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Chronic kidney disease and albuminuria in children with sickle cell disease
Marianne McPherson Yee1, Shameem F Jabbar, Ifeyinwa Osunkwo
1Department of Pediatrics, Division of Hematology/Oncology, Emory University School of Medicine, Atlanta, GA 30322, USA. Marianne.McPherson@choa.org
Insights
Sickle cell nephropathy is common in children, with high rates of albuminuria and chronic kidney disease (CKD) detected early. Early detection of kidney damage in sickle cell disease (SCD) is crucial for preventing future renal failure.
Area of Science:
- Pediatric Nephrology
- Hematology
- Genetics
Background:
- Sickle cell nephropathy is a progressive complication of sickle cell disease (SCD), often beginning in childhood.
- Albuminuria serves as an early indicator of glomerular damage and potential chronic kidney disease (CKD).
Purpose of the Study:
- To determine the prevalence of albuminuria and CKD in pediatric patients with SCD.
- To identify clinical factors associated with albuminuria and CKD in this population.
Main Methods:
- A cross-sectional study of 410 pediatric SCD patients (ages 2-21) over 10 years.
- Measurement of urinary albumin-to-creatinine ratio (ACR) and serum creatinine.
- Classification of CKD stages based on Kidney Disease: Improving Global Outcomes (KDIGO) criteria.
Main Results:
- Abnormal albuminuria was present in 20.7% of patients, higher in HbSS/HbSβ(0) (23.0%) than HbSC/HbSβ(+) (16.8%).
- CKD was identified in 26.5% of patients, with stages 1 and 2 being most common.
- Increasing age and HbSC/HbSβ(+) genotype were associated with CKD in multivariate analysis.
Conclusions:
- This study is the first to stage CKD in children with SCD, revealing a high early prevalence of albuminuria and glomerular injury.
- Early detection and staging of CKD in children with SCD are vital for timely interventions to prevent adult renal failure.
Background And Objectives:
Sickle cell nephropathy begins in childhood and may progress to renal failure. Albuminuria is a sensitive marker of glomerular damage that may indicate early chronic kidney disease (CKD).
Design, Setting, Participants, & Measurements:
The aims of this study were to determine the cross-sectional prevalence and clinical correlates of albuminuria and CKD among children with sickle cell disease (SCD). Over a 10-year period (1995 to 2005) 410 pediatric SCD patients ages 2 to 21 years were enrolled: 261 with hemoglobin SS (HbSS) or HbSβ(0) thalassemia (HbSβ(0)) and 149 with HbSC or HbSβ(+) thalassemia (HbSβ(+)). The albumin/creatinine ratio (ACR) of spot-urine specimens and serum creatinine were measured; abnormal albuminuria was defined as urinary ACR ≥ 30 mg/g.
Results:
The prevalence of abnormal albuminuria was 20.7% (23.0% in HbSS/HbSβ(0), 16.8% in HbSC/HbSβ(+)). Among HbSS/HbSβ(0), abnormal albuminuria was associated with increasing age and lower baseline hemoglobin. GFR, estimated in 189 patients using the updated Schwartz formula, correlated negatively with age (r = -0.27, P = 0.0002). CKD defined according to the Kidney Disease: Improving Global Outcomes study was present in 26.5% (50 of 189) of patients: stage 1 in 27 (14.8%) and stage 2 in 22 (11.6%). In multivariate analysis, age and HbSC/HbSβ(+) genotype were associated with CKD.
Conclusions:
This is the first study to stage CKD in children with SCD and highlights a high prevalence of albuminuria and glomerular injury early in life. Detecting CKD in childhood could allow for earlier intervention and prevention of renal failure in adulthood.
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