CEN Case Reports最新文献

{"title":"IgA nephropathy with rheumatoid arthritis related to adalimumab.","authors":"Büşra Tüfekçi, Rıza Can Kardaş, Betül Öğüt, Abdulsamet Erden","doi":"10.1007/s13730-025-01078-y","DOIUrl":"10.1007/s13730-025-01078-y","url":null,"abstract":"<p><p>The co-occurrence of rheumatoid arthritis (RA) and IgA nephropathy (IgAN) is rare, presenting diagnostic and therapeutic challenges, particularly regarding potential associations with therapies such as TNF inhibitors. We report the case of a 44-year-old man diagnosed with seronegative RA following evaluation for polyarthritis. His prior treatments included rituximab and etanercept, although specific details were unavailable. Adalimumab therapy, initiated in 2014, effectively controlled his RA symptoms for five years. However, in 2019, he developed progressive proteinuria (1 g/day), persistent microscopic hematuria, and low C3/C4 levels. A kidney biopsy revealed IgAN (Oxford classification M1, E0, S1, T1, C0). Suspecting a temporal association with adalimumab, the medication was discontinued, resulting in a significant decrease in proteinuria (to 0.1 g/day) and normalization of serum creatinine. Subsequent treatment with tofacitinib provided inadequate RA control. In 2021, certolizumab was initiated, leading to sustained improvement in RA symptoms (DAS28-CRP 2.6) and continued remission of IgAN (proteinuria 0.2 g/day). This case highlights the potential association between adalimumab use and the development of IgAN in patients with RA and underscores the importance of vigilant renal monitoring during TNF inhibitor therapy.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13129176/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147763671","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":"Carbon dioxide contrast angiography-and computed tomography-guided percutaneous embolization of the renal allograft artery in a patient with graft intolerance syndrome and iodinated contrast allergy: a case report.","authors":"Takahiro Shinzato, Akira Imaizumi, Shigeo Hara, Kojiro Nagai, Jiaowei Guo, Cong Xia, Takao Okawa, Kenta Ito, Masaaki Murakami, Ken Matsuo, Satoshi Tanaka, Kiyoshi Mori","doi":"10.1007/s13730-026-01125-2","DOIUrl":"10.1007/s13730-026-01125-2","url":null,"abstract":"<p><p>Graft intolerance syndrome (GIS) is a well-recognized complication after kidney allograft failure, characterized by fever, graft pain, and systemic inflammation. Management options include continued immunosuppression, graft nephrectomy, and renal artery embolization, each associated with specific risks. We report a case of GIS successfully treated with percutaneous renal allograft artery embolization using carbon dioxide angiography in a patient with iodinated contrast allergy. A 66-year-old woman with autosomal dominant polycystic kidney disease underwent living-donor kidney transplantation and later developed chronic allograft dysfunction, eventually resuming hemodialysis. Following immunosuppression tapering, she presented with recurrent fever and pain over the renal allograft. Despite broad-spectrum antibiotic therapy, her symptoms persisted and imaging showed no abscess formation. Repeated blood cultures were negative. Allograft biopsy revealed extensive tissue necrosis with interstitial inflammation and vascular thrombosis. Multiple donor-specific anti-human leukocyte antigen antibodies with high mean fluorescence intensity were detected, suggesting ongoing alloimmune sensitization. Based on the clinical course and investigations, GIS was diagnosed. Given the high surgical risk and contrast allergy, percutaneous embolization of the renal allograft artery was performed using a combination of carbon dioxide angiography and CT guidance. Clinical symptoms improved promptly after the procedure, allowing discontinuation of calcineurin inhibitor therapy and steroid tapering, with no recurrence during follow-up. This case highlights carbon dioxide contrast angiography- and computed tomography-guided renal artery embolization as a safe and effective minimally invasive treatment option for GIS, particularly in patients unsuitable for iodinated contrast media or surgical nephrectomy.