scholarly journals Screening for Fabry Disease in Kidney Transplant Recipients: Experience of a Multidisciplinary Team

Biomedicines ◽  
2020 ◽  
Vol 8 (10) ◽  
pp. 396 ◽  
Author(s):  
Massimiliano Veroux ◽  
Ines Monte ◽  
Margherita Rodolico ◽  
Daniela Corona ◽  
Rita Bella ◽  
...  

Fabry disease (FD) is a rare cause of end-stage renal disease requiring kidney transplantation. Data on the incidence of unrecognized FD in kidney transplant recipients are scarce and probably underestimated. This study evaluated the incidence of FD in a population of kidney recipients, with a particular focus of the multidisciplinary approach for an early clinical assessment and therapeutic approach. Two hundred sixty-five kidney transplant recipients were screened with a genetic analysis for α-galactosidase A (GLA) mutation, with measurement of α-Gal A enzyme activity and Lyso Gb3 levels. Screening was also extended to relatives of affected patients. Seven patients (2.6%) had a GLA mutation. Two patients had a classic form of FD with Fabry nephropathy. Among the relatives, 15 subjects had a GLA mutation, and two had a Fabry nephropathy. The clinical and diagnostic assessment was completed after a median of 3.2 months, and mean time from diagnosis to treatment was 4.6 months. This study reported a high incidence of unrecognized GLA mutations in kidney transplant recipients. Evaluation and management by a multidisciplinary team allowed for an early diagnosis and treatment, and this would result in a delay in the progression of the disease and, finally, in better long-term outcomes.

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.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Umesh Lingaraj ◽  
Ricken Mehta ◽  
Shivaprasad SM ◽  
Kishan A ◽  
Leelavathi V ◽  
...  

Abstract Background and Aims Glomerulonephritis (GN) is a major cause of end stage renal disease (ESRD)1. It represents the primary cause of end stage renal disease (ESRD) for 25% of the dialysis population1 and 45% of the transplant population. For patients with GN requiring renal replacement therapy, kidney transplantation is associated with superior outcomes compared with dialysis2. The possibility of recurrence of the original disease after transplantation was described in a seminal paper more than 40 years ago, and it is now clear that all forms of GN may recur after kidney transplantation.3 To study the recurrence of glomerulonephritis post-transplant in a tertiary care centre. Method 120 renal transplant recipients were analyzed from September 2015 to August 2019 at the Institute of Nephro-Urology, Bangalore. It was a retrospective analysis of data Results 120 adult patients underwent kidney transplantation, out of these 70 had GN as primary cause of kidney disease. 85.8% were males, 14.2 % females. 58.9 % were biopsy proven GN, remaining 41.1 % diagnosed based on history and clinical presentation. All but one patient had their first transplant. Out of these kidney transplant recipients 08 (11.4%) had recurrence of GN.  From these 4/08 was recurrent IgA N, 2/08 were PGNMID, 1/08 MGN, 1/08 aHUS. Graft loss due to recurrent GN was seen in 1/08 patients (12.5%). Conclusion Our study showed that 11.4 % of kidney transplant recipients with GN as their cause of ESRD had recurrent GN post kidney transplantation. IgAN was the most type of GN that recurred most frequently followed by PGNMID. Recurrence of GN was in par with other studies and did not affect graft survival


2007 ◽  
Vol 39 (4) ◽  
pp. 1091-1094 ◽  
Author(s):  
J. Ameli ◽  
H. Kachuee ◽  
S. Assari ◽  
V.R. Rasta ◽  
H.R. Khoddami-Vishte ◽  
...  

2021 ◽  
Vol 10 (8) ◽  
pp. 1551
Author(s):  
Marta Bodro ◽  
Frederic Cofan ◽  
Jose Ríos ◽  
Sabina Herrera ◽  
Laura Linares ◽  
...  

