Successful Preemptive Kidney Transplantation With Rituximab Induction in a Patient With Focal Segmental Glomerulosclerosis and Massive Nephrotic Syndrome: A Case Report

2016 ◽  
Vol 48 (9) ◽  
pp. 3092-3094 ◽  
Author(s):  
A. Kolonko ◽  
G. Piecha ◽  
A. Więcek
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Transplantation ◽  
Case Report ◽  
Focal Segmental Glomerulosclerosis ◽  
Segmental Glomerulosclerosis ◽  
Preemptive Kidney Transplantation

scholarly journals A clinical case of using therapeutic plasma exchange for the treatment of recurrent focal segmental glomerulosclerosis in a child after kidney transplantation

2021 ◽  
Vol 23 (2) ◽  
pp. 60-66
Author(s):  
I. A. Miloserdov ◽  
V. S. Bogdanov ◽  
P. M. Gadzhieva ◽  
D. A. Saydulaev ◽  
A. A. Kartashev ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Transplantation ◽  
Plasma Exchange ◽  
Kidney Transplant ◽  
Focal Segmental Glomerulosclerosis ◽  
Graft Function ◽  
Therapeutic Plasma Exchange ◽  
Kidney Graft ◽  
Graft Dysfunction ◽  
Segmental Glomerulosclerosis

Background. Focal segmental glomerulosclerosis (FSGS) of the graft in kidney recipients is a rare and difficultto-diagnose post-kidney transplant complication, which can lead to graft loss and death of the recipient. A unified protocol is required for the treatment of this disease.Materials and methods. A 15-year-old female patient C. diagnosed with stage 5 chronic kidney disease as a result of steroid-resistant nephrotic syndrome with hematuria underwent a living related-donor kidney transplantation. On the third day after the operation, laboratory and imaging data showed kidney graft dysfunction. Patient examinations established the cause of the graft dysfunction – idiopathic nephrotic syndrome in FSGS.Results. For the treatment of recurrent FSGS, the patient had her immunosuppressive therapy converted from tacrolimus to cyclosporin A, and received two 500 mg rituximab injections. Ten sessions of therapeutic plasma exchange (Plasauto Sigma) were performed to remove antibodies to podocytes. During the therapy, diuresis was restored, creatinine and urea levels decreased. Six months after the kidney transplant, graft function was fully restored. Conclusion. The absence of recurrent FSGS within six months during a single course of therapeutic plasma exchange with its subsequent cancellation after restoration of graft function allows to recommend the developed method for the treatment of FSGS in pediatric patients after kidney transplantation.

scholarly journals Paraneoplastic Focal Segmental Glomerulosclerosis Associated With Gastrointestinal Stromal Tumors With Cutaneous Metastasis: a Case Report

2021 ◽  
Author(s):  
Jun Zhou ◽  
Zhen Yang ◽  
Cuishun Yang ◽  
Hua Lin ◽  
Wanqiong Yuan
Keyword(s):  
Nephrotic Syndrome ◽  
Case Report ◽  
Focal Segmental Glomerulosclerosis ◽  
Hippo Pathway ◽  
Symptomatic Treatment ◽  
Cutaneous Metastasis ◽  
Negative Regulator ◽  
Hippo Signaling ◽  
Segmental Glomerulosclerosis ◽  
The Hippo Pathway

Abstract Background: Gastrointestinal stromal tumor (GIST) is one of most common mesenchymal neoplasms occurring in different areas of the gastrointestinal tract. GISTs with cutaneous metastasis is very rare and its rarity cutaneous GISTs have not been well characterized. Focal segmental glomerulosclerosis (FSGS) is also rare among paraneoplastic nephritic syndromes (PNS).Case presentation: In this case report, we described a 64-year-old patient with cutaneous metastasis GIST accompanied by nephrotic syndrome as PNS, in whom symptomatic treatment was ineffective, but clinical remission was achieved after surgery. Moreover, the patient has a missense mutation of NPHP4. NPHP4 served as a negative regulator of the Hippo pathway. Hippo signaling pathway is involved in the development and progression of FSGS. NPHP4 is also indeed a driving force for proliferation in tumor cells. Therefore, the mutation of NPHP4 in this patient could explain the occurrence of GIST and FSGS and this was therefore not a random association.Conclusions: This is the first reported case of a GIST with cutaneous metastasis accompanied by nephrotic syndrome as PNS.

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.

scholarly journals Clinical manifestations of focal segmental glomerulosclerosis in Japan from the Japan Renal Biopsy Registry: age stratification and comparison with minimal change disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Takaya Ozeki ◽  
Shoichi Maruyama ◽  
Toshiyuki Imasawa ◽  
Takehiko Kawaguchi ◽  
Hiroshi Kitamura ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Clinical Diagnosis ◽  
Clinical Characteristics ◽  
Minimal Change Disease ◽  
Multivariable Analysis ◽  
Laboratory Data ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

AbstractFocal segmental glomerulosclerosis (FSGS) is a serious condition leading to kidney failure. We aimed to investigate the clinical characteristics of FSGS and its differences compared with minimal change disease (MCD) using cross-sectional data from the Japan Renal Biopsy Registry. In Analysis 1, primary FSGS (n = 996) were stratified by age into three groups: pediatric (< 18 years), adult (18–64 years), and elderly (≥ 65 years), and clinical characteristics were compared. Clinical diagnosis of nephrotic syndrome (NS) was given to 73.5% (97/132) of the pediatric, 41.2% (256/622) of the adult, and 65.7% (159/242) of the elderly group. In Analysis 2, primary FSGS (n = 306) and MCD (n = 1303) whose clinical diagnosis was nephrotic syndrome (NS) and laboratory data were consistent with NS, were enrolled. Logistic regression analysis was conducted to elucidate the variables which can distinguish FSGS from MCD. On multivariable analysis, higher systolic blood pressure, higher serum albumin, lower eGFR, and presence of hematuria associated with FSGS. In Japanese nationwide registry, primary FSGS patients aged 18–64 years showed lower rate of NS than those in other ages. Among primary nephrotic cases, FSGS showed distinct clinical features from MCD.

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