CEN Case Reports最新文献_第4页

Successfully treated C3 glomerulopathy in which protein and genetic analyses were useful for diagnosis. 成功治疗 C3 肾小球病，蛋白质和基因分析有助于诊断。

IF 1

CEN Case Reports Pub Date : 2024-09-12 DOI: 10.1007/s13730-024-00928-5

Motoko Kanzaki, Motoyasu Kurahashi, Kentaro Watanabe, Mana Nishikawa, Kosuke Fukuoka, Noriaki Shimada, Masashi Mizuno, Kenichiro Asano

{"title":"Successfully treated C3 glomerulopathy in which protein and genetic analyses were useful for diagnosis.","authors":"Motoko Kanzaki, Motoyasu Kurahashi, Kentaro Watanabe, Mana Nishikawa, Kosuke Fukuoka, Noriaki Shimada, Masashi Mizuno, Kenichiro Asano","doi":"10.1007/s13730-024-00928-5","DOIUrl":"https://doi.org/10.1007/s13730-024-00928-5","url":null,"abstract":"C3 glomerulopathy is a rare disease that results in nephritis due to complement dysregulation and is characterized by C3 deposition in the glomerulus. Dysregulation of the alternative pathway underlies the pathogenesis, but activation of the terminal pathway is also common. The disease is often caused by acquired rather than genetic factors, i.e., autoantibodies against C3 or C5 converting enzyme (convertase) and other complement-related proteins. We report a case of C3 glomerulopathy diagnosed by renal biopsy that responded well to corticosteroids and went into complete remission within two months. Analysis of complements and complement-related proteins revealed a low level of C3 and a high level of soluble terminal pathway protein complex (sC5b-9). Under genetic analysis about complement-related genes, no pathogenic variant was observed. Based on these findings, we diagnosed this patient with C3 glomerulopathy with autoantibodies. Corticosteroids had a marked effect, which also supports this speculation. Analyses of complements and complement-related proteins, and genetic variants may be useful in understanding the pathogenesis of C3 glomerulopathy and in selecting treatment options.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280882","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Effective management of necrotizing crescentic glomerulonephritis using an aggressive combination therapy including avacopan in a patient double-seropositive for anti-GBM antibodies and ANCA: a case report. 病例报告：对一名抗 GBM 抗体和 ANCA 双血清阳性患者采用包括阿伐戈班在内的积极综合疗法有效治疗坏死性新月体肾小球肾炎。

IF 1

CEN Case Reports Pub Date : 2024-09-10 DOI: 10.1007/s13730-024-00929-4

Rina Tanaka, Takumi Toishi, Reiji Masaki, Hideaki Aihara, Sumie Sakamoto, Mari Ikeda, Tomohiko Inoue, Atsuro Kawaji, Masatoshi Matsunami, Junko Fukuda, Mamiko Ohara, Hiroshi Kuji, Daisuke Ichikawa, Tomo Suzuki

{"title":"Effective management of necrotizing crescentic glomerulonephritis using an aggressive combination therapy including avacopan in a patient double-seropositive for anti-GBM antibodies and ANCA: a case report.","authors":"Rina Tanaka, Takumi Toishi, Reiji Masaki, Hideaki Aihara, Sumie Sakamoto, Mari Ikeda, Tomohiko Inoue, Atsuro Kawaji, Masatoshi Matsunami, Junko Fukuda, Mamiko Ohara, Hiroshi Kuji, Daisuke Ichikawa, Tomo Suzuki","doi":"10.1007/s13730-024-00929-4","DOIUrl":"https://doi.org/10.1007/s13730-024-00929-4","url":null,"abstract":"The prognosis of anti-glomerular basement membrane (anti-GBM) nephritis, often accompanied by the presence of antineutrophil cytoplasmic antibodies (ANCA), is poor, and even with aggressive therapeutic approaches, kidney replacement therapy (KRT) is typically required. Here, we present a case of necrotizing crescentic glomerulonephritis in a patient double-seropositive for anti-GBM antibodies and ANCA who successfully achieved dialysis independence following aggressive treatment, including avacopan. The patient was a 77-year-old woman with rapidly progressive glomerulonephritis and double seropositivity for myeloperoxidase-ANCA and anti-GBM antibodies. A kidney biopsy revealed diffuse cellular crescents with necrosis and immunoglobin (Ig)G1 and IgG3 positivity on immunofluorescence staining, leading to a histological diagnosis of anti-glomerular basement membrane nephritis. Our treatment approach involved a novel combination of glucocorticoids, rituximab, low-dose cyclophosphamide, and plasma exchange complemented by avacopan. Temporary hemodialysis was required, and the patient successfully discontinued dialysis after 12 sessions despite a poor histological prognosis. This case underscores the significance of considering aggressive therapeutic strategies, including avacopan, for severe anti-GBM nephritis, even in the absence of lung involvement, to avert the need for KRT.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Recurrent acute kidney injury with Fanconi syndrome related to red yeast rice supplement. 急性肾损伤并发范可尼综合征的复发性与红麴补充剂有关。

