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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 CKD: An Update
Rima S Zahr1, Santosh L Saraf2
1Division of Pediatric Nephrology and Hypertension, University of Tennessee Health Science Center, Memphis, Tennessee, USA.
American Journal of Nephrology
|October 29, 2023
Summary
Sickle cell disease commonly affects kidneys, leading to early damage like loss of urine concentration and hyperfiltration. Targeted therapies and research are crucial for improving kidney health and survival in affected individuals.
Area of Science:
- Nephrology
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) affects millions globally, causing organ damage through vaso-occlusion and hemolysis.
- Kidneys are frequently impacted in SCD, leading to significant morbidity.
Purpose of the Study:
- To summarize the renal manifestations and management of sickle cell disease-related kidney damage.
- To highlight genetic risk factors and current therapeutic limitations.
Main Methods:
- Literature review of renal complications in sickle cell disease.
- Analysis of genetic associations (APOL1, HMOX1, α-thalassemia) with sickle nephropathy.
- Evaluation of current treatment strategies and transplantation outcomes.
Main Results:
- Early kidney damage includes medullary ischemia, impaired urine concentration, and hyperfiltration.
- Progressive damage involves albuminuria and declining glomerular filtration rate.
- APOL1 variants increase sickle nephropathy risk, while α-thalassemia may offer protection.
Conclusions:
- Kidney disease significantly contributes to mortality in sickle cell disease patients.
- Current therapies have limited efficacy data; novel treatments are needed.
- Kidney transplantation improves survival but is underutilized.
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