Glomerular diseases最新文献

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Glomerular Disease Education Experience across Nephrology Fellowship Programs: An International Survey. 肾脏病奖学金项目的肾小球疾病教育经验:一项国际调查。
Glomerular diseases Pub Date : 2022-04-01 DOI: 10.1159/000521598
Harish Seethapathy, Sayna Norouzi, Kate J Robson, Lida Gharibvand, Ali Poyan Mehr
{"title":"Glomerular Disease Education Experience across Nephrology Fellowship Programs: An International Survey.","authors":"Harish Seethapathy,&nbsp;Sayna Norouzi,&nbsp;Kate J Robson,&nbsp;Lida Gharibvand,&nbsp;Ali Poyan Mehr","doi":"10.1159/000521598","DOIUrl":"https://doi.org/10.1159/000521598","url":null,"abstract":"<p><strong>Introduction: </strong>Glomerulonephritis (GN) education is an important, albeit a challenging, component of nephrology fellowship training. We hypothesized that trainee experience varies widely across programs, leading to differences in self-reported competency levels in the diagnosis and management of glomerular diseases.</p><p><strong>Methods: </strong>The Glomerular Disease Study & Trial Consortium (GlomCon) conducted an anonymous online survey to determine the educational experience of nephrology trainees. We used multiple-choice questions to obtain data about (a) curriculum-based education, (b) dedicated specialty clinic, and (c) exposure to pathology. We leveraged a visual analog scale of 1-100 (with a higher number indicating a higher comfort level) to assess self-reported levels of clinical competency. The survey was disseminated via email to the subscribing members of GlomCon and through Twitter.</p><p><strong>Results: </strong>In total, there were 109 respondents to our survey, and 56% were from training programs in the USA. Exposure to a specialized GN clinic was reported by 45%, while 77% reported the presence of an onsite nephropathologist at their training program. Self-reported competency scores were 59 ± 25 and 52 ± 25 for diagnosis and treatment of glomerular diseases, respectively. Days spent in a GN clinic per year, years of fellowship, and dedicated nephropathology didactics were associated with higher diagnosis and treatment competency scores.</p><p><strong>Conclusion: </strong>Trainees report a wide variation in glomerular disease education across fellowship programs. A lack of nephropathology exposure and a dedicated GN curriculum was associated with lower scores in self-reported clinical competency in caring for patients with glomerular disease.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 2","pages":"89-94"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b1/53/gdz-0002-0089.PMC9670032.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10681231","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}
引用次数: 2
The 13th International Podocyte Conference 第十三届国际足细胞会议
Glomerular diseases Pub Date : 2022-04-01 DOI: 10.1159/000525410
S. Brix, D. Kanigicherla, D. Wallace, Tejas B. Desai, R. Lennon
{"title":"The 13th International Podocyte Conference","authors":"S. Brix, D. Kanigicherla, D. Wallace, Tejas B. Desai, R. Lennon","doi":"10.1159/000525410","DOIUrl":"https://doi.org/10.1159/000525410","url":null,"abstract":"na","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"16 1","pages":"1 - 78"},"PeriodicalIF":0.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88324293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Descriptive Evaluation of Health Literacy and Determinants of COVID-19 Vaccine Acceptance among Patients with IgA Nephropathy with High Vaccine Uptake. 对IgA肾病患者的健康素养和接受COVID-19疫苗的决定因素进行描述性评估,疫苗接种率高。
Glomerular diseases Pub Date : 2022-01-24 eCollection Date: 2022-07-01 DOI: 10.1159/000522158
Cynthia Ciwei Lim, Irene Y J Mok, Jun Jie Leeu, Zhong Hong Liew, Hui Zhuan Tan, Yok Mooi Chin, Wei Ling Teng, Fiona Yeo, Chieh Suai Tan, Jason C J Choo
