scholarly journals Early Focal Segmental Glomerulosclerosis as a Cause of Renal Allograft Primary Nonfunction

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Emma J. Griffin ◽  
Peter C. Thomson ◽  
David Kipgen ◽  
Marc Clancy ◽  
Conal Daly
Keyword(s):  
Renal Transplantation ◽  
Acute Rejection ◽  
Focal Segmental Glomerulosclerosis ◽  
Renal Allograft ◽  
Recurrent Disease ◽  
Graft Function ◽  
Kidney Injury ◽  
Renal Allografts ◽  
Primary Nonfunction ◽  
Segmental Glomerulosclerosis

Background.Primary focal segmental glomerulosclerosis (FSGS) is one of the commonest causes of glomerular disease and if left untreated will often progress to established renal failure. In many cases the best treatment option is renal transplantation; however primary FSGS may rapidly recur in renal allografts and may contribute to delayed graft function. We present a case of primary nonfunction in a renal allograft due to biopsy-proven FSGS.Case Report.A 32-year-old man presented with serum albumin of 22 g/L, proteinuria quantified at 12 g/L, and marked peripheral oedema. Renal biopsy demonstrated tip-variant FSGS. Despite treatment, the patient developed progressive renal dysfunction and was commenced on haemodialysis. Cadaveric renal transplantation was undertaken; however this was complicated by primary nonfunction. Renal biopsies failed to demonstrate evidence of acute rejection but did demonstrate clear evidence of FSGS. The patient was treated to no avail.Discussion.Primary renal allograft nonfunction following transplantation is often due to acute kidney injury or acute rejection. Recurrent FSGS is recognised as a phenomenon that drives allograft dysfunction but is not traditionally associated with primary nonfunction. This case highlights FSGS as a potentially aggressive process that, once active in the allograft, may prove refractory to targeted treatment. Preemptive therapies in patients deemed to be at high risk of recurrent disease may be appropriate and should be considered.

scholarly journals Unsuccessful Treatment with Abatacept in Recurrent Focal Segmental Glomerulosclerosis after Kidney Transplantation

2017 ◽  
Vol 7 (1) ◽  
pp. 1-5 ◽  
Author(s):  
Tilde Kristensen ◽  
Per Ivarsen ◽  
Johan Vestergaard Povlsen
Keyword(s):  
Renal Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Case Reports ◽  
Graft Function ◽  
Therapeutic Drug ◽  
Allograft Survival ◽  
Segmental Glomerulosclerosis ◽  
Unsuccessful Treatment ◽  
Stage Renal Disease ◽  
End Stage

Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation occurs in up to 20–50% of FSGS patients and is associated with inferior allograft survival. Treatment of both primary FSGS as well as recurrent FSGS after transplantation with plasma exchange and immunosuppression is often unsuccessful and remains a major challenge as the disease still leads to end-stage renal disease and decreased graft survival. Previous case reports have described patients with recurrent FSGS who were successfully treated with a B7-1 inhibitor (abatacept) inducing partial or complete remission. The rational basis for believing in abatacept as a new therapeutic drug for the treatment of FSGS is the study by Yu et al. [N Engl J Med 2013;369: 2416–2423] showing B7-1 in immunostainings of the podocytes. The authors speculated that B7-1 immunostaining of renal biopsies might identify a subgroup of patients who would benefit from abatacept treatment. We present a case with recurrent FSGS after renal transplantation. The patient was unsuccessfully treated with B7-1 inhibitors. Although the patient was treated with abatacept 10 mg/kg body weight twice, the proteinuria and decreased graft function remained unchanged, and he never reached remission.

scholarly journals Renal allograft survival in transplant recipients with focal segmental glomerulosclerosis

2019 ◽  
Vol 9 (2) ◽  
pp. e15-e15
Author(s):  
Takahiro Shinzato ◽  
Yoshitaka kinoshita ◽  
Taro Kubo ◽  
Toshihiro Shimizu ◽  
Koji Nanmoku ◽  
...  
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Medical Records ◽  
Renal Allograft ◽  
De Novo ◽  
Transplant Recipients ◽  
Allograft Survival ◽  
Renal Allografts ◽  
Not Otherwise Specified ◽  
Segmental Glomerulosclerosis ◽  
Cadaveric Donors

Introduction: The frequency that idiopathic focal segmental glomerulosclerosis (FSGS) recurs in renal allografts is reportedly 20-50%, but the epidemiology of secondary FSGS in this setting has scarcely been addressed. Objectives: The aim of this study was to examine the incidence, etiology, and subtypes of FSGS in renal allograft recipients and allograft survival in recipients with FSGS. Patients and Methods: As a retrospective review, we examined medical records of 359 consecutive renal allograft recipients (living donors, 329; cadaveric donors, 30). In 121 of these patients, allograft dysfunction or proteinuria prompted biopsies. We compared allograft survival in recipients with and without FSGS. We then determined histologic subtypes of FSGS using the Columbia classification and categorized FSGS as recurrent or de novo, and idiopathic or secondary. Results: Of 121 subjects who were biopsied, six with inadequate specimens (<10 glomeruli) were excluded. Only 17 of those remaining (n=115) were diagnosed as secondary FSGS. Renal allograft survival did not differ significantly in patients with or without FSGS (P=0.953). Subtypes of FSGS were as follows; not otherwise specified (NOS; n=8), collapsing (n=5), cellular (n=2), and perihilar (n=2). Conclusion: Secondary FSGS was observed in 14.5% of biopsies of renal allograft recipients and seemed no significant impact on allograft survival.

scholarly journals Mechanical challenges and cytoskeletal impairments in focal segmental glomerulosclerosis

2018 ◽  
Vol 314 (5) ◽  
pp. F921-F925 ◽  
Author(s):  
Di Feng ◽  
Clark DuMontier ◽  
Martin R. Pollak
Keyword(s):  
Blood Flow ◽  
Focal Segmental Glomerulosclerosis ◽  
Kidney Injury ◽  
Future Research ◽  
Disease States ◽  
Filtration Barrier ◽  
Segmental Glomerulosclerosis ◽  
The Face ◽  
Foot Process ◽  
Future Research Directions

Focal segmental glomerulosclerosis (FSGS) is a histologically defined form of kidney injury typically mediated by podocyte dysfunction. Podocytes rely on their intricate actin-based cytoskeleton to maintain the glomerular filtration barrier in the face of mechanical challenges resulting from pulsatile blood flow and filtration of this blood flow. This review summarizes the mechanical challenges faced by podocytes in the form of stretch and shear stress, both of which may play a role in the progression of podocyte dysfunction and detachment. It also reviews how podocytes respond to these mechanical challenges in dynamic fashion through rearranging their cytoskeleton, triggering various biochemical pathways, and, in some disease states, altering their morphology in the form of foot process effacement. Furthermore, this review highlights the growing body of evidence identifying several mutations of important cytoskeleton proteins as causes of FSGS. Lastly, it synthesizes the above evidence to show that a better understanding of how these mutations leave podocytes vulnerable to the mechanical challenges they face is essential to better understanding the mechanisms by which they lead to disease. The review concludes with future research directions to fill this gap and some novel techniques with which to pursue these directions.

Preventing recurrence of focal segmental glomerulosclerosis following renal transplantation: A case report

2006 ◽  
Vol 10 (8) ◽  
pp. 962-965 ◽  
Author(s):  
Kevin Couloures ◽  
Samuel H. Pepkowitz ◽  
Dennis Goldfinger ◽  
Elaine S. Kamil ◽  
Dechu P. Puliyanda
Keyword(s):  
Case Report ◽  
Renal Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Segmental Glomerulosclerosis
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