CEN Case Reports最新文献

{"title":"Development of IgA nephropathy following risankizumab therapy for psoriatic arthritis: a case report.","authors":"Motohiro Yagasaki, Koshi Yamada, Takeo Koshida, Miyuki Takagi, Harumi Saeki, Kei Ogiwara, Takashi Kobayashi, Masao Kihara, Tomohito Gohda, Yusuke Suzuki","doi":"10.1007/s13730-026-01147-w","DOIUrl":"10.1007/s13730-026-01147-w","url":null,"abstract":"<p><p>IgA nephropathy (IgAN) has been reported in association with several biologic agents; however, renal events related to selective interleukin (IL)-23p19 inhibition remain poorly characterized. We report a case of biopsy-proven IgAN that became clinically apparent after initiation of risankizumab for psoriatic arthritis. A 37-year-old man with psoriatic arthritis achieved marked improvement in joint and skin manifestations after starting risankizumab in October 2022. Thereafter, kidney function gradually declined, with the estimated glomerular filtration rate decreasing from approximately 90-100 to 69.9 mL/min/1.73 m² by August 2023, accompanied by proteinuria and microscopic hematuria. Despite supportive therapy with azilsartan and dapagliflozin, kidney function continued to worsen and proteinuria persisted, prompting a percutaneous kidney biopsy in February 2025. Light microscopy showed mesangial and focal endocapillary hypercellularity without crescents. Immunofluorescence demonstrated granular mesangial deposits of IgA and C3, and KM-55 staining was positive, indicating glomerular deposition of galactose-deficient IgA1; electron microscopy revealed para-mesangial electron-dense deposits. The biopsy was consistent with primary IgAN (Oxford classification M0E1S0T0C0). We administered steroid pulse therapy according to the Pozzi protocol (methylprednisolone 500 mg/day for 3 consecutive days), achieving remission of urinary abnormalities with modest improvement in kidney function, while risankizumab was continued. This case highlights the need for periodic urinalysis and kidney function monitoring during IL-23-targeted therapy and suggests that IgAN-directed treatment may be effective even when continuation of the biologic agent is clinically necessary.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13287197/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148293458","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":"Poststreptococcal acute glomerulonephritis superimposed on chronic kidney disease due to dysplastic kidney in an 11-year-old boy.","authors":"Satoshi Okada, Koichi Kamei, Kentaro Nishi, Hideaki Kitakado, Masao Ogura, Takako Yoshioka, Kentaro Ogata, Takashi Oda","doi":"10.1007/s13730-026-01140-3","DOIUrl":"10.1007/s13730-026-01140-3","url":null,"abstract":"<p><p>We report an 11-year-old boy with chronic kidney disease (CKD) stage 3 due to right multicystic dysplastic kidney and contralateral dysplasia who developed poststreptococcal acute glomerulonephritis (PSAGN) that rapidly progressed to kidney failure despite intensive immunosuppressive treatment. He presented with oliguria, macrohematuria, heavy proteinuria, hypocomplementemia, and an elevated antistreptolysin O titer. Initial kidney biopsy revealed diffuse endocapillary proliferation with prominent C3 deposition, subepithelial humps, and positive staining for nephritis-associated plasmin receptor (NAPlr), consistent with PSAGN. Kidney dysfunction persisted despite the administration of methylprednisolone pulse therapy, prednisolone, cyclosporine, and mycophenolate mofetil. A second biopsy at 4 months revealed focal segmental glomerulosclerosis, diffuse foot process effacement, and negative staining for NAPlr, suggesting transition from a glomerular inflammatory process to a hyperfiltration-driven structural injury. Comprehensive genetic analysis did not identify any pathogenic variants associated with bilateral dysplastic kidney. Peritoneal dialysis was initiated on day 182, and living donor kidney transplantation was performed 10 months after nephritis onset, with favorable early outcomes. This case highlights that PSAGN superimposed on CKD with reduced nephron mass may result in rapid kidney function deterioration despite aggressive treatment.