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Published on: March 14, 2017
Proteinuria in children with sickle cell disease
Olivera Marsenic1, Kevin G Couloures, Joseph M Wiley
1Children's Hospital of Philadelphia, Nephrology, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA. oljamc@mac.com
Summary
Microalbuminuria alone does not detect early kidney damage in children with sickle cell disease (SCD). Testing for total protein and immunoglobulin G (IgG) excretion is recommended for earlier detection and prevention of renal injury.
Area of Science:
- Nephrology
- Pediatrics
- Hematology
Background:
- Sickle cell nephropathy presents with proteinuria, potentially leading to renal failure.
- Microalbuminuria is a marker for glomerular damage, but other proteinuria types in pediatric sickle cell disease (SCD) are understudied.
- Characterizing proteinuria beyond microalbuminuria is crucial for early renal damage recognition and prevention in children with SCD.
Purpose of the Study:
- To characterize glomerular permselectivity and tubular proteinuria in children with sickle cell disease (SCD).
- To assess the utility of albumin, immunoglobulin G (IgG), and retinol-binding protein (RBP) as markers of renal damage in pediatric SCD.
- To identify limitations of microalbuminuria in detecting early renal abnormalities in this population.
Main Methods:
- Investigated 32 stable pediatric patients with sickle cell disease (HbSS) with normal renal function and negative dipstick proteinuria.
- Measured urinary albumin, IgG, and RBP using nephelometry to assess glomerular charge and size selectivity and proximal tubular dysfunction.
- Utilized established values from healthy subjects for comparison, as no control group was included.
Main Results:
- Elevated total protein excretion was found in 41% of patients; 61.5% of these had proteinuria not detected by microalbuminuria testing.
- Increased microalbuminuria was present in 25% of patients, with IgG excretion elevated in 16%, often co-occurring.
- Elevated RBP excretion (tubular dysfunction) was observed in 16% of patients, independent of glomerular damage markers and primarily in younger children.
Conclusions:
- Early glomerular damage, involving both size and charge selectivity, occurs in children with SCD.
- Microalbuminuria alone is insufficient for detecting early renal damage in pediatric SCD.
- Testing for total protein and IgG excretion, in addition to albumin, is recommended for comprehensive assessment and timely intervention in children with SCD.
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