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Navigating Pediatric Atypical Hemolytic Uremic Syndrome: A Two-Year Case Series From Eastern India
Swarnim Swarnim1, Megha Saigal2, Priyanka Priyanka1
1Pediatrics, All India Institute of Medical Sciences, Patna, Patna, IND.
Cureus
|November 25, 2025
Summary
Atypical hemolytic uremic syndrome (aHUS) in Indian children often involves anti-factor H antibodies. Early plasma exchange and immunosuppression improve outcomes, but access to advanced therapies remains a challenge.
Area of Science:
- Nephrology
- Hematology
- Immunology
Background:
- Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy driven by complement system activation, a major cause of pediatric acute kidney injury.
- In India, aHUS is frequently linked to anti-factor H antibodies, posing unique diagnostic and treatment hurdles.
- This study examines pediatric aHUS cases in Eastern India, focusing on clinical features, immunology, management, and outcomes.
Purpose of the Study:
- To characterize the clinical and immunological profiles of pediatric aHUS patients in Eastern India.
- To evaluate the effectiveness of current management strategies, including plasma exchange and immunomodulators.
- To identify challenges and barriers in diagnosing and treating pediatric aHUS in a tertiary care setting.
Main Methods:
- A retrospective observational case series of seven pediatric aHUS patients diagnosed between January 2023 and December 2024.
- Diagnosis involved a clinical triad, exclusion of other causes, and confirmation via complement/anti-factor H antibody testing and genetic analysis.
- Treatment included plasma exchange, immunomodulators, and supportive care, with data analyzed using descriptive statistics.
Main Results:
- Seven pediatric patients (median age 7 years, 6 females) were treated for aHUS.
- CFHR1-CFHR3 deletions and anti-factor H antibodies were found in 43% of patients.
- Hematological remission occurred in 71% within a week; 43% achieved full renal recovery, but 29% developed chronic kidney disease.
Conclusions:
- Pediatric aHUS in Eastern India shows a high prevalence of anti-factor H antibody disease and genetic complement abnormalities.
- Prompt initiation of plasma exchange and immunosuppression correlates with better hematological and renal outcomes.
- Improved access to complement inhibitors and diagnostic tools is crucial for optimizing care in resource-limited settings.
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