Nephrology最新文献_第2页

A Mendelian Randomization Study on the Association Between Sex Hormone-Binding Globulin and Membranous Nephropathy Risk. 性激素结合球蛋白与膜性肾病风险相关性的孟德尔随机研究。

IF 2.4 4区医学

Nephrology Pub Date : 2025-05-01 DOI: 10.1111/nep.70049

Yingxin Fang, Qiuhua Gu, Junya Jia, Tiekun Yan

{"title":"A Mendelian Randomization Study on the Association Between Sex Hormone-Binding Globulin and Membranous Nephropathy Risk.","authors":"Yingxin Fang, Qiuhua Gu, Junya Jia, Tiekun Yan","doi":"10.1111/nep.70049","DOIUrl":"https://doi.org/10.1111/nep.70049","url":null,"abstract":"Aim: Membranous nephropathy (MN) is the leading cause of nephrotic syndrome in adults, with 80% of cases being primary MN of unknown origin. While the influence of sex hormones on chronic kidney disease (CKD) is thoroughly established, the role of sex hormone-binding globulin (SHBG) in MN is still uncertain.Methods: We performed a Mendelian randomization (MR) analysis to assess the causal impact of SHBG on MN, utilising data from genome-wide association studies (GWAS). The main analysis utilised the inverse variance weighted (IVW) method, while various additional and sensitivity analyses were conducted to evaluate the causal estimates.Results: We obtained 51 valid instrumental variables (IVs) of SHBG from large-scale open-access GWASs. Genetic forecasting of SHBG notably raised the likelihood of MN in males (IVW odds ratios [OR] = 2.992, 95% confidence interval [CI] = [1.643, 5.446], p = 3.370 × 10-4). Three additional MR analyses consistently demonstrated a positive causal relationship between SHBG and MN, with all p values being less than 0.05. MR-Egger intercept analysis showed no evidence of horizontal pleiotropy (p > 0.05).Conclusion: Elevated serum SHBG concentrations were directly associated with an increased risk of primary MN in males. Further research is needed to explore the effectiveness of SHBG in assessing and predicting MN risk.","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 5","pages":"e70049"},"PeriodicalIF":2.4,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094450","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Projection of the Prevalence and Economic Burden of Chronic Kidney Disease in Taiwan From 2022 to 2027 (Inside CKD): A Microsimulation Study. 台湾2022至2027年慢性肾脏疾病患病率及经济负担预测（Inside CKD）：微观模拟研究

IF 2.4 4区医学

Nephrology Pub Date : 2025-05-01 DOI: 10.1111/nep.70055

I-Wen Wu, Mei-Yi Wu, Salvatore Barone, Claudia Cabrera, Juan Jose Garcia Sanchez, Lise Retat, Markiyan Mitchyn, Mai-Szu Wu

{"title":"Projection of the Prevalence and Economic Burden of Chronic Kidney Disease in Taiwan From 2022 to 2027 (Inside CKD): A Microsimulation Study.","authors":"I-Wen Wu, Mei-Yi Wu, Salvatore Barone, Claudia Cabrera, Juan Jose Garcia Sanchez, Lise Retat, Markiyan Mitchyn, Mai-Szu Wu","doi":"10.1111/nep.70055","DOIUrl":"https://doi.org/10.1111/nep.70055","url":null,"abstract":"Aim: This study aimed to project the epidemiology of chronic kidney disease (CKD) and its economic impact in Taiwan from 2022 to 2027 using a microsimulation model.Methods: A virtual representative population of Taiwan was generated using demographic data. The cohort then underwent annual progression using a validated patient-level microsimulation technique, projecting CKD onset and progression based on various factors, including age, sex, eGFR, and urine albumin level. The model also incorporated associated comorbidities like type 2 diabetes mellitus and hypertension and complications such as heart failure, myocardial infarction, and stroke. Healthcare costs associated with diagnosed CKD and kidney replacement therapy (KRT) were also projected.Results: In 2022, an estimated 10.6% of the total population (2.5 million individuals) in Taiwan were affected by documented or undiagnosed CKD. Without changes in care standards, this prevalence is projected to rise to 12.4% (3.0 million individuals) by 2027, a relative increase of 17.1%. The prevalence of KRT is expected to grow by 7.0% from 2022 to 2027. Complications related to CKD, including heart failure, myocardial infarction, and stroke, are also projected to increase. The annual healthcare costs for diagnosed CKD and KRT are anticipated to rise by 19.7% from TWD $51.96 billion in 2022 to TWD $62.18 billion in 2027.Conclusion: The projections underscore the escalating burden of CKD in Taiwan, emphasising the need for proactive strategies focusing on early diagnosis, effective management, and public awareness to mitigate the disease's socioeconomic impact.","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 5","pages":"e70055"},"PeriodicalIF":2.4,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144026106","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

