CEN Case Reports最新文献

{"title":"Correction: A pediatric case of membranous-like glomerulopathy with masked IgG kappa deposits.","authors":"Yuichi Uno, Hiroshi Kitamura, Katsuyoshi Kanemoto, Mai Masuda, Yui Otake","doi":"10.1007/s13730-026-01136-z","DOIUrl":"10.1007/s13730-026-01136-z","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13310860/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148344008","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":"Correction: Congophilic fibrillary glomerulonephritis recurrence post-renal transplant: diagnostic challenges and proteomic insights.","authors":"Hiroshi Watanabe, Michiko Nagamine, Yukako Shintani-Domoto, Kunio Kawanishi, Kousuke Ishino, Tsukasa Nakamura, Shinji Sumiyoshi, Eiichi Konishi","doi":"10.1007/s13730-026-01137-y","DOIUrl":"10.1007/s13730-026-01137-y","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13310871/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148343989","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":"Postpartum TMA requiring dialysis with discordant complement tests: a case report.","authors":"Hikari Fujimura, Shun Minatoguchi, Riku Takeuchi, Ryosuke Umeda, Yuka Sato, Shigehisa Koide, Asuka Horinouchi, Noritoshi Kato, Masashi Mizuno, Hiroki Hayashi, Midori Hasegawa, Shoichi Maruyama, Naotake Tsuboi","doi":"10.1007/s13730-026-01138-x","DOIUrl":"10.1007/s13730-026-01138-x","url":null,"abstract":"<p><p>Pregnancy induces vascular, coagulation, and immune alterations that predispose to thrombotic microangiopathy (TMA). Within this spectrum-preeclampsia/ hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP) and complement-mediated TMA (CM-TMA)-overlapping features complicate diagnosis, whereas management and prognosis diverge. A 39-year-old woman developed severe hypertension at 35 weeks' gestation, underwent emergency cesarean section for preeclampsia, and subsequently became anuric. She had schistocytic hemolysis, thrombocytopenia, elevated lactate dehydrogenase (LDH), and transaminitis. One plasma exchange, hemodialysis, and supportive care were provided; urine output recovered and dialysis ceased. Atypical complement tests results persisted for ~ 1 month (undetectable serum CH50 with elevated C3/C4), with normal functional assays and no pathogenic variants in complement-regulatory genes. On subsequent testing with Ethylenediaminetetraacetic acid (EDTA) plasma, preserved at - 80 °C, showed a normal CH50, suggesting that ex vivo complement activation may have contributed to the low serum CH50. In the acute setting, HELLP versus CM-TMA remained indeterminate, so management was guided by the clinical trajectory: brief plasma exchange during TTP evaluation, close monitoring, and deferral of complement inhibition as platelets, LDH, and urine output improved. This case supports a \"trend-first\" approach to peripartum TMA, and underscores ex vivo complement activation as a key pitfall when interpreting complement assays in the peripartum setting.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13315354/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148343948","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":"Nephrin-IgG co-localization expression in pediatric steroid-resistant nephrotic syndrome: a case series from Indonesia.","authors":"Rini Rossanti, Ahmedz Widiasta, Nana Sakakibara, Inoki Yuta, China Nagano, Asahi Yamamoto, Yuka Kimura, Aoyama Shuhei, Tomohiko Yamamura, Tomoko Horinouchi, Shingo Ishimori, Dedi Rachmadi, Kazumoto Iijima, Kandai Nozu, Dany Hilmanto","doi":"10.1007/s13730-026-01144-z","DOIUrl":"10.1007/s13730-026-01144-z","url":null,"abstract":"<p><p>Steroid-resistant nephrotic syndrome (SRNS) remains a major clinical challenge due to its heterogeneous etiologies and poor response to conventional immunosuppressive therapy. Anti-nephrin IgG has been associated with podocyte injury and proteinuria, suggesting a potential pathogenic role in SRNS. This case series was conducted to present real-world data on the evaluation of nephrin-IgG immunofluorescence colocalization in pediatric SRNS in a low-resource setting, and to examine whether this approach may inform treatment selection in routine clinical practice. We describe four pediatric patients with SRNS who underwent kidney biopsy following persistent proteinuria despite immunosuppressive therapy. Case 1 showed severe hypoalbuminemia and anasarca with preserved renal function after full dose steroid; Case 2 remained edematous with nephrotic range proteinuria after seven cycle of cyclophosphamide; Case 3 progressed to chronic kidney disease despite cyclophosphamide and mycophenolate mofetil; Case 4 had normal kidney function and no edema, despite steroid resistant. Nephrin-IgG co-localization was observed in all cases. Clinical outcomes were heterogeneous, and assessment of treatment response was limited by resource constraints and loss to follow-up. These findings are exploratory in nature and suggest the potential applicability of nephrin-IgG co-localization in a low-resource setting, warranting further investigation.