Navegando por Palavras-chave "FSGS"

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    NPHS2 mutations in adult patients with primary focal segmental glomerulosclerosis
    (Wichtig Editore, 2006-05-01) Monteiro, Eduardo Jose Bellotto; Pereira, Alexandre C.; Pereira, Aparecido B.; Krieger, Jose E.; Mastroianni-Kirsztajn, Gianna; Universidade Federal de São Paulo (UNIFESP); Universidade de São Paulo (USP)
    Background. Mutations in the NPHS2 gene encoding the protein podocin have recently been found in a recessive form of steroid-resistant nephrotic syndrome. Focal segmental glomerulosclerosis (FSGS) was the histologic diagnosis in many of the patients harboring these mutations. FSGS is a heterogeneous glomerular lesion with diverse origins and outcomes. Although mutational analysis in children permits the identification of an unresponsive group before initiating treatment, there is not much information on adult-onset patients with FSGS.Methods: We performed NPHS2 gene mutational analysis in 39 adult Brazilian patients with primary FSGS, and evaluated the clinical course of the disease and response to treatment; in addition, we performed urinary screening in 44 relatives of these patients.Results: In this group, only 1 patient (with familial FSGS) had a mutation in the NPHS2 gene with double heterozygosity. The absence of mutations in all other patients evaluated suggests its rarity in sporadic cases of adult-onset (steroid sensitive or resistant) FSGS in our population.Conclusions: Our results suggest that the analysis of the NPHS2 gene mutation is not indicated as a routine diagnostic procedure in our population for adult-onset patients with FSGS.
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    The polysaccharide fraction of Propionibacterium acnes modulates the development of experimental focal segmental glomerulosclerosis
    (Elsevier B.V., 2012-09-01) Reis, Vanessa Oliveira [UNIFESP]; Silva, Janice Costa [UNIFESP]; Souza, Gabriela Trindade [UNIFESP]; Semedo, Patricia [UNIFESP]; Buscariollo, Bruna [UNIFESP]; Pereira, Rafael Luiz [UNIFESP]; Cenedeze, Marcos Antonio [UNIFESP]; Pacheco-Silva, Alvaro [UNIFESP]; Longo-Maugeri, Ieda M. [UNIFESP]; Saraiva Camara, Niels Olsen [UNIFESP]; Keller, Alexandre Castro [UNIFESP]; Universidade Federal de São Paulo (UNIFESP); Inst Israelita Ensino & Pesquisa Albert Einstein; Universidade de São Paulo (USP)
    The pathogenesis of focal segmental glomerulosclerosis (FSGS) appears to be associated with type-2 cytokines and podocyte dysfunction. in this study, we tested the hypothesis that immunization with the polysaccharide fraction of Propionibacterium acnes (PS), a pro-Th1 agonist, may subvert the type-2 profile and protect podocytes from adriamycin-induced glomerulosclerosis. Adriamycin injection resulted in albuminuria and increased serum creatinine in association with loss of glomerular podocin and podoplanin expression, which is consistent with podocyte dysfunction. Renal tissue analysis revealed the expression of transcripts for GATA3 and fibrogenic-related proteins, such as TGF-beta, tissue inhibitor of metalloproteinase-1 (TIMP-1) and metalloproteinase 9 (MMP9). in association with the expression of fibrogenic transcripts, we observed peri-glomerular expression of a-smooth muscle actin (alpha-SMA), indicating epithelial-to-mesenchymal transition, and increased expression of proliferating cell nuclear antigen (PCNA) in tubular cells, suggesting intense proliferative activity. Previous immunization with PS inhibited albuminuria and serum creatinine in association with the preservation of podocyte proteins and inhibition of fibrogenic transcripts and the expression of alpha-SMA and PCNA proteins. Tissue analysis also revealed that PS treatment induced expression of mRNA for GD3 synthase, which is a glycosiltransferase related to the synthesis of GD3, a ganglioside associated with podocyte physiology. in addition, PS treatment inhibited the influx of inflammatory CD8(pos) and CD11b(pos) cells to kidney tissue. Finally, PS treatment on day 4 post-ADM, a period when proteinuria was already established, was able to improve renal function. Thus, we demonstrate that the PS fraction of P. acnes can inhibit FSGS pathogenesis, suggesting that immunomodulation can represent an alternative approach for disease management. (C) 2011 Elsevier GmbH. All rights reserved.