CEN Case Reports最新文献

{"title":"Early administration of caplacizumab combined with plasma exchange for thrombotic microangiopathy due to malignant hypertension: a case report.","authors":"Hiroyuki Okawa, Yukihiro Wada, Kazuhiro Takeuchi, Tomomi Motohashi, Tetsuya Abe, Ryota Uchitsubo, Naohiro Kawamura, Sayumi Kawamura, Shun Sakurabayashi, Kosuke Honda, Masamitsu Morishita, Shokichi Naito, Togo Aoyama, Yasuo Takeuchi","doi":"10.1007/s13730-025-00978-3","DOIUrl":"https://doi.org/10.1007/s13730-025-00978-3","url":null,"abstract":"<p><p>Both thrombotic thrombocytopenic purpura (TTP) and malignant hypertension (MHT) present with thrombotic microangiopathy (TMA). Combination therapy with caplacizumab, anti-von Willebrand factor (VWF) A1 domain antibody, and plasma exchange (PE) has recently been highlighted as a novel therapeutic option for TTP. We treated a 51-year-old woman who showed severe hypertension, retinopathy, and acute kidney injury. Level of consciousness was clear on admission, but low-grade fever was observed. Laboratory tests showed normocytic anemia, thrombocytopenia, renal dysfunction, and a slight decrease in haptoglobin. Neither disseminated intravascular coagulation nor leukemia was diagnosed. The patient emergently received intravenous antihypertensive therapy, continuous hemodiafiltration, and sufficient blood transfusion. However, thrombocytopenia and oliguria remained despite control of blood pressure. On hospital day 8, administration of caplacizumab combined with PE was initiated before receiving results for a disintegrin-like and metalloprotease with thrombospondin type 1 motifs 13 (ADAMTS13) activity and inhibitor levels. We then administered caplacizumab for 5 days and performed 2 sessions of PE until confirming ADAMTS13 activity of 42% and absence of its inhibitor, contributing to increased serum hemoglobin and platelet levels with cessation of dialysis. Renal biopsy findings on hospital day 20 showed arteriolar nephrosclerosis and intimal hyperplasia in small arteries. To the best of our knowledge, this represents the first description of MHT-induced TMA treated with caplacizumab. MHT-induced TMA exhibiting symptoms of TTP tends to show poor renal prognosis, so early administration of caplacizumab with PE before receiving results for ADAMTS13 might prove beneficial for cases in which MHT complicated with TTP is suspected.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143540370","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}

{"title":"Successful outcome of a refractory IgA vasculitis nephritis in children treated with telitacicept.","authors":"Yanyan Jin, Aiqin Sheng, Qian Lin, Xue He, Haidong Fu, Jianhua Mao","doi":"10.1007/s13730-025-00983-6","DOIUrl":"https://doi.org/10.1007/s13730-025-00983-6","url":null,"abstract":"<p><p>IgA vasculitis (IgAV) is the most prevalent form of vasculitis in children. While most cases of IgAV present with mild clinical symptoms and generally have a favorable prognosis, some children with IgAV nephritis may experience persistent heavy proteinuria, which is at risk of progressing to end-stage renal disease. Despite the administration of various immunosuppressive agents, treatment outcomes for these children are often suboptimal. We report the case of an 8-year-and-four-month-old girl who initially presented with rashes on both lower limbs for a duration of four days and abdominal pain persisting for two days. Renal biopsy subsequently confirmed a diagnosis of IgAV nephritis, specifically type IIIb. Despite undergoing treatment with methylprednisolone sodium succinate, cyclophosphamide, mycophenolate mofetil, leflunomide, rituximab, dapagliflozin, and other medications for over two years, her urinary protein levels remained at least 1000 mg/24 h. One month after initiating treatment with telitacicept, the patient's urinary protein levels decreased, and two months later, they became negative. Notably, even after discontinuing immunosuppressants and glucocorticoids, the girl achieved sustained clinical remission. This case serves as a valuable clinical reference for the use of telitacicept in the treatment of refractory IgAV nephritis.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536596","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}