In the context of the coronavirus disease 2019 (COVID-19) pandemic, we aimed to evaluate the impact of anti-cytokine therapies (AT) in kidney transplant recipients requiring hospitalization due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. This is an observational retrospective study, which included patients from March to May 2020. An inverse probability of treatment weighting from a propensity score to receive AT was used in all statistical analyses, and we applied a bootstrap procedure in order to calculate an estimation of the 2.5th and 97.5th percentiles of odds ratio (OR). outcomes were measured using an ordinal scale determination (OSD). A total of 33 kidney recipients required hospitalization and 54% of them received at least one AT, mainly tocilizumab (42%), followed by anakinra (12%). There was no statistical effect in terms of intensive care unit (ICU) admission, respiratory secondary infections (35% vs. 7%) or mortality (16% vs. 13%) comparing patients that received AT with those who did not. Nevertheless, patients who received AT presented better outcomes during hospitalization in terms of OSD ≥5 ((OR 0.31; 2.5th, 97.5th percentiles (0.10; 0.72)). These analyses indicate, as a plausible hypothesis, that the use of AT in kidney transplant recipients presenting with COVID-19 could be beneficial, even though multicenter randomized control trials using these therapies in transplanted patients are needed.


Diseases ◽  
2020 ◽  
Vol 9 (1) ◽  
pp. 2
Author(s):  
Maria L. Gonzalez Suarez ◽  
Charat Thongprayoon ◽  
Panupong Hansrivijit ◽  
Juan Medaura ◽  
Pradeep Vaitla ◽  
...  

Background: Fabry disease (FD) is a rare X-linked lysosomal storage disorder with progressive systemic deposition of globotriaosylceramide, leading to life-threatening cardiac, central nervous system, and kidney disease. Current therapy involves symptomatic medical management, enzyme replacement therapy (ERT), dialysis, kidney transplantation, and, more recently, gene therapy. The aim of this systematic review was to assess outcomes of kidney transplantation among patients with FD. Methods: A comprehensive literature review was conducted utilizing MEDLINE, EMBASE, and Cochrane Database, from inception through to 28 February 2020, to identify studies that evaluate outcomes of kidney transplantation including patient and allograft survival among kidney transplant patients with FD. Effect estimates from each study were extracted and combined using the random-effects generic inverse variance method of DerSimonian and Laird. Results: In total, 11 studies, including 424 kidney transplant recipients with FD, were enrolled. The post-transplant median follow-up time ranged from 3 to 11.5 years. Overall, the pooled estimated rates of all-cause graft failure, graft failure before death, and allograft rejection were 32.5% (95%CI: 23.9%–42.5%), 14.5% (95%CI: 8.4%–23.7%), and 20.2% (95%CI: 15.4%–25.9%), respectively. In the sensitivity analysis, limited only to the recent studies (year 2001 or newer when ERT became available), the pooled estimated rates of all-cause graft failure, graft failure before death, and allograft rejection were 28.1% (95%CI: 20.5%–37.3%), 11.7% (95%CI: 8.4%–16.0%), and 20.2% (95%CI: 15.5%–26.0%), respectively. The pooled estimated rate of biopsy proven FD recurrence was 11.1% (95%CI: 3.6%–29.4%), respectively. There are no significant differences in the risks of all-cause graft failure (p = 0.10) or mortality (0.48) among recipients with vs. without FD. Conclusions: Despite possible FD recurrence after transplantation of 11.1%, allograft and patient survival are comparable among kidney transplant recipients with vs. without FD.


2017 ◽  
Author(s):  
Belinda T. Lee ◽  
Anil Chandraker ◽  
Jamil Azzi ◽  
Martina M McGrath

Kidney transplantation remains the optimal renal replacement therapy for patients with end-stage renal disease (ESRD). A timely referral to kidney transplantation and a thorough pretransplantation evaluation ensure improvement in the morbidity and mortality of ESRD patients. Basic knowledge of immune biology and an in-depth understanding of the different induction and maintenance therapies used post kidney transplantation are imperative for optimal patient management. In this review, we discuss the multidisciplinary process of pretransplantation evaluation of kidney transplant recipients. We also discuss state-of–the-art early management post kidney transplantation with the different immunosuppressive therapies currently available. This review contains 3 figures, 11 tables, and 106 references. Key words: crossmatch, donor-specific antibody, immunosuppression, human leukocyte antigen, immunosuppression, induction, maintenance, medical evaluation, transplantation


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