IF 1

CEN Case Reports Pub Date : 2024-08-24 DOI: 10.1007/s13730-024-00926-7

Yuri Katayama, Reina Miyazaki, Yasuhito Takahashi, Tetsuya Kawamura, Nobuo Tsuboi, Takashi Yokoo

{"title":"Recurrent acute kidney injury with Fanconi syndrome related to red yeast rice supplement.","authors":"Yuri Katayama, Reina Miyazaki, Yasuhito Takahashi, Tetsuya Kawamura, Nobuo Tsuboi, Takashi Yokoo","doi":"10.1007/s13730-024-00926-7","DOIUrl":"https://doi.org/10.1007/s13730-024-00926-7","url":null,"abstract":"A 51-year-old woman was admitted to our hospital for acute kidney injury (AKI) with an elevated serum creatinine level of 5.07 mg/dL and Fanconi syndrome. The patient was discharged after partial recovery of kidney dysfunction with conservative treatment but was readmitted approximately three months later due to a recurrence of AKI with Fanconi syndrome. A kidney biopsy revealed findings consistent with acute tubular necrosis and localized tubulointerstitial nephritis, with no specific vascular or glomerular lesions. The patient's medical history revealed that prior to both AKI episodes, the patient had been taking \"Red Yeast Cholestehelp\", a lipid-lowering supplement for a period of time. Her kidney dysfunction and Fanconi syndrome improved with the discontinuation of the supplement and correction with oral medications. In Japan, a series of similar health hazards related to the red yeast rice supplement has been reported, but the causative toxin and its causal relationship with AKI have not been established. The present case provides firm evidence that clinically supports this relationship.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142046422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

A case of ANCA-negative pauci-immune crescentic glomerulonephritis with lung adenocarcinoma with mediastinal involvement successfully treated by corticosteroid and radiation therapy. 一例 ANCA 阴性、伴有肺腺癌且纵隔受累的新月体肾小球肾炎病例，通过皮质类固醇和放射治疗获得成功。

IF 1

CEN Case Reports Pub Date : 2024-08-20 DOI: 10.1007/s13730-024-00925-8

Yuna Onozawa, Masahiro Koizumi, Yosuke Nakagawa, Go Ogura, Masayuki Oki, Takehiko Wada, Masafumi Fukagawa

{"title":"A case of ANCA-negative pauci-immune crescentic glomerulonephritis with lung adenocarcinoma with mediastinal involvement successfully treated by corticosteroid and radiation therapy.","authors":"Yuna Onozawa, Masahiro Koizumi, Yosuke Nakagawa, Go Ogura, Masayuki Oki, Takehiko Wada, Masafumi Fukagawa","doi":"10.1007/s13730-024-00925-8","DOIUrl":"https://doi.org/10.1007/s13730-024-00925-8","url":null,"abstract":"Pauci-immune crescentic glomerulonephritis (PICGN) is one of the pathologies causing rapidly progressive glomerulonephritis, often associated with anti-neutrophil cytoplasmic antibody (ANCA); however, in 10-30% of cases, ANCAs are negative. While a relatively large number of cases of ANCA-positive PICGN complicated with malignancy have been previously reported, the number of cases of ANCA-negative PICGN with malignancy is limited. The prognosis for such cases was poor, and many patients died within a relatively short period. Here, we report the case of ANCA-negative PICGN complicated with malignancy successfully treated by corticosteroid and radiation therapy. A 63-year-old Japanese man was admitted to our hospital due to spiking fevers in the previous 3 months. Based on the findings of imaging and pathological tests, he was diagnosed with locally advanced lung adenocarcinoma with mediastinal involvement. After admission, his renal function rapidly deteriorated, and urinalysis showed heavy proteinuria. In serological tests, serology for autoantibodies, including ANCAs, was negative. The kidney biopsy revealed PICGN with prominent endocapillary proliferation. We administered corticosteroid therapy for glomerulonephritis and subsequent radiation therapy for lung carcinoma, both of which were effective. He has been alive without progression of malignancy or kidney disease for 5 years after discharge. In patients with malignancy presenting with acute deterioration of kidney function, although infrequent, one of the conceivable pathological conditions to consider is ANCA-negative PICGN associated with malignancy. In such cases, even with negative antibodies such as ANCA, pathological examination is warranted, and a combination of anti-tumor therapy and immunosuppressive therapy is expected to be effective.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142003721","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Parathyroid carcinoma in a dialysis patient definitively diagnosed after parathyroidectomy for uncontrolled secondary hyperparathyroidism. 一名因继发性甲状旁腺功能亢进症未得到控制而接受甲状旁腺切除术的透析患者被明确诊断为甲状旁腺癌。

