A rare case of Alport syndrome, atypical hemolytic uremic syndrome and Pauci-immune crescentic glomerulonephritis.
BMC Nephrol
; 19(1): 355, 2018 12 12.
Article
em En
| MEDLINE
| ID: mdl-30541482
ABSTRACT
BACKGROUND:
Renal thrombotic microangiopathy (TMA) is occasionally seen in biopsies with pauci-immune necrotizing crescentic glomerulonephritis (PCGN). Recent study indicated that the complement activation is more prominent in the ANCA-negative glomerulonephritis. CASE PRESENTATION We report a case of concurrent TMA and PCGN without ANCA positivity. Interestingly, our patient also had biopsy features supportive of Alport syndrome (AS). Genetic studies identified variants and polymorphisms in alternative complement pathway genes that confer substantial risk of developing atypical hemolytic uremic syndrome (aHUS).CONCLUSIONS:
Abnormal activation in complement pathway may represent a common pathogenic link between these three distinct entities.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Síndrome Hemolítico-Urêmica Atípica
/
Glomerulonefrite
/
Nefrite Hereditária
Tipo de estudo:
Prognostic_studies
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article