A pediatric case series of 4 children with steroid-resistant nephrotic syndrome (SRNS) found that thrombotic microangiopathy (TMA) presented with unexplained worsening kidney function and new-onset hypertension; 3 of 4 treated with eculizumab had hematologic improvement, but none recovered kidney function. In the reported study, all patients were negative for shiga toxin, had normal ADAMTS13 activity, and had no recurrent TMA after kidney transplant among the 3 who underwent transplantation.

Why It Matters To Your Practice

• TMA is a rare but important complication to consider in children with SRNS, especially in late-stage chronic kidney disease.

• Clinical clues included worsening renal function, new HTN, hemolytic anemia, and thrombocytopenia.

• This presentation can mimic other causes of CKD progression, so delayed recognition is a risk.

Clinical Benefits

• Earlier recognition may help prompt hematologic evaluation and nephrology escalation.

• Eculizumab improved hemolytic markers in 3 of 4 children, suggesting potential benefit for hematologic control.

• Among 3 children who received kidney transplants, none had recurrent TMA.

Managing Risks

• Do not assume kidney decline in SRNS is only disease progression when new HTN or cytopenias appear.

• Eculizumab did not restore kidney function in this series, so set expectations accordingly.

• Workup in these cases was negative for shiga toxin, ADAMTS13 deficiency, and complement dysregulation, underscoring the need for broad differential assessment.

The Bottom Line

• In pediatric SRNS, unexplained kidney worsening plus new HTN should raise concern for TMA.

• Treatment may improve hemolysis without reversing renal injury.

• Available case data suggest post-transplant recurrence may be low in this entity.