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13315047/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148343943","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":"Purtscher-like retinopathy presenting as acute vision loss in childhood nephrotic syndrome: case report with review of literature.","authors":"Deepthika Sadasivam, Simar Rajan Singh, Sushmitha Poojary, Divya Chauhan, Pujitha Vallabhaneni, Karalanglin Tiewsoh, Lesa Dawman","doi":"10.1007/s13730-026-01151-0","DOIUrl":"10.1007/s13730-026-01151-0","url":null,"abstract":"<p><p>Purtscher-like retinopathy is a rare occlusive microvasculopathy that causes acute, painless visual loss and characteristic retinal lesions. Although typically trauma-related, it can occur in systemic conditions such as nephrotic syndrome (NS), driven by hypercoagulability, complement activation, and microembolization. We report a 3-year-old girl with steroid-resistant NS presenting with sudden vision loss, including inability to fixate and intermittent exotropia, without trauma or hypertension. Fundoscopy revealed bilateral peripapillary retinal whitening with periarteriolar sparing, cotton wool spots, and intraretinal hemorrhages. Laboratory tests showed hypoalbuminemia (1.7 g/dL) and nephrotic-range proteinuria; kidney biopsy suggested podocytopathy. She was receiving prednisolone and cyclosporine (trough 84 ng/mL), which was discontinued due to concern for drug-induced vasculopathy. Treatment included intravenous methylprednisolone pulses followed by oral tapering. Whole-exome sequencing identified a de novo pathogenic WT1 variant (c.1447 + 5G > A), establishing a genetic etiology (NPHS4) for steroid-resistant nephrotic syndrome in this patient. At 6 months, visual acuity improved from light perception to counting fingers at a distance of 2 m. This case highlights the need to consider Purtscher-like retinopathy in NS patients with acute vision loss, particularly in the context of steroid resistance or calcineurin inhibitor therapy. Prompt ophthalmologic evaluation and careful medication review are critical to prevent permanent visual impairment and to optimize recovery.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13315073/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148343951","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":"Pediatric case of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis with immunoglobulin a deposition diagnosed early through school urinary screening.","authors":"Takuto Kawamura, Yuko Kajiho, Keiichi Takizawa, Shoichiro Kanda, Amane Yamamoto, Hiroyuki Abe, Yutaka Harita","doi":"10.1007/s13730-026-01152-z","DOIUrl":"10.1007/s13730-026-01152-z","url":null,"abstract":"<p><p>Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare but severe condition in children, and renal involvement in the form of ANCA-associated glomerulonephritis (AAGN) usually determines prognosis. Although AAGN is characterized by pauci-immune necrotizing crescentic glomerulonephritis, mesangial immunoglobulin A (IgA) deposition occurs in a subset of patients; however, pediatric cases remain exceptionally rare. We aimed to report a case of a 12-year-old girl who was asymptomatic and diagnosed through routine school urinalysis. She presented with hematuria and progressive proteinuria, accompanied by elevated myeloperoxidase (MPO)-ANCA levels, while renal function was preserved. Renal biopsy revealed a cellular crescent with surrounding inflammatory cell infiltration, consistent with AAGN. Immunofluorescence microscopy demonstrated mesangial deposition of IgA, immunoglobulin M, and C3; however, there was no mesangial cell proliferation or matrix expansion to suggest typical IgA nephropathy. Based on the clinical course and pathological findings, the primary pathological process was considered to be AAGN rather than IgA nephropathy. The patient was treated with methylprednisolone pulse therapy, followed by rituximab and maintenance therapy with azathioprine. MPO-ANCA levels and proteinuria normalized, and no relapse has been observed. This case highlights the significance of early detection of pediatric renal-limited AAV and suggests that timely AAV-based immunosuppressive therapy promotes favorable outcomes in pediatric AAGN with IgA deposition.