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Sickle cell disease nephropathy: an update on risk factors and potential biomarkers in pediatric patients
André R Belisário1,2, Ariadna As da Silva2, Cristiane Vm Silva2
1Centro de Tecidos Biológicos de Minas Gerais, Fundação Hemominas, Rua das Goiabeiras, 779, Lagoa Santa, Minas Gerais 33400-000, Brazil.
Insights
Sickle cell nephropathy is a major complication of sickle cell disease (SCD). Early detection and treatment, particularly in children, are crucial for managing kidney complications in SCD patients.
Area of Science:
- Nephrology
- Hematology
- Pediatrics
Background:
- Sickle cell disease (SCD) frequently leads to chronic kidney complications, known as sickle cell nephropathy.
- Understanding the early signs and progression of this complication is vital for timely intervention.
Purpose of the Study:
- To review the pathophysiology, natural history, clinical signs, risk factors, biomarkers, and treatments for sickle cell nephropathy.
- To focus on studies involving pediatric patients with SCD.
Main Methods:
- Literature review focusing on pediatric sickle cell nephropathy.
- Analysis of studies on early renal function changes and potential biomarkers.
- Evaluation of current and emerging therapeutic strategies.
Main Results:
- Early signs of renal disease in SCD include increased glomerular filtration rate and microalbuminuria, observable even in childhood.
- Potential early biomarkers for renal dysfunction in SCD include Nephrin, KIM-1, VGFs, chemokines, and renin-angiotensin system molecules.
- Renin-angiotensin system inhibitors and angiotensin receptor blockers show promise for albuminuria control in adults, but pediatric data is limited.
Conclusions:
- Early detection of renal dysfunction in pediatric SCD patients is critical.
- Further research is needed to establish optimal renoprotective strategies and treatment timing in children with SCD.
Abstract:
One of the major chronic complications of sickle cell disease (SCD) is sickle cell nephropathy. The aim of this review is to discuss the pathophysiology, natural history, clinical manifestations, risk factors, biomarkers and therapeutic approaches for sickle cell nephropathy, focusing on studies with pediatric patients. The earliest manifestation of renal disease is an increase in the glomerular filtration rate. A finding that may also be observed in early childhood is microalbuminuria. Nephrin, KIM-1, VGFs, chemokines and renin-angiotensin system molecules have emerged as potential early markers of renal dysfunction in SCD. In regards to a therapeutic approach, renin-angiotensin system inhibitors and angiotensin receptor blockers seem to be effective for the control of albuminuria in adults with SCD, although new studies in children are needed. The precise moment to begin renoprotection in SCD patients who should be treated remains to be determined.
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