Navegando por Palavras-chave "focal segmental glomerulosclerosis"

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    Glomeruloesclerose segmentar e focal após-transplante renal: evolução clínica e fatores de progressão
    (Universidade Federal de São Paulo (UNIFESP), 2016-12-31) Mata, Gustavo Ferreira da [UNIFESP]; Kirsztajn, Gianna Mastroianni Kirsztajn [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)
    Background: In spite of advances in dialysis therapies, renal transplantation is the best therapy for the treatment of end stage renal disease (ESRD). However, graft survival depends on varied factors: graft age, cold ischemia time, occurrence of rejections, adherence to treatment, immunosuppressive drugs, compatibility, donor type, recipient disease, others. Among glomerular diseases that progress to ESRD, focal segmental glomerulosclerosis (FSGS) occupies a prominent position due to its prevalence and its high recurrence after renal transplantation. After transplantation, the disease can recur in 15-52% of cases and, if left untreated, leads to early graft loss in more than 50% of cases. Objective: To perform a descriptive analysis of a cohort of patients with post-transplant FSGS in terms of recipient and donor demographic characteristics, clinical course, response to treatment and progression of glomerular disease to "graft loss". Material and Methods: This is a retrospective cohort study, including adolescent and adult patients, submitted to renal transplantation with live and deceased donors between January 1999 and September 2014 in the Transplant Section (Division of Nephrology) of the Federal University of São Paulo/Hospital do Rim, São Paulo (SP), Brazil. Results: 88 patients with post-transplant FSGS were identified along the period of this study. The mean age of the patients in this sample was 29.1 ± 13.3 years at the time of transplantation, with predominance of male and white patients. Transplants with deceased donors predominated (60.9%). Of these, 25.6% were performed with an expanded criterion donor. Delay graft function occurred in 47.6% of recipients. The mean time to onset of proteinuria greater than 0.5 g / g was 20.51 ± 20.88 days. The indication of biopsy of the renal graft due to the suspicion of FSGS occurred with a median of 79 days, and histological characteristics of the FSGS were verified, in most cases, only in the subsequent biopsies, with a mean time of appearance of the histological changes of 164.56 ± 375.89 days. Only 21.5% of the patients had histological characteristics of FSGS in the first graft biopsy. The vast majority of patients (90.80%) underwent pulse therapy with methylprednisolone associated with plasmapheresis (70.10%). Taking a period of 60 months after kidney transplantation, 44.16% of the patients had partial remission, 25.97% complete remission and 29.87% had no remission. However, 50.60% of the patients evolved with graft loss (77.27% secondary to FSGS). After 12 months of transplantation, mean serum creatinine was 1.94 ± 1.02 mg/dL. The main complication of the treatment was infection/sepsis (67.5%). Eight patients (9.4%) died in the 60-month period, five (62.5%) deaths were attributed to infection. Conclusion: FSGS after renal transplantation presented a high rate of recurrence, usually early in the course of the disease. The histological changes in light microscopy were not simultaneous to the appearance of proteinuria. The infection rate during follow-up was high and related to mortality. The graft loss rate attributed to the post-transplant FSGS was elevated throughout the follow-up and corresponded to half of the individuals affected. Plasmapheresis therapy was an effective measure for the treatment of post-transplant FSGS and was able to contribute to partial or total response in more than 70% of the patients.
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    Idiopathic focal segmental glomerulosclerosis and HLA antigens
    (Associação Brasileira de Divulgação Científica, 1998-03-01) Gerbase-Lima, Maria [UNIFESP]; Pereira-Santos, A. [UNIFESP]; Sesso, Ricardo de Castro Cintra [UNIFESP]; Temin, Julia; Aragão, E.s.; Ajzen, Horacio [UNIFESP]; A01; Universidade Federal de São Paulo (UNIFESP)
    The objective of the present study was to investigate a possible association between HLA class II antigens and idiopathic focal segmental glomerulosclerosis (FSGS). HLA-A, -B, -DR and -DQ antigens were determined in 19 Brazilian patients (16 white subjects and three subjects of Japanese origin) with biopsy-proven FSGS. Comparison of the HLA antigen frequencies between white patients and white local controls showed a significant increase in HLA-DR4 frequency among FSGS patients (37.7 vs 17.2%, P<0.05). In addition, the three patients of Japanese extraction, not included in the statistical analysis, also presented HLA-DR4. In conclusion, our data confirm the association of FSGS with HLA-DR4 previously reported by others, thus providing further evidence for a role of genes of the HLA complex in the susceptibility to this disease
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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.