CEN Case Reports最新文献

{"title":"A case of membranous nephropathy during asymptomatic syphilis.","authors":"Yudai Kobayashi, Tomohito Doke, Mako Hayashi, Kazunori Goto, Chisato Tukamoto, Hiroshi Kojima, Kazuhiro Furuhashi, Shoichi Maruyama","doi":"10.1007/s13730-026-01183-6","DOIUrl":"10.1007/s13730-026-01183-6","url":null,"abstract":"<p><p>We experienced membrane nephropathy associated with syphilis infection. A 51-year-old woman had an increase in body weight with leg edema. Blood and urine examination showed low serum protein and heavy proteinuria. She was diagnosed with nephrotic syndrome. The light microscope showed normal glomeruli with no bubbling appearance or spike lesions. Immunofluorescence analysis on the renal biopsy sample showed granular deposition of IgG, C3, C1q, κ and λ on capillary walls. The electron microscope showed a hump-like subepithelial and mesangial electron-dense deposit. Her pathologic diagnosis was Ehrenreich-Churg stage I membrane nephropathy. Negative staining of IgG4 on glomerular walls indicates secondary membrane nephropathy. The immunology test indicated active syphilis infection despite the lack of any skin lesions or constitutional symptoms related to syphilis. NDNF, which was recently identified as an antigen of syphilis-associated MN, was positive on capillary walls. The antibiotic treatment led to rapid, complete remission without using immunosuppressive treatment.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13612784/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149036584","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":"Successful hemodialysis withdrawal with glucocorticoid treatment after achieving infection control in a case of crescentic IgA-dominant staphylococcus-associated glomerulonephritis.","authors":"Yuto Nishiyama, Mimiko Matsumura, Yukiko Yasui, Nozomi Omizo, Takahiro Saito, Miho Kawashima, Nayuta Seto, Yuichiro Nagase, Shinichiro Morioka, Daisuke Katagiri, Hideki Takano","doi":"10.1007/s13730-026-01182-7","DOIUrl":"10.1007/s13730-026-01182-7","url":null,"abstract":"<p><p>Staphylococcus-associated glomerulonephritis (SAGN) is a subtype of infection-related glomerulonephritis (IRGN) that often affects older adults and can progress rapidly. The role of immunosuppressive therapy in IRGN remains controversial due to the risk of worsening infection and limited evidence from randomized controlled trials. In this report, we present the case of a 70 year-old woman who developed bacteremia, pyogenic sacroiliitis, and multiple iliopsoas abscesses caused by methicillin-susceptible Staphylococcus aureus. While receiving pathogen-directed antibiotics, the patient developed acute kidney injury (peak serum creatinine level, 5.48 mg/dL) with gross hematuria and nephrotic-range proteinuria and subsequently required hemodialysis. Renal biopsy revealed endocapillary hypercellularity and cellular-to-fibrocellular crescents with mesangial expansion and hypercellularity. Immunofluorescence tests demonstrated IgA-dominant deposits with C3, and electron microscopy revealed mesangial-to-subendothelial electron-dense deposits, supporting the diagnosis of IgA-dominant SAGN. Glucocorticoid treatment was initiated after confirming regression of the iliopsoas abscesses and objective evidence of infection control, while continuing pathogen-directed antibiotics. Renal function gradually improved, and hemodialysis was discontinued. In patients with crescentic IgA-dominant SAGN with objectively confirmed infection control and progressive renal dysfunction despite adequate antimicrobial therapy, a carefully timed glucocorticoid treatment strategy, with continued antibiotic therapy and close monitoring, may lead to recovery of renal function and hemodialysis withdrawal.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13586130/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148988254","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":"Highly viscous ascites prompting reconsideration of cardiorenal syndrome in a patient with advanced CKD after TAVI.","authors":"Koshi Yamada, Takeo Koshida, Kenichiro Abe, Takashi Kobayashi, Masao Kihara, Tomohito Gohda, Yusuke Suzuki","doi":"10.1007/s13730-026-01179-2","DOIUrl":"10.1007/s13730-026-01179-2","url":null,"abstract":"<p><p>Volume management is challenging in patients with advanced chronic kidney disease (CKD) complicated by cardiovascular disease, particularly when systemic congestion cannot be explained solely by cardiac or renal dysfunction. We report an elderly man with type 2 diabetes mellitus, stage 4 CKD, hypertension, and ischemic heart disease who developed