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Abstract: FR-PO0725

Sparsentan Ameliorates Proteinuria in Children with Glomerular Diseases

Session Information

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Wang, Fang, Department of Pediatrics, Children's Medical Center, Peking University First Hospital, Beijing, China
  • Zhang, Yanqin, Department of Pediatrics, Children's Medical Center, Peking University First Hospital, Beijing, China
  • Ding, Jie, Department of Pediatrics, Children's Medical Center, Peking University First Hospital, Beijing, China
Background

It is unclear whether Sparsentan can reduce nephrotic-range proteinuria as measured by 24-hour urine protein excretion. Here, we present data from a case series of children with glomerular diseases, examining the early efficacy and safety of Sparsentan.

Methods

This observational case series began with four patients aged 10–17 years with a diagnosis of Alport syndrome or hereditary nephrotic syndrome. Prior to the start of Sparsentan, the patients were stopped from taking angiotensin-converting-enzyme inhibitor (ACEi), angiotensin II receptor blockers (ARB), finerenone and dapagliflozin. Twenty-four-hour urine samples were collected for analysis of 24-hour urine protein excretion (24-h UP), 24-hour urine albumin excretion, the protein-to-creatinine ratio (PCR) and the albumin-to-creatinine ratio (ACR). Kidney function and blood pressure were measured at each visit.

Results

Case 1 developed nephrotic-range proteinuria (24-h UP 5.72 g/d, PCR 10.23 g/g, ACR 8338.17 mg/g) after receiving an ACEi for five months. However, after being treated with 400 mg of Sparsentan once daily for 3 weeks, proteinuria significantly decreased by 33%-49%. Case 2 had nephrotic-range proteinuria (24-h UP 16.88 g/d, PCR 16.44 g/g, ACR 12265.5 mg/g) after receiving ACEi and ARB for 10 months and finerenone for three months. Treatment with Sparsentan at a dose of 400 mg once daily for three weeks reduced proteinuria by 43.8%-55.3%. Case 3, who was treated with dapagliflozin for 13.5 months and ARB and finerenone for 9 months, did not improve with regard to nephrotic-range proteinuria (24-h UP of 6.84 g/d and 24-h urine albumin excretion of 6.471 g/d) . However, treatment with Sparsentan at a dose of 400 mg once daily for 4 weeks reduced proteinuria by 66.5% and 71%. Case 4 had a 24-hour UP of 8.575 g/d after receiving an ACEi with or without an ARB for 13 months and finerenone for seven months. Treatment with Sparsentan at a dose of 400 mg once daily for four weeks reduced proteinuria by 74%. Cases 1, 2 and 4 had normal kidney function. Kidney dysfunction in case 3 remained stable. All cases had normal blood pressure. No one had oedema.

Conclusion

Significant reductions in proteinuria were observed in children with glomerular diseases, even after 3-4 weeks of treatment with sparsentan.

Digital Object Identifier (DOI)