Glomerular diseases最新文献

{"title":"An Alport Syndrome Journey: From Powerless to Empowered - A Patient Perspective.","authors":"Lisa Bonebrake","doi":"10.1159/000529433","DOIUrl":"https://doi.org/10.1159/000529433","url":null,"abstract":"<p><p>As an Alport syndrome patient, caregiver, and executive director of Alport Syndrome Foundation, I am aware of the frequently challenging road in seeking an accurate diagnosis. Our journeys are scattered with misdiagnosis, missed opportunities for accurate diagnosis, counterproductive medications, and overwhelming guilt when our children are diagnosed. We understand that most of our healthcare providers know very little about our disease. Typically, it is incumbent upon us to become empowered through education and connection with our patient community to be sure that our physical and emotional health is well managed.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"42-46"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f5/35/gdz-2023-0003-0001-529433.PMC9939918.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9313963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ridge Regression for Functional Form Identification of Continuous Predictors of Clinical Outcomes in Glomerular Disease.","authors":"Jeremy Rubin,&nbsp;Laura Mariani,&nbsp;Abigail Smith,&nbsp;Jarcy Zee","doi":"10.1159/000528847","DOIUrl":"https://doi.org/10.1159/000528847","url":null,"abstract":"<p><strong>Introduction: </strong>Penalized regression models can be used to identify and rank risk factors for poor quality of life or other outcomes. They often assume linear covariate associations, but the true associations may be nonlinear. There is no standard, automated method for determining optimal functional forms (shapes of relationships) between predictors and the outcome in high-dimensional data settings.</p><p><strong>Methods: </strong>We propose a novel algorithm, ridge regression for functional form identification of continuous predictors (RIPR) that models each continuous covariate with linear, quadratic, quartile, and cubic spline basis components in a ridge regression model to capture potential nonlinear relationships between continuous predictors and outcomes. We used a simulation study to test the performance of RIPR compared to standard and spline ridge regression models. Then, we applied RIPR to identify top predictors of Patient-Reported Outcomes Measurement Information System (PROMIS) adult global mental and physical health scores using demographic and clinical characteristics among <i>N</i> = 107 glomerular disease patients enrolled in the Nephrotic Syndrome Study Network (NEPTUNE).</p><p><strong>Results: </strong>RIPR resulted in better predictive accuracy than the standard and spline ridge regression methods in 56-80% of simulation repetitions under a variety of data characteristics. When applied to PROMIS scores in NEPTUNE, RIPR resulted in the lowest error for predicting physical scores, and the second-lowest error for mental scores. Further, RIPR identified hemoglobin quartiles as an important predictor of physical health that was missed by the other models.</p><p><strong>Conclusion: </strong>The RIPR algorithm can capture nonlinear functional forms of predictors that are missed by standard ridge regression models. The top predictors of PROMIS scores vary greatly across methods. RIPR should be considered alongside other machine learning models in the prediction of patient-reported outcomes and other continuous outcomes.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"47-55"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/df/0d/gdz-0003-0047.PMC10126734.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9364659","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"HIV-Associated Nephropathy in 2022.","authors":"Frederick Berro Rivera,&nbsp;Marie Francesca Mapua Ansay,&nbsp;Jem Marie Golbin,&nbsp;Pia Gabrielle I Alfonso,&nbsp;Gerard Francis E Mangubat,&nbsp;Rajiv Hans Solita Menghrajani,&nbsp;Siena Placino,&nbsp;Marianne Katharina Vicera Taliño,&nbsp;Deogracias Villa De Luna,&nbsp;Nicolo Cabrera,&nbsp;Carlo Nemesio Trinidad,&nbsp;Amir Kazory","doi":"10.1159/000526868","DOIUrl":"https://doi.org/10.1159/000526868","url":null,"abstract":"<p><strong>Background: </strong>HIV-associated nephropathy (HIVAN) is a renal parenchymal disease that occurs exclusively in people living with HIV. It is a serious kidney condition that may possibly lead to end-stage kidney disease, particularly in the HIV-1 seropositive patients.