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13287538/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148293454","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":"Does pregnancy modify disease activity in C3 glomerulopathy?","authors":"Yuya Yamaguchi, Yusuke Okabayashi, Masahiro Okabe, Nobuo Tsuboi, Takashi Yokoo","doi":"10.1007/s13730-026-01135-0","DOIUrl":"10.1007/s13730-026-01135-0","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13287173/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148293433","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":"Membranous nephropathy secondary to very early-onset inflammatory bowel disease in a 3-year-old boy: case report.","authors":"Shuhei Aoyama, Tomohiko Yamamura, Tomoko Horinouchi, Yuka Kimura, Yuta Inoki, Nana Sakakibara, China Nagano, Shingo Ishimori, Yasuyuki Kameoka, Keisuke Kajihara, Harunori Miyauchi, Yuna Ku, Haruka Miyazaki, Norihiro Okamoto, Norishige Yoshikawa, Kandai Nozu","doi":"10.1007/s13730-026-01116-3","DOIUrl":"10.1007/s13730-026-01116-3","url":null,"abstract":"<p><p>Membranous nephropathy (MN) is uncommon in children overall, but secondary MN is relatively common in younger children. Inflammatory bowel disease (IBD) can be complicated by kidney disease, but IBD complicated by MN is rarely reported. A 3-year-old boy diagnosed with very early onset IBD (VEO-IBD) a year earlier was incidentally found to have proteinuria via urine screening system. Laboratory tests revealed nephrotic syndrome with microscopic hematuria and signs of ongoing inflammation associated with IBD activity. Kidney biopsy showed mesangial cell proliferation and dense subepithelial immune complex deposits, along with negative PLA2R staining, which supported a diagnosis of secondary MN. Oral steroid therapy was initiated for its potential benefit for both nephrotic syndrome and the underlying VEO-IBD. As a result, four months later, proteinuria and hematuria had resolved, and kidney function remained stable. This is the youngest reported patient with MN associated with IBD. Routine urinalysis is suggested by this case to be useful for detecting kidney complications of IBD, including MN. Moreover, steroid therapy is likely to be useful for MN secondary to IBD. Our report suggests that potential kidney complications should also be considered in patients with VEO-IBD.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13280327/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148276018","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":"Refractory hyponatremia after traumatic brain injury unmasks adrenal insufficiency in a patient with remote steroid use.","authors":"Toshikazu Ozeki, Seiya Ito, Yuna Muto, Fumika Nagase, Keita Iwasaki, Yuki Ito, Hiroki Ikai, Mari Yamamoto, Waka Yokoyama-Kokuryo, Yoshiro Fujita","doi":"10.1007/s13730-026-01143-0","DOIUrl":"10.1007/s13730-026-01143-0","url":null,"abstract":"<p><p>A man in his early 60 s developed persistent hyponatremia following lumbar spine surgery complicated by traumatic brain injury with subarachnoid hemorrhage. Initial evaluation demonstrated hypotonic hyponatremia with inappropriately concentrated urine, consistent with the syndrome of inappropriate antidiuretic hormone secretio. Standard therapy including hypertonic saline and oral sodium supplementation resulted in only transient improvement. Given a history of secondary adrenal insufficiency due to long-term topical corticosteroid use, adrenal function was reassessed. An adrenocorticotropic hormone stimulation test confirmed secondary adrenal insufficiency. Initiation of hydrocortisone therapy led to sustained normalization of serum sodium. This case highlights the diagnostic difficulty of post-TBI hyponatremia and underscores the importance of considering adrenal insufficiency, even years after apparent recovery from steroid-induced hypothalamic-pituitary-adrenal axis suppression.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13272691/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148257464","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":"Heavily ossified renal mass as the initial presentation of primary renal osteosarcoma: a case report.","authors":"Michael Kumar Sah, Monika Singh, Densia Rons Thampi, Ankur Mittal, Rahul Dev","doi":"10.1007/s13730-026-01141-2","DOIUrl":"10.1007/s13730-026-01141-2","url":null,"abstract":"<p><p>Primary renal osteosarcoma (PRO) is a rare extraskeletal malignancy, accounting for less than 1% of soft-tissue sarcomas and fewer than 50 reported cases to date. We present the case of a 47-year-old male who presented with six months of progressive right flank pain and an abdominal lump. Initial imaging with plain radiography and contrast-enhanced computed tomography urography revealed a large, heterogeneously enhancing right renal mass characterised by extensive, confluent, dense intratumoral calcifications with a bone-like appearance, mediastinal displacement of adjacent viscera, calcified retroperitoneal lymph nodes, and peritoneal deposits suggestive of metastatic disease. The radiologic differential diagnosis included renal cell carcinoma with dystrophic calcification, calcified renal tuberculosis, and