{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,29]],"date-time":"2026-06-29T16:45:14Z","timestamp":1782751514920,"version":"3.54.5"},"reference-count":48,"publisher":"S. Karger AG","license":[{"start":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T00:00:00Z","timestamp":1777334400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T00:00:00Z","timestamp":1777334400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nephron"],"abstract":"<jats:p>Introduction: Thrombotic microangiopathy (TMA) with associated nephrotic syndrome is a unique and rare condition that is infrequently described in pediatrics. Case Presentations: Four children with steroid-resistant nephrotic syndrome (SRNS), immune-mediated and of monogenic origin, presented with TMA during late stages of chronic kidney disease. All patients tested negative for Shiga toxin and had normal A Disintegrin-like and Metalloprotease with Thrombospondin type 1 repeats 13 (ADAMTS13) activity. Genetic and functional studies of complement dysregulation were negative. Three of the 4 patients treated with Eculizumab showed good hematologic response but no kidney function recovery. None of the three children who underwent kidney transplantation had recurrent TMA. Conclusions: TMA may develop in patients with SRNS, manifesting as an unexplained worsening of kidney function, new-onset hypertension, hemolytic anemia, and thrombocytopenia. Eculizumab was effective in improving hemolytic markers without kidney function recovery. TMA did not recur after kidney transplantation in this entity.<\/jats:p>","DOI":"10.1159\/000552049","type":"journal-article","created":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T16:03:05Z","timestamp":1777392185000},"page":"1-13","source":"Crossref","is-referenced-by-count":0,"title":["Thrombotic Microangiopathy in Childhood Steroid-Resistant Nephrotic Syndrome: Case 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