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Renal lesions in sickle cell nephropathy in children
Insights
Sickle cell nephropathy can cause glomerular damage, including focal segmental glomerulosclerosis and mesangial proliferation. This study suggests hyperfiltration may contribute to these kidney lesions in children with sickle cell disease.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Hematology
Background:
- Sickle cell nephropathy involves glomerular lesions, which are less studied than tubular alterations in sickle cell disease.
- Proteinuria and nephrotic syndrome are key indicators of sickle cell nephropathy.
Purpose of the Study:
- To investigate the spectrum of glomerular lesions in pediatric sickle cell nephropathy.
- To explore the relationship between clinical presentation and histological findings in these patients.
Main Methods:
- Retrospective review of 13 children with sickle cell nephropathy over 14 years.
- Analysis of clinical data, including age at onset, nephrotic syndrome, and renal clearances.
- Histopathological examination of kidney biopsies.
Main Results:
- Eight children had focal and segmental glomerulosclerosis (FSGS), and five had mesangial proliferation.
- FSGS patients were older and more frequently presented with nephrotic syndrome (p<0.05).
- Supranormal renal clearances (suggesting hyperfiltration) were observed in all mesangial proliferation and half of FSGS patients.
Conclusions:
- Sickle cell disease can lead to FSGS and mesangial proliferation in the glomeruli.
- Hyperfiltration may be a contributing factor to the development of these glomerular lesions, similar to diabetic nephropathy.
Abstract:
Sickle cell nephropathy characterized by proteinuria and predominantly glomerular lesions has not been studied as extensively as renal tubular alterations in sickle cell disease. We reviewed our experience with this entity over a 14-year period. Of 13 children with either proteinuria or the nephrotic syndrome, 8 showed focal and segmental glomerulosclerosis, and 5 had mesangial proliferation. Children with focal and segmental glomerulosclerosis were older at onset of nephropathy and presented with the nephrotic syndrome more frequently than those with mesangial proliferation (p less than 0.05). All patients with mesangial proliferation and half of the focal and segmental glomerulosclerosis patients had supranormal renal clearances at onset of nephropathy suggesting hyperfiltration. Hyperfiltration seen in animals with reduced renal mass, and in human diabetic nephropathy before reduction in nephron units leads to mesangial proliferation and sclerosis. Our study suggests that sickle cell disease produces similar lesions in patients with sickle cell nephropathy.