{"title":"A Descriptive Evaluation of Health Literacy and Determinants of COVID-19 Vaccine Acceptance among Patients with IgA Nephropathy with High Vaccine Uptake.","authors":"Cynthia Ciwei Lim, Irene Y J Mok, Jun Jie Leeu, Zhong Hong Liew, Hui Zhuan Tan, Yok Mooi Chin, Wei Ling Teng, Fiona Yeo, Chieh Suai Tan, Jason C J Choo","doi":"10.1159/000522158","DOIUrl":"10.1159/000522158","url":null,"abstract":"<p><strong>Aims: </strong>Shared decision-making regarding COVID-19 vaccination in IgA nephropathy involves the ability to handle health information regarding potential benefits and risk of flare, but few studies have evaluated health literacy in the context of vaccination. We aimed to evaluate the health literacy and COVID-19 vaccination uptake and acceptance in IgA nephropathy.</p><p><strong>Methods: </strong>Single-center cross-sectional study of 126 consecutive patients with IgA nephropathy. Health literacy was assessed using the HLS-EU-47 questionnaire. Determinants of vaccine acceptance such as contextual influences, individual and group influences, and vaccine-specific issues were adapted from the World Health Organization framework.</p><p><strong>Results: </strong>Forty-eight patients (38.1%) with IgAN nephropathy completed the survey between June and August 2021. The participants' median age was 40.5 (31.6, 52.8) years with median disease duration of 2.8 (1.3, 4.3) years. The median general health literacy index was 31.74 (29.88, 35.82) with significantly greater difficulty in the competency of appraising health information and in the domain of disease prevention (<i>p</i> < 0.001). Forty-five patients (93.8%) received at least one dose of COVID-19 vaccine between January and August 2021. Among the 3 unvaccinated patients, 2 intended to receive the vaccination while and 1 did not intend to get vaccinated. There was a high level of trust and belief that their government and healthcare providers had their best interests at heart and that the healthcare providers were honest about the vaccine's risk and benefits, although 31.2% did not understand how the vaccine works and 22.9% believed that there were other ways to prevent infection. Most thought there was adequate safety information, were confident in the system for tracking adverse events and had no issues with access to the vaccine.</p><p><strong>Conclusion: </strong>Participants with IgA nephropathy had high health literacy scores and low vaccine hesitancy. The determinants for vaccine acceptance can potentially guide efforts to optimize vaccination coverage.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 3","pages":"132-138"},"PeriodicalIF":0.0,"publicationDate":"2022-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/48/47/gdz-0002-0132.PMC9059031.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10724710","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}
引用次数: 0
IgA Nephropathy: A Chinese Perspective. IgA肾病:中国视角。
Glomerular diseases Pub Date : 2022-01-01 DOI: 10.1159/000520039
Zhao Zhang, Yuemiao Zhang, Hong Zhang
{"title":"IgA Nephropathy: A Chinese Perspective.","authors":"Zhao Zhang,&nbsp;Yuemiao Zhang,&nbsp;Hong Zhang","doi":"10.1159/000520039","DOIUrl":"https://doi.org/10.1159/000520039","url":null,"abstract":"<p><strong>Background: </strong>IgA nephropathy (IgAN) is the most common primary glomerular disease worldwide and remains a leading cause of chronic kidney disease and end-stage renal disease. The disease prevalence, clinical and pathological phenotypes, the underlying pathogenic molecular mechanisms, and the response to treatments are highly heterogeneous in different ethnic populations, which raise the concern that IgAN may differ across different parts of the world.</p><p><strong>Summary: </strong>From a Chinese perspective, we stated the disease burden of IgAN, summarized genome-wide association studies and research into pathological molecules, and compared them with findings based on other populations. The emerging biomarkers, indigenous clinical trials, and major challenges for Chinese researchers and nephrologists in studying IgAN are also discussed.</p><p><strong>Key messages: </strong>In this review, we described a higher risk of major susceptible loci in mucosal immunity, IgA production, and complement activation pathways in Chinese patients with IgAN. With our understanding of the pathogenesis of IgAN, novel biomarkers are emerging. Although there are challenges for conducting high-quality clinical trials in China, it is still feasible to conduct innovative and well-designed studies of IgAN. In the future, international collaborations on research infrastructure would be helpful to advance clinical and basic research in China.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 1","pages":"30-41"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/09/9a/gdz-0002-0030.PMC9677733.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10676271","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}
