End-Stage Kidney Disease Due to Fibrillary Glomerulonephritis and Immunotactoid Glomerulopathy - Outcomes in 66 Consecutive ANZDATA Registry Cases

2015 ◽  
Vol 42 (3) ◽  
pp. 177-184 ◽  
Author(s):  
Andrew Mallett ◽  
Wen Tang ◽  
Gareth Hart ◽  
Stephen P. McDonald ◽  
Carmel M. Hawley ◽  
...  

Background: Fibrillary glomerulonephritis (FGN) and immunotactoid glomerulopathy (IG) are uncommon and characterised by non-amyloid fibrillary glomerular deposits. The aim of this study was to investigate characteristics and outcomes of patients undergoing renal replacement therapy (RRT) for end-stage kidney disease (ESKD) secondary to FGN and IG. Methods: All ESKD patients who commenced RRT in Australia and New Zealand 1 January 1990 to 31 December 2010 were included. Outcomes were assessed by Kaplan-Meier, multivariate logistic-regression analysis and multivariable Cox proportional-hazards survival analysis. Results: Of 45,216 individuals with ESKD, 55 (0.12%) had FGN and 11 (0.02%) had IG. The median survival of FGN patients on dialysis (5.63 years, 95% CI 3.31-7.96) was not significantly different from patients with other ESKD causes (median 4.01 years, 95% CI 4.34-4.47; log-rank 1.32, p = 0.25), but was significantly longer than that of IG patients (median 2.93 years, 95% CI 0.00-6.17; log-rank 4.8, p = 0.03). Thirteen (24%) FGN patients received 13 renal-allografts, 4 (36%) IG patients received 4 renal-allografts and 11,528 (26%) other ESKD patients received 12,278 renal-allografts. FGN patients experienced comparable outcomes to other ESKD patients for both 10-year patient survival (100 vs. 84%, p = 0.93) and renal-allograft survival (67 vs. 76%, p = 0.06). For IG, the median follow-up was 3.66 years with 75% patient survival and 100% renal-allograft survival. One (8%) FGN patient and 1 (25%) IG patient experienced recurrent FGN and IG respectively in their allograft. Conclusion: Patients with FGN have comparable dialysis and renal transplant outcomes to patients with other causes of ESKD. IG patients have inferior survival on dialysis, although renal transplant outcomes are acceptable. Disease recurrence in renal-allografts was low for both FGN and IG.

2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Gregory J. Wilson ◽  
Yeoungjee Cho ◽  
Armando Teixiera-Pinto ◽  
Nicole Isbel ◽  
Scott Campbell ◽  
...  

Abstract Background Membranoproliferative glomerulonephritis (MPGN) is an uncommon cause of end stage kidney disease (ESKD) and the clinical outcomes of patients with MPGN who commence kidney replacement therapy have not been comprehensively studied. Methods All adult patients with ESKD due to glomerulonephritis commencing kidney replacement therapy in Australia and New Zealand from January 1, 1996 to December 31, 2016 were reviewed. Patients with ESKD due to MPGN were compared to patients with other forms of glomerulonephritis. Patient survival on dialysis and following kidney transplantation, kidney recovery on dialysis, time to transplantation, allograft survival, death-censored allograft survival and disease recurrence post-transplant were compared between the two groups using Kaplan Meier survival curves and Cox proportional hazards regression. Results Of 56,481 patients included, 456 (0.8%) had MPGN and 12,660 (22.4%) had another form of glomerulonephritis. Five-year patient survival on dialysis and following kidney transplantation were similar between patients with ESKD from MPGN and other forms of glomerulonephritis (Dialysis: 59% vs. 62% p = 0.61; Transplant: 93% vs. 93%, p = 0.49). Compared to patients with other forms of glomerulonephritis, patients with MPGN had significantly poorer 5-year allograft survival (70% vs. 81% respectively, p = 0.02) and death censored allograft survival (74% vs. 87%, respectively; p < 0.01). The risk of disease recurrence was significantly higher in patients with MPGN compared to patients with other glomerulonephritidites (18% vs. 5%; p < 0.01). In patients with MPGN who had allograft loss, patients with MPGN recurrence had a significantly shorter time to allograft loss compared to patients with MPGN who had allograft loss due to any other cause (median time to allograft loss 3.2 years vs. 4.4 years, p < 0.01). Conclusions Compared with other forms of glomerulonephritis, patients with MPGN experienced comparable rates of survival on dialysis and following kidney transplantation, but significantly higher rates of allograft loss due to disease recurrence.


2021 ◽  
Vol 10 (14) ◽  
pp. 3063
Author(s):  
Napat Leeaphorn ◽  
Charat Thongprayoon ◽  
Pradeep Vaitla ◽  
Panupong Hansrivijit ◽  
Caroline C. Jadlowiec ◽  
...  

