C3 Glomerulonephritis in a patient with AlportSyndrome: a rare case
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Alport Syndrome
hereditary nephritis
C3 glomerulonephritis
nephrotic syndrome
glomerular basement membrane

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Sánchez Solano MA, Guerrero Tinoco GA. C3 Glomerulonephritis in a patient with AlportSyndrome: a rare case. Rev. Colomb. Nefrol. [Internet]. 2022 Jan. 20 [cited 2022 May 18];9(1):e491. Available from: https://revistanefrologia.org/index.php/rcn/article/view/491


Alport Syndrome is a hereditary entity that occurs mainly due to a mutation in the genes that encode type IV collagen. C3 glomerulonephritis is a rare entity with a pattern of membranoproliferative glomerulonephritis and its etiology is based on abnormal control of the activation of the alternative complement pathway. We describe a case of a male patient who presents with a corticosteroid nephrotic syndrome in which a pattern of membranoproliferative glomerulonephritis is documented in the renal biopsy with C3 deposits in the immunofluorescence, associated with a heterozygous deletion in the gene CFHR1 in the genetic study of complement regulatory proteins. Furthermore, a variant COL4A5 associated with X-linked Alport syndrome is found in the genetic panel for corticosteroid resistance. These entities can present with a diverse clinical course, but when associated they can accelerate progression to chronic kidney disease, which is why makes it necessary to do a more strict clinical follow-up.

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