Scholarlink

Background <p>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.</p> Methods <p>NEPHRODREPA is a prospective pilot study including 17 patients (age 4–21&#xa0;years) followed at the University Hospital of Nice. In addition to the annual check-up, serum cystatin C and <sup>99m</sup>Tc-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.</p> Results <p>The median GFR measured by <sup>99m</sup>Tc-DTPA was 111 [102–118] mL/min/1.73 m<sup>2</sup>. 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.</p> Conclusion <p>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.</p> Graphical abstract <p></p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

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

  • 期刊论文

  • 发表日期：

    2026年1月4日

查看全文
  • Élise Larché,
  • Joy Benadiba,
  • Pierre Simon Rohrlich,
  • Camille Faudeux,
  • Etienne Bérard,
  • Fabrice Monpoux,
  • Caroline Grangeon,
  • Carine Cerato-Blanc,
  • Jennifer Battista,
  • Marie Meyronnet,
  • Jeanne Bernat,
  • Valeriia Rezapova,
  • Laurence Derain Dubourg,
  • Claus Peter Schmitt,
  • Julie Bernardor

摘要

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.

Graphical abstract