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Pathology of the glomerulus in sickle cell anemia with and without nephrotic syndrome
Insights
Sickle cell disease (SS) can cause kidney damage, including glomerular basement membrane reduplication and enlargement, leading to nephrotic syndrome (NS). These kidney changes were observed in patients with SS and NS, and to a lesser extent in SS without NS.
Area of Science:
- Nephrology
- Hematology
- Pathology
Background:
- Sickle cell disease (SS) is a genetic blood disorder with known renal complications.
- Nephrotic syndrome (NS) is a kidney disorder characterized by protein in the urine.
Purpose of the Study:
- To compare the glomerular histology and quantitative features in patients with sickle cell disease and nephrotic syndrome to those with sickle cell disease without nephrotic syndrome, sickle cell trait, other hemoglobinopathies, and normal controls.
- To investigate the relationship between sickle cell disease and the development of nephrotic syndrome.
Main Methods:
- Histological examination of glomeruli from patients with various hemoglobinopathies and controls.
- Quantitative analysis of glomerular size and glomerular area per unit area of cortex.
- Comparison of findings between groups.
Main Results:
- Five of six patients with SS and NS showed extensive glomerular basement membrane reduplication and mild mesangial proliferation.
- Similar, but milder, glomerular lesions were found in SS patients without NS, but not in sickle cell trait (SCT) or controls.
- Glomeruli in SS were significantly larger (>70%) than in SCT and controls.
- Mean total glomerular area per unit area of cortex was significantly greater in SS with normal BUN compared to SCT and controls.
Conclusions:
- Nephrotic syndrome in sickle cell disease is likely secondary to the effects of the disease.
- Sickle cell disease is associated with significant glomerular hypertrophy and characteristic histologic lesions.
- The findings suggest underlying mechanisms for glomerular pathology in sickle cell disease.
Abstract:
Glomeruli from 6 cases of sickle cell disease (SS) with the nephrotic syndrome (NS) were compared histologically and quantitatively with glomeruli from 9 cases of SS, 10 cases of sickle cell trait (SCT), 4 cases of other hemoglobinopathies, all without NS, and normal controls. Five of 6 patients with SS and NS had extensive reduplication of their glomerular basement membranes and mild mesangial proliferation. Similar but milder lesions occurred in SS without NS but not in SCT or controls. Incidental renal disease occurred in 1 patient with SS and NS. Nephrotic syndrome was probably secondary to effects of sickle cell disease. Glomeruli in SS were significantly larger (>70%) than in SCT and controls. Mean total glomerular area per unit area of cortex in SS with normal BUN significantly exceeded that of SCT, which, in turn, was significantly greater than that of controls. Mechanisms for the histologic lesions and hypertrophy of the glomeruli were suggested.