Iranian Journal of Pathology

Crescentic Glomerulonephritis, A Rare Presentation of Alport Syndrome

Document Type : Case Reports

Authors

Tahereh Malakoutian 1
Fatemeh Nili 2
Sholeh Ghasemi Darbrood 1
Samaneh Salarvand 2
Mitra Mehrazma 3

1 Department of Nephrology, Shahid Hasheminejad Kidney Center, Iran University of Medical Sciences, Tehran, Iran

2 Department of Pathology, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran

3 Department of Pathology, Shahid Hasheminejad Kidney Center, Iran University of Medical Sciences, Tehran, Iran

https://doi.org/10.30699/ijp.2022.554160.2899
Abstract
Crescentic glomerulonephritis (GN) is a feature of severe glomerular injury. Anti-GBM disease, immune-complex mediated glomerulonephritis, and ANCA-associated vasculitis are the main causes of crescentic GN. Alport syndrome is a progressive form of hereditary nephritis presenting with hematuria and progression to proteinuria and renal failure. Herein we present a 16-year-old male with rapidly progressive glomerulonephritis syndrome, sensory-neural hearing loss, and a family history of hematuria and proteinuria in his mother and aunt. Light microscopic examination shows cellular crescent in glomeruli. In an electron microscopy study, GBM changes compatible with Alport syndrome were identified. Alport syndrome rarely can be presented as crescentic GN. Electron microscopy is necessary for the diagnosis of this type of pauci-immune crescentic glomerulonephritis.

Keywords

Alport
Crescentic glomerulonephritis
Hereditary nephritis

Subjects

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Iranian Journal of Pathology
Volume 17, Issue 4
Autumn 2022
Pages 496-499

  • Receive Date 20 May 2022
  • Accept Date 04 July 2022

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