CEN Case Reports最新文献

{"title":"Multilocular cystic nephroma in an adult: a diagnostic quandary.","authors":"Ritu Verma, Jyoti Verma, Neelima Gupta","doi":"10.1007/s13730-024-00852-8","DOIUrl":"10.1007/s13730-024-00852-8","url":null,"abstract":"<p><p>Multilocular cystic nephroma (MLCN) is an unusual, benign slow-growing renal cystic neoplasm which mimics other cystic renal lesions and has such clinical, radiological, and morphological features that causes diagnostic dilemma. MLCN lies in the spectrum of mixed epithelial and stromal tumor (MEST) family of kidney. According to World Health Organization (WHO 2016 classification), MEST encompasses spectrum of tumors ranging from predominantly cystic tumors, adult cystic nephroma (ACN) to tumors that are variably solid (MEST), thus creating diagnostic dilemma. Moreover, it has several benign and malignant differentials due to its several overlapping histomorphological features which when not cautiously dealt with may result in misdiagnosing it as malignant lesion. We hereby present a case of a woman in late twenties who presented with left flank swelling and pain since 6 months which was misdiagnosed as renal cell carcinoma on radiology which turned out to be ACN on histology and further verified on immunohistochemistry.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"356-365"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139982486","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 non-lupus full-house nephropathy diagnosed by kidney biopsy but observed IgA nephropathy on second biopsy.","authors":"Yoichi Iwafuchi, Tetsuo Morioka, Yuko Oyama, Ichiei Narita","doi":"10.1007/s13730-024-00860-8","DOIUrl":"10.1007/s13730-024-00860-8","url":null,"abstract":"<p><p>We describe a case of full-house nephropathy without any underlying disease, including systemic lupus erythematosus. A 40-year-old woman was referred to our hospital with mild proteinuria and microscopic hematuria. The patient was diagnosed with immune complex-mediated glomerulonephritis with a predominant mesangioproliferative pattern based on renal histopathological results using full-house immunofluorescence staining. She showed no clinical criteria for the diagnosis of systemic lupus erythematosus, except for kidney disorders, and tested negative for antinuclear antibodies throughout her clinical course. However, in the second kidney biopsy, no C1q or C4 were detected in the immunofluorescence study, suggesting an immunoglobulin A nephropathy-like pattern. The patient responded favorably to corticosteroid treatment. We found a heterozygous CFHR3-CFHR1 deletion. The association between full-house nephropathy and CFHR3-CFHR1 deletion is unknown, but its influence on the histological pattern in our case is suspected. This indicates the diversity in the pathogenesis of non-lupus full-house nephropathy and warrants further investigation.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"373-380"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442713/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139989440","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":"Effective calcineurin inhibitor treatment in adult-onset steroid-resistant nephrotic syndrome with a novel splice donor site variant of TRPC6: a case report.","authors":"Tomoki Nagasaka, Kiyotaka Uchiyama, Eriko Yoshida Hama, Daiki Kojima, Kenji Kaneko, Norifumi Yoshimoto, Itaru Yasuda, Mamiko Yamada, Fuyuki Miya, Hisato Suzuki, Takaya Tajima, Shintaro Yamaguchi, Kaori Hayashi, Takeshi Kanda, Akinori Hashiguchi, Kenjiro Kosaki, Hiroshi Itoh","doi":"10.1007/s13730-024-00935-6","DOIUrl":"https://doi.org/10.1007/s13730-024-00935-6","url":null,"abstract":"<p><p>Transient receptor potential canonical 6 (TRPC6) variants, which were initially detected in adult-onset familial focal segmental glomerulosclerosis (FSGS), were also identified in pediatric-onset one. Here, we present a patient with adult-onset steroid-resistant nephrotic syndrome (SRNS) who harbored a likely pathogenic TRPC6 variant and partially responded to calcineurin inhibitors (CNIs). A 44-year-old woman with stable rheumatoid arthritis, systemic lupus erythematosus, and Sjögren's syndrome was presented with nephrotic syndrome. Her renal biopsy results showed minor glomerular abnormalities. Upon admission, she was treated with steroids for around 4 weeks, but it was ineffective. After 1-2 weeks of cyclosporine A (CyA) administration, urine output increased, renal function improved without a decrease in proteinuria, and she was discharged. Her renal function was maintained for 2 months, but after a CyA dose reduction, she was again admitted to the hospital due to relapsing edema, decreased urine output, and worsening renal function. CyA was replaced by tacrolimus (TAC). A second renal biopsy showed nearly the same findings as the first except for tubulointerstitial lesions. After 1-2 weeks of TAC administration, urine output increased, and renal function improved. However, urinary protein levels did not decrease as before. After discharge, a whole exome analysis revealed a heterozygous splice donor site variant NM_004621.6;c.2644 + 1G > A in TRPC6. Genetic testing identified a novel splice donor site variant of TRPC6 in a patient with adult-onset SRNS, which prevented unnecessary steroid continuation. The safety and efficacy of CNI in TRPC6 glomerulopathy must be evaluated in future larger studies with longer follow-up.