scholarly journals Clinical outcomes and temporal trends of immunological and non-immunological rare diseases in adult kidney transplant

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Ester Gallo ◽  
Silvia Mingozzi ◽  
Alberto Mella ◽  
Fabrizio Fop ◽  
Roberto Presta ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Kidney Transplant ◽  
Rare Diseases ◽  
De Novo ◽  
Case Reports ◽  
Temporal Trends ◽  
Case Series ◽  
High Volume ◽  
Post Transplant ◽  
Adult Kidney

Abstract Background Rare diseases (RDs) encompass many difficult-to-treat conditions with different characteristics often associated with end-stage renal disease (ESRD). However, data about transplant outcomes in adult patients are still lacking and limited to case reports/case series without differentiation between immunological/non-immunological RDs. Methods Retrospective analysis among all adult kidney transplanted patients (KTs) with RDs (RDsKT group) performed in our high-volume transplantation center between 2005 and 2016. RDs were classified according to the Orphanet code system differentiating between immunological and non-immunological diseases, also comparing clinical outcomes and temporal trends to a control population without RDs (nRDsKT). Results Among 1381 KTs, 350 patients (25.3%) were affected by RDs (RDsKTs). During a f/up > 5 years [median 7.9 years (4.8–11.1)], kidney function and graft/patient survival did not differ from nRDsKTs. Considering all post-transplant complications, RDsKTs (including, by definition, patients with primary glomerulopathy except on IgA nephropathy) have more recurrent and de-novo glomerulonephritis (14.6% vs. 9.6% in nRDsKTs; p = 0.05), similar rates of de-novo cancers, post-transplant diabetes, dysmetabolism, hematologic disorders, urologic/vascular problems, and lower infectious episodes than nRDsKTs (63.7% vs 72.7%; p = 0.013). Additional stratification for immunological and non-immunological RDsKTs or transplantation periods (before/after 2010) showed no differences or temporal trends between groups. Conclusions Kidney transplant centers are deeply involved in RDs management. Despite their high-complex profile, both immunological and non-immunological RDsKTs experienced favorable patients’ and graft survival.

P1726C3 GLOMERULOPATHY RECURRENCE AFTER KIDNEY TRANSPLANT: A SYSTEMATIC REVIEW

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Oumayma Taroua ◽  
Yassir Selouani
Keyword(s):  
Systematic Review ◽  
Kidney Transplant ◽  
Large Scale ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Qualitative Synthesis ◽  
Post Transplantation ◽  
Post Transplant ◽  
Disease Free

Abstract Background and Aims C3 glomerulopathy (C3G) is a recently defined entity, characterized by the dysregulation of the complement pathway, leading to deposition of C3 complement in the glomeruli, with no immunoglobulins, leading to glomerular inflammation. C3G encompasses 2 disorders: C3 glomerulonephritis (C3GN) and Dense Deposit Disease (DDD), distinguished by their morphological pattern. Although multiple pharmacological treatments exist to control the progression of the disease, for patients reaching End-Stage Kidney Disease, kidney transplantation remains the last resort. While it is known that membranoproliferative glomerulopathies carry a risk of recurrence after transplant, no large-scale meta-analysis was done after 2015 to assess if the precise recurrence risk and remission duration for C3 glomerulopathies. The goal of this work is to determine if there is currently enough literature specific for C3G to conduct such a meta-analysis. Method Our research protocol was guided by the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA-P), and the Joanna Briggs Institute Critical Appraisal Tools. A search was conducted in PubMed, Web of Science and Scopus databases, using a specific search string, at the conclusion of which, 185 papers were found. The identified papers were subsequently screened by the 2 authors independently using precise inclusion and exclusion criteria. The screening resulted in the final inclusion of 7 papers, on which a qualitative synthesis was performed. The information extracted was organized on basis of demographics, time of transplantation, disease recurrence and disease-free period post-transplantation. Results Among the 7 papers selected, 2 were case series and 5 were case reports. In total, 23 patients were reported as having recurrence of C3G. For the patients whose age at diagnosis was known, it ranged between 9 and 51 years of age. Among the 23 patients, 16 of them were male, while 7 of them were females. The C3G subtype was determined for 21 patients, with 7 being classified as having DDD, and 14 having C3GN. The age of transplant was reported for 20 patients, ranging from 11 to 70 years old. The disease-free period between the kidney transplant and the recurrence of the disease ranged from 14 days to 101 months, with 1 case series paper only reported the median time to recurrence in months (14[0-80] for C3GN patients and 15[2-32] for DDD patients). The same paper reported the post-transplant recurrence rate among transplanted patients, determined as 10 to 12 for C3GN patients and 6 to 7 for DDD patients. Conclusion While C3G, with its 2 subtypes, have been well defined entities for 5 years, our review reveals that very little research about the post-transplant evolution and recurrence of these disease has been done. While extensive research can be found on the recurrence risks of Membranoproliferative Glomerulonephritis, we believe that with the new classification, more data on the new subtypes is necessary to guide the decision-making of clinicians for these patients.