stone disease. Diagnostic laparoscopy with biopsy revealed pleomorphic spindle-to-epithelioid cells in disorganised sheets with characteristic lace-like eosinophilic osteoid. Immunohistochemical analysis confirmed the diagnosis by demonstrating SATB2 positivity and negativity for MDM2, cytokeratin, PAX8, and CD34, with a high Ki-67 proliferation index, supporting a mesenchymal origin rather than sarcomatoid carcinoma. In view of peritoneal and lymph node involvement indicating metastatic disease, the patient was initiated on platinum-based combination chemotherapy with doxorubicin and cisplatin, completing three cycles with good tolerance. This case highlights the clinical, radiological, and pathological features essential for diagnosing PRO, a uniformly aggressive malignancy that typically presents at an advanced stage with poor prognosis. Definitive diagnosis requires comprehensive histopathological examination combined with targeted immunohistochemical panels to exclude alternative etiologies, including sarcomatoid renal cell carcinoma and renal tuberculosis. Multimodal management with radical surgical resection and platinum-based chemotherapy represents the current standard of care, though long-term outcomes remain limited.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13265438/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148249554","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 metformin-associated encephalopathy in a patient undergoing maintenance hemodialysis.","authors":"Fumihiro Fukata, Seina Iwami, Kensei Yasuda, Mana Masunaga, Motonobu Yasukawa","doi":"10.1007/s13730-026-01146-x","DOIUrl":"10.1007/s13730-026-01146-x","url":null,"abstract":"<p><p>Metformin is a major drug for the treatment of type 2 diabetes mellitus; however, its use is not recommended in dialysis patients due to the risk of lactic acidosis. Consequently, there are very few reports of metformin-associated lactic acidosis (MALA) or metformin-associated encephalopathy in dialysis patients. We report a case of a 67-year-old man undergoing maintenance hemodialysis who was admitted to our hospital with impaired consciousness. Blood tests revealed marked hypoglycemia (blood glucose level of 18 mg/dL) in addition to severe metabolic acidosis and lactic acidosis (pH 6.989, HCO3- 5.8 mmol/L, and lactate 10.7 mmol/L). The patient had a history of metformin use, and was diagnosed with MALA. We immediately initiated glucose correction and emergency hemodialysis. On the same day, magnetic resonance imaging (MRI) showed symmetrical swelling in the bilateral basal ganglia, with low signal intensity on T1-weighted images and high signal intensity on T2-weighted and fluid-attenuated inversion recovery images, leading to a diagnosis of metformin-associated encephalopathy. The patient's condition improved with frequent dialysis sessions, and subsequent MRI showed improvement in the lesions. The patient was discharged on the 17th day, after confirming recovery to the pre-admission level. The present case reinforces the recommendation that metformin should not be prescribed to patients undergoing dialysis.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13265439/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148249571","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":"Successful treatment of non-occlusive mesenteric ischemia in a patient undergoing combined therapy with peritoneal dialysis and hemodialysis: a case report.","authors":"Takahiko Hoshino, Takahiro Uchida, Minami Koizumi, Tadasu Kojima, Noriko Yoshikawa, Muneharu Yamada, Hiroyuki Higuchi, Toru Sano, Shigeyuki Kawachi, Takashi Oda","doi":"10.1007/s13730-026-01139-w","DOIUrl":"10.1007/s13730-026-01139-w","url":null,"abstract":"<p><p>A Japanese man in his 60 s with end-stage kidney disease due to diabetic nephropathy, who initially underwent peritoneal dialysis (PD) and was transferred to combined PD and hemodialysis (HD) therapy 4 years prior, was admitted to our hospital for surgical treatment of lumbar spinal canal stenosis. His rehabilitation continued well after successful surgery; however, he suddenly presented with abdominal pain and cloudy peritoneal dialysate, along with an increased white blood cell count in the peritoneal dialysate effluent, suggesting PD-associated peritonitis. Intraperitoneal administration of antibiotics was ineffective, and contrast-enhanced computed tomography showed areas of poor contrast enhancement and pneumatosis intestinalis in the colon. Emergency laparotomy revealed non-occlusive mesenteric ischemia (NOMI), leading to intestinal resection with ileostomy creation. He made good progress after the surgery and was eventually discharged while receiving thrice-weekly HD. NOMI is a well-known life-threatening condition, and its outcomes in patients receiving dialysis therapy are reportedly unfavorable. Furthermore, to our knowledge, there have been no reported cases of NOMI in patients receiving combined PD and HD therapy. Further accumulation of similar cases is needed to clarify appropriate approaches for treating NOMI in these patients.