Sickle cell nephropathy

Renal Complications of Sickle Cell Syndrome in Southern Iran

“…Micro albuminuria, an early manifestation, has been reported in 30% -60% in SCD patients (5,6). Also proteinuria, which begins early in life and is associated with severity of disease, was seen in 20% -25% of SCD patients.…”

“…The patients have impaired urinary concentrating ability, supra normal proximal tubular function, defects in urinary acidification and potassium excretion (5). Young SCD patients have supra normal renal hemodynamics with elevations in both effective renal plasma flow and glomerular filtration rate (GFR).…”

“…Sickle cell anemia and the related hemoglobinopathies (including sickle β thalassemia which is common in southern Iran) causes micro vascular occlusion, which is manifested in most organ systems with a large spectrum of renal abnormalities (5). About 4% -12% of these patients will progress to end-stage renal disease (ESRD) and survival is significantly decreased in sickle cell patients who develop renal failure (5,6).…”

“…It is usually caused by inheritance of the sickle β globin gene, either in homozygous form (hemoglobin SS), Sickle trait (SA) or in combination with hemoglobin C (SC), β-thalassemia (Sβ-thalassemia) or several less common hemoglobin variants (1). Sickle cell anemia and the related hemoglobinopathies (including sickle β thalassemia which is common in southern Iran) causes micro vascular occlusion, which is manifested in most organ systems with a large spectrum of renal abnormalities (5). About 4% -12% of these patients will progress to end-stage renal disease (ESRD) and survival is significantly decreased in sickle cell patients who develop renal failure (5,6).…”

“…Sickle cell disease (SCD) is one of the most common genetic causes of morbidity and mortality in the world, especially in the central regions of Africa with an estimated 1 out of 500 births in African Americans (1)(2)(3)(4)(5). It is usually caused by inheritance of the sickle β globin gene, either in homozygous form (hemoglobin SS), Sickle trait (SA) or in combination with hemoglobin C (SC), β-thalassemia (Sβ-thalassemia) or several less common hemoglobin variants (1).…”