CEN Case Reports最新文献

{"title":"A case of effective tocilizumab for arthropathy due to dialysis-related amyloidosis.","authors":"Susumu Tsunoda, Yusuke Yoshimura, Yuki Oba, Daisuke Ikuma, Hiroki Mizuno, Masayuki Yamanouchi, Tatsuya Suwabe, Izuru Kitajima, Kei Kono, Kenichi Ohashi, Yoshifumi Ubara, Naoki Sawa","doi":"10.1007/s13730-025-01009-x","DOIUrl":"10.1007/s13730-025-01009-x","url":null,"abstract":"<p><p>A 79-year-old male patient on hemodialysis for 29 years was admitted to the hospital with unexplained fever (C-reactive protein, 16.1 mg/dL), anemia (hemoglobin 7.6 g/dL), and multiple joint swelling that began 7 months earlier. Infection was ruled out, both rheumatoid factor and anti-cyclic citrullinated peptide antibodies were negative. Positron emission tomography-computed tomography showed positive findings in bilateral shoulder joints, palmar region, femoral head area, and cervical spine. Magnetic resonance imaging showed low intensity on T1 and low-normal intensity on T2, especially in the shoulder joint. Musculoskeletal ultrasound revealed hypoechoic material with surrounding fluid collection and Doppler signals indicating blood flow in the shoulder joint, resembling synovitis associated with rheumatoid arthritis. Biopsy of the shoulder joint mass showed positive Congo-red and direct fast scarlet staining, positive β2-microglobulin, and infiltration of CD68-positive macrophages. Given the history of previous carpal tunnel release and cervical destructive spondyloarthropathy, dialysis-related amyloidosis, and amyloid-related arthritis were diagnosed. However, as the case had the feature of rheumatoid arthritis-like synovitis, tocilizumab was initiated, resulting in improved anemia and Clinical Disease Activity Index. We speculate that dialysis-related amyloidosis can present with arthritis involving cytokines, similar to rheumatoid arthritis, and tocilizumab may be effective for the resulting synovitis.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"726-731"},"PeriodicalIF":0.7,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12457238/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144504932","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":"Diagnosing apparent mineralocorticoid excess in a child with hypertension and nephrocalcinosis: from symptoms to genetics.","authors":"Pujitha Vallabhaneni, S K Safikul Hasan, Karalanglin Tiewsoh, Aarchie Gupta, Priyanka Srivastava, Lesa Dawman","doi":"10.1007/s13730-025-01015-z","DOIUrl":"10.1007/s13730-025-01015-z","url":null,"abstract":"<p><p>Apparent mineralocorticoid excess (AME) is a rare genetic disorder caused by reduced activity of the 11β-hydroxysteroid dehydrogenase type 2 (11β-HSD2) enzyme. It is characterized by hypertension, hypokalemia, and low levels of renin and aldosterone. This study presents the case of a 2-year-old female patient who exhibited abdominal distension, hypertension, recurrent hypokalemia with metabolic alkalosis, failure to thrive, polyuria, and features of rickets. Furthermore, the patient presented with nephrocalcinosis and enlarged kidneys with a normal renal Doppler study. The genetic analysis revealed a homozygous mutation in the HSD11B2 gene, confirming the diagnosis of AME. The treatment with amiloride and spironolactone resulted in normalized urine output, stabilized blood pressure, and balanced electrolyte levels. This case emphasizes the importance of considering AME in pediatric patients presenting with unexplained refractory hypertension associated with hypokalemia and nephrocalcinosis, and it underscores the crucial role of genetic testing in diagnosis and management.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"786-792"},"PeriodicalIF":0.7,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12457250/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144728282","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":"Phenotype-dependent heterogeneity of THSD7A expression in gastric cancer tissue in a patient with THSD7A-associated membranous nephropathy.","authors":"Chisato Minezaki, Tatsuhiko Azegami, Akinori Hashiguchi, Takashin Nakayama, Norifumi Yoshimoto, Akihito Hishikawa, Aika Hagiwara, Kaori Hayashi","doi":"10.1007/s13730-025-01006-0","DOIUrl":"10.1007/s13730-025-01006-0","url":null,"abstract":"<p><p>An 88-year-old Japanese man with benign prostatic hyperplasia was presented to our hospital because of proteinuria and generalized edema. He was diagnosed with nephrotic syndrome and underwent a kidney biopsy, which revealed thickening of the capillary wall, spike formation, and subepithelial deposits, leading to histopathological diagnosis of membranous nephropathy. IgG4-dominant