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Favorable Outcome With Eculizumab in Hemolytic Uremic Syndrome Presenting With Severe Neurological Complications: A

Maryam Zaitoun1, Abdulkarim Alanazi2, Tasneem Kattash1

  • 1College of Medicine, Alfaisal University, Riyadh, Saudi Arabia, alfaisal.edu.

Case Reports in Nephrology
|August 5, 2026
PubMed
Abstract

Insights

This case study shows that severe neurological symptoms can be an early sign of hemolytic uremic syndrome (HUS). Eculizumab treatment was life-saving for a child with HUS and neurological involvement, though recovery was slow.

Area of Science:

  • Pediatric Nephrology
  • Hematology
  • Neurology

Background:

  • Thrombotic microangiopathies (TMAs) are rare, severe conditions including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS).
  • HUS is classified as typical or atypical based on its cause.
  • TMAs present with microangiopathic hemolytic anemia (MAHA), low platelets, and organ damage.

Purpose of the Study:

  • To report a pediatric case of atypical hemolytic uremic syndrome (aHUS) with severe neurological manifestations.
  • To evaluate the efficacy of eculizumab in treating pediatric TMA with significant central nervous system (CNS) involvement.

Main Methods:

  • A 4-year-old child with symptoms of bloody diarrhea, vomiting, and fever was treated with plasma infusion and peritoneal dialysis.
  • Eculizumab therapy was initiated one week after admission.
  • The patient was monitored for hematological, renal, and neurological recovery over a 6-year follow-up period.

Main Results:

  • Rapid improvement in hematological and renal parameters was observed after eculizumab initiation.
  • Neurological recovery was gradual but significant over the 6-year follow-up.
  • The patient's severe neurological manifestations were managed effectively, highlighting aHUS as a potential cause.

Conclusions:

  • Severe neurological involvement can be an initial presentation of HUS, not exclusively TTP.
  • Eculizumab is a potentially life-saving therapy for pediatric TMA patients with severe neurological complications.
  • While eculizumab can be effective, central nervous system recovery in pediatric TMA may require extended follow-up.
Keywords:
CNS involvementcomplement-mediated TMAhemolytic uremic syndrome (HUS)infection-associated HUS (STEC-HUS)seizures

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