A Case of IgA Nephropathy With Membranoproliferative Glomerulonephritis-Like Features. IgA肾病伴膜增生性肾小球肾炎样特征1例。

IF 2.4 4区医学

Nephrology Pub Date : 2025-05-01 DOI: 10.1111/nep.70057

Miyu Kanazawa, Kenji Tsuji, Ryoya Aoki, Mihiro Sue, Hiromasa Miyake, Naruhiko Uchida, Hiroyuki Nakanoh, Kazuhiko Fukushima, Haruhito A Uchida, Jun Wada

{"title":"A Case of IgA Nephropathy With Membranoproliferative Glomerulonephritis-Like Features.","authors":"Miyu Kanazawa, Kenji Tsuji, Ryoya Aoki, Mihiro Sue, Hiromasa Miyake, Naruhiko Uchida, Hiroyuki Nakanoh, Kazuhiko Fukushima, Haruhito A Uchida, Jun Wada","doi":"10.1111/nep.70057","DOIUrl":"https://doi.org/10.1111/nep.70057","url":null,"abstract":"A 73-year-old man was referred due to the onset of nephrotic-range proteinuria. He had been diagnosed with rheumatoid arthritis 18 years prior and had achieved remission with treatment, including methotrexate and janus kinase (JAK) inhibitor. Although routine follow-ups had not revealed any urinary abnormalities, subsequent tests detected proteinuria and hematuria in the absence of infection or other symptoms. As the urinary abnormalities persisted, with a serum albumin decrease and proteinuria measuring 5.7 g/day, indicating nephrotic syndrome, the patient was referred to our hospital for further evaluation, and a renal biopsy was performed. Light microscopy revealed mesangial cell proliferation, endocapillary proliferation and double-contoured basement membranes. Immunofluorescence microscopy showed IgA-dominant deposits in both mesangial areas and glomerular capillary walls. Transmission electron microscopy demonstrated electron-dense deposits in the mesangium and subendothelial regions, leading to the diagnosis of membranoproliferative glomerulonephritis (MPGN)-type IgA nephropathy. Immunostaining with the Gd-IgA1 (galactose-deficient IgA1)-specific antibody (KM55) was positive, consistent with the diagnosis. Following the initiation of steroid therapy, proteinuria rapidly decreased, achieving complete remission within 5 months. IgA nephropathy with MPGN-like features often presents as nephrotic syndrome, differing from the typical pathological and clinical presentation of IgA nephropathy, making differentiation from secondary MPGN and other diseases sometimes challenging. This case suggests that KM55 staining may offer additional information in differentiating atypical IgA nephropathy with non-classical pathological features.","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 5","pages":"e70057"},"PeriodicalIF":2.4,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144079277","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Recognizing a Rare Presentation: Hypokalemic Periodic Paralysis Secondary to Amphetamine Use. 认识到一种罕见的表现：使用安非他明后继发的低钾性周期性麻痹。

IF 2.4 4区医学

Nephrology Pub Date : 2025-05-01 DOI: 10.1111/nep.70056

Hamza Naveed, Crystal Ike, Laith M Haj-Ahmad, Srinidhi Shyamkumar, Damon Cao

{"title":"Recognizing a Rare Presentation: Hypokalemic Periodic Paralysis Secondary to Amphetamine Use.","authors":"Hamza Naveed, Crystal Ike, Laith M Haj-Ahmad, Srinidhi Shyamkumar, Damon Cao","doi":"10.1111/nep.70056","DOIUrl":"https://doi.org/10.1111/nep.70056","url":null,"abstract":"Hypokalemic periodic paralysis is a hereditary or acquired temporary flaccid paralysis of skeletal muscles, affecting proximal musculature more than distal, as a result of hypokalemia. In this report, we describe a unique case of amphetamine-induced hypokalemic periodic paralysis that has only been described twice in the current literature. A 31-year-old male with a history of substance abuse presented with altered mental status and acute tetraparesis after inhalation of methamphetamine. Labs were significant for marked hypokalemia (1.8 mmol/L), elevated creatine kinase and a positive urine drug screen for methamphetamines. Aggressive potassium replacement was initiated, yet the patient remained hypokalemic, with worsening neurological status and aphasia. Intubation and mechanical ventilation became a necessity after the patient developed acute hypercapnic respiratory failure (pCO2 103 mmHg, pH 7.02). Our patient then received potassium replacement via multiple routes, including intravenous and orogastric administration. After 15 h, his potassium normalised, and he was successfully extubated the next day, regaining full motor strength. This case highlights the potential for amphetamine-induced hypokalemic periodic paralysis to cause life-threatening diaphragmatic paralysis and respiratory failure. How methamphetamine exactly induces hypokalemic periodic paralysis remains unclear, yet its indirect sympathomimetic effects with subsequent intracellular potassium shifts possibly explain the resultant hypokalemia. Given that methamphetamine use is on the rise, physicians should have a high index of suspicion for amphetamine-induced hypokalemic periodic paralysis in patients presenting with acute paralysis and a substance use history. Rapid recognition, potassium repletion and respiratory monitoring are essential for patient recovery.","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 5","pages":"e70056"},"PeriodicalIF":2.4,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144036879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

