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A previously healthy seven-year-old boy presented with clinical and laboratory findings consistent with atypical hemolytic uremic syndrome (aHUS) given thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and acute kidney injury in the setting of influenza A. Notably, he also met diagnostic criteria for disseminated intravascular coagulation (DIC) at the time of presentation with clinical findings including prolonged prothrombin time (PT), markedly elevated D-dimer, and low fibrinogen. While aHUS and DIC share overlapping clinical features, they are traditionally regarded as distinct entities, with aHUS driven by complement dysregulation and DIC by the widespread activation of the coagulation cascade resulting in microvascular thrombosis, consumptive coagulopathy, and secondary fibrinolysis. The patient was treated with both supportive care and eculizumab, a terminal complement inhibitor, leading to rapid and sustained clinical and laboratory improvement without recurrence. This case highlights the diagnostic and therapeutic complexity of concurrent aHUS and DIC, lends clinical support to emerging hypotheses that uncontrolled complement activation may contribute to DIC, and underscores the value of early recognition and complement-directed therapy in atypical hemolytic uremic syndrome (HUS).
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http://dx.doi.org/10.7759/cureus.87437 | DOI Listing |
Intern Med
September 2025
Department of Gastroenterology and Nephrology, Tottori University Hospital, Japan.
The clinical manifestations of atypical hemolytic uremic syndrome (aHUS) vary depending on the genetic background. A 19-year-old man with the C3 p.Asp1115Asn variant experienced 2 episodes of recurrent aHUS following respiratory tract infections caused by influenza and COVID-19.
View Article and Find Full Text PDFPediatr Nephrol
September 2025
AIIMS Jodhpur: All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.
Ann Med Surg (Lond)
September 2025
Department of Biomedical and Laboratory Science, Africa University, Mutare, Zimbabwe.
Thrombotic microangiopathies (TMAs) encompass a diverse group of syndromes marked by microvascular thrombosis, thrombocytopenia, and organ injury, primarily affecting the kidneys and central nervous system. While the etiologies differ-ranging from genetic mutations to infectious and autoimmune triggers-a unifying pathogenic mechanism is endothelial dysfunction. Recent advances have illuminated the pivotal role of cytokine dysregulation in initiating and sustaining this vascular injury.
View Article and Find Full Text PDFFront Immunol
September 2025
Department of Immunology, La Rabta Hospital, Tunis, Tunisia.
Introduction: Atypical Hemolytic Uremic Syndrome (aHUS) is the prototype of renal diseases secondary to dysregulation of the alternative complement pathway. Our previous studies demonstrated that factor I deficiency appears to be common in Tunisian aHUS patients with the recurrence of a rare variant c.1071T>G (p.
View Article and Find Full Text PDFBMC Nephrol
September 2025
Paediatric Department, Athens Medical Group, Athens, Greece.
Background: Post-infectious glomerulonephritis (PIGN) is one of the leading causes of acute nephritis in children worldwide. C3 glomerulopathy (C3G) is a rare form of membranoproliferative glomerulonephritis (MPGN) characterised by either genetic or acquired dysregulation of the alternative complement pathway resulting in predominant C3 deposition within the glomeruli. The overlap between atypical post-streptococcal glomerulonephritis (PSGN), a subset of PIGN primarily induced by streptococcal species, and C3G has attracted considerable attention in recent clinical trials.
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