scholarly journals Pediatric focal segmental glomerulosclerosis: favorable transplantation outcome with plasma exchange

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Fatina I. Fadel ◽  
Hafez M. Bazaraa ◽  
Mohamed A. Abdel Mawla ◽  
Doaa M. Salah
Keyword(s):  
Serum Creatinine ◽  
Plasma Exchange ◽  
Focal Segmental Glomerulosclerosis ◽  
Survival Data ◽  
Late Recurrence ◽  
Early Recurrence ◽  
Kidney Transplant Recipients ◽  
Segmental Glomerulosclerosis ◽  
Kaplan Meier ◽  
End Stage

Abstract Background Although kidney transplantation (KTX) is the treatment of choice for pediatric end stage kidney disease (ESKD); concerns for recurrence in cases of focal segmental glomerulosclerosis (FSGS) are still present. This study aimed to investigate the outcome of KTX in children with ESKD secondary to FSGS, with implementation of preemptive perioperative plasma exchange (PE) for non-genetically proven patients. Methods Forty FSGS pediatric kidney transplant recipients were studied. Of them: 12 patients (30%) had genetically proven NPHS2 mutations/familial and 28 (70%) were sporadic FSGS patients. All sporadic patients electively received 6 perioperative PE sessions. Patients with recurrence of proteinuria (n = 13; including 3 patients with genetic/familial and 10 patients with sporadic FSGS) were managed with PE and Rituximab (RTX). Kaplan-Meier curves were used to analyze graft and recurrence free survival data. Results The mean follow-up duration after KTX was 3.8 ± 2.86 years. Recurrence of proteinuria was encountered early postoperative in 11 patients (27.5%) and late (1.6 and 2.9 years after KTX) in 2 patients (5%). All patients with early recurrence achieved complete remission, while patients with late recurrence developed graft failure. Current serum creatinine and proteinuria levels were not different in patients received PE (n = 31) and patients did not PE (n = 9) (p = 0.308 and 0.287 respectively). Current serum creatinine and proteinuria levels in sporadic patients (n = 28) after prophylactic perioperative PE were not different from those of genetic/ familial patients (n = 12) (p = 0.303 and 0.144 respectively). Proteinuria was less in patients underwent native nephrectomy than others immediately postoperative and at assessment (p = 0.002 & 0.0031 respectively). One-year graft and patient survival was 93.8% with a mean 1-year serum creatinine of 0.67 ± 0.25 mg/dl. Three graft losses (7.5%) were due to chronic rejection 3.3, 3.75 and 4.17 years after KTX and 2 patients’ mortality (5%) occurred early postoperative (first 2 weeks). Conclusion FSGS transplanted children have favorable outcomes with perioperative PE for non-genetically proven cases. Early recurrence after KTX can be successfully managed with PE and RTX.

scholarly journals Pediatric Focal Segmental Glomerulosclerosis: Favorable Transplantation Outcome With Plasma Exchange

2021 ◽  
Author(s):  
Fatina I Fadel ◽  
Hafez M Bazaraa ◽  
Mohamed A Abdel Mawla ◽  
Doaa M Salah
Keyword(s):  
Plasma Exchange ◽  
Focal Segmental Glomerulosclerosis ◽  
Survival Data ◽  
Early Recurrence ◽  
Transplant Recipients ◽  
Kidney Transplant Recipients ◽  
Segmental Glomerulosclerosis ◽  
Kaplan Meier ◽  
One Year

Abstract Background: Although kidney transplantation (KT) is the treatment of choice for pediatric kidney failure (KF); concerns for recurrence in cases of focal segmental glomerulosclerosis (FSGS) are still present. This study aimed to investigate the outcome of KT in children with KF secondary to FSGS, with implementation of preemptive perioperative plasma exchange (PE) for non-genetically proven patients.Methods: Forty FSGS pediatric kidney transplant recipients were studied. Of them: 12 patients (30%) had genetically proven NPHS2 mutations/familial and 28 (70%) were sporadic FSGS patients. Sporadic patients electively received 6 preoperative PE sessions. Recurrence of proteinuria was managed with PE and Rituximab (RTX). Kaplan-Meier curves were used to analyze graft and recurrence free survival data.Results: The mean follow-up duration after KT was 3.8 ± 2.86 years. Recurrence of proteinuria was encountered early postoperative in 11 patients (27.5%) and late (1.6 and 2.9 years after KT) in 2 patients (5%). Proteinuria was less in patients underwent native nephrectomy than others immediately postoperative and at assessment (p= 0.002 & 0.0031 respectively). One-year graft and patient survival was 93.8% with a mean 1-year serum creatinine of 0.67 ± 0.25 mg/dl. Three graft losses (7.5%) were due to chronic rejection 3.3, 3.75 and 4.17 years after KT and 2 patients' mortality (5%) occurred early postoperative (first 2 weeks) due to infection.Conclusion: FSGS transplanted children have favorable outcomes with perioperative PE for non-genetically proven cases. Early recurrence after KT can be successfully managed with PE and RTX.

