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Reduction of polycystic kidney and liver volumes in a patient with tolvaptan-resistant autosomal dominant polycystic kidney disease and acromegaly during lanreotide therapy. 在lanreotide治疗期间,托伐普坦耐药常染色体显性多囊肾病和肢端肥大症患者的多囊肾和肝脏体积减少
IF 1
CEN Case Reports Pub Date : 2025-06-27 DOI: 10.1007/s13730-025-01013-1
Shun Takenaka, Fumihiko Hattanda, Takuro Kawamura, Kanako Watanabe-Kusunoki, Daigo Nakazawa, Hiraku Kameda, Olga Amengual, Tatsuya Atsumi, Saori Nishio
{"title":"Reduction of polycystic kidney and liver volumes in a patient with tolvaptan-resistant autosomal dominant polycystic kidney disease and acromegaly during lanreotide therapy.","authors":"Shun Takenaka, Fumihiko Hattanda, Takuro Kawamura, Kanako Watanabe-Kusunoki, Daigo Nakazawa, Hiraku Kameda, Olga Amengual, Tatsuya Atsumi, Saori Nishio","doi":"10.1007/s13730-025-01013-1","DOIUrl":"https://doi.org/10.1007/s13730-025-01013-1","url":null,"abstract":"<p><p>A 35-year-old female was diagnosed with autosomal dominant polycystic kidney disease (ADPKD) in 2002 and referred to the Nephrology department of our hospital for follow-up and management. Treatment with tolvaptan was started in 2016, which failed to reduce her total kidney volume (TKV) and slow the decline in kidney function. In 2018, she experienced bitemporal hemianopia. Examination revealed enlarged fingers, characteristic facial features, and elevated serum insulin-like growth factor 1 (IGF-1) and growth hormone (GH) levels. A pituitary tumor was identified through the brain, and she was subsequently diagnosed with acromegaly. Lanreotide treatment was initiated, effectively reducing IGF-1 and GH serum levels and TKV within six months. After transsphenoidal surgery, lanreotide was discontinued. However, her oral glucose tolerance test after surgery did not show remission (GH nadir > 0.4 ng/mL), and serum GH levels gradually increased. Furthermore, TKV increased and kidney function decreased rapidly. Lanreotide treatment was restarted, resulting in further TKV reduction, suppression of liver volume enlargement and slowing kidney function decline. Compared to tolvaptan, the evidence for the effectiveness of somatostatin analogs in reducing TKV in ADPKD is not sufficiently established. Our case demonstrates that lanreotide effectively suppressed the progression of TKV, liver volume and kidney dysfunction with correlation to IGF-1 or GH in a patient with ADPKD. Our experience indicates that elevated levels of IGF-1 or GH may contribute to increased kidney and liver volume. Herein, we report the first case of tolvaptan-resistant ADPKD with concomitant acromegaly successfully managed with lanreotide.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144511610","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}
引用次数: 0
A case of acute tubulointerstitial nephritis with suspected Saikokaryukotsuboreito involvement responding to glucocorticoid therapy. 急性肾小管间质性肾炎疑似Saikokaryukotsuboreito受累1例,经糖皮质激素治疗。
IF 1
CEN Case Reports Pub Date : 2025-06-26 DOI: 10.1007/s13730-025-01012-2
Shun Ishibashi, Toshiaki Usui, Masaki Baba, Tsuyoshi Tsukada, Kohei Inoue, Shintaro Furuno, Masahiro Niisaka, Soichiro Nomura, Takuya Harada, Tomoki Akiyama, Ryota Ishii, Ryoya Tsunoda, Akiko Fujita, Hirayasu Kai, Naoki Morito, Kaori Mase, Chie Saito, Joichi Usui, Motohiro Sekiya, Kunihiro Yamagata
{"title":"A case of acute tubulointerstitial nephritis with suspected Saikokaryukotsuboreito involvement responding to glucocorticoid therapy.","authors":"Shun Ishibashi, Toshiaki Usui, Masaki Baba, Tsuyoshi Tsukada, Kohei Inoue, Shintaro Furuno, Masahiro Niisaka, Soichiro Nomura, Takuya Harada, Tomoki Akiyama, Ryota Ishii, Ryoya Tsunoda, Akiko Fujita, Hirayasu Kai, Naoki Morito, Kaori Mase, Chie Saito, Joichi Usui, Motohiro Sekiya, Kunihiro Yamagata","doi":"10.1007/s13730-025-01012-2","DOIUrl":"https://doi.org/10.1007/s13730-025-01012-2","url":null,"abstract":"<p><p>Drug-induced kidney injury is a major cause of acute tubulointerstitial nephritis (AIN). The standard treatment for AIN is discontinuation of the causative agent; however, if discontinuation alone is insufficient, glucocorticoid therapy may be effective. A 51-year-old woman was admitted to our hospital with acute kidney injury, presenting with an estimated glomerular filtration rate (eGFR) of 6.3 mL/min/1.73m<sup>2</sup>. She was diagnosed with AIN after