引用次数: 4
Clinical Predictors and Prognosis of Recurrent IgA Nephropathy in the Kidney Allograft. 同种异体肾移植术后IgA肾病复发的临床预测因素及预后。
Glomerular diseases Pub Date : 2022-01-01 DOI: 10.1159/000519834
Catherine R Kavanagh, Francesca Zanoni, Rita Leal, Namrata G Jain, Megan Nicole Stack, Elena-Rodica Vasilescu, Geo Serban, Carley Shaut, Jeanne Kamal, Satoru Kudose, António Martinho, Rui Alves, Dominick Santoriello, Pietro A Canetta, David Cohen, Jai Radhakrishnan, Gerald B Appel, Michael B Stokes, Glen S Markowitz, Vivette D D'Agati, Krzysztof Kiryluk, Nicole K Andeen, Ibrahim Batal
{"title":"Clinical Predictors and Prognosis of Recurrent IgA Nephropathy in the Kidney Allograft.","authors":"Catherine R Kavanagh,&nbsp;Francesca Zanoni,&nbsp;Rita Leal,&nbsp;Namrata G Jain,&nbsp;Megan Nicole Stack,&nbsp;Elena-Rodica Vasilescu,&nbsp;Geo Serban,&nbsp;Carley Shaut,&nbsp;Jeanne Kamal,&nbsp;Satoru Kudose,&nbsp;António Martinho,&nbsp;Rui Alves,&nbsp;Dominick Santoriello,&nbsp;Pietro A Canetta,&nbsp;David Cohen,&nbsp;Jai Radhakrishnan,&nbsp;Gerald B Appel,&nbsp;Michael B Stokes,&nbsp;Glen S Markowitz,&nbsp;Vivette D D'Agati,&nbsp;Krzysztof Kiryluk,&nbsp;Nicole K Andeen,&nbsp;Ibrahim Batal","doi":"10.1159/000519834","DOIUrl":"https://doi.org/10.1159/000519834","url":null,"abstract":"<p><strong>Introduction: </strong>Although IgA nephropathy (IgAN) is the most common recurrent glomerulonephritis encountered in the kidney allograft, the clinical and immunogenetic characteristics remain poorly understood. We sought to study determinants and prognosis of recurrent IgAN with special focus on HLA antigens.</p><p><strong>Materials and methods: </strong>Between 2005 and 2019, we identified 282 transplanted patients with failure secondary to IgAN from two North American and one European Medical Centers, including 80 with recurrent IgAN and 202 without recurrence. Prevalence of HLA antigens was compared to external healthy controls of European ancestry (n=15,740). Graft survival was assessed by Kaplan-Meier method and log rank test. Cox proportional hazards were used for multivariable analyses.</p><p><strong>Results: </strong>Compared to external controls of European ancestry, kidney transplant recipients of European ancestry with kidney failure secondary to IgAN had higher frequency of HLA-DQ5 (42% vs. 30%, OR=1.68, P=0.002) and lower frequency of HLA-DR15 (15% vs. 28%, OR=0.46, P<0.001) and HLA-DQ6 (32% vs. 45%, OR=0.59, P=0.003); however, the frequency of these HLA antigens were similar in recurrent versus non-recurring IgAN. Younger recipient age at transplantation was an independent predictor of recurrence. HLA-matching was an independent predictor for recurrent IgAN only in recipients of living-related but not deceased or living unrelated transplants. Recurrent IgAN was an independent predictor of allograft failure, along with acute rejection. In patients with recurrent IgAN, serum creatinine at biopsy, degree of proteinuria, and concurrent acute rejection were associated with inferior allograft survival.</p><p><strong>Discussion/ conclusion: </strong>Recurrent IgAN negatively affects allograft survival. Younger recipient age at transplantation is an independent predictor of recurrent IgAN, while the presence of HLA antigens associated with IgAN in the native kidney and HLA-matching in recipients of deceased or living unrelated transplants are not.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 1","pages":"42-53"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/18/14/gdz-0002-0042.PMC9017582.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10807212","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}