scholarly journals Focal Segmental Glomerulosclerosis

1999 ◽  
Vol 10 (9) ◽  
pp. 1900-1907
Author(s):  
MELVIN M. SCHWARTZ ◽  
JONI EVANS ◽  
RAY BAIN ◽  
STEPHEN M. KORBET
Keyword(s):  
Serum Creatinine ◽  
Focal Segmental Glomerulosclerosis ◽  
End Stage Renal Disease ◽  
Poor Response ◽  
Steroid Treatment ◽  
Response To Treatment ◽  
Therapeutic Trial ◽  
Segmental Glomerulosclerosis ◽  
Stage Renal Disease ◽  
End Stage

Abstract. The cellular lesion (CELL), seen in some patients with primary focal segmental glomerulosclerosis (FSGS), comprises proliferation, hypertrophy, and pathologic changes in the cells overlying the glomerular scar. The prognosis of the cellular lesion was retrospectively studied in 100 patients with FSGS (43 had FSGS-CELL and 57 had FSGS without the cellular lesion (classic segmental scar [CS]). Patients with the FSGS-CELL lesion were more often black and severely proteinuric and developed more end-stage renal disease (ESRD). Nephrotic patients with FSGS-CELL (n = 39) were more proteinuric at presentation than patients with FSGS-CS (n = 36). ESRD developed more frequently in patients with the FSGS-CELL (17 of 39, 44% versus 5 of 36, 14%, P = 0.005), and patients with extensive FSGS-CELL (≥ 20% glomeruli) were mainly black (94%), severely nephrotic (67%, >10 g/d), and had a poor response to treatment (23% remission). In nephrotic patients, initial serum creatinine, interstitial expansion ≥20%, and CELL independently predicted ESRD. However, the rates of remission in treated nephrotic patients with FSGS-CELL and FSGS-CS were the same (9 of 17, 53% versus 17 of 39, 52%), and patients in both groups who achieved a remission had a 5-yr survival of 100%. Steroid treatment was the only variable that predicted remission. Patients with the FSGS-CELL have an increased prevalence of ESRD, but the improved prognosis associated with remission is so significant that a therapeutic trial is warranted in all nephrotic FSGS patients, regardless of the presence of the cellular lesion.

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

2016 ◽  
Vol 6 (1) ◽  
pp. 53-60 ◽  
Author(s):  
Minh-Ha Tran ◽  
Cynthia Chan ◽  
Whitney Pasch ◽  
Philip Carpenter ◽  
Hirohito Ichii ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Focal Segmental Glomerulosclerosis ◽  
Renal Allograft ◽  
Therapeutic Plasma Exchange ◽  
Spot Urine ◽  
Segmental Glomerulosclerosis ◽  
Glomerular Lesions ◽  
Stage Renal Disease ◽  
End Stage

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.

scholarly journals Novel diagnostic and therapeutic techniques reveal changed metabolic profiles in recurrent focal segmental glomerulosclerosis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Janina Müller-Deile ◽  
George Sarau ◽  
Ahmed M. Kotb ◽  
Christian Jaremenko ◽  
Ulrike E. Rolle-Kampczyk ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Raman Spectra ◽  
Focal Segmental Glomerulosclerosis ◽  
Disease Recurrence ◽  
Early Recurrence ◽  
Metabolome Analysis ◽  
Novel Technologies ◽  
Filtration Barrier ◽  
Segmental Glomerulosclerosis ◽  
Living Related

