0-53.4) mU/m`L·creatinine, p < 0.05.
Our data revealed a high urinary ACE 1 activity, different than plasmatic level, in SCD patients suggesting a dissociation between the intrarenal and systemic RAAS. The increase of urinary ACE 1 activity in SCD patients suggests higher levels of Ang II with a predominance of classical RAAS axis, that can induce kidney damage.
镰状细胞肾病始于儿童期，表现为肾小球滤过率的早期增加，长期来看，这可能导致慢性肾衰竭。几种疾病会增加循环和尿液中的血管紧张素转换酶（ACE）活性，但关于镰状细胞病（SCD）患儿 ACE 活性的变化知之甚少。
我们检测了 SCD 患儿的循环和尿液 ACE 1 活性。
这项横断面研究比较了 SCD 携带者患儿和对照组（CG）患儿。评估了血清和尿液 ACE 活性、生化因素、尿白蛋白/肌酐比和估算肾小球滤过率。
SCD 患儿的尿液 ACE 活性明显高于健康儿童（中位数 0.01；范围 0.00-0.07 与中位数 0.00；范围 0.00-0.01 mU/mL·肌酐，p < 0.001）。SCD 和 CG 组之间血清 ACE 活性无显著差异（中位数 32.25；范围 16.2-59.3 与中位数 40.9；范围 18.0-53.4 mU/m`L·肌酐，p < 0.05）。
我们的数据显示 SCD 患者的尿液 ACE 1 活性升高，与血浆水平不同，提示肾内和全身 RAAS 之间存在分离。SCD 患者尿液 ACE 1 活性增加表明 Ang II 水平升高，经典 RAAS 轴占优势，可能导致肾脏损伤。