引用次数: 8
Fibrillary Glomerulonephritis, DNAJB9, and the Unfolded Protein Response. 原纤维性肾小球肾炎,DNAJB9和未折叠蛋白反应。
Glomerular diseases Pub Date : 2022-01-01 DOI: 10.1159/000525542
Nicole K Andeen, Vanderlene L Kung, Josh Robertson, Susan B Gurley, Rupali S Avasare, Sneha Sitaraman
{"title":"Fibrillary Glomerulonephritis, DNAJB9, and the Unfolded Protein Response.","authors":"Nicole K Andeen,&nbsp;Vanderlene L Kung,&nbsp;Josh Robertson,&nbsp;Susan B Gurley,&nbsp;Rupali S Avasare,&nbsp;Sneha Sitaraman","doi":"10.1159/000525542","DOIUrl":"https://doi.org/10.1159/000525542","url":null,"abstract":"Background: Fibrillary glomerulonephritis (FGN) is found in approximately 1% of native kidney biopsies and was traditionally defined by glomerular deposition of fibrils larger than amyloid (12–24 nm diameter) composed of polyclonal IgG. Recent identification of DNAJB9 as a sensitive and specific marker of FGN has revolutionized FGN diagnosis and opened new avenues to studying FGN pathogenesis. In this review, we synthesize recent literature to provide an updated appraisal of the clinical and pathologic features of FGN, discuss diagnostic challenges and pitfalls, and propose molecular models of disease in light of DNAJB9. Summary: DNAJB9 tissue assays, paraffin immunofluorescence studies, and IgG subclass testing demonstrate that FGN is distinct from other glomerular diseases with organized deposits and highlight FGN morphologic variants. Additionally, these newer techniques show that FGN is only rarely monoclonal, and patients with monoclonal FGN usually do not have a monoclonal gammopathy. DNAJB9 mutation does not appear to affect the genetic architecture of FGN; however, the accumulation of DNAJB9 in FGN deposits suggests that disease is driven, at least in part, by proteins involved in the unfolded protein response. Treatments for FGN remain empiric, with some encouraging data suggesting that rituximab-based therapy is effective and that transplantation is a good option for patients progressing to ESKD. Key Messages: DNAJB9 aids in distinguishing FGN from other glomerular diseases with organized deposits. Further investigations into the role of DNAJB9 in FGN pathogenesis are necessary to better understand disease initiation and progression and to ultimately develop targeted therapies.","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 4","pages":"164-175"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a5/f4/gdz-0002-0164.PMC9936766.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10772190","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}
引用次数: 1
Gluten-Free Diet in Childhood Difficult-to-Treat Nephrotic Syndrome: A Pilot Feasibility Study. 无麸质饮食治疗儿童难治性肾病综合征:一项试点可行性研究。
Glomerular diseases Pub Date : 2022-01-01 DOI: 10.1159/000525587
Tarak Srivastava, Katherine M Dell, Kevin V Lemley, Debbie S Gipson, Frederick J Kaskel, Kevin Edward Meyers, Christian Faul, Ayelet Goldhaber, LauraJane Pehrson, Howard Trachtman
{"title":"Gluten-Free Diet in Childhood Difficult-to-Treat Nephrotic Syndrome: A Pilot Feasibility Study.","authors":"Tarak Srivastava,&nbsp;Katherine M Dell,&nbsp;Kevin V Lemley,&nbsp;Debbie S Gipson,&nbsp;Frederick J Kaskel,&nbsp;Kevin Edward Meyers,&nbsp;Christian Faul,&nbsp;Ayelet Goldhaber,&nbsp;LauraJane Pehrson,&nbsp;Howard Trachtman","doi":"10.1159/000525587","DOIUrl":"https://doi.org/10.1159/000525587","url":null,"abstract":"<p><strong>Introduction: </strong>Minimal change disease in childhood can follow a frequently relapsing or steroid-dependent course in up to 40% of cases. Second-line immunosuppressive medications that are used to manage these patients are associated with significant adverse effects. There is a need for safer alternative treatments for difficult-to-treat nephrotic syndrome. Therefore, we conducted an open-label feasibility study to assess the safety and efficacy of a gluten-free diet as treatment for pediatric patients with difficult-to-treat nephrotic syndrome. As a second aim, we sought to determine if the plasma zonulin concentration can identify those who are more likely to respond to this intervention.