| Autor: |
Domenico Santoro, Rossella Siligato, Carmela Vadalà, Mariacristina Lucanto, Simona Cristadoro, Giovanni Conti, Michele Buemi, Stefano Costa, Ettore Sabadini, Giuseppe Magazzù |
| Jazyk: |
angličtina |
| Rok vydání: |
2018 |
| Předmět: |
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| Zdroj: |
BMC Nephrology, Vol 19, Iss 1, Pp 1-5 (2018) |
| Druh dokumentu: |
article |
| ISSN: |
1471-2369 |
| DOI: |
10.1186/s12882-018-0880-y |
| Popis: |
Abstract Background C3 glomerulonephritis is a rare glomerulopathy characterized at renal biopsy by C3 deposition, alone or with scanty immunoglobulins, as well as by an electron-dense material in mesangium, subendothelial and subepithelial space. An abnormal systemic activation of the alternative pathway of the complement cascade is responsible for the development of the disease if triggered by several possible environmental conditions. We report the first case in literature of a patient affected by cystic fibrosis and C3GN. Case presentation Our case involves a young woman with cystic fibrosis, who had persistent microscopic hematuria, proteinuria and hypocomplementemia C3 for over three months. Renal biopsy confirmed the diagnosis of C3 glomerulopathy. Complement system dysregulation was tested and resulted in a strong terminal pathway activation proved by high levels of sC5b-9 complex, amounting to 1588 ng/ml (normal value |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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