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13128999/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147763651","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":"Elevated hemoglobin levels associated with severe sleep-disordered breathing in hemodialysis patients not receiving erythropoiesis-stimulating agents: a case series.","authors":"Hiroshi Matsumoto, Seizo Murai","doi":"10.1007/s13730-026-01124-3","DOIUrl":"10.1007/s13730-026-01124-3","url":null,"abstract":"<p><p>Elevated hemoglobin levels in hemodialysis patients not receiving erythropoiesis-stimulating agents (ESA) are uncommon and may suggest underlying hypoxic conditions. However, the clinical significance of sleep-disordered breathing (SDB) in this context remains underrecognized. We report three non-obese male patients undergoing maintenance hemodialysis without ESA or hypoxia-inducible factor prolyl hydroxylase inhibitor therapy who developed elevated hemoglobin levels. None of the patients exhibited typical symptoms of SDB. All were diagnosed with severe SDB based on sleep studies and were treated with continuous positive airway pressure (CPAP). CPAP adherence was assessed using device-recorded parameters, including average usage time, residual apnea–hypopnea index, and 95th percentile leak over a 3-month period. The degree of hemoglobin reduction appeared to correlate with CPAP usage time. Notably, elevated hemoglobin levels were observed despite relatively low ferritin levels in all cases. Hypoxia-inducible erythropoiesis and altered iron metabolism may explain these findings. In contrast to obstructive sleep apnea, where inflammation-associated hepcidin elevation may limit erythropoiesis, our cases—characterized by non-obesity, relative youth, and suspected central sleep apnea—may have involved less inflammation, allowing hypoxia-induced suppression of hepcidin and enhanced iron utilization. Elevated hemoglobin levels in hemodialysis patients not receiving ESA may be associated with unrecognized SDB, even in the absence of typical symptoms. The combination of elevated hemoglobin and relatively low ferritin may serve as a clinical clue to underlying SDB in this population.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13129149/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147763734","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":"Acute kidney injury with interstitial hemorrhage in renal heavy chain amyloidosis: a clinicopathologic case report.","authors":"Nozomi Mori, Kayaho Maeda, Rie Ito, Yusuke Handa, Hibiki Tagawa, Suzuho Onagi, Fuyuki Kametani, Masahide Yazaki, Tomohiko Isaki, Asuka Kropp, Yusuke Kakizaki, Michiko Yamazaki, Tomoharu Watanabe","doi":"10.1007/s13730-026-01117-2","DOIUrl":"10.1007/s13730-026-01117-2","url":null,"abstract":"<p><p>Heavy chain (AH) amyloidosis is a rare subtype of immunoglobulin-derived amyloidosis, comprising less than 1% of all systemic cases. While kidney involvement is frequently observed in systemic amyloidosis, presentations dominated by acute kidney injury (AKI) with glomerular bleeding and interstitial hemorrhage are uncommon. We report the case of a Japanese man in his 50s who developed AKI requiring dialysis. He had a history of proteinuria noted during previous health checkups but no known underlying diseases. Renal biopsy revealed amorphous mesangial and capillary wall deposits with weak periodic acid-Schiff and negative periodic acid-methenamine silver staining, and positivity for Congo red and direct fast scarlet. Electron microscopy showed non-branching fibrils, and laser microdissection followed by liquid chromatography-tandem mass spectrometry confirmed a diagnosis of AH amyloidosis. Of note, the biopsy also revealed numerous intratubular red blood cells and patchy interstitial hemorrhage in the absence of crescents or necrotizing lesions. The patient was initially treated with corticosteroids under the presumption of rapidly progressive glomerulonephritis. After histologic diagnosis, clone-directed daratumumab-based chemotherapy was introduced. Renal function gradually recovered, and dialysis was discontinued within 3 weeks of admission. This case highlights a rare but important phenotype of AH amyloidosis characterized by disruption of the glomerular capillaries and interstitial hemorrhage leading to reversible AKI. Early biopsy and appropriate therapeutic intervention may allow renal function to recover, even in severe presentations.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13125667/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147763595","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":"Correction: Mixed cryoglobulinaemia vasculitis secondary to marginal zone lymphoma in a patient with end‑stage renal failure on haemodialysis.","authors":"Craig Peter Coorey, Amirhossein Aarabi, Karthik Kumar","doi":"10.1007/s13730-026-01109-2","DOIUrl":"10.1007/s13730-026-01109-2","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103024/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147763592","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":"Pierson syndrome with numerous dilated tubules masquerading as autosomal recessive polycystic kidney disease: a case report.","authors":"Natsumi