</p><p><strong>Methods: </strong>Seventeen patients were placed on a gluten-free diet for 6 months. A positive response was defined as a 50% reduction in the relapse rate compared to the preceding 6 months or the ability to discontinue 1 immunosuppressive drug.</p><p><strong>Results: </strong>Five (29%) participants had a positive response to the dietary intervention. The gluten-free diet was well tolerated with no clinical or laboratory adverse events. Plasma zonulin concentration was elevated in patients who failed to benefit from the gluten-free diet.</p><p><strong>Discussion/conclusion: </strong>A gluten-free diet may be a useful adjunctive intervention for patients with difficult-to-treat nephrotic syndrome that can be implemented prior to resorting to second-line immunosuppressive therapy. Development of the plasma zonulin level as a biomarker to predict efficacy would facilitate rational use of a gluten-free diet in the management of nephrotic syndrome.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 4","pages":"176-183"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a5/dd/gdz-0002-0176.PMC9936750.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9073959","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}
引用次数: 1
IgA Nephropathy: "The Times They Are a-Changin". IgA肾病:“时代正在改变”。
Glomerular diseases Pub Date : 2022-01-01 DOI: 10.1159/000515199
Richard J Glassock
{"title":"IgA Nephropathy: \"The Times They Are a-Changin\".","authors":"Richard J Glassock","doi":"10.1159/000515199","DOIUrl":"https://doi.org/10.1159/000515199","url":null,"abstract":"<p><strong>Background: </strong>Primary IgA Nephropathy (IgA N) is a very common and often progressive glomerular disease. At present, the diagnosis of IgA N is totally dependent on kidney biopsy, but the prospect for a future diagnosis by means of a \"liquid\" biopsy is promising. A great deal is now understood regarding its diverse clinical and pathological features as well as its epidemiology, genetics, prognosis, and pathogenesis. Treatment approaches are now on increasingly solid evidence-based grounds, but many uncertainties continue to be devil the field. Better means of categorization of patients into a hierarchy of progression risk at the time of diagnosis will undoubtedly refine and personalize treatment decisions.</p><p><strong>Summary: </strong>The panorama of treatment strategies is undergoing a rapid transformation, largely due to an increase in large randomized clinical trials testing available agents and novel therapeutic classes. It is anticipated that the combination of better prognostic tools and new strategies for treatment of IgA N will alter the landscape of therapeutic algorithms for patients with IgA N.</p><p><strong>Key messages: </strong>This review seeks to describe some of the evolutionary changes in the approach to treatment of IgA N, to place them in the context of current management, and to identify knowledge gaps that need to be addressed.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 1","pages":"4-14"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000515199","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9228657","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}
引用次数: 1
IgA Nephropathy and Atypical Anti-GBM Disease: A Rare Dual Pathology in a Pediatric Rapidly Progressive Glomerulonephritis. IgA肾病和非典型抗gbm疾病:儿童快速进展性肾小球肾炎的罕见双重病理。
Glomerular diseases Pub Date : 2022-01-01 DOI: 10.1159/000521582
Varun Bajaj, Shilpi Thakur, Adarsh Barwad, Aditi Sinha, Arvind Bagga, Geetika Singh