</p><p><strong>Summary: </strong>The African-American population has increased susceptibility to this comorbidity due to a strong association found in the <i>APOL1</i> gene, specifically two missense mutations in the G1 allele and a frameshift deletion in the G2 allele, although a \"second-hit\" event is postulated to have a role in the development of HIVAN. HIVAN presents with proteinuria, particularly in the nephrotic range, as with other kidney diseases. The diagnosis requires biopsy and typically presents with collapsing subtype focal segmental glomerulosclerosis and microcyst formation in the tubulointerstitial region. Gaps still exist in the definitive treatment of HIVAN - concurrent use of antiretroviral therapy and adjunctive management with like renal-angiotensin-aldosterone system inhibitors, steroids, or renal replacement therapy showed benefits.</p><p><strong>Key message: </strong>This study reviews the current understanding of HIVAN including its epidemiology, mechanism of disease, related genetic factors, clinical profile, and pathophysiologic effects of management options for patients.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"1-11"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1d/ef/gdz-0003-0001.PMC9936764.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9314490","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-Term Safety, Clinical and Immunological Outcomes in Primary Membranous Nephropathy with Severe Renal Impairment Treated with Cyclophosphamide and Steroid-Based Regimen.","authors":"Omar Ragy,&nbsp;Patrick Hamilton,&nbsp;Anjali Pathi,&nbsp;Adil Abdalla Mohamed Ahmed,&nbsp;Sandip Mitra,&nbsp;Durga A K Kanigicherla","doi":"10.1159/000529605","DOIUrl":"https://doi.org/10.1159/000529605","url":null,"abstract":"<p><strong>Introduction and aims: </strong>Therapy of primary membranous nephropathy (PMN) with progressive advanced kidney dysfunction is challenging with limited literature and no clear therapeutic strategies. This is due to the scant evidence of effectiveness and uncertainty around the risk-benefit profile of immunosuppression (ImS) when eGFR is less than 30 mL/min. We aimed to determine long-term clinical outcomes in patients with PMN and severe renal impairment treated with combined cyclophosphamide and steroids.</p><p><strong>Methods: </strong>The study is a single-center retrospective longitudinal cohort study. All patients (between 2004 and 2019) with biopsy confirmed PMN who initiated combination therapy with steroids and cyclophosphamide and had an eGFR of ≤30 mL/min/1.73 m² at the time of initiation of therapy were included for analysis. Clinical and laboratory parameters including anti-PLA₂R-Ab were monitored as per standard clinical guidance. Primary outcome was achievement of partial remission. Secondary outcomes included immunological remission, need for renal replacement therapy, and adverse effects.</p><p><strong>Results: </strong>Eighteen patients with median age of 68 (IQR 58-73) years and 5:1 M:F ratio received the combination therapy when eGFR was ≤30 mL/min/1.73 m² (CKD-EPI). At time of ImS, median eGFR and uPCR were 23 (IQR 18-27) mL/min/1.73 m² and 8.4 (IQR 6.9-10.7) g/g, respectively. Median follow-up was for 67 (IQR 27-80) months. 16 patients (89%) achieved partial remission and 7 (39%) achieved complete remission. eGFR increased by 7 mL/min/1.73 m² (27%) after 1 year of starting ImS treatment and 12 mL/min/1.73 m² at end of follow-up. Two patients (11%) developed end-stage renal disease needing renal replacement therapy. 67% achieved both immunological and clinical remission. At the end of the follow-up period, 2 (11%) patients required hospitalization secondary to infections, 4 (22%) patients developed cancer and 4 patients died (22%).</p><p><strong>Conclusion: </strong>Combination therapy with cyclophosphamide and steroids is effective in achieving partial remission and improving renal function in PMN with advanced renal dysfunction. Prospective controlled studies are required to provide further evidence to rationalize treatment and improve outcomes in such patients.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"88-97"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/2a/e9/gdz-0003-0088.PMC10126738.