New Onset of Primary Membranous Nephropathy After COVID-19 mRNA Vaccination in Affected Sjögren's Syndrome. 受感染Sjögren综合征患者接种COVID-19 mRNA后新发原发性膜性肾病

IF 2.4 4区医学

Nephrology Pub Date : 2025-05-01 DOI: 10.1111/nep.70048

Chia-Wei Tseng, Jing-Huan Liao, Tai-Kuang Chao, Shun-Neng Hsu

{"title":"New Onset of Primary Membranous Nephropathy After COVID-19 mRNA Vaccination in Affected Sjögren's Syndrome.","authors":"Chia-Wei Tseng, Jing-Huan Liao, Tai-Kuang Chao, Shun-Neng Hsu","doi":"10.1111/nep.70048","DOIUrl":"https://doi.org/10.1111/nep.70048","url":null,"abstract":"The global administration of mRNA vaccines in response to the coronavirus disease 2019 (COVID-19) pandemic has been crucial in mitigating the spread of the virus. While these vaccines are generally safe and effective, there have been occasional reports of rare adverse effects, including new-onset nephropathies. Primary Sjögren's syndrome (pSS), an autoimmune disorder primarily affecting the exocrine glands, can also present with renal involvement, most commonly as tubulointerstitial nephritis (TIN). A 52-year-old female with a history of pSS developed shortness of breath, generalised edema, and oliguria 1 month after receiving her fourth dose of the COVID-19 mRNA vaccine. Initial evaluation revealed bilateral pleural effusion on chest X-ray. Laboratory evaluations revealed rapidly progressive glomerulonephritis (RPGN) and nephrotic syndrome. Renal biopsy findings showed mesangial expansion, focal crescent formation, pronounced tubulointerstitial nephritis, and positive staining for anti-phospholipase A2 receptor (PLA2R). The temporal association, coupled with renal biopsy findings, strongly suggested a vaccine-related trigger, and the diagnosis of new-onset primary membranous nephropathy (MN) following COVID-19 mRNA vaccination was made. The patient was treated with haemodialysis, plasma exchange, corticosteroid pulse therapy, and immunosuppressive agents, resulting in complete remission of proteinuria within 3 months. This case underscores the potential for COVID-19 mRNA vaccines to precipitate primary MN in patients with pre-existing familial autoimmune conditions such as pSS. It also emphasises the importance of recognising vaccine-related renal complications in autoimmune patients and the necessity for close monitoring and prompt intervention to prevent serious complications.","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 5","pages":"e70048"},"PeriodicalIF":2.4,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12060212/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144021597","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

A Case of Myeloproliferative Neoplasm-Related Glomerulopathy Secondary to Primary Myelofibrosis With Acute Leukaemic Transformation. 骨髓增生性肿瘤相关肾小球病变继发于原发性骨髓纤维化伴急性白血病转化1例。