{"title":"IgA Nephropathy and Atypical Anti-GBM Disease: A Rare Dual Pathology in a Pediatric Rapidly Progressive Glomerulonephritis.","authors":"Varun Bajaj,&nbsp;Shilpi Thakur,&nbsp;Adarsh Barwad,&nbsp;Aditi Sinha,&nbsp;Arvind Bagga,&nbsp;Geetika Singh","doi":"10.1159/000521582","DOIUrl":"https://doi.org/10.1159/000521582","url":null,"abstract":"<p><strong>Introduction: </strong>Anti-GBM nephritis in the pediatric age group is exceedingly rare with concurrent additional pathologies being even rarer. Tissue diagnosis requires a combination of crescentic histomorphology, immunofluorescence showing \"paint brush stroke\" pattern of linear IgG or rarely IgA, and serum anti-GBM antibodies subject to the disease course and treatment. The authors describe one such case with a dual pathology involving IgA nephropathy and atypical anti-GBM disease.</p><p><strong>Case presentation: </strong>A 13-year-old girl presenting with features of rapidly progressive glomerulonephritis underwent a renal biopsy showing a mesangioproliferative histology with crescents and an immunofluorescence pattern indicating a dual pathology of IgA nephropathy and anti-GBM nephritis. Additional ancillary testing including staining for IgG subclasses and galactose-deficient IgA (KM55) helped to confirm the diagnosis. She responded to steroid pulses and plasma exchange therapy, was off dialysis after 8 weeks with a serum creatinine level of 1.5 mg/dL, and however remains proteinuric at last follow-up.</p><p><strong>Conclusion: </strong>Concurrent anti-GBM nephritis and IgA nephropathy is a rare occurrence and possibly arises from a complex interaction between the anti-GBM antibodies and the basement membrane unmasking the antigens for IgA antibodies. Additional newer techniques like immunofluorescence for KM55 are helpful in establishing the dual pathology.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 1","pages":"54-57"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ae/06/gdz-0002-0054.PMC9677737.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9243515","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}
引用次数: 2
New Insights into the Pathogenesis and Treatment Strategies in IgA Nephropathy. IgA肾病发病机制及治疗策略的新认识
Glomerular diseases Pub Date : 2022-01-01 DOI: 10.1159/000519973
Katrin Scionti, Karen Molyneux, Haresh Selvaskandan, Jonathan Barratt, Chee Kay Cheung
{"title":"New Insights into the Pathogenesis and Treatment Strategies in IgA Nephropathy.","authors":"Katrin Scionti,&nbsp;Karen Molyneux,&nbsp;Haresh Selvaskandan,&nbsp;Jonathan Barratt,&nbsp;Chee Kay Cheung","doi":"10.1159/000519973","DOIUrl":"https://doi.org/10.1159/000519973","url":null,"abstract":"<p><strong>Background: </strong>Immunoglobulin A nephropathy (IgAN) is the most common form of primary glomerulonephritis worldwide. It is defined by mesangial IgA deposition, with consequent mesangial cell proliferation, inflammation, and tubulointerstitial fibrosis.</p><p><strong>Summary: </strong>Approximately 30% of affected patients will progress to end-stage kidney disease within 20 years of diagnosis. Currently, there is no disease-specific treatment available and management recommendations are, in general, limited to optimization of lifestyle measures and use of renin-angiotensin-aldosterone system blockers. More recently, advances in the understanding of the pathogenesis of IgAN have informed the development of novel therapeutic strategies that are now being tested in clinical trials. These have focused on different areas that include modulating the production of poorly galactosylated IgA1, which is central to the development of IgAN, and inhibiting the downstream signaling pathways and complement activation that are triggered following mesangial IgA1 deposition. In this review, we will summarize important pathogenic mechanisms in IgAN and highlight important areas of interest where treatment strategies are being developed.</p><p><strong>Key messages: </strong>IgAN is a common form of primary glomerulonephritis for which there is no current approved specific therapy. Recent advances in the understanding of its pathogenesis have led to the development of novel therapies, with the hope that new treatment options will be available soon to treat this condition.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 1","pages":"15-29"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ad/38/gdz-0002-0015.PMC9677740.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9228658","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}
引用次数: 5
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