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9363895","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Super-Resolution Microscopy: A Technique to Revolutionize Research and Diagnosis of Glomerulopathies.","authors":"Florian Siegerist,&nbsp;Vedran Drenic,&nbsp;Thor-Magnus Koppe,&nbsp;Nihal Telli,&nbsp;Nicole Endlich","doi":"10.1159/000528713","DOIUrl":"https://doi.org/10.1159/000528713","url":null,"abstract":"<p><strong>Background: </strong>For decades, knowledge about glomerular (patho)physiology has been tightly linked with advances in microscopic imaging technology. For example, the invention of electron microscopy was required to hypothesize about the mode of glomerular filtration barrier function.</p><p><strong>Summary: </strong>Super-resolution techniques, defined as fluorescence microscopy approaches that surpass the optical resolution limit of around 200 nm, have been made available to the scientific community. Several of these different techniques are currently in use in glomerular research. Using three-dimensional structured illumination microscopy, the exact morphology of the podocyte filtration slit can be morphometrically analyzed and quantitatively compared across samples originating from animal models or human biopsies.</p><p><strong>Key messages: </strong>Several quantitative image analysis approaches and their potential influence on glomerular research and diagnostics are discussed. By improving not only optical resolution but also information content and turnaround time, super-resolution microscopy has the potential to expand the diagnosis of glomerular disease. Soon, these approaches could be introduced into glomerular disease diagnosis.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"19-28"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9936760/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9314486","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Counselling for Kidney Biopsy and Immunosuppression in Glomerulonephritis Using Simulation.","authors":"Cynthia Ciwei Lim,&nbsp;Irene Mok,&nbsp;Zhihua Huang,&nbsp;Hui Zhuan Tan,&nbsp;Jason Choo","doi":"10.1159/000528816","DOIUrl":"https://doi.org/10.1159/000528816","url":null,"abstract":"not applicable for letter in response to recent publication in this journal","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"29-31"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6a/df/gdz-0003-0029.PMC9936759.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9328827","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autoimmunity in Infection-Related Glomerulonephritis.","authors":"Hae Yoon Grace Choung,&nbsp;Rickinder Grewal","doi":"10.1159/000528712","DOIUrl":"https://doi.org/10.1159/000528712","url":null,"abstract":"<p><strong>Introduction: </strong>Autoimmune (AI) reactivity in the setting of infection-related GN (IRGN) is often viewed as an epiphenomenon and is not well described.</p><p><strong>Methods: </strong>We report a cohort of 17 patients with IRGN during a 7-year period that highlights cases with AI reactivity and describes the clinical and pathologic characteristics of IRGN cases associated with AI reactivity.</p><p><strong>Results: </strong>Of the IRGN cases, 76% had clinical evidence of an autoimmune disease (AD) and/or positive AI serologies. Within the IRGN group with AI reactivity, 12 had positive AI serologies (92%) and 10 had AD (77%). 30% had a prior diagnosis of AD, while the remaining 70% did not have a history of AD and were either diagnosed or suspected of having an AD at the time of biopsy. The most common autoantibody detected was anti-nuclear antibody followed by anti-neutrophil cytoplasmic antibodies and autoantibodies associated with antiphospholipid syndrome.</p><p><strong>Conclusion: </strong>The study is not sufficiently powered to determine any significance but demonstrates the frequency with which AI features occur in IRGN and should prompt further future investigation. In summary, our findings suggest AI manifestations are common in IRGN.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"32-41"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/07/bf/gdz-0003-0032.PMC9936758.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9328828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Readability, Understandability, and Actionability of Online Cardiovascular Risk Assessment Tools and Patient Educational Material: A Systematic Review.","authors":"Ashraf Roshan,&nbsp;Jason Choo,&nbsp;Cynthia Lim","doi":"10.1159/000528118","DOIUrl":"https://doi.org/10.1159/000528118","url":null,"abstract":"<p><strong>Introduction: </strong>Individuals with kidney diseases have increased risk of cardiovascular disease and death. Online cardiovascular risk assessment tools can educate patients on risks and modifiable factors. Since patients have variable health literacy, we evaluated the readability, understandability, and actionability of publicly available online cardiovascular risk assessment tools.