a renal biopsy. Drug-induced lymphocyte stimulation tests were performed on the herbal medicine she had been taking for 5 months, Saikokaryukotsuboreito (comprising Bupleurum root, Pinellia tuber, Scutellaria root, and Ginseng as key ingredients). The test results were positive for all four components. The patient discontinued the causative agent, and her renal function improved with oral glucocorticoid therapy (prednisolone 30 mg/day). After 6 months of treatment, her renal function stabilized, with only minor residual tubular acidosis. This case of AIN induced by Saikokaryukotsuboreito, which responded to glucocorticoid therapy, highlights the potential for recovery with appropriate treatment.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144494893","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}
引用次数: 0
A case of effective tocilizumab for arthropathy due to dialysis-related amyloidosis. 托珠单抗治疗透析相关淀粉样变性所致关节病变1例。
IF 1
CEN Case Reports Pub Date : 2025-06-26 DOI: 10.1007/s13730-025-01009-x
Susumu Tsunoda, Yusuke Yoshimura, Yuki Oba, Daisuke Ikuma, Hiroki Mizuno, Masayuki Yamanouchi, Tatsuya Suwabe, Izuru Kitajima, Kei Kono, Kenichi Ohashi, Yoshifumi Ubara, Naoki Sawa
{"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":"https://doi.org/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":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144504932","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}
引用次数: 0
NELL-1-positive membranous nephropathy following gastric cancer in an older living kidney donor: a case report. 高龄活体肾供者胃癌后nell -1阳性膜性肾病1例报告。
IF 1
CEN Case Reports Pub Date : 2025-06-24 DOI: 10.1007/s13730-025-01011-3
Mamiko Hashimoto, Erika Hishida, Yuko Ono, Takuya Murakami, Mami Hirata, Maki Kinoshita, Toshimi Imai, Tetsu Akimoto, Daisuke Nagata
{"title":"NELL-1-positive membranous nephropathy following gastric cancer in an older living kidney donor: a case report.","authors":"Mamiko Hashimoto, Erika Hishida, Yuko Ono, Takuya Murakami, Mami Hirata, Maki Kinoshita, Toshimi Imai, Tetsu Akimoto, Daisuke Nagata","doi":"10.1007/s13730-025-01011-3","DOIUrl":"https://doi.org/10.1007/s13730-025-01011-3","url":null,"abstract":"<p><p>Living kidney transplantation is an established treatment for end-stage kidney disease (ESKD). While rare, the donors may develop de novo glomerular diseases post-donation. We report a case of membranous nephropathy (MN) in an 80-year-old male, diagnosed 9 years post-donation. The patient had a history of early gastric cancer treated with endoscopic submucosal dissection one year post-donation. Routine follow-up revealed asymptomatic proteinuria (4.27 g/gCr). The kidney biopsy demonstrated MN with IgG1/IgG3-dominant deposits and NELL-1 positivity. Recent evidence suggests NELL-1-positive MN may occur independently of malignancy in older individuals. Notably, MN developed 8 years after cancer remission with no evidence of recurrence. The patient achieved complete remission with conservative therapy consisting of an angiotensin receptor blocker and dietary modification. This case underscores the need for vigilant long-term follow-up in older kidney donors and illustrates how early proteinuria detection facilitates timely management of de novo glomerulopathies.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144474073","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}
引用次数: 0
Renal and gastrointestinal complications following tosufloxacin therapy for suspected mycoplasma infection in a 10-year-old child. 托氟沙星治疗疑似支原体感染的10岁儿童的肾脏和胃肠道并发症。
IF 1
CEN Case Reports Pub Date : 2025-06-17 DOI: 10.1007/s13730-025-01007-z
Shigehiro Sainokami, Hirofumi Watanabe, Shoichiro Kanda, Natsuho Adachi, Hiroyuki Tanaka, Keiichi Takizawa, Naoko Sone, Yuko Kajiho, Akiko Kinumaki, Yoshifumi Morita, Teruhiko Yoshida, Makoto Kurano, Yutaka Harita