deposits were observed in IgG subclass staining, and immunostaining for thrombospondin type 1 domain-containing 7A (THSD7A) demonstrated granular staining along the capillary wall. A malignancy screening was performed, which led to the detection of gastric cancer. Malignancy-associated membranous nephropathy secondary to gastric cancer was suspected, and priority was given to the treatment of gastric cancer. Three months after undergoing radical surgery for gastric cancer, his nephrotic syndrome achieved remission. The histopathological diagnosis of gastric cancer was papillary adenocarcinoma. In the surgical specimen of the gastric cancer, THSD7A was positive in the tumor cells with intestinal phenotype and negative in gastric foveolar phenotype. These findings suggest that the acquisition of the ability to express THSD7A by cancer transformation in multi-step carcinogenesis of gastric cancer may be involved in the development of malignancy-associated membranous nephropathy.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"693-699"},"PeriodicalIF":0.7,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12457271/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144246645","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":"Williams syndrome presenting as infantile hypercalcemia with acute kidney injury: a case report.","authors":"Esther Park, Soon Chul Kim","doi":"10.1007/s13730-025-01010-4","DOIUrl":"10.1007/s13730-025-01010-4","url":null,"abstract":"<p><p>Williams syndrome is an autosomal-dominant multisystem disorder. Infantile hypercalcemia has been documented in 15% of infants with Williams syndrome, and is commonly transient and mild. The need for pamidronate therapy has been reported in a few cases of Williams syndrome with severe hypercalcemia. Few cases of acute kidney injury due to severe hypercalcemia during Williams syndrome have also been recorded. We report the case of an infant with Williams syndrome who presented with acute kidney injury and severe hypercalcemia and was treated with intravenous pamidronate. In addition, we aimed to consider management strategies that maintain renal function and promote growth until adulthood.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"764-767"},"PeriodicalIF":0.7,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12457234/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144583201","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":"Granulomatous interstitial nephritis due to sarcoidosis following SARS-CoV-2 infection: a rare case report.","authors":"Megumi Otani, Kiyotaka Nagahama, Kento Nakamura, Akiko Nagatomo, Suzu Yoshida, Kazuhiro Okai, Ayaka Suzuki, Masato Sasaki, Suguru Hirasawa, Shota Aki, Hiroyuki Tanaka","doi":"10.1007/s13730-025-01008-y","DOIUrl":"10.1007/s13730-025-01008-y","url":null,"abstract":"<p><p>We report the first documented case of renal sarcoidosis developing after SARS-CoV-2 infection. The patient, a 59-year-old woman with no significant medical history, was diagnosed with COVID-19 after experiencing persistent fatigue and insomnia. Approximately three weeks after the infection, she developed a skin rash, prompting a biopsy that revealed epithelioid granulomas. A systemic evaluation using computed tomography identified enlarged bilateral hilar lymph nodes, and a needle aspiration biopsy confirmed the presence of epithelioid granulomas, leading to a diagnosis of sarcoidosis. Nine months after recovering from COVID-19, she was referred to the nephrology department due to progressive renal dysfunction. A renal biopsy demonstrated granulomatous interstitial nephritis with Schaumann bodies, consistent with renal sarcoidosis. Treatment with prednisolone (30 mg/day, 0.5 mg/kg/day) resulted in significant improvement in renal function, suggesting a potential link between SARS-CoV-2 infection and immune dysregulation. This case highlights the importance of monitoring renal function in patients with post-COVID-19 sarcoidosis, even in the absence of disease progression in other organs.