refractory pleural effusion, ascites, and peripheral edema after transcatheter aortic valve implantation (TAVI). Right heart catheterization showed preserved cardiac output, whereas echocardiography suggested right-sided pressure overload and systemic venous congestion. Intensified diuretic therapy led to partial body weight reduction but was accompanied by worsening renal function, and adequate volume control remained difficult. Although ascites was initially attributed to cardiorenal syndrome or liver disease, diagnostic paracentesis revealed highly viscous, protein-rich ascitic fluid with a low serum-ascites albumin gradient and a markedly elevated carcinoembryonic antigen level. In conjunction with non-contrast magnetic resonance imaging findings showing multiple loculated cystic collections and fluid-fluid levels, these ascitic fluid characteristics raised suspicion of a mucinous peritoneal process suggestive of pseudomyxoma peritonei, despite negative cytology. Recurrent deterioration of renal function was temporally associated with the reaccumulation of massive ascites, whereas right-sided venous congestion may have acted as an aggravating hemodynamic factor. This case highlights the importance of evaluating atypical ascitic fluid characteristics and considering etiologies beyond cardiac, renal, or hepatic dysfunction in patients with refractory volume overload after TAVI.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13586112/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148988237","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":"A shared immunological context underlying IgA nephropathy.","authors":"Akihiro Shimizu, Nobuo Tsuboi","doi":"10.1007/s13730-026-01178-3","DOIUrl":"10.1007/s13730-026-01178-3","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13570835/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148954950","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":"Experience with early treatment using febuxostat in a patient with adenine phosphoribosyltransferase deficiency after pediatric kidney transplantation: a case report.","authors":"Yujiro Aoki, Maho Maeda, Junya Hashimoto, Ayuko Zaitsu, Kazuyuki Ueno, Satoshi Furukawa, Shintaro Ochiai, Kei Sakurabayashi, Takashi Yonekura, Yoshihiro Itabashi, Masaki Muramatsu, Takeshi Kawamura, Yuko Hamasaki, Ken Sakai","doi":"10.1007/s13730-026-01176-5","DOIUrl":"10.1007/s13730-026-01176-5","url":null,"abstract":"<p><p>Kidney transplantation (KT) for adenine phosphoribosyltransferase (APRT) deficiency is performed using the xanthine dehydrogenase (XDH) inhibitor allopurinol to prevent the recurrence of dihydroxyadenine (DHA) nephropathy. However, there are few reports on the use of febuxostat after pediatric KT for APRT deficiency. Herein, we report the case of a 12-year-old boy with congenital kidney and urinary tract abnormalities who underwent deceased-donor KT for end-stage kidney disease caused by APRT deficiency. The patient developed acute kidney injury during infancy due to urinary tract stones and was diagnosed with APRT deficiency based on stone analysis and genetic testing. Treatment with allopurinol was initiated. However, the patient had persistent renal dysfunction and required peritoneal dialysis at the age of 9 years. Grade IV left vesicoureteral reflux (VUR) and a large bladder diverticulum were observed. Although the VUR resolved, the patient underwent bladder diverticulectomy and ureteroneocystostomy before KT due to recurrent complicated urinary tract infections associated with an enlarged bladder diverticulum and residual urine. Subsequently, the patient underwent KT, and the XDH inhibitor was switched from allopurinol to febuxostat. Transplant renal biopsy at 4 months post-KT showed no recurrence of DHA nephropathy; graft function remained stable. This case demonstrated that pediatric patients with APRT deficiency undergoing KT could be successfully managed with febuxostat.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13538336/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148879259","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":"Rapidly progressive steroid-resistant focal segmental glomerulosclerosis associated with an INF2 exon 6 variant.","authors":"Syuhei Watanabe, Kenichi Tanaka, Tasuku Ishikawa, Takumi Nishiyama, Yuto Otsuki, Hirotaka Saito, Hiroshi Kimura, Atsushi Ono, Junichiro James Kazama","doi":"10.1007/s13730-026-01177-4","DOIUrl":"10.1007/s13730-026-01177-4","url":null,"abstract":"<p><p>Variants in the inverted formin-2 (INF2) gene are a known cause of hereditary focal segmental glomerulosclerosis (FSGS) and Charcot-Marie-Tooth disease. We report a case of rapidly progressive FSGS associated with a rare INF2 variant. A 12-year-old boy developed proteinuria and was diagnosed with FSGS at age 14 following a renal biopsy. Steroid therapy and subsequent immunosuppressive treatments, including plasma exchange, were ineffective. At age 15, a heterozygous missense variant in exon 6 of the INF2 gene (c.763G>A, p.Asp255Asn) was identified. Despite conservative management, the patient progressed to end-stage kidney disease at age 17. Although exon 6 variants are rarely reported, the present case showed a relatively aggressive renal course.