</p><p><strong>Methods: </strong>We systematically searched, reviewed, characterized, and assessed English-language cardiovascular risk assessment tools online for readability (Flesch-Kincaid Grade Level [FKGL] score), understandability, and actionability (Patient Education Materials Assessment Tool for printable materials [PEMAT-P]).</p><p><strong>Results: </strong>After screening 969 websites, 69 websites employing 76 risk tools were included. The most frequently used tools were the Framingham Risk Score (<i>n</i> = 13) and the Atherosclerotic Cardiovascular Disease score (<i>n</i> = 12). Most tools were intended for the general population and estimated the 10-year incident cardiovascular risk. Patient education was provided in the form of targets for blood pressure (<i>n</i> = 17), lipids (<i>n</i> = 15), or glucose (<i>n</i> = 5); and advice regarding diet (<i>n</i> = 18), exercise (<i>n</i> = 19), and smoking cessation (<i>n</i> = 20). The median FKGL, PEMAT understandability, and actionability scores were 6.2 (4.7, 8.5), 84.6% (76.9%, 89.2%), and 60% (40%, 60%), respectively.</p><p><strong>Conclusion: </strong>The online cardiovascular risk tools were generally easy to read and understand, but only a third provided education on risk modification. Judicious selection of an online cardiovascular risk assessment tool may help patients in self-management.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"56-68"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/30/71/gdz-0003-0056.PMC10126735.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9364657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Crescentic Fibrillary Glomerulonephritis in the Setting of Immune Checkpoint Inhibitor Therapy: A Report of Two Cases.","authors":"Lanny T DiFranza,&nbsp;Eleas Chafouleas,&nbsp;Swapna Katipally,&nbsp;M Barry Stokes,&nbsp;Satoru Kudose,&nbsp;Miroslav Sekulic","doi":"10.1159/000528881","DOIUrl":"https://doi.org/10.1159/000528881","url":null,"abstract":"<p><strong>Introduction: </strong>Immune checkpoint inhibitor (ICPI) therapy is used to treat various malignancies; however, it can be associated with off-target effects including kidney injury. Acute tubulointerstitial nephritis is the most commonly described renal pathology associated with ICPIs, although less frequently, glomerulopathies may be identified when a kidney biopsy is performed in the work-up of acute kidney injury (AKI).</p><p><strong>Case presentation: </strong>Two patients with small cell carcinoma of the lung were treated with etoposide, carboplatin, and the ICPI atezolizumab. During 2 and 1.5 months of atezolizumab therapy, respectively, patients developed AKI, hematuria, and proteinuria, and kidney biopsies were performed. Both biopsies showed fibrillary glomerulonephritis with focal crescentic features. One patient died 5 days after the kidney biopsy, while the second showed improvement of renal function after discontinuation of atezolizumab and initiation of corticosteroid therapy.</p><p><strong>Discussion: </strong>We describe two cases of fibrillary glomerulonephritis with crescents after administration of atezolizumab. Development of impaired kidney function following initiation of ICPI therapy in both cases raises the possibility that ICPI therapy may potentiate the development of endocapillary proliferation and crescents (i.e., an \"active\" glomerulitis) <i>via</i> immune modulation. Thus, exacerbation of underlying glomerulonephritis should be kept in the differential diagnosis of patients who develop AKI, proteinuria, and hematuria following ICPI therapy.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"3 1","pages":"69-74"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/eb/b8/gdz-0003-0069.PMC10126733.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9364658","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erratum.","authors":"","doi":"10.1159/000525534","DOIUrl":"https://doi.org/10.1159/000525534","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1159/000521233.].</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 3","pages":"151"},"PeriodicalIF":0.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d3/a7/gdz-0002-0151.PMC9710313.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10733089","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}