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"706-712"},"PeriodicalIF":0.7,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12457235/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144301191","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":"Nephrotic syndrome after switching from irinotecan, S-1, and bevacizumab combination to trifluridine/tipiracil.","authors":"Jun Takami, Yuta Nakano, Eriko Harada, Shota Nakakuma, Ryosuke Kawamoto, Eisaku Ito, Kayoko Matsukawa","doi":"10.1007/s13730-025-01014-0","DOIUrl":"10.1007/s13730-025-01014-0","url":null,"abstract":"<p><p>Trifluridine/tipiracil (FTD/TPI) has shown efficacy in treating advanced colorectal and gastric cancers. Although renal injury due to FTD/TPI is rare, its renal effects have not been well documented. We present the case of a 74-year-old man with metastatic colon cancer who developed nephrotic syndrome with hematuria after transitioning from a regimen of irinotecan, S-1, and bevacizumab to FTD/TPI. Renal biopsy revealed IgA nephropathy with glomerular microangiopathy (GMA). His condition improved with steroid therapy. To our knowledge, this is the first documented case of nephrotic syndrome associated with FTD/TPI. Renal toxicity should be monitored through urinalysis during FTD/TPI treatment.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"768-773"},"PeriodicalIF":0.7,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12457258/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144599551","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":"Efficacy of hemodialysis and importance of tonicity monitoring in mannitol-induced acute kidney injury with hyponatremia.","authors":"Ayaka Soejima, Masatomo Ogata, Ryo Takaki, Takuya Matsuda, Shiika Watanabe, Yugo Shibagaki, Masahiko Yazawa","doi":"10.1007/s13730-025-01017-x","DOIUrl":"10.1007/s13730-025-01017-x","url":null,"abstract":"<p><p>Mannitol is an osmotic diuretic that can induce acute kidney injury (AKI) and hypertonic hyponatremia. Rapid mannitol removal and the avoidance of osmotic demyelination syndrome (ODS) by overcorrecting hyponatremia during dialysis are paramount. We present a case of mannitol-induced AKI and hyponatremia in a man in his 50 s with chronic kidney disease and heart failure who was undergoing chemotherapy for seminoma. After mannitol administration as a part of the chemotherapy protocol for forced diuresis, sudden anuric AKI and subsequent volume expansion developed. An estimated mannitol concentration of 728 mg/dL calculated using the osmolar gap (OG) was treated with hemodialysis (HD). Because of concerns regarding ODS caused by rapid serum sodium (sNa) correction by HD, extracorporeal ultrafiltration was initially considered for volume reduction. However, HD was ultimately chosen for mannitol removal; therefore, instead of the measured sNa, tonicity or corrected sodium (cNa) was monitored to account for transcellular free-water shifts between the intracellular and extracellular compartments. In this case, HD effectively removed mannitol, as reflected by the decreased OG, thereby resolving AKI and hyponatremia. Furthermore, tonicity (or cNa) remained stable throughout treatment, and complications were avoided. Prioritizing tonicity (or cNa) over measured sNa is important when managing hypertonic hyponatremia caused by mannitol intoxication.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"746-750"},"PeriodicalIF":0.7,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12457231/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144526586","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":"Proteinuria and influence on monoclonal antibody excretion: a pembrolizumab case report and literature review.","authors":"T S S Teuntje van Es, B J M Bas Peters, G Gurbey Ocak, E A Elisabeth Kastelijn, S L Sabine Croonen, F C Floris Loeff, M P H Marcel van den Broek","doi":"10.1007/s13730-025-01000-6","DOIUrl":"10.1007/s13730-025-01000-6","url":null,"abstract":"<p><p>Therapeutic monoclonal antibodies (mAbs) have revolutionized the treatment landscape of various diseases, offering targeted therapy options with high specificity. Under normal physiological conditions, their size prevents renal excretion. However, there is limited information about mAbs pharmacokinetics in patients with massive proteinuria, a condition often associated with a nephrotic syndrome. In this case report, we describe a 68-year-old man with non-small-cell lung carcinoma (NSCLC) and a paraneoplastic nephrotic syndrome, who was treated with pembrolizumab 200 mg every 3 weeks. Since there is limited data on pembrolizumab disposition in patients with nephrotic syndrome, we monitored pembrolizumab serum and urine concentrations to ensure adequate systemic exposure. Therapeutic drug monitoring results showed no renal excretion of pembrolizumab and therapeutic drug exposure. Treatment of the NSCLC led to an amelioration of the paraneoplastic nephrotic syndrome. We conducted a literature review on the various types of proteinuria and their effects on the excretion of mAbs. Existing literature shows that increased renal clearance of monoclonal antibodies in patients with glomerular proteinuria is possible, but it probably depends on the amount of glomerular proteinuria. Based on literature findings and our own, we suggest that in cases of severe glomerular proteinuria, like nephrotic range proteinuria, the likelihood of renal loss of monoclonal antibodies is higher than in other cases.