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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2021  |  Volume : 32  |  Issue : 6  |  Page : 1782-1789
Recurrent C3 Glomerulonephritis with an ADAMTS 13 Gene Variant: A Case Report and Literature Review


1 Department of Pathology, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
2 Department of Kidney and Pancreas Transplantation and Department of Pathology and Laboratory Medicine (MBC-10), Riyadh, Saudi Arabia
3 Department of Pathology and Laboratory Medicine (MBC-10), King Faisal Specialist Hospital and Research Center; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia

Correspondence Address:
Reem A. Al Zahrani
Department of Pathology, Faculty of Medicine, King Abdulaziz University, P. O. Box 80205, Jeddah 21589, Saudi Arabia.
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-2442.352441

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C3 glomerulonephritis (C3GN) is a recently described form of GN that mainly occurs in children and young adults. It results from dysregulation of the alternative complement pathway. Studies have shown that dense deposit disease has a high recurrence rate; however, since C3GN is a recently described disorder, its recurrence rate is still variable. A 28-year-old male with end-stage renal disease caused by C3GN underwent renal transplantation. After 19 months, the patient experienced recurrent C3GN (rC3GN) that involved a variant of unknown significance in the ADAMTS13 gene. Over a short span of time, the patient suffered from rapid deterioration of the graft function that required renal replacement therapy. This is the first case of rC3GN that possibly involved genetic alteration, a variant within the ADAMTS 13 gene.


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