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Atypical hemolytic uremic syndrome and C3 glomerulopathy/immune complex membranoproliferative glomerulonephritis are ultra-rare chronic, complement-mediated diseases with childhood manifestation in a majority of cases. Transition of clinical care of patients from pediatric to adult nephrologists-typically with controlled disease in native or transplant kidneys in case of atypical hemolytic uremic syndrome and often with chronic progressive disease despite treatment efforts in case of C3 glomerulopathy/immune complex membranoproliferative glomerulonephritis-identifies a challenging juncture in the journey of these patients. Raising awareness for the vulnerability of this patient cohort; providing education on disease pathophysiology and management including the use of new, high-precision complement antagonists; and establishing an ongoing dialog of patients, families, and all members of the health care team involved on either side of the age divide will be inevitable to ensure optimal patient outcomes and a safe transition of these patients to adulthood.
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http://dx.doi.org/10.1053/j.ackd.2022.04.003 | 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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