scholarly journals Clinicopathological Implications of Proteinuria after Long-Term Isolated Hematuria due to Thin Basement Membrane Nephropathy and Focal Segmental Glomerulosclerosis

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Ryo Togashi ◽  
Yoshikazu Nemoto ◽  
Kaito Waki ◽  
Michito Nagura ◽  
Shigeyuki Arai ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Focal Segmental Glomerulosclerosis ◽  
Angiotensin Receptor Blocker ◽  
Kidney Biopsy ◽  
Angiotensin Receptor ◽  
Thin Basement Membrane Nephropathy ◽  
Segmental Glomerulosclerosis ◽  
Features Reduction ◽  
Urinary Abnormalities

A 45-year-old obese man presented with persistent hematuria for 21 years. At the age of 37, he developed hypertension and proteinuria which later increased up to 1.6 g/g creatinine. Kidney biopsy revealed thin basement membrane nephropathy (TBMN) and focal segmental glomerulosclerosis (FSGS), which explained his urinary abnormalities. Although a subgroup of TBMN can be complicated by FSGS, his FSGS was associated with obesity because of its histological features. Reduction of body weight and increasing a dose of angiotensin-receptor blocker could transiently reduce the amount of proteinuria. Clinicopathological implications of proteinuria after long-term hematuria by TBMN and FSGS were further discussed.

scholarly journals COL4A3/COL4A4 Mutations Producing Focal Segmental Glomerulosclerosis and Renal Failure in Thin Basement Membrane Nephropathy

2007 ◽  
Vol 18 (11) ◽  
pp. 3004-3016 ◽  
Author(s):  
Konstantinos Voskarides ◽  
Loukas Damianou ◽  
Vassos Neocleous ◽  
Ioanna Zouvani ◽  
Stalo Christodoulidou ◽  
...  
Keyword(s):  
Renal Failure ◽  
Basement Membrane ◽  
Focal Segmental Glomerulosclerosis ◽  
Thin Basement Membrane Nephropathy ◽  
Segmental Glomerulosclerosis

scholarly journals Successful long-term management of recurrent focal segmental glomerulosclerosis after kidney transplantation with costimulation blockade

2020 ◽  
Author(s):  
Thomas Mühlbacher ◽  
Kerstin Amann ◽  
Moritz Mahling ◽  
Silvio Nadalin ◽  
Nils Heyne ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Costimulation Blockade ◽  
Segmental Glomerulosclerosis ◽  
Remission Maintenance ◽  
Maintenance Immunosuppression ◽  
Therapeutic Concepts ◽  
Allograft Outcome

Abstract Recurrence of primary focal segmental glomerulosclerosis (FSGS) occurs in up to 50% of patients after kidney transplantation and is associated with poor allograft outcome. Novel therapeutic concepts directly target podocyte function via B7-1 with inconsistent response. We present the case of a 19 yr. old patient with recurrent primary FSGS early after living donor kidney transplantation. Plasmapheresis and rituximab did not induce remission. Repetitive abatacept administration was able to achieve partial remission. Maintenance immunosuppression was subsequently switched to a belatacept-based CNI-free immunosuppression, resulting in sustained complete remission with excellent allograft function throughout a follow-up of more than 56 months.

P041 Angiotensin receptor type 1, MICA and vimentin antibodies in focal segmental glomerulosclerosis

2017 ◽  
Vol 78 ◽  
pp. 84
Author(s):  
Jamila McClinton ◽  
Cristina Fhied ◽  
David Cimbaluk ◽  
Maria Oppermann ◽  
Maribeth L. Flaws Flaws ◽  
...  
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Receptor Type ◽  
Angiotensin Receptor ◽  
Segmental Glomerulosclerosis

MO939RECURRENCE AND OUTCOME OF ANTI-GLOMERULAR BASEMENT MEMBRANE GLOMERULONEPHRITIS AFTER KIDNEY TRANSPLANTATION: A BELGIAN MULTICENTER STUDY

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Sophie Coche ◽  
Ben Sprangers ◽  
Steven Van Laecke ◽  
Laurent Weekers ◽  
Vicky De Meyer ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Graft Survival ◽  
Glomerular Basement Membrane ◽  
Kidney Biopsy ◽  
Graft Loss ◽  
Survival Rates ◽  
Kidney Graft ◽  
Clinical Recurrence ◽  
Patient And Graft Survival

Abstract Background and Aims Recurrence of anti-glomerular basement membrane (anti-GBM) glomerulonephritis in the kidney graft is a rare event, described in limited case reports and registry analysis. The aim of this study was to evaluate in a large cohort of patients with detailed data collection and long follow-up the risk of recurrence of anti-GBM disease and graft loss caused by recurrence, the risk factors associated with clinical recurrence and the long-term patient and graft survival. Method Multicenter retrospective study. Inclusion criteria: patients with anti-GBM glomerulonephritis transplanted with a kidney between 1977 and 2015. Exclusion criteria: systemic vasculitis (except ANCA), lupus erythematosus and cryoglobulinemia. Clinical recurrence was defined as reappearance of signs of glomerulonephritis along with histological signs of proliferative glomerulonephritis and linear IgG staining on kidney biopsy, with or without anti-GBM antibodies. Results Fifty-three patients were included. Clinical recurrence in a first kidney transplant occurred in only one patient five years after transplantation -a prevalence rate of 1.9%- in the context of cessation of immunosuppressive drugs. The graft was lost due to recurrence. Histological recurrence with linear IgG staining on kidney biopsy in the absence of histologic signs of proliferative glomerulonephritis was observed in four patients, in the context of cellular rejection. Two patients lost their kidney graft from severe acute rejection; the others fully recovered. Patient survival was 100%, 94% and 89% at 5, 10 and 15 years, respectively. Overall, death-censored first graft survival rates were 88%, 83% and 79% at 5, 10 and 15 years, respectively. Conclusion Recurrence rate of anti-GBM glomerulonephritis after transplantation is very low, and associated with graft loss. The long-term patient and graft survival rates are excellent.

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