IF 1

CEN Case Reports Pub Date : 2024-08-19 DOI: 10.1007/s13730-024-00924-9

Ryoko Tatsumi, Yusuke Tomita, Shinya Takiguchi, Saeko Uehara, Michio Nakamura

{"title":"Parathyroid carcinoma in a dialysis patient definitively diagnosed after parathyroidectomy for uncontrolled secondary hyperparathyroidism.","authors":"Ryoko Tatsumi, Yusuke Tomita, Shinya Takiguchi, Saeko Uehara, Michio Nakamura","doi":"10.1007/s13730-024-00924-9","DOIUrl":"https://doi.org/10.1007/s13730-024-00924-9","url":null,"abstract":"Secondary hyperparathyroidism (SHPT) is a well-known complication in chronic kidney disease patients undergoing maintenance dialysis. In 2006, the Japanese Society for Dialysis Therapy recommended parathyroidectomy (PTx) for medically resistant SHPT cases, resulting in an increase in the performance of PTx. However, after calcimimetics were added to the treatment options in 2008, the number of cases requiring PTx has decreased. Presented here is the case of a dialysis patient with SHPT under medical treatment with calcimimetics, who was normocalcemic but showed persistently high levels of parathyroid hormone (PTH), suggesting the possibility of parathyroid carcinoma. Parathyroid carcinoma is a very rare endocrine malignancy characterized by hypercalcemia and increased PTH level. With appropriately performed PTx at the proper time, the definitive diagnosis was made and the patient has not developed any recurrences or metastases to date. In cases of SHPT refractory to medical therapy, the possibility of parathyroid carcinoma should be considered as an alternative. We report a case in which parathyroid carcinoma was diagnosed after appropriate conversion from medical therapy to PTx with reference to ultrasonographic images.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141999447","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Lipoprotein glomerulopathy with a novel apolipoprotein E variant, APOE Kanto (Asp 151dup). 新型载脂蛋白 E 变异体 APOE Kanto（Asp 151dup）引起的脂蛋白肾小球病。

IF 1

CEN Case Reports Pub Date : 2024-08-14 DOI: 10.1007/s13730-024-00920-z

Akio Yokochi, Akira Matsunaga, Keiko Kanemoto, Naoto Tominaga, Susumu Uda, Takao Saito

引用次数: 0

Clinical manifestations and renal pathology of ethylene glycol. 乙二醇的临床表现和肾脏病理学。

IF 1

CEN Case Reports Pub Date : 2024-08-12 DOI: 10.1007/s13730-024-00921-y

Shogo Taira, Shiori Tamayose, Tasuku Kikumura, Morikuni Nishihira

{"title":"Clinical manifestations and renal pathology of ethylene glycol.","authors":"Shogo Taira, Shiori Tamayose, Tasuku Kikumura, Morikuni Nishihira","doi":"10.1007/s13730-024-00921-y","DOIUrl":"https://doi.org/10.1007/s13730-024-00921-y","url":null,"abstract":"Ethylene glycol (EG) poisoning is a critical medical emergency often associated with suicide attempts in adults. EG is metabolized by alcohol dehydrogenase, leading to the formation of toxic metabolites that cause metabolic acidosis, renal failure, hypocalcemia, aciduria, and disorders of the central nervous and cardiovascular systems. Calcium oxalate, a metabolite of EG, contributes to acute tubular necrosis. Despite limited reports on human renal pathology, we present a case detailing renal pathology following EG ingestion. A 44-year-old male, admitted due to loss of consciousness, had ingested a lethal dose of EG. Blood tests indicated metabolic acidosis, while urinary examination revealed calcium oxalate crystals. Continuous renal replacement therapy corrected the acidosis; however, nephrogenic diabetes insipidus subsequently developed. A renal biopsy on day 31 revealed calcium oxalate crystal deposition and tubulointerstitial damage. Notably, various stages of crystal deposition, adherence, and degradation were observed. This case underscores the importance of considering EG poisoning in cases of unexplained metabolic acidosis and renal dysfunction, with renal biopsy serving as a valuable diagnostic tool. Understanding the renal effects of EG is essential for timely intervention and effective management of poisoning cases.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141970697","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Correction to: Fanconi syndrome with karyomegalic interstitial nephritis after ifosfamide treatment for osteosarcoma: a case report. Correction to：骨肉瘤伊佛酰胺治疗后伴巨核细胞间质性肾炎的范可尼综合征：病例报告。

