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Kidney function monitoring in pediatric sickle cell disease: evidence from the NEPHRODREPA study

Élise Larché1,2, Joy Benadiba3, Pierre Simon Rohrlich2,3

  • 1Service de Néphrologie Pédiatrique, CHU de Nice, Hôpital Archet, 151 Route Saint-Antoine de Ginestière, 06202, Nice, France.

Pediatric Nephrology (Berlin, Germany)
|January 4, 2026
PubMed

Insights

Estimating kidney function in children with sickle cell disease (SCD) is crucial. The CKiDU25 equation using cystatin C best approximates isotopic glomerular filtration rate (GFR) measurements in young SCD patients.

Area of Science:

  • Nephrology
  • Pediatrics
  • Hematology

Background:

  • Sickle cell disease (SCD) is associated with early-onset kidney abnormalities, including glomerular hyperfiltration and progression to chronic kidney disease.
  • Accurate estimation of glomerular filtration rate (GFR) in pediatric SCD patients is challenging due to limitations of traditional creatinine-based formulas.

Purpose of the Study:

  • To evaluate kidney function in pediatric and young adult SCD patients.
  • To compare isotopic GFR measurements with various creatinine and cystatin C-based estimation equations.

Main Methods:

  • Prospective pilot study (NEPHRODREPA) involving 17 patients aged 4-21 years.
  • Measured serum cystatin C and 99mTc-DTPA plasmatic clearance for isotopic GFR.
  • Estimated GFR using 2009 Schwartz and CKiDU25 (creatinine, cystatin C, or both) equations.

Main Results:

  • Isotopic GFR measurement showed a median of 111 mL/min/1.73 m².
  • Creatinine-based formulas (Schwartz 2009, CKiDU25) overestimated GFR, while the cystatin C-only CKiDU25 equation showed the lowest bias (5%).
  • Prevalent early tubular dysfunction (hyposthenuria) and renin-angiotensin-aldosterone system imbalance were observed.

Conclusions:

  • The CKiDU25 equation utilizing serum cystatin C provides reliable GFR estimates in young SCD patients without diagnosed nephropathy.
  • Early detection of tubular dysfunction in SCD warrants further investigation and potential therapeutic interventions.
  • Larger cohort studies are needed to confirm these findings.
Abstract

Keywords:
ChildrenChronic kidney diseaseHyposthenuriaKidney functionSickle cell disease

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