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13538315/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148879181","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":"Biopsy-proven kidney-limited thrombotic microangiopathy associated with bevacizumab and pegylated liposomal doxorubicin: a case with a biphasic clinical course.","authors":"Naoki Chigusa, Tatsuhiko Azegami, Takashin Nakayama, Norifumi Yoshimoto, Akihito Hishikawa, Aika Hagiwara, Akinori Hashiguchi, Kaori Hayashi","doi":"10.1007/s13730-026-01175-6","DOIUrl":"10.1007/s13730-026-01175-6","url":null,"abstract":"<p><p>Drug-induced thrombotic microangiopathy (TMA) is an important cause of kidney injury in patients receiving anticancer therapy, although kidney-limited forms without systemic manifestations are often difficult to recognize. Pegylated liposomal doxorubicin (PLD)-associated TMA remains under-recognized, particularly in patients receiving concomitant vascular endothelial growth factor (VEGF) inhibitors. We report a case of biopsy-proven kidney-limited TMA associated with bevacizumab and PLD in a 52-year-old woman with metastatic ovarian serous adenocarcinoma, characterized by a biphasic clinical course. She developed progressive kidney dysfunction and proteinuria during treatment with bevacizumab followed by PLD. Despite discontinuation of bevacizumab, kidney dysfunction persisted. Kidney biopsy demonstrated findings consistent with TMA, including mesangiolysis, glomerular basement membrane duplication, and focal PAS-positive pseudothrombi suggestive of VEGF inhibitor-associated endothelial injury. Electron microscopy further revealed endothelial detachment, accompanied by a substance containing liposome-like particles, suggestive of the characteristic ultrastructural findings previously reported in PLD-associated TMA. The patient had no evidence of microangiopathic hemolytic anemia or thrombocytopenia, and no alternative cause of TMA was identified. Following discontinuation of PLD, kidney function improved markedly with complete resolution of proteinuria. This case highlights the importance of kidney biopsy and careful clinicopathological correlation in diagnosing drug-induced kidney-limited TMA.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13538319/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148879231","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":"Durvalumab-associated nephrotic syndrome and organizing pneumonia: a case report.","authors":"Takashi Nawata, Masaki Shibuya, Maki Asami-Noyama, Noriko Uesugi, Motoaki Sano","doi":"10.1007/s13730-026-01180-9","DOIUrl":"10.1007/s13730-026-01180-9","url":null,"abstract":"<p><p>Immune checkpoint inhibitors have improved cancer prognosis, but are associated with immune-related adverse events, including rare renal complications. Here, we report the case of a 68-year-old Japanese man with unresectable advanced non-small cell lung cancer who developed nephrotic syndrome and organizing pneumonia associated with durvalumab therapy. The patient developed significant proteinuria during glucocorticoid treatment for organizing pneumonia. Renal biopsy led to a diagnosis of durvalumab-associated nephrotic syndrome. Histological examination revealed complex features, characterized by lesions resembling focal segmental glomerulosclerosis, marked foot process effacement and detachment, and prominent tubulointerstitial nephritis. Notably, his proteinuria improved following discontinuation of durvalumab and treatment with losartan potassium, dapagliflozin, and finerenone, without a substantial increase in the glucocorticoid dose. The clinical course highlights the heterogeneity of durvalumab-associated nephrotic syndrome.