CEN Case Reports最新文献

{"title":"Pelvic inflammatory pseudotumor with calcium polystyrene sulfonate crystal deposition in a kidney transplant recipient.","authors":"Tomoaki Hirata, Yu Mihara, Ryo Kurose, Natsuko Okuno, Noriyoshi Ota, Akiyuki Iwamoto, Yuta Inoue, Tetsuro Kusaba, Masayoshi Okumi, Osamu Ukimura, Keiichi Tamagaki","doi":"10.1007/s13730-025-01033-x","DOIUrl":"https://doi.org/10.1007/s13730-025-01033-x","url":null,"abstract":"<p><p>A 63-year-old woman underwent living-donor kidney transplantation three years earlier for end-stage renal disease due to diabetic nephropathy, with her younger sister as the donor. She was prescribed calcium polystyrene sulfonate for the management of hyperkalemia, which had been discontinued two years earlier. At this time, she developed recurrent abdominal and urinary symptoms, which were managed empirically with antibiotics. Four months prior to admission, she noted mucoid vaginal discharge. Pelvic MRI revealed a pelvic mass. She underwent an open biopsy and pathological examination revealed an appendiceal abscess with deposition of calcium polystyrene sulfonate crystals and an associated inflammatory pseudotumor. The lesion resolved with antibiotic therapy and drainage. Calcium polystyrene sulfonate is known to cause gastrointestinal complications; however, it may be associated with the development of inflammatory pseudotumors in rare cases. Surgical resection is generally considered the first-line treatment for inflammatory pseudotumors; however, treatment strategies for unresectable cases have not been established. Herein, we report a rare case of an inflammatory pseudotumor with calcium polystyrene sulfonate crystal deposition that was successfully managed with antibiotics.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144999770","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":"Light chain proximal tubulopathy in Sjögren syndrome: the critical role of tubular function tests in revealing a hidden threat.","authors":"Haruki Mae, Go Kanzaki, Yumeka Inamura, Nanae Matsuo, Shiko Honma, Kazuhito Suzuki, Nobuo Tsuboi, Kensuke Joh, Shingo Yano, Takashi Yokoo","doi":"10.1007/s13730-025-01034-w","DOIUrl":"https://doi.org/10.1007/s13730-025-01034-w","url":null,"abstract":"<p><p>A 65-year-old female patient with Sjögren syndrome (SJS) and tubulointerstitial nephritis (TIN) confirmed by kidney biopsy results 7 years previously presented to our department with progressive kidney function deterioration. Laboratory findings revealed increased serum creatinine level accompanied with deterioration of tubular function. Although she already had signs of proximal tubular dysfunction due to TIN from 7 years before, deterioration of the proximal tubule related parameters was particularly prominent. Owing to the substantial increase in the IgG level, monoclonal gammopathy was considered. Subsequent immunoelectrophoresis of the serum and urine revealed the presence of monoclonal IgG-κ. A repeat kidney biopsy revealed crystalline inclusions in the proximal tubules that tested positive for κ light chain. Along with findings from the bone marrow biopsy, this led to the diagnosis of light chain proximal tubulopathy (LCPT) secondary to multiple myeloma. Chemotherapy was initiated promptly which resulted in stabilization of renal function and improvement of tubular function. Vigilant monitoring of the tubular function is essential for patients with SJS. Deterioration of proximal tubule parameters, especially when accompanied by increased IgG levels, should prompt the investigation of monoclonal gammopathy and the consideration of kidney biopsy.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144944454","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":"Orthostatic hypotension after kidney transplantation in a patient with diabetic kidney disease: an underrecognized and therapeutic challenge.","authors":"Masafumi Sakai, Masatomo Ogata, Jun Tanabe, Yuko Sakurai, Kazunobu Shinoda, Yugo Shibagaki, Masahiko Yazawa","doi":"10.1007/s13730-025-01030-0","DOIUrl":"https://doi.org/10.1007/s13730-025-01030-0","url":null,"abstract":"<p><p>Orthostatic hypotension (OH) is characterized by an excessive drop in blood pressure upon standing, leading to impaired quality of life, increased fall risk, and potential cardiovascular complications. It is frequently associated with autonomic dysfunction in patients with neurodegenerative diseases, diabetes mellitus, and aging. Despite its potential impact, OH may be underrecognized in kidney transplant (KT) recipients, particularly in the early post-transplant period, when diuresis-induced hypovolemia may serve as a precipitating factor. We present a case of severe OH in a woman in her 50s who underwent living-donor KT for diabetic kidney disease. Pre-transplant