COMBINATION OF ATYPICAL HEMOLYTIC UREMIC SYNDROME AND IGA NEPHROPATHY : A CASE REPORT AND LITERATURE REVIEW
Atypical hemolytic uremic syndrome (aHUS), which is characterized by thrombotic microangiopathy, is distinct from Shiga-toxin-induced HUS and thrombotic thrombocytopenic purpura. aHUS is associated with dysregulation of the alternative complement system. Eculizumab, an anti-C5 antibody, is effective in limiting complement activation in patients with aHUS. In recent years, a link has been identified between aHUS and some other complement-mediated nephropathies, in particular C3 glomerulopathy and immune-complex–mediated membranoproliferative glomerulonephritis. A common genetic component may be a unifying factor in these situations. At the same time, the possible combination of aHUS with IgA nephropathy (IgAN) remains poorly understood. We herein report the case of a 10-year-old girl, who was admitted to our hospital with symptoms of jaundice, anemia, thrombocytopenia, and acute kidney injury. Clinical and laboratory assessments led to a diagnosis of atypical hemolytic uremic syndrome (aHUS). The patient was initially treated withplasma exchange and hemodialysis. Eculizumab treatment was initiated on hospital day 6.Remission of her thrombotic microangiopathy was achieved, however during her follow-up, the patient experienced several episodes of acute respiratory infections with macrohematuria. Kidney biopsy was performed and her histological findings were consistent with immunoglobulin A nephropathy (IgAN). The patient showed improvement following treatment with eculizumab and ramipril. This case prompts a discussion on the potential relationship between aHUS and IgAN.
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Kagan M.Yu., Issanguzhina Zh.H., Seipenova A.N., Puxovikova N.N. Combination of atypical hemolytic uremic syndrome and IGA nephropathy: a case report and literature review // Nauka i Zdravookhranenie [Science & Healthcare]. 2025. Vol.27 (6), pp. 244-250. doi 10.34689/SH.2025.27.6.027Related publications:
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