scholarly journals Incidence and Impacts of Inflammatory Bowel Diseases among Kidney Transplant Recipients: A Meta-Analysis

2020 ◽  
Vol 8 (3) ◽  
pp. 39
Author(s):  
Panupong Hansrivijit ◽  
Max M. Puthenpura ◽  
Charat Thongprayoon ◽  
Himmat S. Brar ◽  
Tarun Bathini ◽  
...  
Keyword(s):  
Kidney Transplant ◽  
De Novo ◽  
Meta Analysis ◽  
Transplant Recipients ◽  
Kidney Transplant Recipients ◽  
Post Transplant ◽  
Adult Kidney ◽  
Bowel Diseases ◽  
Inflammatory Bowel

Background: The incidence of inflammatory bowel diseases (IBD) and its significance in kidney transplant recipients is not well established. We conducted this systematic review and meta-analysis to assess the incidence of and complications from IBD in adult kidney transplant recipients. Methods: Eligible articles were searched through Ovid MEDLINE, EMBASE, and the Cochrane Library from inception through April 2020. The inclusion criteria were adult kidney transplant patients with reported IBD. Effect estimates from the individual studies were extracted and combined using the fixed-effects model when I2 ≤ 50% and random-effects model when I2 > 50%. Results: of 641 citations, a total of seven studies (n = 212) were included in the systematic review. The mean age was 46.2 +/− 6.9 years and up to 51.1% were male. The mean duration of follow-up was 57.8 +/− 16.8 months. The pooled incidence of recurrent IBD was 27.6% (95% CI, 17.7–40.5%; I2 0%) while the pooled incidence of de novo IBD was 18.8% (95% CI, 10.7–31.0%; I2 61.3%). The pooled incidence of post-transplant IBD was similar across subgroup analyses. Meta-regression analyses showed no association between the incidence of IBD and age, male sex, and follow-up duration. For post-transplant complications, the pooled incidence of post-transplant infection was 4.7% (95% CI, 0.5–33.3%; I2 73.7%). The pooled incidence of graft rejection and re-transplantation in IBD patients was 31.4% (95% CI, 14.1–56.1%; I2 76.9%) and 30.4% (95% CI, 22.6–39.5%; I2 0%). Conclusion: Recurrent and de novo IBD is common among kidney transplant recipients and may result in adverse outcomes.

scholarly journals Laminopathies’ Treatments Systematic Review: A Contribution Towards a ‘Treatabolome’

2021 ◽  
pp. 1-21
Author(s):  
Antonio Atalaia ◽  
Rabah Ben Yaou ◽  
Karim Wahbi ◽  
Annachiara De Sandre-Giovannoli ◽  
Corinne Vigouroux ◽  
...  
Keyword(s):  
Rare Diseases ◽  
Case Reports ◽  
Phenotypic Expression ◽  
Genetic Diagnosis ◽  
Specific Treatment ◽  
Case Series ◽  
Treatment Recommendations ◽  
Scientific Data ◽  
Bibliographic Databases ◽  
Central Registry

Background: Variants in the LMNA gene, encoding lamins A/C, are responsible for a growing number of diseases, all of which complying with the definition of rare diseases. LMNA-related disorders have a varied phenotypic expression with more than 15 syndromes described, belonging to five phenotypic groups: Muscular Dystrophies, Neuropathies, Cardiomyopathies, Lipodystrophies and Progeroid Syndromes. Overlapping phenotypes are also reported. Linking gene and variants with phenotypic expression, disease mechanisms, and corresponding treatments is particularly challenging in laminopathies. Treatment recommendations are limited, and very few are variant-based. Objective: The Treatabolome initiative aims to provide a shareable dataset of existing variant-specific treatment for rare diseases within the Solve-RD EU project. As part of this project, we gathered evidence of specific treatments for laminopathies via a systematic literature review adopting the FAIR (Findable, Accessible, Interoperable, and Reusable) guidelines for scientific data production. Methods: Treatments for LMNA-related conditions were systematically collected from MEDLINE and Embase bibliographic databases and clinical trial registries (Cochrane Central Registry of Controlled Trials, clinicaltrial.gov and EudraCT). Two investigators extracted and analyzed the literature data independently. The included papers were assessed using the Oxford Centre for Evidence-Based Medicine 2011 Levels of Evidence. Results: From the 4783 selected articles by a systematic approach, we identified 78 papers for our final analysis that corresponded to the profile of data defined in the inclusion and exclusion criteria. These papers include 2 guidelines/consensus papers, 4 meta-analyses, 14 single-arm trials, 15 case series, 13 cohort studies, 21 case reports, 8 expert reviews and 1 expert opinion. The treatments were summarized electronically according to significant phenome-genome associations. The specificity of treatments according to the different laminopathic phenotypical presentations is variable. Conclusions: We have extracted Treatabolome-worthy treatment recommendations for patients with different forms of laminopathies based on significant phenome-genome parings. This dataset will be available on the Treatabolome website and, through interoperability, on genetic diagnosis and treatment support tools like the RD-Connect’s Genome Phenome Analysis Platform.