therapy with a glucagon-like peptide-1 (GLP-1) receptor agonist led to significant weight loss, followed by post-transplant diuresis, ultimately resulting in volume depletion. One month postoperatively, the patient developed persistent dizziness and fatigue. Orthostatic testing confirmed neurogenic OH, and assessment of cardiac autonomic function using the coefficient of variation of R-R intervals (CVRR) revealed significant autonomic dysfunction. Despite initial treatment with midodrine, symptoms persisted. Given concurrent mild hyperkalemia, fludrocortisone was administered. Unfortunately, no improvement in OH was observed during the observation period. This case underscores the importance of considering OH in KT recipients, particularly in the early post-transplant period when diuresis may exacerbate autonomic dysfunction. OH would be more common than recognized in routine clinical practice and is potentially underdiagnosed. Given the increasing number of elderly and diabetic KT recipients, heightened awareness and appropriate diagnostic evaluation of OH are essential for timely intervention. Fludrocortisone should also be considered in cases where volume depletion coexists with hyperkalemia, although its effectiveness may be limited, highlighting the therapeutic challenge in managing OH after KT.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144944449","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 kidney transplantation using eltrombopag in a patient with MYH9-related disease.","authors":"Aya Kato, Yoko Shirai, Shoichiro Kanda, Shinji Kunishima, Tomokazu Shimizu, Hideki Ishida, Motoshi Hattori, Kenichiro Miura","doi":"10.1007/s13730-025-01032-y","DOIUrl":"https://doi.org/10.1007/s13730-025-01032-y","url":null,"abstract":"<p><p>MYH9-related disease is an autosomal dominant genetic disease characterized by congenital macrothrombocytopenia, sensorineural hearing loss, and progressive kidney failure. Severe cases require kidney replacement therapy in the patient's second decade of life, and thrombocytopenia constitutes a major risk factor for hemorrhagic complications during dialysis initiation or kidney transplantation. Serious bleeding complications have been reported to occur in the perioperative period despite adequate platelet transfusions. Additionally, platelet transfusion may induce the production of anti-platelet antibodies and donor-specific anti-human leukocyte antigen antibodies. We report a case of a 19-year-old woman with MYH9-related disease due to an R702C missense variant in the motor domain of the MYH9 gene who used prophylactic eltrombopag, a thrombopoietin receptor agonist, from 6 weeks before kidney transplantation. Before transplantation, her platelet count increased from 10 × 10³/μL to 156 × 10³/μL. No major bleeding complications occurred after living donor kidney transplantation. Although perioperative eltrombopag use has been reported in other surgeries, this is the first successful case of living donor kidney transplantation in which perioperative eltrombopag administration allowed platelet transfusions to be avoided. Eltrombopag may serve as a valuable alternative in kidney transplant recipients with MYH9-related disease by avoiding platelet transfusion and reducing the risk of severe bleeding complications.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144944406","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":"Membranous nephropathy as a segmental pattern with solitary immunoglobulin A deposition: a case report.","authors":"Shinya Yokote, Saeko Hatanaka, Akihiro Shimizu, Masahiro Okabe, Kotaro Haruhara, Takaya Sasaki, Hiroyuki Ueda, Kensuke Joh, Nobuo Tsuboi, Takashi Yokoo","doi":"10.1007/s13730-025-01028-8","DOIUrl":"https://doi.org/10.1007/s13730-025-01028-8","url":null,"abstract":"<p><p>A 67-year-old man with a history of hypertension and dyslipidemia presented with edema and heavy proteinuria. Light microscopic analysis of kidney biopsy revealed a diffuse segmental membranous feature. Immunofluorescence stain was segmentally positive for IgA, galactose-deficient IgA1, both κ and λ light chains, and C3 along the glomerular capillary walls, but negative for IgG, IgM, or C1q. Electron microscopy showed subepithelial and intramembranous electron-dense deposits (EDD) in the segmental glomerular capillary walls, along with foot process effacement in the corresponding areas. No EDD was observed in the mesangial or para-mesangial areas. The patient's histopathology revealed membranous nephropathy with a solitary IgA deposition. No clinical findings suggested a secondary cause of membranous nephropathy. Combination therapy with corticosteroids and cyclosporine resulted in proteinuria remission. To our knowledge, this is the first reported case of membranous nephropathy with a segmental pattern associated with solitary IgA and galactose-deficient IgA1 deposition. Further case reports and studies are required to elucidate the pathogenesis of membranous nephropathy, which shows these unique histopathological features.