IF 2.4 4区医学

Nephrology Pub Date : 2025-04-01 DOI: 10.1111/nep.70033

Peter-Joon Lee, Jacinta Perram, Min Li Huang, Jacob Sevastos, Namrata Khanal

{"title":"A Case of Myeloproliferative Neoplasm-Related Glomerulopathy Secondary to Primary Myelofibrosis With Acute Leukaemic Transformation.","authors":"Peter-Joon Lee, Jacinta Perram, Min Li Huang, Jacob Sevastos, Namrata Khanal","doi":"10.1111/nep.70033","DOIUrl":"https://doi.org/10.1111/nep.70033","url":null,"abstract":"Glomerular diseases associated with myeloproliferative neoplasms (MPN) are rare, and most often present with proteinuria and kidney impairment. Its natural history is not well described, although it has been associated with poor prognosis in described cases. Here, we present a case of MPN-related focal segmental glomerulosclerosis (FSGS) secondary to primary myelofibrosis (PMF) and describe its progression with transformation of PMF to leukaemia. A 52-year-old gentleman was referred for lower limb swelling on a background of primary myelofibrosis requiring splenectomy 3 months prior. Kidney function was normal, but there was nephrotic-range proteinuria of 3.6 g (normal range, NR < 0.15 g) and mild hypoalbuminaemia of 29 g/L (NR 33-48 g/L). Urine microscopy was bland with no haematuria or pyuria. A kidney biopsy confirmed secondary FSGS with dysmorphic megakaryocytes in the glomerular capillaries, as well as immunohistochemistry demonstrating the presence of megakaryocytes and erythroid precursors in the interstitium, indicating the presence of extramedullary haematopoiesis. No deposits were seen on immunofluorescence or electron microscopy. Despite an initial response to high-dose corticosteroids, a relapse in proteinuria to 10.9 g was seen 5 months after diagnosis. This coincided with leukaemic transformation, which was confirmed on bone marrow biopsy. We describe a case of FSGS secondary to PMF presenting with normal kidney function and nephrotic syndrome. As far as the authors are aware, this is the first case to detail the progression of kidney disease before and after leukaemic transformation. Ongoing follow-up may provide useful insights into the natural history of this infrequent association.","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70033"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144028921","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Posterior Reversible Encephalopathy Syndrome Triggered by a Rapid Rise of Cyclosporine Levels in a Renal Transplant Recipient. 肾移植受者环孢素水平快速升高引发的后部可逆性脑病综合征

IF 2.4 4区医学

Nephrology Pub Date : 2025-04-01 DOI: 10.1111/nep.70036

Evangelia Charitaki, Triantaphyllia Bounta, Charalampos Dimitrakopoulos, Pelagia Kriki, Euthymia Mourvati, Marios Theodoridis, Konstantia Kantartzi, Stylianos Panagoutsos

{"title":"Posterior Reversible Encephalopathy Syndrome Triggered by a Rapid Rise of Cyclosporine Levels in a Renal Transplant Recipient.","authors":"Evangelia Charitaki, Triantaphyllia Bounta, Charalampos Dimitrakopoulos, Pelagia Kriki, Euthymia Mourvati, Marios Theodoridis, Konstantia Kantartzi, Stylianos Panagoutsos","doi":"10.1111/nep.70036","DOIUrl":"https://doi.org/10.1111/nep.70036","url":null,"abstract":"Posterior reversible encephalopathy syndrome (PRES) is an acute neurologic syndrome characterised by headache, confusion, visual changes, seizures, and neuroimaging findings of posterior cerebral white matter oedema. Cytotoxic and immunosuppressive drugs, such as cyclosporine, are known factors associated with PRES and, along with hypertensive encephalopathy and eclampsia, are the most common causes of the syndrome. So far, studies in cyclosporine-associated PRES have not shown an association between serum cyclosporine levels and the onset of neurologic symptoms, whereas many cases seem to be related to hypertension caused by cyclosporine. We report here, a renal-transplant recipient receiving cyclosporine, who developed acute blindness and seizures 3 days after the initiation of voriconazole for pulmonary aspergillosis. The onset of PRES coincided with an acute rise in cyclosporine levels. This case illustrates that the rapid elevation of cyclosporine levels-even in the therapeutic range-may trigger the onset of PRES and clinicians should be extremely careful when introducing drugs that may interfere with cytochrome P450, especially strong P450 inhibitors such as voriconazole, which reduce cyclosporine metabolism.","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70036"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144012526","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The Kidney and Extracorporeal Therapies in Acute-on-Chronic Liver Failure: What the Nephrologist Needs to Know. 急性和慢性肝衰竭的肾脏和体外治疗：肾病专家需要知道的。

IF 2.4 4区医学

Nephrology Pub Date : 2025-04-01 DOI: 10.1111/nep.70034

Ashika Bangera, Pooja Mohan Basthi, Balaji Musunuri, Shankar Prasad Nagaraju, Shiran Shetty, Indu Ramachandra Rao