{"title":"A case of IgG-type heavy chain amyloidosis with membranous nephropathy-like changes with long-term survival.","authors":"Kei Kono, Naoki Sawa, Yuki Oba, Koichi Kikuchi, Hiroki Mizuno, Masayuki Yamanouchi, Eiko Hasegawa, Tatsuya Suwabe, Atsushi Wake, Yukako Shintani-Domoto, Kenichi Ohashi, Fuyuki Kametani, Masahide Yazaki, Takehiko Wada, Yoshifumi Ubara","doi":"10.1007/s13730-025-00982-7","DOIUrl":"https://doi.org/10.1007/s13730-025-00982-7","url":null,"abstract":"<p><p>We performed a kidney biopsy on a 68-year-old man with 2.6 g/day proteinuria. Immunofluorescence (IF) study showed a positive finding of IgG (IgG1) along the glomerular capillary wall, suggesting membranous nephropathy. However, electron microscopy showed no subepithelial electron dense deposits and amorphous deposits of 8-12 nm fibrillar structure, and positive DFS/Congo red staining confirmed the diagnosis of amyloidosis. Amyloid deposits were localized only in the glomeruli. No overt extrarenal amyloid lesions were identified. Serum and urine were positive for monoclonal IgG-lambda, but IF was negative for kappa and lambda, ruling out AL-amyloidosis. At 75 years of age, he underwent a second kidney biopsy, and the IgG-positive findings at IF became more pronounced, with progressive glomerular sclerosing lesions. A proteomic analysis was performed focusing on the positive IgG finding in IF. The amyloid was proven to be composed of fragments of a heavy chain variable region sequence. AH-amyloidosis was the final diagnosis. He was treated according to the treatment for AL-amyloidosis, but hemodialysis was started at the age of 81, and died at the age of 86. We report a valuable case of AH-amyloidosis with an observed long-term prognosis.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143531333","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}

{"title":"IgA nephropathy and IgA vasculitis in a pediatric Crohn's disease patient with early IgA deposition in vascular walls of intestines.","authors":"Junqian Tang, Lan Wang, Wei Zhou, Youying Mao, Chenxing Zhang, Jiayao Shen, Minzhi Yin, Lei Yin","doi":"10.1007/s13730-025-00970-x","DOIUrl":"https://doi.org/10.1007/s13730-025-00970-x","url":null,"abstract":"<p><p>Patients with inflammatory bowel disease may present with extraintestinal manifestations. Crohn's disease complicated with IgA nephropathy or IgA vasculitis is relatively rare. In this case, an 11-year-old girl was diagnosed with Crohn's disease and infliximab was administered. 1 year after treatment, she presented with asymptomatic but persistent microscopic hematuria. The child was diagnosed with IgA vasculitis and IgA nephropathy at the fourth year of follow-up. To explore the association between Crohn's disease and IgA associated diseases, immunostaining for IgA and GdIgA1 deposition was retrospectively conducted in intestinal biopsy tissues obtained at the time of initiation and relapse of Crohn's disease. GdIgA1 deposition in intestinal tissues was found not only at the time of relapse of Crohn's disease, but also at the beginning of Crohn's disease when patient had neither exposure to any drug nor any symptom of IgA vasculitis or IgA nephropathy. The early appearance of GdIgA1 deposition indicated that Crohn's disease played a greater role in its formation than infliximab induction and the child might be prone to IgA associated diseases. Patients with Crohn's disease, especially those who receive tumor necrosis factor-alpha inhibitors are recommended to receive regular kidney examinations.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143514792","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}

{"title":"Peritoneal lymphomatosis as a rare entity of post-transplant lymphoproliferative disorder after kidney transplantation: a case report.","authors":"Kana Shirai, Masatomo Ogata, Marie Murata-Hasegawa, Masataka Miyauchi, Yuko Sakurai, Kazunobu Shinoda, Junki Koike, Yugo Shibagaki, Masahiko Yazawa","doi":"10.1007/s13730-025-00976-5","DOIUrl":"https://doi.org/10.1007/s13730-025-00976-5","url":null,"abstract":"<p><p>Post-transplant lymphoproliferative disorder (PTLD) is a severe complication of solid organ transplantation, and its manifestation as peritoneal lymphomatosis (PL) is extremely rare. This report describes the case of a 65-year-old kidney transplant recipient who presented with massive ascites as the initial symptom of PTLD 8 years post-transplantation. The patient experienced a sudden onset of ascites, and both his serum and ascitic fluid showed elevated lactate dehydrogenase (LDH) levels along with characteristic computed tomography (CT) findings, both of which raised the suspicion of lymphoma. The cytology of the ascitic fluid confirmed the diagnosis of peritoneal primary diffuse large B-cell lymphoma, leading to an early diagnosis of PL and management strategies. Although the patient developed tumor lysis syndrome soon after chemotherapy, prompt management led to complete remission. When ascites is observed in post-transplant recipients, it is essential to consider PTLD, recognize the diagnostic value of LDH levels and CT imaging, and emphasize the importance of early intervention to achieve favorable outcomes. To the best of our knowledge, this is the first reported case of PL in a kidney transplant recipient.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143448322","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}