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144944441","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":"Microscopic polyangiitis with histopathologic evolution in serial renal biopsies during treatment of idiopathic pulmonary fibrosis.","authors":"Yuya Yamaguchi, Takeshi Tosaki, Takaya Sasaki, Daisuke Nakashima, Yu Honda, Shinya Yokote, Nobuo Tsuboi, Takashi Yokoo","doi":"10.1007/s13730-025-01023-z","DOIUrl":"https://doi.org/10.1007/s13730-025-01023-z","url":null,"abstract":"<p><p>Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease associated with inflammation implicated in the development of vasculitis, specifically microscopic polyangiitis (MPA). Herein, we report a case of MPA complicated by IPF. A woman in her 70s with a history of IPF treated with nintedanib presented with a serum creatinine level of 2.22 mg/dL, microscopic hematuria, and a serum myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) titer level of 78.5 IU/mL. The initial renal biopsy revealed diffuse tubulointerstitial nephritis without glomerular crescent formation; therefore, prednisolone was initiated. However, the serum MPO-ANCA titer level increased to 91.1 IU/mL after tapering the prednisolone dose. A second renal biopsy revealed pauci-immune necrotizing glomerulonephritis with crescents, confirming MPA. Treatment was adjusted to include a resumed steroid regimen and combination therapy with rituximab and avacopan, resulting in stable kidney function. In conclusion, this case underscores the importance of monitoring serum ANCA titer levels as a surrogate marker for subclinical vasculitis in patients with IPF. The sequential occurrence of tubulointerstitial nephritis followed by crescentic glomerulonephritis suggests a potential progression pattern in MPA, warranting careful clinical and histopathological evaluations.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144834099","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 endoluminal balloon dilation for difficult removal of tunneled dialysis catheters with long-term indwelling for 8 years.","authors":"Keisuke Onishi, Eisuke Nakamura, Takafumi Shiga, Aiko Shiraishi, Yasushi Kunisho, Tadashi Sofue, Takahisa Noma, Yoichi Yamashita, Tetsuo Minamino","doi":"10.1007/s13730-025-01026-w","DOIUrl":"https://doi.org/10.1007/s13730-025-01026-w","url":null,"abstract":"<p><p>This case report highlights the effectiveness of endoluminal balloon dilation for the difficult removal of tunneled dialysis catheters. Recently, the use of tunneled catheters for vascular access has increased, and cases of difficult removal due to prolonged indwelling have been reported. We describe the case of a 66-year-old man on maintenance dialysis with a tunneled dialysis catheter that had been placed in the right internal jugular vein 8 years prior, who presented with post-dialysis fever. The procedure to remove the catheter due to a catheter-related infection using the traditional method was unsuccessful. Catheter adhesion due to the fibrin sheath was suspected. The catheter was removed using endoluminal balloon dilation. The patient had no postoperative fever or other complications. Another tunneled catheter was subsequently inserted into the same internal jugular vein. This case illustrates that prolonged catheter placement can lead to intravascular adhesions due to the formation of a fibrin sheath. Endoluminal balloon dilation is a minimally invasive treatment option that should be considered before surgical removal.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144834097","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":"Hereditary distal renal tubular acidosis with chronic granulomatous disease: a rare coincidence.","authors":"Keerthana Srinivas, Vernika Tyagi, Akanksha Mahajan, Mukta Mantan","doi":"10.1007/s13730-025-01025-x","DOIUrl":"https://doi.org/10.1007/s13730-025-01025-x","url":null,"abstract":"<p><p>The primary defect in distal renal tubular acidosis (dRTA) is impaired H⁺ ion secretion in the distal nephron, resulting in a normal anion gap metabolic acidosis. The solute carrier family 4-member 1 (SLC4A1) gene encodes the erythroid and renal anion exchanger 1 (AE1) protein for chloride-bicarbonate exchange. Mutations in the gene can result in hereditary dRTA, red blood cell membrane defect, and hemolytic anemia. Chronic granulomatous disease (CGD) is a rare primary immunodeficiency syndrome caused by NADPH oxidase deficiency, leading to impaired neutrophil and phagocyte function, and thus predisposing the patient to multiple bacterial infections. Melioidosis is a rare infection caused by Burkholderia pseudomallei and is often linked to CGD. Here we present an interesting case of an 8-year-old girl with melioidosis secondary to CGD. Also, she had nephrocalcinosis, metabolic acidosis, hypercalciuria, and anemia. The simultaneous presence of distal RTA (Pathogenic homozygous SLC4A1 mutation on whole exome sequencing) and CGD has not been reported previously and reiterates the importance of detailed clinical evaluation combined with investigations for the long-term management of such complex cases.