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13538327/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148879174","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":"MPO-ANCA-associated glomerulonephritis with immune complex deposition and membranous features in a patient with Sjögren's syndrome: a case report.","authors":"Koshi Yamada, Ryo Fukunaga, Takeo Koshida, Miyuki Takagi, Harumi Saeki, Masayuki Maiguma, Takashi Kobayashi, Masao Kihara, Tomohito Gohda, Yusuke Suzuki","doi":"10.1007/s13730-026-01174-7","DOIUrl":"10.1007/s13730-026-01174-7","url":null,"abstract":"<p><p>Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is classically characterized by pauci-immune necrotizing crescentic glomerulonephritis; however, immune complex deposition is increasingly recognized in a subset of patients, and its clinical significance remains incompletely defined. Coexistence with membranous nephropathy (MN)-like features is uncommon and may complicate diagnosis and treatment. We report a 37-year-old woman with persistent urinary abnormalities detected during routine health screening. Laboratory evaluation showed proteinuria (1.83 g/gCr), microscopic hematuria (> 50 red blood cells/high-power field), mildly impaired kidney function [estimated glomerular filtration rate (eGFR) 51.4 mL/min/1.73 m²], elevated myeloperoxidase (MPO)-ANCA levels, and no hypocomplementemia. Kidney biopsy revealed crescentic glomerulonephritis with granular IgG, C3, and C1q deposition in mesangial and capillary wall regions, together with subepithelial electron-dense deposits suggestive of MN-like features. IgG subclass staining was positive for IgG1-3 but negative for IgG4. Glomerular staining for PLA2R, THSD7A, and NELL1 was negative, whereas glomerular EXT1 staining was positive and EXT2 staining was negative. Overall, the clinicopathological profile was not typical of primary MN. Further evaluation demonstrated anti-SSA antibody positivity and objective ocular findings consistent with Sjögren's syndrome (SjS). Following treatment with high-dose corticosteroids, rituximab, and avacopan, kidney function and urinary abnormalities gradually improved, although proteinuria declined relatively slowly. This case was characterized by MPO-ANCA-associated crescentic glomerulonephritis with immune complex deposition and membranous features in the setting of SjS. The findings highlight the diagnostic difficulty of attributing the membranous component to a single disease process and suggest the involvement of complex and overlapping autoimmune mechanisms.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13518758/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148825611","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":"Sequential changes in urinary crystal morphology in ethylene glycol poisoning: first human LV-SEM characterization of recovery-phase crystals.","authors":"Hiroki Ito, Takuo Hirose, Yuya Suzuki, Kentaro Yano, Risa Ishikawa, Tsubasa Yuki, Hideaki Hashimoto, Go Anan, Takashi Shimada, Hideya Itagaki, Yoshinobu Abe, Tomoyuki Endo, Takefumi Mori","doi":"10.1007/s13730-026-01173-8","DOIUrl":"10.1007/s13730-026-01173-8","url":null,"abstract":"<p><p>Ethylene glycol (EG) poisoning typically causes severe metabolic acidosis and acute kidney injury (AKI), characterized by urinary calcium oxalate (CaOx) crystals. Although needle-shaped calcium oxalate monohydrate (COM) crystals are a recognized diagnostic marker in the acute phase, sequential changes in urinary crystal morphology during recovery have not been well characterized in human cases. We present a 32-year-old man who developed profound metabolic acidosis (pH 6.747) and AKI (serum creatinine 1.56 mg/dL) after ingesting a lethal dose of EG. Following emergency hemodialysis and fomepizole treatment, urinalysis showed a sequential transition of urinary CaOx crystals from abundant needle-shaped COM in the acute phase to dumbbell-shaped COM and octahedral calcium oxalate dihydrate (COD) by day 14. This transition was observed during recovery, and renal function fully recovered by day 68 (serum creatinine 0.78 mg/dL). Low-vacuum scanning electron microscopy (LV-SEM) provided detailed three-dimensional visualization of these recovery-phase crystals after simple cytospin preparation, without chemical fixation or conductive coating. This report provides the first human LV-SEM visualization and characterization of recovery-phase urinary calcium oxalate crystals in ethylene glycol poisoning. These morphological changes may reflect recovery-related changes in the intratubular environment and offer pathophysiological insight into the transition from the acute toxic phase to recovery.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 5","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13473040/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148727659","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}