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"757-763"},"PeriodicalIF":0.7,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12457255/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144552441","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 kidney disease related to Staphylococcus epidermidis infection soon after insertion of a central venous catheter.","authors":"Kei Kono, Naoki Sawa, Daisuke Ikuma, Yuki Oba, Hiroki Mizuno, Akinari Sekine, Noriko Inoue, Kiho Tanaka, Masayuki Yamanouchi, Eiko Hasegawa, Tatsuya Suwabe, Takeshi Fujii, Yutaka Takazawa, Kenichi Ohashi, Yutaka Yamaguchi, Takehiko Wada, Yoshifumi Ubara","doi":"10.1007/s13730-025-01029-7","DOIUrl":"10.1007/s13730-025-01029-7","url":null,"abstract":"<p><p>Central venous catheter (CVC)-related kidney disease has been well documented in cases of long-term catheter use, typically manifesting as membranoproliferative glomerulonephritis with immunoglobulin (Ig)M and C3 deposits after months to years of catheterization. We report an exceptional case of rapidly progressive kidney disease that developed just 7 days after CVC insertion in a 74-year-old man who underwent laparoscopic rectal cancer surgery. The patient presented with high fever (39 ℃) and acute kidney injury, with serum creatinine rapidly escalating from a baseline of 1.1-2.7 mg/dL. Laboratory evaluation revealed marked hypocomplementemia (C3 35 mg/dL, C4 5 mg/dL), significant proteinuria (1.5 g/day), and hematuria. Catheter tip culture isolated multidrug-resistant Staphylococcus epidermidis. Kidney biopsy demonstrated distinctive pathological findings characterized by endocapillary proliferative glomerulonephritis with massive fibrin deposition, confirmed by phosphotungstic acid-hematoxylin (PTAH) staining. Immunofluorescence revealed granular deposits of IgG and C3 along the capillary wall, notably without IgA or IgM deposits. Electron microscopy identified characteristic fibrin deposits measuring 7-8 nm in thickness, predominantly in the subendothelial region. DnaJ homolog subfamily B member 9 (DNAJB9) staining was negative, definitively excluding fibrillary glomerulonephritis (FGN). Following catheter removal and targeted antibiotic therapy without steroid treatment, the patient's renal function showed remarkable improvement, with creatinine decreasing to 1.8 mg/dL at discharge and further improving to 1.0 mg/dL after 1 year. This case demonstrates that S. epidermidis can induce severe glomerular lesions through fibrin deposition within an extraordinarily short timeframe, contrasting dramatically with the immune complex-mediated injury typically observed in long-term CVC use.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145063583","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":"Good response with nivolumab and cabozantinib combination therapy in patients with metastatic collecting duct carcinoma with high expression of PD-L1, c-MET, and AXL.","authors":"Tatsuya Umemoto, Masanori Hasegawa, Jun Naruse, Tatsuo Kano, Nobuyuki Nakajima, Masahiro Nitta, Yoshiaki Kawamura, Hiroshi Kajiwara, Sunao Shoji","doi":"10.1007/s13730-025-01027-9","DOIUrl":"https://doi.org/10.1007/s13730-025-01027-9","url":null,"abstract":"<p><p>Collecting duct carcinoma (CDC) is a rare subtype of renal cell carcinoma with a poor prognosis. Moreover, despite various chemotherapeutic strategies and administration of several tyrosine kinase inhibitors for metastatic CDC, the outcomes remain unfavorable, with no established treatment. Herein, we report the cases of two patients with CDC who exhibited a good response to nivolumab and cabozantinib combination therapy. Both patients were diagnosed with CDC via a needle biopsy of the renal tumor, revealing high expression levels of programmed death ligand 1 (PD-L1), c-MET, and AXL. After 10 and 12 courses of combination therapy for Cases 1 and 2, respectively, significant response was observed against the primary and metastatic lesions. Subsequently, the patients underwent laparoscopic nephrectomy. To the best of our knowledge, this is the first report documenting the favorable therapeutic response of nivolumab and cabozantinib combination therapy against metastatic CDC in patients with high expression of the corresponding molecular targets. These findings may have a strong implication in the selection of first-line systemic therapies for metastatic CDC.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145022827","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}