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342437","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 treatment of severe renal failure caused by malignant hypertension using a combination of renin-angiotensin-aldosterone system inhibitors: a case report.","authors":"Wataru Harada, Yujiro Maeoka, Akira Takahashi, Mahoko Yoshida, Yosuke Osaki, Naoki Ishiuchi, Kensuke Sasaki, Takao Masaki","doi":"10.1007/s13730-024-00934-7","DOIUrl":"https://doi.org/10.1007/s13730-024-00934-7","url":null,"abstract":"<p><p>Marked activation of the renin-angiotensin-aldosterone system (RAAS) plays an important role in malignant hypertension (MHT) by worsening hypertension and renal function. The rates of readmission for severe hypertension and cardiovascular disease in such emergencies are high, suggesting that suppression of the RAAS may be inadequate during the acute phase in some cases. This report presents a case of MHT complicated with renal insufficiency (creatinine 3.93 mg/dL) and massive proteinuria, in which antihypertensive therapy, including an angiotensin receptor blocker, aliskiren, and spironolactone, normalized blood pressure (BP) and preserved renal function. Plasma renin activity was extremely high (131.9 ng/mL/h) on admission but normalized within almost 2 weeks. Although aliskiren and spironolactone were discontinued before discharge, BP was well controlled and renal function was further improved (creatinine 1.14 mg/dL) at follow-up 24 months later. This case of renal failure induced by MHT was successfully treated with a combination of RAAS inhibitors during the acute phase. The controlled BP and improved renal function in this patient suggest that adequate suppression of the RAAS cascade during the acute phase is potentially effective in terms of breaking the vicious cycle of MHT with hyperreninemia.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342438","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":"Multiple relapses of sigmoid volvulus in a patient on automated peritoneal dialysis: the role of an elongated sigmoid colon and peritoneal dialysis as potential predisposing factors.","authors":"Keitaro Uehara, Shunsuke Yamada, Yusuke Mizuuchi, Takanari Kitazono, Toshiaki Nakano","doi":"10.1007/s13730-024-00930-x","DOIUrl":"https://doi.org/10.1007/s13730-024-00930-x","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280881","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":"MPO-ANCA-positive rapidly progressive glomerulonephritis after COVID-19 vaccination during treatment of plaque psoriasis with bimekizumab.","authors":"Takuya Sugiura, Tomohito Doke, Akihito Tanaka, Yuka Sato, Kayaho Maeda, Kazuhiro Furuhashi, Noritoshi Kato, Tomoki Kosugi, Shoichi Maruyama","doi":"10.1007/s13730-024-00927-6","DOIUrl":"https://doi.org/10.1007/s13730-024-00927-6","url":null,"abstract":"<p><p>A 75-year-old man presented with MPO-ANCA-positive rapidly progressive glomerulonephritis after COVID-19 vaccination during the treatment of plaque psoriasis vulgaris with bimekizumab. Bimekizumab, an anti-IL17 monoclonal antibody, was regularly administered to control the activity of plaque psoriasis. After receiving the sixth COVID-19 vaccine, his kidney function rapidly declined over the course of weeks. Urinalysis showed microscopic hematuria and proteinuria with deformed red blood cells and granular cast. The immunology test was positive for MPO-ANCA. The patient was clinically diagnosed with MPO-ANCA-associated glomerulonephritis. As the patient lost his appetite and developed lower extremity edema with low eGFR (< 15 ml/min/1.73m²) on admission day, hemodialysis induction was initiated along with methylprednisolone pulse, followed by oral prednisolone. The kidney function and urine volume were improved in response to immunosuppressive therapy, and withdrawal from hemodialysis was considered. However, the patient developed a catheter infection due to methicillin-sensitive Staphylococcus aureus 2 weeks after the initial prednisolone treatment, causing a decline in kidney function. Antibiotics treatment for the catheter infection was effective, but kidney function remained low, resulting in dependence on regular hemodialysis. COVID-19 vaccination provides significant improvement in overall prognosis; however, there have been reports of kidney function decline and exacerbation of hematuria in patients with IgA nephropathy following vaccination. The incidence of MPO-ANCA-associated glomerulonephritis after COVID-19 vaccination was rare. Data accumulation is warranted to understand the risk factors for secondary MPO-ANCA glomerulonephritis after COVID-19 vaccination. Regular monitoring of urinalysis and kidney function after COVID-19 vaccination is recommended in patients with psoriasis vulgaris treated with IL17 monoclonal antibodies.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280880","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":"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":"<p><p>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.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"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}

{"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":"<p><p>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.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"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}

{"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":"<p><p>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.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"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}

{"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":"<p><p>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.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"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}