Background: Lower patient survival has been observed in sickle cell disease (SCD) patients who go on to receive a kidney transplant. This study aimed to assess the post-transplant outcomes of SCD kidney transplant recipients in the contemporary era. Methods: We used the OPTN/UNOS database to identify first-time kidney transplant recipients from 2010 through 2019. We compared patient and allograft survival between recipients with SCD (n = 105) vs. all other diagnoses (non-SCD, n = 146,325) as the reported cause of end-stage kidney disease. We examined whether post-transplant outcomes improved among SCD in the recent era (2010–2019), compared to the early era (2000–2009). Results: After adjusting for differences in baseline characteristics, SCD was significantly associated with lower patient survival (HR 2.87; 95% CI 1.75–4.68) and death-censored graft survival (HR 1.98; 95% CI 1.30–3.01), compared to non-SCD recipients. The lower patient survival and death-censored graft survival in SCD recipients were consistently observed in comparison to outcomes of recipients with diabetes, glomerular disease, and hypertension as the cause of end-stage kidney disease. There was no significant difference in death censored graft survival (HR 0.99; 95% CI 0.51–1.73, p = 0.98) and patient survival (HR 0.93; 95% CI 0.50–1.74, p = 0.82) of SCD recipients in the recent versus early era. Conclusions: Patient and allograft survival in SCD kidney recipients were worse than recipients with other diagnoses. Overall SCD patient and allograft outcomes in the recent era did not improve from the early era. The findings of our study should not discourage kidney transplantation for ESKD patients with SCD due to a known survival benefit of transplantation compared with remaining on dialysis. Urgent future studies are needed to identify strategies to improve patient and allograft survival in SCD kidney recipients. In addition, it may be reasonable to assign risk adjustment for SCD patients.


2019 ◽  
Vol 12 (1) ◽  
pp. 35-40
Author(s):  
Mahesha Vankalakunti ◽  
Ashish Parekh ◽  
Anil K. B. Thimmegowda ◽  
Rajanna Sreedhara

Anti-Glomerular Basement Membrane (anti-GBM) crescentic glomerulonephritis developing in an allograft is a rare phenomenon. A patient with Alport syndrome receiving a renal transplant is at risk of developing anti-GBM glomerulonephritis, due to the absence of normal COL4α3, COL4α4 and COL4α5 trimer of the collagen network. Two unique challenges with planning kidney transplant in such a patient include- ideal donor selection; and risk of developing anti-GBM nephritis. We report a case of post-transplant anti-GBM crescentic glomerulonephritis in a female recipient with unknown native kidney disease who was diagnosed with Alport disease when she presented with hematuria and proteinuria nearly 2 years postrenal transplant. Allograft outcome in our case was unfavourable, patient reaching end-stage kidney disease within 6-month of diagnosis. The patient remains on continuous ambulatory peritoneal dialysis and currently active on the deceased donor transplant waiting list.


2016 ◽  
Vol 30 (3) ◽  
pp. 256-262 ◽  
Author(s):  
Monica Saumoy ◽  
Arun B. Jesudian ◽  
Brandon Aden ◽  
David Serur ◽  
Subha Sundararajan ◽  
...  

2020 ◽  
Vol 40 (11) ◽  
pp. 6525-6530
Author(s):  
JANOS DOCS ◽  
DANIEL BANYAI ◽  
TIBOR FLASKO ◽  
ARPAD SZANTO ◽  
GYULA KOVACS

Author(s):  
Eva Pella ◽  
Afroditi Boutou ◽  
Aristi Boulmpou ◽  
Christodoulos E Papadopoulos ◽  
Aikaterini Papagianni ◽  
...  

Abstract Chronic kidney disease (CKD), especially end-stage kidney disease (ESKD), is associated with increased risk for cardiovascular events and all-cause mortality. Exercise intolerance as well as reduced cardiovascular reserve are extremely common in patients with CKD. Cardiopulmonary exercise testing (CPET) is a non-invasive, dynamic technique that provides an integrative evaluation of cardiovascular, pulmonary, neuropsychological and metabolic function during maximal or submaximal exercise, allowing the evaluation of functional reserves of these systems. This assessment is based on the principle that system failure typically occurs when the system is under stress and, thus, CPET is currently considered to be the gold-standard for identifying exercise limitation and differentiating its causes. It has been widely used in several medical fields for risk stratification, clinical evaluation and other applications but its use in everyday practice for CKD patients is scarce. This article describes the basic principles and methodology of CPET and provides an overview of important studies that utilized CPET in patients with ESKD, in an effort to increase awareness of CPET capabilities among practicing nephrologists.


Author(s):  
Micaella Sotera Hansen ◽  
Wubshet Tesfaye ◽  
Beena Sewlal ◽  
Bharati Mehta ◽  
Kamal Sud ◽  
...  

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