Sickle Hemoglobin-Related Nephropathy: an Overview on Pathophysiology, Diagnosis, and Treatment
Journal of Pediatric Nephrology,
Vol. 7 No. 4 (2019),
29 December 2019
Sickle cell disease (SCD) is a common hemoglobinopathy in the world with progressive multi organ failure. Many organs have been affected in this disease, but sickle cell nephropathy (SCN) is a major complication which affects the quality of life in these patients. SCN has a wide range of renal manifestations such as asymptomatic microalbuminuria, hyposthenuria, hematuria, frank proteinuria, nephrotic syndrome and end stage renal disease (ESRD). Nowadays novel biomarkers such as N-acetyl-beta-D-glucosaminidase (NAG), kidney injury molecule-1 (KIM-1) and transforming growth factor β (TGF-β) has allowed the early detection of the kidney involvement in sickle cell disease. The angiotensin converting enzyme (ACE) inhibitor drugs such as captopril or enalapril and also ACE receptor blockers (losartan) have beneficial effects in albuminuria or proteinuria. Also endothelin-1 (ET-1) receptor antagonists have a promising role in glomerular injury in SCD. In sickle patients who develop ESRD, renal replacement therapy can be life- saving. In recent years, kidney transplantation is the only curative treatment for advanced chronic kidney disease in these patients.
Keywords: Sickle cell; Nephropathy; Kidney.
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