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144834098","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 anticoagulation-related nephropathy complicated by IgA nephropathy that developed following the long-term use of anticoagulants.","authors":"Yuko Oyama, Yoichi Iwafuchi, Yumi Ito, Naofumi Imai, Ichiei Narita","doi":"10.1007/s13730-025-01020-2","DOIUrl":"https://doi.org/10.1007/s13730-025-01020-2","url":null,"abstract":"<p><p>Anticoagulant-related nephropathy (ARN) is a critical disease with clinical manifestations including acute kidney injury (AKI), which develops months after drug administration commences. The risk of ARN associated with direct oral anticoagulant agents (DOACs) is lower than that for warfarin, but there have been reports of ARN induced by DOACs other than dabigatran, such as edoxaban. We describe a patient with ARN caused by edoxaban, after the long-term use of warfarin and a switch from dabigatran, who had underlying IgA nephropathy with normal renal function. A 75-year-old man presented with AKI, with hematuria and proteinuria. He had never previously experienced urinary abnormalities or renal impairment. He was started on warfarin for atrial fibrillation 17 years previously, then switched to dabigatran for 11 years, and then edoxaban for 3 years. Three months before admission, he developed melena and hematuria. On the basis of his medical history and renal pathologic findings, he was diagnosed with ARN complicated by IgA nephropathy, with interstitial lesions and marked arteriosclerosis. After discontinuing edoxaban and methylprednisolone pulse therapy followed by oral prednisolone, his proteinuria diminished, and his renal dysfunction and hematuria were ameliorated. ARN can develop at any time in association with any DOAC, even in patients with normal renal function, and may be associated with anticoagulant overdose and subclinical IgA nephropathy. Therefore, careful monitoring of renal function and urinalysis is necessary for the prevention and early recognition of ARN, and dose reduction or a change in anticoagulant should occur when anticoagulant overdose or ARN is suspected.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144798298","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":"Sustained eGFR improvement after dapagliflozin in a patient with antiretroviral therapy-related chronic kidney disease.","authors":"Hirotaka Arae, Shinichi Hoshino, Nao Moromi, Kouichi Tokumine, Kentaro Kohagura","doi":"10.1007/s13730-025-01024-y","DOIUrl":"https://doi.org/10.1007/s13730-025-01024-y","url":null,"abstract":"<p><p>Chronic kidney disease (CKD) is an emerging concern in the aging population of people living with HIV, especially in those with history of nephrotoxic antiretroviral therapy. Sodium-glucose cotransporter 2 (SGLT2) inhibitors have shown renoprotective effects in various clinical settings, but their utility in patients with antiretroviral-induced kidney impairment remains unexplored. We report the case of a 59-year-old man with well-controlled HIV infection who developed progressive decline in estimated glomerular filtration rate (eGFR, mL/min/1.73 m²) over 15 years of taking antiretroviral therapy (ΔeGFR - 2.3 mL/min/1.73 m² per year), including 8 years of tenofovir disoproxil fumarate (TDF) intake. After discontinuation of TDF and its successor drug, the eGFR stabilized at approximately 52 for approximately 4 years. The SGLT2 inhibitor dapagliflozin was started to prevent renal decline. Subsequently, the eGFR increased to 57.3 after 3 months and 60.2 after 6 months, then was maintained at approximately 57 thereafter. After dapagliflozin initiation, the overall eGFR slope was + 1.2 mL/min/1.73 m² per year. To the best of our knowledge, this was the first report demonstrating improvement in eGFR slope after SGLT2 inhibitor use in a patient with antiretroviral-associated renal impairment. This highlighted the potential role of SGLT2 inhibitors in mitigating CKD progression in people living with HIV.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":0.7,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144803612","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}