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Prevalence and clinical correlates of glomerulopathy in children with sickle cell disease

D R Wigfall1, R E Ware, M R Burchinal

  • 1Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA.

The Journal of Pediatrics
|June 6, 2000
PubMed

Insights

Proteinuria, a marker of sickle nephropathy, affects children with sickle cell disease (SCD) early in life. Early detection of proteinuria in pediatric SCD patients is crucial for preventing kidney damage.

Area of Science:

  • Pediatric Nephrology
  • Hematology
  • Sickle Cell Disease Research

Background:

  • Sickle cell disease (SCD) is associated with significant kidney complications, including glomerular disease and renal failure.
  • Proteinuria is an early indicator of sickle nephropathy, but its prevalence and clinical associations in children with SCD remain unclear.

Purpose of the Study:

  • To determine the prevalence of proteinuria in children with SCD.
  • To investigate the clinical correlations of proteinuria in pediatric SCD patients.

Main Methods:

  • Prospective 10-year data collection on pediatric SCD patients, including physical measurements, lab results, and clinical complications.
  • Persistent proteinuria defined as >=1+ on urinalysis for at least 6 months.
  • Glomerular filtration rate (GFR) estimated using serum creatinine and height; proteinuria correlated with other variables using chi(2) analysis.

Main Results:

  • Proteinuria observed in 20 of 442 pediatric SCD patients (4.5%), with 15 (6.2%) having sickle cell anemia.
  • Proteinuria prevalence increased with age, affecting 12% of older teenagers with sickle cell anemia.
  • Proteinuria associated with lower hemoglobin, higher mean corpuscular volume, higher leukocyte count, and complications like stroke, acute chest syndrome, cholelithiasis, and hospitalizations. Early GFR hyperfiltration followed by normalization observed.

Conclusions:

  • Sickle nephropathy, indicated by persistent proteinuria, manifests early in life across all SCD types and correlates with disease severity.
  • Early identification of proteinuria in pediatric SCD patients is vital for timely therapeutic interventions to prevent progressive renal insufficiency.
Abstract

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