{"title":"Renal and gastrointestinal complications following tosufloxacin therapy for suspected mycoplasma infection in a 10-year-old child.","authors":"Shigehiro Sainokami, Hirofumi Watanabe, Shoichiro Kanda, Natsuho Adachi, Hiroyuki Tanaka, Keiichi Takizawa, Naoko Sone, Yuko Kajiho, Akiko Kinumaki, Yoshifumi Morita, Teruhiko Yoshida, Makoto Kurano, Yutaka Harita","doi":"10.1007/s13730-025-01007-z","DOIUrl":"https://doi.org/10.1007/s13730-025-01007-z","url":null,"abstract":"<p><p>Tosufloxacin, a fluoroquinolone antibiotic, is increasingly prescribed for pediatric patients, particularly for macrolide-resistant Mycoplasma pneumoniae infections in Japan. While its efficacy is well-documented, adverse effects such as renal impairment and gastrointestinal symptoms have raised growing concerns. We report a case of a 10-year-old girl who developed sequential symptoms following tosufloxacin administration. On the day after starting tosufloxacin, she experienced right-sided flank pain, followed by the onset of gastrointestinal symptoms, including abdominal pain, diarrhea, and vomiting, on the subsequent day. Contrast-enhanced computed tomography revealed increased fatty tissue opacity surrounding the kidney and duodenal wall thickening, suggestive of localized inflammation extending from the kidney to adjacent structures. Urine microscopy demonstrated needle- and sea urchin-shaped drug crystals, strongly implicating tosufloxacin in the development of renal and gastrointestinal symptoms. This case provides the first evidence suggesting that kidney inflammation may contribute to gastrointestinal symptom onset via localized inflammatory extension. The patient's symptoms resolved promptly with the discontinuation of tosufloxacin and supportive care. This report underscores the importance of monitoring pediatric patients for renal and gastrointestinal adverse effects following tosufloxacin administration.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144315965","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}
引用次数: 0
Granulomatous interstitial nephritis due to sarcoidosis following SARS-CoV-2 infection: a rare case report. SARS-CoV-2感染后结节病致肉芽肿性间质性肾炎1例
IF 1
CEN Case Reports Pub Date : 2025-06-16 DOI: 10.1007/s13730-025-01008-y
Megumi Otani, Kiyotaka Nagahama, Kento Nakamura, Akiko Nagatomo, Suzu Yoshida, Kazuhiro Okai, Ayaka Suzuki, Masato Sasaki, Suguru Hirasawa, Shota Aki, Hiroyuki Tanaka
{"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":"https://doi.org/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":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144301191","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}
引用次数: 0
Life-threatening baclofen toxicity in ESRD: a case report. ESRD中危及生命的巴氯芬毒性:一例报告。
IF 1
CEN Case Reports Pub Date : 2025-06-13 DOI: 10.1007/s13730-025-01005-1
Shogo Taira, Shiori Tamayose, Tasuku Kikumura, Morikuni Nishihira
{"title":"Life-threatening baclofen toxicity in ESRD: a case report.","authors":"Shogo Taira, Shiori Tamayose, Tasuku Kikumura, Morikuni Nishihira","doi":"10.1007/s13730-025-01005-1","DOIUrl":"https://doi.org/10.1007/s13730-025-01005-1","url":null,"abstract":"<p><p>Baclofen, a GABA-B receptor agonist, is widely used to manage spasticity, alcohol use disorders, and chronic hiccups. However, baclofen toxicity is a significant concern, particularly in patients with renal impairment due to its primary renal excretion. This case report describes the diagnostic and management challenges of baclofen toxicity in an 82-year-old man with end-stage renal disease (ESRD) undergoing maintenance hemodialysis. The patient presented with symptoms of vomiting, decreased consciousness, and involuntary movements 5 days after initiating a low dose of baclofen (5 mg/day) for hiccups. Despite the low dosage, the patient exhibited severe neurological symptoms, including atypical athetosis-like involuntary movements, which are not commonly associated with baclofen toxicity. Given his impaired renal clearance, baclofen accumulation was suspected as the underlying cause. Hemodialysis was performed under mechanical ventilation due to concerns about respiratory suppression, leading to significant clinical improvement. The patient was successfully extubated and discharged after 14 days of hospitalization. This case underscores the critical need for heightened vigilance and cautious medication management when prescribing baclofen to patients with renal dysfunction, even at low doses, as they face a significantly elevated risk of toxicity. Early recognition and timely intervention, including hemodialysis, are essential in managing baclofen toxicity in this vulnerable population. Increased awareness of atypical neurological manifestations of baclofen toxicity is crucial for accurate diagnosis and effective treatment.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144282552","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}
引用次数: 0