{"title":"Two adolescents with frequently relapsing nephrotic syndrome newly diagnosed after SARS-CoV-2 vaccination: case report and literature review.","authors":"Eriko Nakazawa, Toru Uchimura, Rie Ohyama, Hayato Togashi, Aya Inaba, Kentaro Shiga, Shuichi Ito","doi":"10.1007/s13730-025-00967-6","DOIUrl":"https://doi.org/10.1007/s13730-025-00967-6","url":null,"abstract":"<p><p>Even though several cases of new-onset nephrotic syndrome following vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been reported, none have included the medium- to long-term prognosis of the patients. Here, we report the prognoses of two adolescents, aged 14 and 15 years, who developed nephrotic syndrome soon after receiving the Pfizer-BioNTech SARS-CoV-2 vaccine. Both patients were diagnosed with nephrotic syndrome after developing edema within a few days post-SARS-CoV-2 vaccination. Although they achieved rapid and complete remission with prednisolone therapy, they developed frequently relapsing nephrotic syndrome and were initiated on cyclosporine. In one patient, frequent relapses occurred while taking cyclosporine, requiring rituximab to maintain remission. Measurements of antibody titers against the spike protein of the SARS-CoV-2 vaccine taken over time revealed significantly lower titers in both patients compared with those in healthy individuals. Furthermore, each patient was infected with SARS-CoV-2 about 12 months post vaccination, with mild symptoms. Nephrotic syndrome did not recur in either patient. We also reviewed 49 published cases of patients who developed nephrotic syndrome after SARS-CoV-2 vaccination, compared to our pediatric cases, there are no cases of recurrence with the same frequency in adult cases, and it is desirable to accumulate and compare more pediatric cases in the future.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440029","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}

{"title":"Tonsillectomy in Immunoglobulin A vasculitis with nephritis: case series.","authors":"Eisuke Kubo, Kotaro Haruhara, Hirokazu Marumoto, Takaya Sasaki, Masahiro Okabe, Shinya Yokote, Akihiro Shimizu, Hiroyuki Ueda, Nobuo Tsuboi, Takashi Yokoo","doi":"10.1007/s13730-025-00975-6","DOIUrl":"https://doi.org/10.1007/s13730-025-00975-6","url":null,"abstract":"<p><p>There is no consensus-based treatment for adult-onset immunoglobulin A vasculitis with nephritis (IgAV nephritis). Tonsillectomy is a treatment option for primary IgA nephropathy, which has similar histopathological features and pathogenesis to IgAV nephritis. The present case series aimed to describe the clinical course of patients with IgAV nephritis who underwent tonsillectomy in our institution. Adult patients with biopsy-proven IgAV nephritis who received tonsillectomy from 2015 to 2022 were systematically reviewed at six hospitals in Japan. Hematuria, proteinuria and slope of the estimated glomerular filtration rate (eGFR) were evaluated before and after tonsillectomy. Patients with IgAV nephritis who underwent tonsillectomy was identified in 12 of 2626 kidney biopsies performed during the study period. The median observation periods before and after tonsillectomy were 20.7 and 48.6 months, respectively. The following drugs were administered concurrent with tonsillectomy: corticosteroids (n = 8), mizoribin (n = 1), and rituximab (n = 1). Three patients were not treated with corticosteroids or immunosuppressants. During post-tonsillectomy observation, 5 patients showed remission of hematuria. Of the 10 patients whose proteinuria was not at a remission level prior to tonsillectomy, 7 showed remission of proteinuria after tonsillectomy. The eGFR slope was attenuated in 9 patients after tonsillectomy relative to before tonsillectomy. This study suggests that some patients may benefit from tonsillectomy in the treatment of IgAV nephritis. The efficacy of tonsillectomy or combination therapy with immunosuppression for IgAV nephritis requires further case series to clarify the clinicopathologic picture of patients associated with a response to tonsillectomy.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440026","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}