Multi-institutional evaluation of the clinical outcomes and genomic correlates of African Americans with metastatic castration-sensitive prostate cancer (mCSPC).

2021 ◽  
Vol 39 (6_suppl) ◽  
pp. 17-17
Author(s):  
Meredith MI Freeman ◽  
Ellen Jaeger ◽  
Jason Zhu ◽  
Audrey Phone ◽  
Roberto Nussenzveig ◽  
...  
Keyword(s):  
Prostate Cancer ◽  
Retrospective Analysis ◽  
Clinical Outcomes ◽  
De Novo ◽  
High Volume ◽  
Abiraterone Acetate ◽  
Phase Iii ◽  
Treatment Intensification ◽  
Radium 223 ◽  
Incidence And Mortality

17 Background: Prostate cancer incidence and mortality is higher in African American (AA) as compared with non-AA men. The outcomes of mCSPC have significantly improved through treatment intensification yet, AA representation in those studies was suboptimal. We aimed to report the clinical, treatment outcomes and genomic data of AA men with mCSPC. Methods: Retrospective analysis of consecutive AA men with mCSPC at six Academic Institutions. The primary objective was to report the baseline characteristics and treatment patterns of mCSPC AA patients. The secondary objectives included the germline and somatic data and the clinical outcomes including PSA response, progression-free survival and subsequent treatments. Results: A total of 71 patients, median age 63 years (range, 41-84) with 58% Gleason 8-10, initial PSA of 69.8 ng/mL (0.02-7650), 59% with de-novo and 55% with high-volume (CHAARTED criteria; 20% visceral) disease, were included in this analysis. Twenty-two patients (31%) were treated with androgen deprivation therapy (ADT; 67% prior to year 2017), while 24%, 45% and 3% received docetaxel (median 6 cycles), abiraterone acetate and enzalutamide, respectively. Two patients received triplet therapy with ADT/docetaxel plus abiraterone or enzalutamide. Undetectable PSA was achieved in 35% after a median of 8.9 months (1.8-22.3). Among patients with mCSPC who received radiation therapy to prostate (n = 8), 89% had low volume disease. At time of cut off, thirty-two patients developed CRPC and the estimated median time to CRPC was 2.9 years (95% CI, 1.6-4.2). Subsequent therapies (n = 29) included abiraterone acetate (41%), enzalutamide (24%), bicalutamide (10%), radium-223 (7%), chemotherapy (7%), sipuleucel-T (3%) and others (7%). Five patients (8%) had pathogenic germline alterations (n = 2 BRCA1; n = 1 HOXB13, PALB2 and PMS2). Additionally, the most common somatic alterations among tested patients (n = 27) included CDK12, SPOP, TMPRSS2-ERG fusion, and TP53, all in 11% frequency. Of note, n = 2 BRCA1 and n = 1 high MSI/TMB. Conclusions: In one of the largest reported cohorts to our knowledge, mCSPC AA presented with a high number of de-novo and high-volume disease and might harbor a different germline and somatic genomic profile. The outcomes were comparable to contemporary phase III trials with treatment intensification, yet 31% were treated with ADT. Despite the known limitations associated with retrospective analysis, these data support prior observations where AA might have better initial PSA responses to ADT-based strategies compared with Caucasians, requiring further validation.