AbstractIdiopathic forms of Focal Segmental Glomerulosclerosis (FSGS) are caused by circulating permeability factors, which can lead to early recurrence of FSGS and kidney failure after kidney transplantation. In the past three decades, many research endeavors were undertaken to identify these unknown factors. Even though some potential candidates have been recently discussed in the literature, “the” actual factor remains elusive. Therefore, there is an increased demand in FSGS research for the use of novel technologies that allow us to study FSGS from a yet unexplored angle. Here, we report the successful treatment of recurrent FSGS in a patient after living-related kidney transplantation by removal of circulating factors with CytoSorb apheresis. Interestingly, the classical published circulating factors were all in normal range in this patient but early disease recurrence in the transplant kidney and immediate response to CytoSorb apheresis were still suggestive for pathogenic circulating factors. To proof the functional effects of the patient’s serum on podocytes and the glomerular filtration barrier we used a podocyte cell culture model and a proteinuria model in zebrafish to detect pathogenic effects on the podocytes actin cytoskeleton inducing a functional phenotype and podocyte effacement. We then performed Raman spectroscopy in the < 50 kDa serum fraction, on cultured podocytes treated with the FSGS serum and in kidney biopsies of the same patient at the time of transplantation and at the time of disease recurrence. The analysis revealed changes in podocyte metabolome induced by the FSGS serum as well as in focal glomerular and parietal epithelial cell regions in the FSGS biopsy. Several altered Raman spectra were identified in the fractionated serum and metabolome analysis by mass spectrometry detected lipid profiles in the FSGS serum, which were supported by disturbances in the Raman spectra. Our novel innovative analysis reveals changed lipid metabolome profiles associated with idiopathic FSGS that might reflect a new subtype of the disease.

scholarly journals Clinical and laboratory features that differentiate familial from sporadic focal segmental glomerulosclerosis in adolescents and adults

2021 ◽  
Vol 6 (1) ◽  
pp. 1-5
Author(s):  
Maria Goretti Polito ◽  
Michelle Tiveron Passos ◽  
Danilo Euclides Fernandes ◽  
Gianna Mastroianni-Kirsztajn
Keyword(s):  
Serum Albumin ◽  
Focal Segmental Glomerulosclerosis ◽  
Steroid Resistance ◽  
End Stage Kidney Disease ◽  
Steroid Resistant ◽  
Best Management ◽  
Segmental Glomerulosclerosis ◽  
Laboratory Features ◽  
Adolescents And Adults ◽  
End Stage

Background: Focal segmental glomerulosclerosis (FSGS) is an important cause of end-stage kidney disease in children and adults. Although most cases are sporadic (s), familial (f) presentation is also described. The purpose of the present study was to establish clinical and laboratory profiles of fFSGS vs. primary sFSGS, contributing to the distinguishing diagnosis in clinical practice and best management, in particular when mutation analysis is not available. Methods: Demographic, clinical and laboratorial parameters were studied in 124 patients 12 years and older with FSGS, subdivided in sFSGS (n=89) and fFSGS (n=35). Results: General and clinical features were similar, as well as serum creatinine at disease presentation. Proteinuria levels were more frequently ≥ 3g/day in sFSGS (63.8%) than in fFSGS (44%, p=0.080), and serum albumin levels were < 3.0 g/dL in 45.8% and 20%, respectively (p=0.046). The groups were statistically different regarding steroid resistance, corresponding to 60% in sFSGS and 100% in fFSGS (p=0.001). Conclusions: The studied groups were clinically similar, except that proteinuria tended to be higher (nephrotic range) and serum albumin was lower in sFSGS vs. fFSGS. In addition, all treated fFSGS patients were steroid resistant. At presentation it is important to characterize if the patient has fFSGS, that will contribute to further disease management, and disease history will be the first clue for such differential diagnosis.

scholarly journals Recent Advances in Treatments of Primary Focal Segmental Glomerulosclerosis in Children

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Kyoung Hee Han ◽  
Seong Heon Kim
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Calcineurin Inhibitors ◽  
Steroid Treatment ◽  
Renal Survival ◽  
Steroid Resistant ◽  
Segmental Glomerulosclerosis ◽  
Risk Of Progression ◽  
Traditional Therapies ◽  
Stage Renal Disease ◽  
End Stage

Focal segmental glomerulosclerosis (FSGS) is a nephrotic syndrome. Up to around 80% of cases of primary FSGS are resistant to steroid treatment. A large proportion of patients with steroid-resistant FSGS progress to end-stage renal disease. The purpose of treatment is to obtain a complete remission of proteinuria, a necessary step that precedes improved renal survival and reduces the risk of progression to chronic kidney disease. When this is not possible, the secondary goal is a partial remission of proteinuria. Reduction or remission of proteinuria is the most important factor predictive of renal survival. We will review the current updated strategies for treatment of primary FSGS in children, including traditional therapies consisting of corticosteroids and calcineurin inhibitors and novel therapies such as rituximab, abatacept, adalimumab, and fresolimumab.

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