Phenotype-dependent heterogeneity of THSD7A expression in gastric cancer tissue in a patient with THSD7A-associated membranous nephropathy. THSD7A相关膜性肾病患者胃癌组织中THSD7A表达的表型依赖异质性
IF 1
CEN Case Reports Pub Date : 2025-06-09 DOI: 10.1007/s13730-025-01006-0
Chisato Minezaki, Tatsuhiko Azegami, Akinori Hashiguchi, Takashin Nakayama, Norifumi Yoshimoto, Akihito Hishikawa, Aika Hagiwara, Kaori Hayashi
{"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":"https://doi.org/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":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144246645","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}
引用次数: 0
A rare case of coronin-1A deficiency with IgM dominant membranoproliferative glomerulonephritis. 冠状蛋白1a缺乏伴IgM显性膜增生性肾小球肾炎1例。
IF 1
CEN Case Reports Pub Date : 2025-06-04 DOI: 10.1007/s13730-025-01004-2
Zeynep Ural, Betül Ogüt, Gülsüm Kayhan, Ipek Işık Gönül, Ulver Derici
{"title":"A rare case of coronin-1A deficiency with IgM dominant membranoproliferative glomerulonephritis.","authors":"Zeynep Ural, Betül Ogüt, Gülsüm Kayhan, Ipek Işık Gönül, Ulver Derici","doi":"10.1007/s13730-025-01004-2","DOIUrl":"https://doi.org/10.1007/s13730-025-01004-2","url":null,"abstract":"<p><p>Coronin-1A deficiency, caused by mutations in the CORO1A gene, is an autosomal recessive immunodeficiency characterized by T-cell dysfunction and is classified as severe combined immunodeficiency (SCID). This condition presents with lymphopenia, hypogammaglobulinemia, recurrent Epstein-Barr virus (EBV) infections, EBV-associated B-cell lymphoma and epidermodysplasia verruciformis. This case report presents a 32-year-old female with Coronin-1A deficiency, who developed IgM-dominant membranoproliferative glomerulonephritis (MPGN) alongside recurrent viral infections. This is the first reported case linking Coronin-1A deficiency with MPGN. The patient was treated with corticosteroids, which improved her renal function, but she succumbed to recurrent infections within a year. This case emphasizes the potential for renal disease in immunodeficient patients with persistent infections.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144215030","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}
引用次数: 0
Severe hypokalemia through urinary retention: a case report of a patient with sigmoid neobladder replacement. 重度低钾血症伴尿潴留:乙状结肠新膀胱置换术1例报告。
IF 1
CEN Case Reports Pub Date : 2025-06-03 DOI: 10.1007/s13730-025-01002-4
Midori Kobayashi, Katsuyuki Nagatoya, Masafumi Wada, Maho Tokuchi, Soichiro Misegawa, Rina Saito, Hiroki Nomi, Ryota Haga, Daisuke Mori, Atsushi Yamauchi
{"title":"Severe hypokalemia through urinary retention: a case report of a patient with sigmoid neobladder replacement.","authors":"Midori Kobayashi, Katsuyuki Nagatoya, Masafumi Wada, Maho Tokuchi, Soichiro Misegawa, Rina Saito, Hiroki Nomi, Ryota Haga, Daisuke Mori, Atsushi Yamauchi","doi":"10.1007/s13730-025-01002-4","DOIUrl":"https://doi.org/10.1007/s13730-025-01002-4","url":null,"abstract":"<p><p>Severe hypokalemia with respiratory distress is rarely caused by intestinal potassium (K) excretion from the intestinal tract because most of the ingested K is excreted in the urine and the kidney primarily maintains K balance. However, we recently experienced a rare case in which severe hypokalemia may have been caused by K excretion from a portion of the intestinal tract used as a neobladder. This case was characterized by the massive dilatation of the sigmoid neobladder estimated to hold 2-3 L of urine, due to urinary retention. The patient developed respiratory distress and required K dosages in excess of 300 mEq per day. Recent studies have demonstrated that the physical stimulus of membrane stretch increases the probability of opening the BK channel, a type of K channel expressed on the luminal side of the colon's wall. Therefore, we presumed that, in our case, much potassium was excreted through the urine in the sigmoid neobladder due to colonic wall extension. Additionally, immunostaining of the sigmoid colon tissue of his bladder demonstrated a higher level of expression of BK channel protein than the patient with normal renal function. We speculated that BK channel upregulation also contributed to severe hypokalemia.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144207764","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}
引用次数: 0
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