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Early Predictors of Renal Dysfunction in Pediatric Patients with Sickle Cell Disease

Mohamed Abdelaziz El-Gamasy1, Wageh S El-Naghy2

  • 1Department of Pediatrics, Faculty of Medicine, Tanta University, Egypt.

Indian Journal of Nephrology
|March 1, 2019
PubMed

Insights

Serum cystatin-C and beta 2 microglobulin are effective early biomarkers for detecting kidney dysfunction in children with sickle cell disease (SCD). These markers show higher sensitivity and specificity than traditional creatinine tests.

Area of Science:

  • Nephrology
  • Pediatric Hematology
  • Biomarker Discovery

Background:

  • Sickle cell disease (SCD) is a genetic disorder causing hemolytic anemia and vaso-occlusion, leading to chronic organ damage, particularly affecting the urinary system.
  • Early detection of glomerular and renal tubular dysfunction in pediatric SCD patients is crucial for timely intervention and management.
  • Traditional markers like serum creatinine may not be sufficiently sensitive for early detection of renal impairment in SCD.

Purpose of the Study:

  • To measure serum levels of cystatin-C and beta 2 microglobulin in pediatric patients with sickle cell disease (SCD).
  • To evaluate the significance of cystatin-C and beta 2 microglobulin as early biomarkers for glomerular and/or renal tubular dysfunction in SCD.
  • To compare the diagnostic accuracy of cystatin-C and beta 2 microglobulin with serum creatinine in this population.

Main Methods:

  • A case-control study involving 70 children with SCD and 40 healthy age- and sex-matched controls.
  • Comprehensive laboratory investigations included blood urea, serum creatinine, serum ferritin, eGFR, creatinine clearance, urinary albumin/creatinine ratio, serum cystatin-C, and β-2 microglobulin.
  • Statistical analysis to compare marker levels between groups and assess correlations with clinical parameters and diagnostic accuracy.

Main Results:

  • Pediatric SCD patients exhibited significantly elevated serum cystatin-C and β-2 microglobulin levels compared to controls.
  • These elevated levels positively correlated with markers of renal dysfunction (blood urea, serum creatinine, urinary albumin/creatinine ratio) and disease severity (ferritin, transfusion frequency, iron chelation duration).
  • Serum cystatin-C and β-2 microglobulin demonstrated higher sensitivity and specificity (91%, 90% and 85.7%, 100% respectively) for detecting renal dysfunction than serum creatinine (79%, 85% respectively).

Conclusions:

  • Serum cystatin-C and β-2 microglobulin are sensitive and specific early biomarkers for assessing glomerular and tubular dysfunction in children with SCD.
  • These markers offer superior diagnostic performance compared to serum creatinine for early renal impairment detection in pediatric SCD.
  • Routine monitoring of cystatin-C and β-2 microglobulin may improve the management of renal complications in children with sickle cell disease.

Keywords:
Cystatin-CSickle cell diseasebeta 2microglobulin

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