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13315392/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148343926","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":"Late-onset oligomeganephronia in a young adult born extremely preterm with lifestyle-related hyperfiltration stress.","authors":"Satoko Abe, Wako Yumura, Ayano Izawa, Masatoshi Oka, Shiho Matsuno, Noriko Yamanaka, Noriyuki Suzuki, Akiko Komatsu, Hirofumi Rokutan, Tomio Arai, Mitsuyo Itabashi, Takashi Takei","doi":"10.1007/s13730-026-01150-1","DOIUrl":"10.1007/s13730-026-01150-1","url":null,"abstract":"<p><p>Oligomeganephronia (OMN) is a rare congenital renal hypoplasia characterized by markedly reduced nephron number with compensatory glomerular hypertrophy. Although typically diagnosed in childhood, adult-onset OMN is uncommon and often under-recognized. A 29-year-old man born at 28 weeks of gestation as one of triplets, with a birth weight of 740 g, was referred for evaluation of persistent proteinuria. Proteinuria had been intermittently detected for 10 years but remained uninvestigated. Three months before admission, the urinary protein-to-creatinine ratio increased to 1.20 g/gCr with serum creatinine 1.54 mg/dL. He had engaged in regular resistance exercise and consumed protein supplements. On admission, urinary β2-microglobulin was mild elevated, and a discrepancy between creatinine- and cystatin C-based estimated glomerular filtration rate (eGFR) was observed, whereas inulin clearance was preserved. Kidney biopsy revealed markedly enlarged glomeruli, reduced glomerular density, and perihilar variant focal segmental glomerulosclerosis, consistent with OMN with secondary hyperfiltration injury. Treatment with losartan resulted in a rapid reduction in proteinuria and stable renal function over 9 months. This case illustrates that latent OMN in individuals born extremely preterm may remain subclinical until additional hyperfiltration stressors precipitate overt renal injury. Early recognition and renin-angiotensin system inhibition can ameliorate proteinuria and stabilize renal function.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13315388/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148343938","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 case of lipoprotein glomerulopathy in which complete remission was achieved by pemafibrate therapy.","authors":"Chika Tomita, Taichi Sato, Yuki Suzuki, Mayumi Arakawa, Yoshitaka Shimizu, Yumiko Suzuki, Hideo Yasuda, Taro Misaki","doi":"10.1007/s13730-026-01148-9","DOIUrl":"10.1007/s13730-026-01148-9","url":null,"abstract":"<p><p>Lipoprotein glomerulopathy (LPG) is a rare hereditary glomerular disease characterized by lipoprotein thrombi within dilated glomerular capillaries for which effective treatments remain limited. This report concerns a 25-year-old Japanese woman with an 8-year history of persistent proteinuria and severe dyslipidemia who presented with heavy proteinuria (3.31 g/gCr) despite preserved renal function. Kidney biopsy revealed features typical of LPG on light and electron microscopy, and serum apoE levels were markedly elevated. The patient was started on pemafibrate, a selective peroxisome proliferator-activated receptor-alpha modulator known to exert stronger triglyceride-lowering effects and to have a more favorable hepatic and renal safety profile compared with conventional fibrates. Following treatment, the patient's triglyceride levels decreased substantially and her proteinuria progressively improved. Complete remission (0.05 g/gCr) was achieved within 7 months, and she remained stable thereafter. Although partial remission with fibrates has been previously reported in patients with LPG, there have been no reports of complete remission in those treated with pemafibrate. To our knowledge, this is the first documented case of complete remission of proteinuria induced by pemafibrate in a patient with LPG. Pemafibrate may be a promising targeted therapy for this rare glomerular disorder.