Yamamura-Miyazaki, Toshimi Michigami, Nobuhiko Okamoto, Takaya Iida, Kumiko Yanagi, Tadashi Kaname, Sayaka Fukuda, Minato Baba, Kaori Fujiwara, Takuo Kubota","doi":"10.1007/s13730-026-01105-6","DOIUrl":"10.1007/s13730-026-01105-6","url":null,"abstract":"<p><p>Pierson syndrome, characterized by congenital nephrotic syndrome, ocular abnormalities, and neurological defects, is caused by biallelic pathogenic variants in LAMB2. LAMB2 encodes laminin β2, a key component of basement membranes that is predominantly expressed in the glomeruli, eyes, and neuromuscular junctions. The renal histopathology of Pierson syndrome typically shows diffuse mesangial sclerosis (DMS), with occasional tubulointerstitial atrophy and fibrosis. We report a case of Pierson syndrome characterized by DMS and prominent tubular dilatation. A fetal ultrasound at 23 weeks of gestation revealed hyperechoic kidneys, which gradually enlarged, accompanied by the onset of anhydramnios from 31 weeks. The patient was delivered at 39 weeks of gestation, weighing 3,132 g, without placentomegaly. Postnatal respiratory failure due to pulmonary hypoplasia required extracorporeal membrane oxygenation, and hemodialysis was initiated for anuria. Left nephrectomy was performed on day 8 of life, revealing replacement of the renal parenchyma by numerous irregularly dilated tubules with eosinophilic casts. The right kidney reached maximal enlargement by 1 month of age and subsequently began to shrink. Ocular findings included bilateral microcoria and cataracts. Whole-exome sequencing identified compound heterozygous truncating variants in LAMB2 (p.Gln1507Ter and p.Gln1622Ter). This case highlights the need to consider Pierson syndrome in the differential diagnosis of prenatally detected hyperechoic and enlarged kidneys, in addition to polycystic kidney disease.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13091815/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716290","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":"Measurement of complement proteins may aid interpretation of pathophysiology in kidney transplant recipients with atypical hemolytic uremic syndrome.","authors":"Masayoshi Miura, Hiroshi Higashiyama, Takahiro Tsuji, Katsuki Ohtani, Nobutaka Wakamiya","doi":"10.1007/s13730-026-01114-5","DOIUrl":"10.1007/s13730-026-01114-5","url":null,"abstract":"<p><p>A 53-year-old woman with kidney failure and low-titer donor-specific antibody underwent a living-unrelated donor kidney transplant. Presentation of severe thrombotic microangiopathy (TMA) resulted in early graft loss at day 8 post-transplant and initiation of hemodialysis. Thorough examination of the medical data revealed the patient to have had atypical hemolytic uremic syndrome (aHUS). Hemolytic anemia persisted for several months, even after complete embolization of the kidney graft. The patient subsequently received prophylactic eculizumab treatment and underwent a successful second transplant from a familial donor. Withdrawal of eculizumab resulted in development of TMA in the second graft, while reintroduction of eculizumab reversed the TMA. We examined complement-related profiles over time, and serial measurement of factors such as factor Ba, soluble C5b-9, and C3, and of total complement activity (CH50), clearly reflected the underlying pathophysiological condition and treatment status of the patient before, during, and after transplant. Longitudinal observation of complement protein levels may therefore aid in identifying candidates for kidney transplant who are at risk of aHUS, and monitoring patient status during transplant and follow-up.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13091814/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716314","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 hypertensive emergency in a patient who had a history of very low birth weight.","authors":"Yoichi Kadoh, Jun Yoshino, Tomohiro Oka, Fumika Kamei, Ai Uchida, Maki Hanada, Mamiko Nagase, Daisuke Niino, Takeshi Kanda","doi":"10.1007/s13730-026-01119-0","DOIUrl":"10.1007/s13730-026-01119-0","url":null,"abstract":"<p><p>Low birth weight (LBW) is a risk factor for development of hypertension and chronic kidney disease in adulthood. Here, we report the case of a forty-year-old man with a history of very low birth weight (VLBW) (1300 g) who had hypertensive emergency (233/142 mmHg), acute kidney injury (AKI) (serum creatinine, 8.47 mg/dL), elevated plasma renin activity (102 ng/mL/h), and posterior reversible encephalopathy syndrome. He received temporary hemodialysis but was eventually liberated following intensive antihypertensive therapy. A renal biopsy revealed focal segmental glomerulosclerosis (FSGS) with endothelial injury. In addition, the patient had pre-existing, undertreated hypertension. Taken together, the reduced nephron number and impaired vascular development due to LBW may be involved in the development of adult-onset, near fatal hypertensive emergency and subsequent AKI in our case. This case reinforces the long-term cardio-renal risks associated with LBW and illustrates the importance of evaluating perinatal history in the diagnostic workup and successful management of adult-onset nephropathy.