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 4","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13265440/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148249593","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 deep intronic IFT172 variant causing pseudoexon inclusion identified by whole-genome sequencing in nephronophthisis.","authors":"Perry Martel Sy, Minato Baba, Kaori Fujiwara, Natsumi Yamamura, Kazumi Kawato, Eriko Nishi, Shuhei Aoyama, Yuka Kimura, Yuta Inoki, China Nagano, Nana Sakakibara, Tomoko Horinouchi, Tomohiko Yamamura, Shingo Ishimori, Naoya Morisada, Kandai Nozu","doi":"10.1007/s13730-026-01132-3","DOIUrl":"10.1007/s13730-026-01132-3","url":null,"abstract":"<p><p>Nephronophthisis is an autosomal recessive ciliopathy and a major genetic cause of end-stage kidney disease in children and young adults. Although next-generation sequencing panels have improved diagnostic yield, some patients remain genetically unresolved, partly due to deep intronic variants that disrupt pre-mRNA splicing and are not captured by exon-focused approaches. We report a 13-year-old boy who presented with advanced kidney dysfunction, small renal cysts, and kidney histopathology consistent with nephronophthisis. Targeted gene panel sequencing failed to identify causative pathogenic variants beyond a missense variant of uncertain significance. Whole-genome sequencing subsequently revealed compound heterozygous variants in IFT172 (NM_015662.3): a missense variant (c.4696C > T, p.Arg1566Cys) and a deep intronic variant (c.4915-94A > G). In silico analysis predicted activation of cryptic splice sites leading to inclusion of an 86-bp pseudoexon, which was confirmed by a minigene splicing assay. These findings established a molecular diagnosis of IFT172-related nephronophthisis. To our knowledge, this is the first report demonstrating pseudoexon inclusion in IFT172, thereby expanding its mutational spectrum. Our case underscores the importance of evaluating deep intronic regions using whole-genome sequencing and functional validation in genetically unresolved nephronophthisis.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13221558/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148052946","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":"Trimethoprim/sulfamethoxazole-triggered drug-induced hypersensitivity syndrome in an HLA B*13:01-positive kidney transplant recipient: a case report with implications for HLA-severe cutaneous adverse reaction associations in transplant care.","authors":"Mitsuru Tomizawa, Shunta Hori, Kuniaki Inoue, Tatsuo Yoneda, Nobuya Akioka, Yuki Nishimura, Hideo Asada, Yasushi Nakai, Makito Miyake, Kiyohide Fujimoto","doi":"10.1007/s13730-026-01134-1","DOIUrl":"10.1007/s13730-026-01134-1","url":null,"abstract":"<p><p>Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DIHS/DRESS) is a severe cutaneous adverse reaction (SCAR) with a reported mortality rate of approximately 2-10%. DIHS/DRESS typically develops 2-8 weeks after exposure to an offending drug. An important focus of contemporary SCAR research is the growing evidence that specific human leukocyte antigen (HLA) alleles confer a markedly increased risk of drug-specific hypersensitivity reactions. We report a case of a kidney transplant recipient who developed DIHS/DRESS after prolonged trimethoprim/sulfamethoxazole (TMP/SMX) prophylaxis and carried the HLA-B13:01 allele. HLA-B13:01 is a strong genetic risk factor for TMP/SMX-induced DIHS/DRESS, particularly in Southeast Asian populations. Herein, we highlight the potential clinical relevance of pre-transplant HLA typing in predicting SCAR risk in transplant recipients. TMP/SMX-associated DIHS/DRESS may be under-recognized in transplant settings, where awareness of HLA-associated risk remains limited despite robust evidence from non-transplant populations. Transplant clinicians should be aware that DIHS/DRESS can occur outside the typical latency period, especially during immunosuppressant tapering, highlighting the need to integrate pharmacogenomic risk assessments into transplant care.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13221509/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148052944","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}