Thrombotic Microangiopathy in Children: Experience from a Tertiary Care Center in South India

Abstract Introduction: Thrombotic microangiopathy (TMA) is a rare and serious disorder that can lead to long-term nephrological consequences. Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are the clinical manifestations of TMA. This study aimed to describe the clinical profile of children with TMA and assess their renal outcomes. Materials and Methods: This single-center, ambidirectional observational study analyzed 15 pediatric patients with TMA over 6 years from June 2018 to June 2024. Nine children were enrolled prospectively from January 2022 to January 2024, and all were followed up until June 2024. Results: Diarrhea-associated HUS (D + HUS) was observed in four children, whereas two children had TTP and nine children had atypical HUS (a-HUS). Seven cases of atypical HUS showed positive anti-complement factor H (CFH) antibodies. Most (73.3%) of the patients were treated with plasma exchange, whereas three received eculizumab. Mortality was observed in two patients: one patient with diarrhea-associated HUS (D + HUS) and one patient with TTP. Conclusion: Anti-CFH antibody-mediated atypical HUS is the most common form of TMA seen in our setting and is associated with a good prognosis.