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Updated: Sep 5, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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Sickle Cell Disease and Kidney.

Pooja Amarapurkar1, Levard Roberts1, Jose Navarrete1

  • 1Division of Renal Medicine, Emory University School of Medicine, Atlanta, GA.

Advances in Chronic Kidney Disease
|July 11, 2022
PubMed
Summary

Sickle cell disease (SCD) can cause significant kidney problems, including albuminuria and chronic kidney disease. Early screening and management are crucial for better outcomes in patients with SCD kidney disease.

Keywords:
AlbuminuriaGlomerular hyperfiltrationSickle cell diseaseSickle cell nephropathyVaso-occlusion

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Area of Science:

  • Nephrology
  • Hematology
  • Genetics

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder with diverse kidney manifestations.
  • These include impaired urine concentration, electrolyte imbalances, albuminuria, acute kidney injury, and chronic kidney disease.
  • Mechanisms involve glomerular hyperfiltration, tubular dysfunction, endothelial damage, and inflammation.

Purpose of the Study:

  • To review the kidney manifestations of sickle cell disease.
  • To discuss the underlying mechanisms of kidney damage in SCD.
  • To outline screening, treatment, and management strategies for SCD-related kidney disease.

Main Methods:

  • Literature review of studies on sickle cell disease and kidney disease.
  • Analysis of pathophysiological mechanisms contributing to renal damage.
  • Synthesis of current recommendations for screening and management.

Main Results:

  • Albuminuria is a common and progressive feature of SCD-related kidney disease.
  • Kidney disease in SCD is linked to increased mortality.
  • Annual proteinuria screening from age 10, ACE inhibitors, and hydration are key interventions.

Conclusions:

  • Kidney disease is a major complication of sickle cell disease, impacting patient survival.
  • Early detection through screening and proactive management, including specific hemoglobin targets and multidisciplinary care, can delay disease progression.
  • Controlling sickle cell disease and avoiding nephrotoxic agents are vital for preserving kidney function.