Pediatric nephrology (Berlin, Germany) · 2026
Kidney function monitoring in pediatric sickle cell disease: evidence from the NEPHRODREPA study.
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Larché Élise, Benadiba Joy, Rohrlich Pierre Simon, Faudeux Camille, Bérard Etienne, Monpoux Fabrice, Grangeon Caroline, Cerato-Blanc Carine, Battista Jennifer, Meyronnet Marie, Bernat Jeanne, Rezapova Valeriia, Dubourg Laurence Derain, Schmitt Claus Peter, Bernardor Julie
Аннотация
BACKGROUND: Sickle cell disease (SCD) induces early kidney abnormalities, beginning in childhood with glomerular hyperfiltration and progressing toward chronic kidney disease. Estimating glomerular filtration rate (GFR) in these patients remains challenging due to limitations of creatinine-based formulas. This study aimed to assess kidney function in children and young adults with SCD using isotopic GFR measurement and various estimation equations, including creatinine and cystatin C-based formulas.
METHODS: NEPHRODREPA is a prospective pilot study including 17 patients (age 4-21 years) followed at the University Hospital of Nice. In addition to the annual check-up, serum cystatin C and 99mTc-DTPA plasmatic clearance were measured. GFR was estimated using the 2009 Schwartz formula, and the CKiDU25 equations based on creatinine, cystatin C, or both. Additional kidney markers were assessed.
RESULTS: The median GFR measured by 99mTc-DTPA was 111 [102-118] mL/min/1.73 m2. The Schwartz 2009 and CKiDU25 creatinine-based formulas overestimated GFR by 23% and 17%, respectively. The CKiDU25 combined formula overestimated by 10%, while the cystatin C-only formula appeared closer to measured GFR (5%); it yielded the lowest mean bias and showed higher dispersion than the combined equation. Hyposthenuria was observed in 7/17 patients and renin-angiotensin-aldosterone system imbalance in 8/17, with elevated blood pressure in two cases.
CONCLUSION: This study suggests that in young patients with SCD without known nephropathy, the CKiDU25 equation using serum cystatin C, provides GFR estimates close to the gold standard isotopic measurement. Early tubular dysfunction is prevalent and may justify therapeutic interventions. These findings warrant confirmation in larger cohorts.
METHODS: NEPHRODREPA is a prospective pilot study including 17 patients (age 4-21 years) followed at the University Hospital of Nice. In addition to the annual check-up, serum cystatin C and 99mTc-DTPA plasmatic clearance were measured. GFR was estimated using the 2009 Schwartz formula, and the CKiDU25 equations based on creatinine, cystatin C, or both. Additional kidney markers were assessed.
RESULTS: The median GFR measured by 99mTc-DTPA was 111 [102-118] mL/min/1.73 m2. The Schwartz 2009 and CKiDU25 creatinine-based formulas overestimated GFR by 23% and 17%, respectively. The CKiDU25 combined formula overestimated by 10%, while the cystatin C-only formula appeared closer to measured GFR (5%); it yielded the lowest mean bias and showed higher dispersion than the combined equation. Hyposthenuria was observed in 7/17 patients and renin-angiotensin-aldosterone system imbalance in 8/17, with elevated blood pressure in two cases.
CONCLUSION: This study suggests that in young patients with SCD without known nephropathy, the CKiDU25 equation using serum cystatin C, provides GFR estimates close to the gold standard isotopic measurement. Early tubular dysfunction is prevalent and may justify therapeutic interventions. These findings warrant confirmation in larger cohorts.