{"title":"A case of renal infiltration by Bence Jones protein kappa-producing lymphoplasmacytic lymphoma.","authors":"Kazuhiro Sonomura, Hozue Ehara, Hiroya Adachi, Yusuke Yamane, Eri Kawata, Yasukiyo Mori","doi":"10.1007/s13730-025-00977-4","DOIUrl":"https://doi.org/10.1007/s13730-025-00977-4","url":null,"abstract":"<p><p>Lymphoplasmacytic lymphoma (LPL) is a type of low-grade B-cell lymphoma, with 90-95% of cases associated with Waldenström macroglobulinemia, characterized by the presence of IgM-type M-protein. We report, for the first time, a case of LPL-producing Bence Jones (BJ) protein kappa. The patient was a 78-year-old woman admitted to our department due to general fatigue and proteinuria that had persisted for 2 months. No M-protein was detected by blood immunofixation, but kappa-type BJ protein was detected in the urine. Light microscopy of a kidney biopsy sample revealed infiltration of lymphocytes and plasma cells into the perirenal adipose tissue and renal interstitium. The infiltrating cells exhibited kappa light chain restriction. Bone marrow examination revealed clusters of immature plasmacytoid lymphocytes that were CD20 positive, CD5 negative, and exhibited light chain restriction. Genetic analysis detected a MYD88 mutation, leading to the diagnosis of LPL in the patient. Six months after starting treatment with tirabrutinib, urinary protein levels improved to 0.2 g/gCr. Renal infiltration was identified due to urinary protein, and currently, no extramedullary lesions outside the kidneys are observed. Tirabrutinib has been extremely effective, but careful follow-up is still required.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143406081","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}

{"title":"Edoxaban-induced acute interstitial nephritis.","authors":"Shiko Gen, Ririko Higashi, Natsuki Nagae, Ryuta Kigure, Yuta Kamikubo, Kanako Nobe, Naofumi Ikeda","doi":"10.1007/s13730-025-00974-7","DOIUrl":"https://doi.org/10.1007/s13730-025-00974-7","url":null,"abstract":"<p><p>A 76 year-old man with no specific medical history was diagnosed with atrial fibrillation and was started on edoxaban. Seven days after starting edoxaban, renal dysfunction was observed. He was suspected of having anticoagulant-related nephropathy due to acute kidney injury that occurred after starting edoxaban, and a renal biopsy was performed on day 5 of the illness to confirm the diagnosis. The renal biopsy results showed no red blood cell casts in the renal tubules, and inflammatory cell infiltration of lymphocytes and plasma cells was found in the interstitium, leading to a diagnosis of acute interstitial nephritis. There were no findings suggestive of autoimmune disease, and a drug-induced lymphocyte stimulation test to edoxaban was positive, leading to a diagnosis of edoxaban-induced acute interstitial nephritis. In addition to anticoagulant-related nephropathy, drug-induced interstitial nephritis should be considered as a cause of acute kidney injury after initiation of anticoagulant drugs.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413577","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}

{"title":"Delayed rectum perforation by a peritoneal dialysis catheter in a peritoneal dialysis patient: a case report and literature review.","authors":"Mayu Iwata, Tadashi Uramatsu, Ryosuke Sakamoto, Kenta Torigoe, Ayuko Yamashita, Shinichi Abe, Yuki Ota, Mineaki Kitamura, Atsuhiro Maeda, Yasushi Mochizuki, Kumiko Muta, Ryoichi Imamura, Tomoya Nishino","doi":"10.1007/s13730-025-00971-w","DOIUrl":"https://doi.org/10.1007/s13730-025-00971-w","url":null,"abstract":"<p><p>A 60-year-old woman with diabetic nephropathy was diagnosed with end-stage kidney disease and underwent peritoneal dialysis (PD) (X-3 years). Due to decreased residual renal function, she commenced combined PD and hemodialysis (HD) for 6 h once a week (Jan. X year). Seven months later, she visited her maintenance dialysis facility because of fluid leakage from the pubic region and abdominal pain during the dialysate infusion. Tests of the leakage fluid revealed a high glucose concentration, leading to discontinuation of PD and transfer to HD three times a week. She had been referred to a general hospital in her city, where vesical and uterine fistulas were excluded. Consequently, she was referred to our department to investigate the cause of the leakage. She was diagnosed with peritonitis by a physician and was receiving treatment. The lumen of the PD catheter was brownish, indicating possible contamination, leading us to plan its removal. At the time of catheter removal, laparoscopy and intraoperative lower gastrointestinal endoscopy revealed a rectal perforation at the tip of the PD catheter; this was removed after suturing the damaged area. This was a rare case of delayed perforation of the rectum by a PD catheter, during combined PD and HD therapy.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413575","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}