Abstract: FR-PO0950
A Case of Complement-Mediated Membranoproliferative Glomerulonephritis in a Pediatric Patient
Session Information
- Pediatric Nephrology: Genetic Diseases, Development, Neonatal Nephrology, Glomerular Diseases, and More
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1800 Pediatric Nephrology
Authors
- Tugman, Matthew James, ECU Health, Greenville, North Carolina, United States
- Nguyen, Sophia T., ECU Health, Greenville, North Carolina, United States
- Gomez Mendez, Liliana Michelle, East Carolina University Brody School of Medicine, Greenville, North Carolina, United States
Introduction
Atypical hemolytic uremic syndrome is a rare disease of complement dysregulation that is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. It has high morbidity and mortality, but treatment outcomes have improved with the use of ravulizumab.
Case Description
A 13-year-old previously healthy female presented to the emergency department with periorbital edema and hypertension in the setting of a positive rapid test for Streptococcus pyogenes. The initial workup noted an elevated urine protein to creatinine ratio, hematuria, hypoalbuminemia, and a normal serum creatinine. She was diagnosed clinically with post-streptococcus glomerulonephritis and treated with amoxicillin and hydrochlorothiazide. The edema and hypertension improved, and she was discharged home with close follow-up arranged with pediatric nephrology. When her complement levels and proteinuria did not improve within three months, she was scheduled for a kidney biopsy that revealed findings consistent with immune complex-mediated membranoproliferative glomerulonephritis. She subsequently had genetic testing that showed contiguous gene deletions of CFHR1/CFHR3 and of CFHR1/CFHR4, which confirmed the diagnosis of atypical hemolytic uremic syndrome. She was started on ravulizumab treatment and has been responding well.
Discussion
This case is an example of how a complement mutation can cause membranoproliferative glomerulonephritis. Classically, the presentation of atypical hemolytic uremic syndrome is microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, but that was not the case for our patient. Genetic testing was key to the diagnosis and to starting appropriate complement blockade treatment with ravulizumab. The treatment has been effective as the hypoalbuminemia is resolved, and the proteinuria is nearly resolved.