Abstract
Introduction: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).Objective:We examined circulating and urinary ACE 1 activity in children with SCD.Methods: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.Results: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.Conclusion: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.
Highlights
Sickle cell nephropathy begins in childhood and presents early increases in glomerular filtration, which, over the long term, can lead to chronic renal failure
The present study aimed to evaluate the modulation of serum and urinary angiotensin-converting enzymes 1 (ACE 1) and angiotensin converting enzyme (ACE) 2 activities in pediatric Sickle cell disease (SCD), important for understanding the role of these enzymes in the sickle cell nephropathy
There were no significant differences in the urinary albumin to urinary creatinine concentration ratio (UACR) between the SCD group and the Control Group
Summary
Sickle cell nephropathy begins in childhood and presents early increases in glomerular filtration, which, over the long term, can lead to chronic renal failure. Results: Urinary ACE activity was significantly higher in patients with SCD than in healthy children 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. Hyperfiltration is a risk factor for developing proteinuria and chronic kidney disease in SCD.[6] These renal changes may be accompanied by changes in the renin angiotensin aldosterone system (RAAS), and studies have described how SCD patients experience decreases in microalbuminuria and proteinuria with the use of angiotensin I-converting enzyme (ACE1) inhibitors[7]. Thrower et al (2019) demonstrated that patients with proteinuria who received RAAS blockade presented delayed loss of kidney function in patients with SCD8
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