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13091823/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716226","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 giant renal angiomyolipoma and diabetic nephropathy with abnormalities in the genes TSC2 and HNF1B.","authors":"Hisashi Sugimoto, Naoki Sawa, Yuki Oba, Hiroki Mizuno, Akinari Sekine, Masayuki Yamanouchi, Kiho Tanaka, Eiko Hasegawa, Tatsuya Suwabe, Takehiko Wada, Takayoshi Yokoyama, Katsuyuki Miki, Yuki Nakamura, Yasuo Ishii, Kei Kono, Kenichi Ohashi, Yutaka Takazawa, Takuya Fujimaru, Takayasu Mori, Eisei Sohara, Yoshifumi Ubara","doi":"10.1007/s13730-026-01107-4","DOIUrl":"10.1007/s13730-026-01107-4","url":null,"abstract":"<p><p>A 57-year-old female patient with tuberous sclerosis and end-stage renal failure who had a history of diabetes mellitus and repeated renal hemorrhage after induction of dialysis underwent surgical nephrectomy. The renal tissue that caused the hemorrhage consisted of adipocytes, blood vessels, and smooth muscle cells, so angiomyolipoma was diagnosed. The cause of the hemorrhage was thought to be an abnormality in the vascular structure, i.e., disruption of the elastic plates that make up the vessel wall, and the presence of a hematoma in the same area. Diabetic nephropathy, which was observed in the atrophied native kidney, was presumed to be the cause of end-stage renal failure. Genetic analysis confirmed the presence of TSC2 mutation, which is linked to tuberous sclerosis, and HNF1B mutation, which is linked to diabetes. To our knowledge, this is the first report of a patient with HNF1B mutation who may have developed end-stage renal failure because of diabetic nephropathy and who also had histologic evidence of renal hemorrhage.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13091817/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716254","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":"Lansoprazole as a possible trigger of restless legs syndrome with suspected periodic limb movement disorder in a hemodialysis patient: a case report.","authors":"Hirosuke Nakata, Koichi Nishiwaki","doi":"10.1007/s13730-026-01099-1","DOIUrl":"10.1007/s13730-026-01099-1","url":null,"abstract":"<p><p>Restless legs syndrome (RLS) and periodic limb movement disorder (PLMD) are common in patients undergoing hemodialysis and can significantly impair sleep and daily functioning. Several medications are known to induce or exacerbate RLS/PLMD, but proton pump inhibitors (PPIs) have not been clearly implicated. We report a hemodialysis patient with dopaminergic-refractory RLS/PLMD whose symptoms improved markedly following discontinuation of lansoprazole, suggesting a possible drug-related trigger. A 76-year-old woman with end-stage renal disease due to diabetic nephropathy began hemodialysis. Lansoprazole 15 mg/day was initiated two months earlier for peptic ulcer disease. Soon afterward, she developed irresistible leg discomfort and involuntary movements during rest and sleep, occasionally extending into daytime. Symptoms were relieved by movement and fulfilled the International RLS Study Group diagnostic criteria. Rotigotine therapy was ineffective, and symptoms gradually worsened. Laboratory findings showed preserved iron status, magnesium levels, and dialysis efficiency. Due to atypical daytime symptoms and dopaminergic refractoriness, drug-induced RLS/PLMD was suspected. Methyldopa, introduced later, was discontinued first, resulting in partial improvement. Subsequent withdrawal of lansoprazole and transition to vonoprazan led to near-complete symptom resolution within 14 days, with a reduction in the RLS-6 score from 28 to 4. The Naranjo score was 5, indicating a probable adverse drug reaction. This case suggests that lansoprazole may contribute to RLS/PLMD symptoms in susceptible hemodialysis patients. When RLS/PLMD presents atypically or responds poorly to dopaminergic agents, clinicians should reassess PPI necessity and consider structured withdrawal.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13091812/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147716221","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}