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Updated: May 5, 2026

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Glomerular filtration rate is altered in children with sickle cell disease: a comparison between Hb SS and Hb SC
Rafael Pereira de Paula1, Alana Ferreira Nascimento, Sandra Mara Bispo Sousa
1Universidade Estadual do Sudoeste da Bahia - UESB, Jequié, BA, Brazil.
Insights
Glomerular hyperfiltration, a precursor to renal failure in sickle cell disease, is present in children. This condition is more pronounced in homozygotic (SS) sickle cell disease patients than heterozygotic (SC) patients.
Area of Science:
- Pediatric Nephrology
- Hematology
- Sickle Cell Disease Research
Background:
- Renal failure is a common complication in older adults with sickle cell disease (SCD).
- Subclinical glomerular hyperfiltration often precedes renal failure in SCD.
- Limited data exists on renal function in children with SCD, particularly comparing different genotypes.
Purpose of the Study:
- To investigate and compare the glomerular filtration rate (GFR) in children with sickle cell disease.
- To differentiate renal function between heterozygotic (SC) and homozygotic (SS) pediatric SCD patients.
Main Methods:
- Evaluated the GFR in 11 children diagnosed with sickle cell disease.
- Included 7 homozygotic (SS) and 4 heterozygotic (SC) patients, with a mean age of 11 years (±5 years).
- Employed standard laboratory techniques for GFR assessment and descriptive analysis for results.
Main Results:
- Glomerular hyperfiltration was observed in pediatric patients with sickle cell disease.
- The degree of glomerular hyperfiltration was notably higher in homozygotic (SS) children compared to heterozygotic (SC) children.
Conclusions:
- Findings indicate the presence of glomerular hyperfiltration in children with sickle cell disease.
- Highlights the need for regular renal function monitoring in pediatric SCD patients.
- Emphasizes increased vigilance for homozygotic (SS) patients due to higher risk.
Background:
Renal failure is common among older patients with sickle cell disease; this is preceded by subclinical glomerular hyperfiltration. Data about renal function of adults with sickle cell disease have been reported, but data on children is scarce, especially when comparing heterozygotic and homozygotic patients.
Objective:
The goal of this study was to investigate the glomerular filtration rate of heterozygotic and homozygotic children with sickle cell disease.
Methods:
The glomerular filtration rate of 11 children with sickle cell disease [7 homozygotic (SS) and 4 heterozygotic (SC)] with a mean age of 11 years (standard deviation: ± 5 years) was evaluated using standard laboratory techniques. Results are presented as descriptive analysis.
Results:
Our results suggest that glomerular hyperfiltration is present in children with sickle cell disease; this is more evident in homozygotic than heterozygotic children.
Conclusion:
There is evidence of a need to monitor the renal function of children with sickle cell disease when special attention should be paid to homozygotic patients.
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