IF 1

CEN Case Reports Pub Date : 2024-08-12 DOI: 10.1007/s13730-024-00916-9

Yohei Kita, Sayuri Shirai, Teppei Koyama, Ryuichiro Makinouchi, Shinji Machida, Katsuomi Matsui, Junki Koike, Naohiko Imai

引用次数: 0

Identification of CUBN variants in triplets with a 20-year history of proteinuria. 在有 20 年蛋白尿病史的三胞胎中鉴定 CUBN 变体。

IF 1

CEN Case Reports Pub Date : 2024-08-05 DOI: 10.1007/s13730-024-00919-6

Natsumi Yamamura-Miyazaki, Nana Sakakibara, Kandai Nozu, Yuko Shima, Kenichi Satomura, Satoko Yamamoto, Minato Baba, Kaori Fujiwara, Katsusuke Yamamoto, Toshimi Michigami

{"title":"Identification of CUBN variants in triplets with a 20-year history of proteinuria.","authors":"Natsumi Yamamura-Miyazaki, Nana Sakakibara, Kandai Nozu, Yuko Shima, Kenichi Satomura, Satoko Yamamoto, Minato Baba, Kaori Fujiwara, Katsusuke Yamamoto, Toshimi Michigami","doi":"10.1007/s13730-024-00919-6","DOIUrl":"https://doi.org/10.1007/s13730-024-00919-6","url":null,"abstract":"CUBN encodes cubilin, which plays a role in the reabsorption of glomerular-filtered albumin in the proximal tubule. CUBN-related proteinuria was recently established as a new disease concept and may be present in proteinuric cases that were previously undiagnosed either genetically or histologically. We herein report a case of triplets diagnosed with chronic benign proteinuria due to CUBN variants 20 years after its onset. The proband, the first child of triplets, tested positive for urinary protein several times during the neonatal period. A urine screening test at 3 years old was positive. Proteinuria persisted for years within a non-nephrotic range. Kidney biopsy at 8 years old revealed minor glomerular abnormalities. Renin-angiotensin system inhibitors were started for albumin-based proteinuria but were ineffective. Since the two other triplets had similar courses, analyses of the NPHS1/2 and WT1 genes were performed but revealed no abnormalities. The triplets transitioned to adult care at 15 years old. CUBN-related proteinuria was reported in 2020; therefore, we re-analyzed their DNA samples and identified compound heterozygous variants in CUBN in all three triplets. The molecular diagnosis of CUBN-related proteinuria will save patients from unnecessary treatments and concerns about renal prognosis.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141888563","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Recurrent localized fever caused by cryoglobulinemic vasculitis following hemodialysis: A case report. 血液透析后冷球蛋白血症血管炎引起的反复局部发热：病例报告。

IF 1

CEN Case Reports Pub Date : 2024-08-05 DOI: 10.1007/s13730-024-00923-w

Mitsuharu Kojima, Maki Shibata, Saori Tomita, Reina Ueda, Rina Kasai, Eriko Yamamoto, Ayako Ban, Satoshi Suzuki, Shoichi Maruyama

{"title":"Recurrent localized fever caused by cryoglobulinemic vasculitis following hemodialysis: A case report.","authors":"Mitsuharu Kojima, Maki Shibata, Saori Tomita, Reina Ueda, Rina Kasai, Eriko Yamamoto, Ayako Ban, Satoshi Suzuki, Shoichi Maruyama","doi":"10.1007/s13730-024-00923-w","DOIUrl":"https://doi.org/10.1007/s13730-024-00923-w","url":null,"abstract":"Post-dialysis fever is commonly reported in patients undergoing hemodialysis (HD). However, it is often challenging to identify the underlying cause owing to the wide variety of potential factors that can lead to fever. In this case, a 66-year-old Japanese man experienced recurrent fever after HD treatment. Initially, antibiotics were prescribed to treat pneumonia, but it was later discovered that the pneumonia was an alveolar hemorrhage caused by cryoglobulinemic vasculitis. It is believed that cryoglobulin was sensitized by cold exposure owing to the dialysate temperature, which resulted in fever being experienced only after HD. Although treatment for vasculitis required prednisolone and rituximab, simple plasma exchange and a dialysate temperature of 37.5 °C dramatically suppressed the occurrence of post-dialysis fever. Cryoglobulinemia should be considered as a potential cause of fever, as it may be a common occurrence in patients undergoing HD and could be overlooked as a possible cause of localized fever following HD treatment.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141888565","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0