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From post-infectious glomerulonephritis to complement-mediated aHUS: a diagnostic challenge.
Saeed Alzabali1, Reem AlRasheed2, Abdulkarim Alanazi2
1Pediatric Nephrology Section, Children's Specialized Hospital, King Fahad Medical City, Riyadh, Saudi Arabia. alzablis@ymail.com.
Pediatric Nephrology (Berlin, Germany)
|March 5, 2026
Summary
Post-infectious glomerulonephritis can trigger atypical hemolytic uremic syndrome in children with genetic complement pathway issues. Early complement inhibition with eculizumab is crucial for kidney recovery.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Complement System Biology
Background:
- Post-infectious glomerulonephritis (PIGN) and atypical hemolytic uremic syndrome (aHUS) share overlapping clinical features due to complement pathway dysregulation.
- Children with genetic predispositions are susceptible to severe complement-mediated kidney diseases.
Purpose of the Study:
- To report a case of PIGN triggering aHUS in a genetically susceptible child.
- To highlight the importance of recognizing aHUS in PIGN and the role of complement inhibition.
Main Methods:
- Case report of a 4-year-old boy presenting with PIGN.
- Monitoring of clinical and laboratory parameters, including kidney function and hematologic markers.
- Genetic testing for complement regulatory genes (CFHR1/CFHR3, CD46) and treatment with eculizumab.
Main Results:
- Initial PIGN diagnosis with impaired kidney function despite standard treatment.
- Development of thrombotic microangiopathy (TMA) and worsening kidney function.
- Rapid hematologic normalization and kidney recovery following eculizumab initiation.
- Identification of CFHR1/CFHR3 and CD46 mutations indicating aHUS susceptibility.
Conclusions:
- PIGN can precipitate aHUS in genetically predisposed children.
- Prompt recognition of TMA and initiation of complement inhibition (eculizumab) are vital for favorable outcomes.
- Genetic testing is essential for identifying underlying complement dysregulation in such cases.
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