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13315064/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148344005","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":"Renal vein thrombosis as a reversible vascular renal complication of CLOVES syndrome: a case report.","authors":"Zeynep Ural","doi":"10.1007/s13730-026-01149-8","DOIUrl":"10.1007/s13730-026-01149-8","url":null,"abstract":"<p><p>PIK3CA-related overgrowth spectrum (PROS) comprises mosaic gain-of-function variants in PIK3CA that lead to segmental overgrowth and complex vascular malformations. Renal involvement has been inconsistently described and mainly includes glomerulomegaly and focal segmental glomerulosclerosis. We report a 39-year-old woman with a phenotypic diagnosis of CLOVES syndrome molecularly supported by a somatic mosaic PIK3CA variant, NM_006218.4:c.1634 A > C, p.Glu545Ala, detected in affected hypertrophic tissue. She presented with acute kidney injury and subnephrotic-range proteinuria secondary to left renal vein thrombosis. Imaging revealed intrarenal venous congestion in the setting of extensive abdominal venous malformations. Anticoagulation resulted in complete radiologic and functional recovery. Renal vein thrombosis should be considered as a potentially reversible vascular cause of acute kidney injury and proteinuria in patients with CLOVES syndrome/PROS, particularly in the presence of extensive venous malformations.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13291370/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148301147","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":"Cold agglutinin syndrome secondary to Epstein-Barr virus reactivation during corticosteroid therapy for minimal change nephrotic syndrome in a patient with human immunodeficiency virus infection.","authors":"Harui Bamba, Daisuke Katagiri, Keiki Shimada, Hitomi Otani, Naoto Eriguchi, Yuri Katayama, Mikako Koizumi, Minami Suzuki, Hideki Takano","doi":"10.1007/s13730-026-01145-y","DOIUrl":"10.1007/s13730-026-01145-y","url":null,"abstract":"<p><p>Epstein-Barr virus (EBV), a member of the herpesvirus family, establishes lifelong latency following primary infection and may be reactivated under conditions of immunosuppression. EBV reactivation is classically associated with infectious mononucleosis and Burkitt lymphoma and rarely associated with cold agglutinin syndrome. Here, we describe a case of minimal change nephrotic syndrome complicated by cold agglutinin syndrome secondary to EBV reactivation in the setting of human immunodeficiency virus (HIV) infection and corticosteroid-induced immunosuppression. A 63-year-old man with well-controlled HIV infection owing to antiretroviral therapy was admitted for nephrotic syndrome. He was treated with prednisolone (80 mg/day), and renal biopsy performed on day 9 confirmed minimal change nephrotic syndrome. Complete remission was achieved by day 18; however, the patient subsequently developed hyperbilirubinemia, elevated transaminase and lactate dehydrogenase levels, progressive anemia, and thrombocytopenia, accompanied by pharyngitis, malaise, and fever. Laboratory investigations revealed a positive cold agglutinin test, positive direct Coombs test, reduced haptoglobin level, and positive urine urobilinogen, consistent with cold agglutinin syndrome. CMV reactivation was also suggested by transient CMV antigenemia positivity, although no organ-invasive CMV disease was evident. Although EBV serology indicated a past infection, the EBV DNA load was elevated, which suggested viral reactivation. The patient responded to supportive measures, along with early tapering of the corticosteroid dose to reduce immunosuppression. This case highlights that HIV infection in combination with corticosteroid therapy may create an immunosuppressive milieu, predisposing patients to EBV reactivation and subsequent development of cold agglutinin syndrome. Clinicians should be vigilant for EBV-related complications in patients with minimal change nephrotic syndrome requiring corticosteroid therapy, particularly in those with underlying HIV infection.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13287368/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148293418","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}