{"title":"The Kidney and Extracorporeal Therapies in Acute-on-Chronic Liver Failure: What the Nephrologist Needs to Know.","authors":"Ashika Bangera, Pooja Mohan Basthi, Balaji Musunuri, Shankar Prasad Nagaraju, Shiran Shetty, Indu Ramachandra Rao","doi":"10.1111/nep.70034","DOIUrl":"https://doi.org/10.1111/nep.70034","url":null,"abstract":"In this review, we discuss the pathophysiology and management of acute kidney injury (AKI) in the setting of acute-on-chronic liver failure (ACLF). ACLF is characterised by the occurrence of acute hepatic and/or extrahepatic organ failure, induced by immune dysregulation and systemic inflammation in patients with chronic liver disease. Kidney involvement is common, with AKI occurring in 30% to > 95% of ACLF patients, depending on the definition used. Since there is a lack of kidney biopsy data in these patients, the underlying pathophysiological basis of AKI remains incompletely understood, and systemic inflammation is believed to be the primary driver of organ injury. The management of AKI has been largely extrapolated from studies in decompensated cirrhosis, and there is little data specifically in the ACLF setting. However, available evidence suggests that structural kidney injury is more common in ACLF than in decompensated CLD, and therefore, AKI in ACLF is less likely to respond to volume repletion and vasopressors. Treatment options remain limited for those who are non-responsive to intravenous fluids and vasopressors. Liver transplantation (LT), with or without kidney transplantation, is the definitive treatment for these patients. At present, extracorporeal therapies such as therapeutic plasma exchange and kidney replacement therapies play a supportive role in ACLF as a bridge to LT; however, the optimal timing and dosing remain unclear. While theoretically, extracorporeal therapies have the potential to reverse or halt progression of organ damage in ACLF, there is limited evidence currently.","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70034"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143991709","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Gitelman Syndrome in a Toddler With Normal Blood Test Findings Except on Sick Days. 除病假外，血液检查结果正常的幼儿患有吉特尔曼综合征。

IF 2.4 4区医学

Nephrology Pub Date : 2025-04-01 DOI: 10.1111/nep.70040

Shoichiro Shirane, Shogo Amemiya, Yuya Saito, Toshimasa Obonai

引用次数: 0

Fibrillary Glomerulonephritis: Clinicopathological Characteristics and Outcome-Case Series From a Multicentre Australasian Cohort. 原纤维性肾小球肾炎：来自澳大利亚多中心队列的临床病理特征和结果病例系列。

IF 2.4 4区医学

Nephrology Pub Date : 2025-04-01 DOI: 10.1111/nep.70022

Muralikrishna Gangadharan Komala, Angela Bayly, Adrian Y S Lee, Brian Nankivell, Levina Neill, Seethalakshmi Viswanathan

{"title":"Fibrillary Glomerulonephritis: Clinicopathological Characteristics and Outcome-Case Series From a Multicentre Australasian Cohort.","authors":"Muralikrishna Gangadharan Komala, Angela Bayly, Adrian Y S Lee, Brian Nankivell, Levina Neill, Seethalakshmi Viswanathan","doi":"10.1111/nep.70022","DOIUrl":"10.1111/nep.70022","url":null,"abstract":"Aim: Fibrillary glomerulonephritis (FGN) is a rare deposition disease with unclear aetiology. There are limited case series of FGN described in the literature. Here, we describe the clinicopathological characteristics and outcomes of a series of 26 patients with FGN diagnosed at an Australian tertiary centre for renal diseases over a decade.Method(s): The present study includes 26 patients with biopsy-proven FGN diagnosed between January 2011 and December 2021.Results: The average age at presentation was 60 years, with a female predominance. The mean creatinine at presentation was 205 μmol/L. Most of the patients had significant proteinuria, with an average 24-h urine protein of 3.76 g. Associated conditions included four patients with autoimmune disease, one patient with malignancy, and two patients with Hepatitis C infection. Serum electrophoresis demonstrated monoclonality in three patients, although immunofluorescence did not reveal clonal restriction on the renal biopsy. Most patients had mesangial expansion, with an increase in mesangial cellularity and variable degrees of capillary wall thickening. An established membranoproliferative pattern was seen in 10 patients. The median follow-up period was 33 months. Three patients received therapy targeted at FGN. End-stage kidney disease developed in 10 patients, with 6 patients dying during the follow-up period, mostly due to additional cardiovascular disease or sepsis.Conclusion: This case series of FGN demonstrates that a significant proportion of patients progress towards end-stage kidney disease. The mortality is significant although the cause of death is due to additional conditions rather than directly due to FGN.","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"30 4","pages":"e70022"},"PeriodicalIF":2.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11949550/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143730906","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0