Abstract P101: Mechanical Thrombectomy in COVID-19 Positive Patients With Large Vessel Occlusion: Pooled Review of Individual Case Series

Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Muhammad Z Memon ◽  
Taha Nisar ◽  
Amit Singla ◽  
Anil Nanda ◽  
Gaurav Gupta ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Mechanical Thrombectomy ◽  
Case Reports ◽  
Case Series ◽  
Individual Case ◽  
Large Vessel ◽  
Large Vessel Occlusion ◽  
Vessel Occlusion ◽  
Historic Data ◽  
Meta Analyses

Background: COVID-19 has been shown to induce a hypercoagulable state thereby increasing the risk of arterial thrombosis resulting in Large Vessel Occlusion Stroke (LVOs) Objective: We performed a systematic review of published reports to study the clinical characteristics, and outcomes of COVID-19 acute ischemic stroke (AIS) patients with LVO treated with mechanical thrombectomy (MT) and compared them with historical controls. Methods: We conducted a systematic literature search from December 2019 to July 2020 using multiple combinations of keywords from PubMed and Ovid databases according to the PRISMA meta-analyses and systemic reviews guidelines and then pooled data from individual case series. We included studies where COVID -19 associated LVO cases were treated with MT and their clinical outcomes were reported. We then compared these findings with the historic patient data from the five landmark randomized MT trials, the Hermes collaborators (HC). Results: An initial search generated 12 studies but after excluding case reports and multiple reports comprising of the same series of patients, a total of five reports consisting of 51 patients were analyzed. The mean age of patients was 59 years (IQR 36-75), and 40 (78 %) were men. Median NIHSS on presentation was 20 (IQR 10-29). AIS with LVO was the presenting manifestation of COVID-19 in 16 (20%) of patients. Intracranial ICA was the most common site of occlusion found in 27 (53%) of patients with multi-territory occlusion in 10 (20 %). Final recanalization TICI ≥ 2b was achieved in 33 (64%) of patients but reocclusion was noted in 7 (14 %). Modified Rankin score (mRS) 0-2 was reported in 12 (23 %) of patients with 40 % in-hospital mortality. When compared to historic data from HC, COVID -19 patients were younger (59 vs 69 years), presented with a higher median NIHSS score (20 vs 17), and had a higher prevalence of ICA terminus occlusion (53% vs 21% ). Similarly, patient outcomes were poor in the COVID -19 group with mRs 0-2 in (23 % versus 46 %) and mortality (40 % vs 15 %) compared to Hermes group. Conclusion: COVID -19 AIS patients with LVO who underwent MT were younger, had multiple territory occlusions with a propensity for ICA terminus location, and had poor angiographic and clinical outcomes as compared to historic data.

scholarly journals BK polyomavirus nephropathy in two kidney transplant patients with distinct diagnostic strategies for BK virus and similar clinical outcomes: two case reports

2017 ◽  
Vol 11 (1) ◽  
Author(s):  
Ana Luisa Figueira Gouvêa ◽  
Rachel Ingrid Juliboni Cosendey ◽  
Ana Lucia Rosa Nascimento ◽  
Fabiana Rabe Carvalho ◽  
Andrea Alice Silva ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Kidney Transplant ◽  
Case Reports ◽  
Bk Virus ◽  
Diagnostic Strategies ◽  
Transplant Patients ◽  
Bk Polyomavirus ◽  
Kidney Transplant Patients

scholarly journals Post-transplant Lymphoproliferative Disorders: Single Center Case Series and Literature Review

2020 ◽  
pp. 1-6
Author(s):  
Sumayyah Altamimi ◽  
◽  
Mohamed Al Shuaibi ◽  
Mohamed Alnahdi ◽  
Abdulrahman Altheaby ◽  
...  
Keyword(s):  
De Novo ◽  
Organ Transplant ◽  
Tumor Development ◽  
Case Series ◽  
Standard Of Care ◽  
Lymphoproliferative Disorders ◽  
Outcome Analysis ◽  
Solid Organ ◽  
Hematopoietic Stem ◽  
Post Transplant

Post-transplant lymphoproliferative disorders (PTLD) are referred to lymphoid and/or plasmacytic proliferation which occur as result of immunosuppression therapy in patient underwent solid organ or allogeneic hematopoietic stem cell transplantation. Among solid organ transplant recipients, it accounts approximately 20% of all cancers. Epstein-Barr virus (EBV) has been linked to the pathogenesis of PTLD when EBV was identified in the tumor biopsies. In most affected patients, PTLD occurs as a result of proliferation of EBV positive B cell following immunosuppression and impaired Tcell immune activity. EBV negative PTLD has been documented but no clear etiology has been confirmed, however theories of previous exposure to EBV which is completely cleared at the time of PTLD is diagnosed, different viruses or chronic antigenic stimulation all been considered as provoking factors for tumor development. Clinical symptomatology at presentation of patients with PTLD is usually indistinct from de novo lymphoproliferative disorders and chemotherapy protocols in this group of patients are generally similar to the standard of care of lymphoma treatment according to the subtype in addition to cessation or dose reduction of immunosuppressive therapy. We report our experience and outcome analysis in PTLD management among 23 patients from our institution treated between 2011 and 2019.

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