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Treatment of Focal Segmental Glomerulosclerosis Recurrence in the Renal Allograft: A Report of Two Cases.

Tran MH, Chan C, Pasch W, Carpenter P, Ichii H, Foster C - Case Rep Nephrol Dial (2016)

Bottom Line: Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of <0.5 was achieved.In patient number two, a second peak in proteinuria and azotemia was ultimately attributable to ureteral stenosis and these values normalized following repair.In conclusion, therapeutic plasma exchange is an effective treatment for FSGS recurring following renal transplant.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Laboratory Medicine, UC Irvine Health School of Medicine, USA.

ABSTRACT
Focal segmental glomerulosclerosis (FSGS) causes glomerular lesions that can progress to end-stage renal disease. It is suspected to be caused by a circulating factor that is amenable to plasmapheresis removal and exhibits a risk for recurrence in the renal allograft. We present two patients with FSGS recurrence in their allograft kidneys diagnosed by biopsy after significant proteinuria developed in the posttransplant setting. Treatment with therapeutic plasma exchange induced long-term remission in both patients. Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of <0.5 was achieved. In patient number two, a second peak in proteinuria and azotemia was ultimately attributable to ureteral stenosis and these values normalized following repair. In conclusion, therapeutic plasma exchange is an effective treatment for FSGS recurring following renal transplant.

No MeSH data available.


Related in: MedlinePlus

Histologic response. The pretreatment biopsy demonstrates moderate effacement (arrow) of the foot processes of the glomerular visceral epithelial cells and microvillous transformation changes, evidence of recurrent FSGS. There were patent capillary loops, normal endothelial fenestration, and no immune deposits. There was effacement of approximately half of the epithelial cell foot processes on electron microscopy. After improvement of Pr:Cr ratio from plasma exchange therapy, the posttreatment glomeruli show features of epithelial foot process recovery. There is markedly less effacement and minimal microvillous transformation changes. Electron microscopy shows less than one quarter of the foot processes effaced and the majority having a normal arrangement.
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Figure 2: Histologic response. The pretreatment biopsy demonstrates moderate effacement (arrow) of the foot processes of the glomerular visceral epithelial cells and microvillous transformation changes, evidence of recurrent FSGS. There were patent capillary loops, normal endothelial fenestration, and no immune deposits. There was effacement of approximately half of the epithelial cell foot processes on electron microscopy. After improvement of Pr:Cr ratio from plasma exchange therapy, the posttreatment glomeruli show features of epithelial foot process recovery. There is markedly less effacement and minimal microvillous transformation changes. Electron microscopy shows less than one quarter of the foot processes effaced and the majority having a normal arrangement.

Mentions: A 39-year-old Hispanic male with a history of end-stage renal disease (ESRD), hypertension and hypertrophic cardiomyopathy underwent deceased donor renal transplantation with thymoglobulin induction and standard steroid taper. He developed poor graft function and nephrotic-range proteinuria beginning on posttransplant day 1. He required posttransplant hemodialysis on posttransplant days 3, 6, 8, and 11, as well as initiation of therapeutic plasma exchange and IVIG on postoperative day 5. Renal transplant biopsies obtained on posttransplant day 7 confirmed the team's suspicion for FSGS recurrence in the renal allograft. The patient's treatment course is depicted in figure 1. Pretreatment and posttreatment biopsies are depicted in figure 2. The patient is currently 4 years posttransplant and continues to demonstrate excellent renal allograft function (serum creatinine of 1.6) with continued absence of proteinuria.


Treatment of Focal Segmental Glomerulosclerosis Recurrence in the Renal Allograft: A Report of Two Cases.

Tran MH, Chan C, Pasch W, Carpenter P, Ichii H, Foster C - Case Rep Nephrol Dial (2016)

Histologic response. The pretreatment biopsy demonstrates moderate effacement (arrow) of the foot processes of the glomerular visceral epithelial cells and microvillous transformation changes, evidence of recurrent FSGS. There were patent capillary loops, normal endothelial fenestration, and no immune deposits. There was effacement of approximately half of the epithelial cell foot processes on electron microscopy. After improvement of Pr:Cr ratio from plasma exchange therapy, the posttreatment glomeruli show features of epithelial foot process recovery. There is markedly less effacement and minimal microvillous transformation changes. Electron microscopy shows less than one quarter of the foot processes effaced and the majority having a normal arrangement.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4836136&req=5

Figure 2: Histologic response. The pretreatment biopsy demonstrates moderate effacement (arrow) of the foot processes of the glomerular visceral epithelial cells and microvillous transformation changes, evidence of recurrent FSGS. There were patent capillary loops, normal endothelial fenestration, and no immune deposits. There was effacement of approximately half of the epithelial cell foot processes on electron microscopy. After improvement of Pr:Cr ratio from plasma exchange therapy, the posttreatment glomeruli show features of epithelial foot process recovery. There is markedly less effacement and minimal microvillous transformation changes. Electron microscopy shows less than one quarter of the foot processes effaced and the majority having a normal arrangement.
Mentions: A 39-year-old Hispanic male with a history of end-stage renal disease (ESRD), hypertension and hypertrophic cardiomyopathy underwent deceased donor renal transplantation with thymoglobulin induction and standard steroid taper. He developed poor graft function and nephrotic-range proteinuria beginning on posttransplant day 1. He required posttransplant hemodialysis on posttransplant days 3, 6, 8, and 11, as well as initiation of therapeutic plasma exchange and IVIG on postoperative day 5. Renal transplant biopsies obtained on posttransplant day 7 confirmed the team's suspicion for FSGS recurrence in the renal allograft. The patient's treatment course is depicted in figure 1. Pretreatment and posttreatment biopsies are depicted in figure 2. The patient is currently 4 years posttransplant and continues to demonstrate excellent renal allograft function (serum creatinine of 1.6) with continued absence of proteinuria.

Bottom Line: Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of <0.5 was achieved.In patient number two, a second peak in proteinuria and azotemia was ultimately attributable to ureteral stenosis and these values normalized following repair.In conclusion, therapeutic plasma exchange is an effective treatment for FSGS recurring following renal transplant.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Laboratory Medicine, UC Irvine Health School of Medicine, USA.

ABSTRACT
Focal segmental glomerulosclerosis (FSGS) causes glomerular lesions that can progress to end-stage renal disease. It is suspected to be caused by a circulating factor that is amenable to plasmapheresis removal and exhibits a risk for recurrence in the renal allograft. We present two patients with FSGS recurrence in their allograft kidneys diagnosed by biopsy after significant proteinuria developed in the posttransplant setting. Treatment with therapeutic plasma exchange induced long-term remission in both patients. Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of <0.5 was achieved. In patient number two, a second peak in proteinuria and azotemia was ultimately attributable to ureteral stenosis and these values normalized following repair. In conclusion, therapeutic plasma exchange is an effective treatment for FSGS recurring following renal transplant.

No MeSH data available.


Related in: MedlinePlus