CEN Case Reports最新文献_第9页

A case of posterior and reversible encephalopathy syndrome in a patient previously undiagnosed with lupus nephritis. 一例后路可逆性脑病综合征患者先前未确诊狼疮肾炎。

IF 1

CEN Case Reports Pub Date : 2025-06-01 Epub Date: 2025-02-07 DOI: 10.1007/s13730-025-00973-8

Yoichi Kadoh, Jun Yoshino, Tomohiro Oka, Kenichi Itoga, Maki Hanada, Daisuke Niino, Atsushi Nagai, Kunihiro Ichinose, Takeshi Kanda

{"title":"A case of posterior and reversible encephalopathy syndrome in a patient previously undiagnosed with lupus nephritis.","authors":"Yoichi Kadoh, Jun Yoshino, Tomohiro Oka, Kenichi Itoga, Maki Hanada, Daisuke Niino, Atsushi Nagai, Kunihiro Ichinose, Takeshi Kanda","doi":"10.1007/s13730-025-00973-8","DOIUrl":"10.1007/s13730-025-00973-8","url":null,"abstract":"Posterior reversible encephalopathy syndrome (PRES) is a rare clinico-neuroradiologic disease associated with various conditions, such as hypertension, eclampsia, chronic kidney disease, and autoimmune diseases. Here, we present the case of the unusual occurrence of PRES with hypertensive emergency and renal insufficiency in a 37-year-old woman previously undiagnosed with systemic lupus erythematosus (SLE) and lupus nephritis. The patient was emergently admitted to our hospital with sudden onset of visual impairment, headache, and high blood pressure, and she was eventually diagnosed with PRES by brain magnetic resonance imaging (MRI). Her PRES-associated clinical symptoms and MRI abnormalities were improved following anti-hypertensive treatment with calcium channel blocker. A kidney biopsy revealed diffuse proliferative glomerulonephritis with a full-house immunofluorescence pattern and fibrinoid necrosis in small blood vessels, suggesting a class IV-G (A) lupus nephritis with vasculitis. The immunosuppressive therapy with intravenous methylprednisolone pulse followed by oral prednisolone, mycophenolate mofetil, and intravenous belimumab, attenuated SLE-associated clinical manifestations including butterfly rush, edema, renal dysfunction, and proteinuria. Our case highlights the need to consider PRES as an initial clinical presentation of lupus nephritis and provide the early diagnosis and timely treatment to achieve a favorable outcome.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"328-334"},"PeriodicalIF":1.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12126384/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143363871","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}

引用次数: 0

Fatal visceral disseminated varicella zoster virus infection during initial remission induction therapy in a patient with lupus nephritis: a case report and review of the literature. 狼疮肾炎患者初始缓解诱导治疗期间致死性内脏弥散性水痘带状疱疹病毒感染：1例报告及文献回顾。

IF 1

CEN Case Reports Pub Date : 2025-06-01 Epub Date: 2024-12-12 DOI: 10.1007/s13730-024-00950-7

Runa Takehara, Itaru Ebihara, Yoshifumi Honda, Norimasa Ooba, Hiromi Kurosawa, Chihiro Sato, Haruo Ohtani, Yutaka Tsutsumi, Masato Nose, Masaki Kobayashi

{"title":"Fatal visceral disseminated varicella zoster virus infection during initial remission induction therapy in a patient with lupus nephritis: a case report and review of the literature.","authors":"Runa Takehara, Itaru Ebihara, Yoshifumi Honda, Norimasa Ooba, Hiromi Kurosawa, Chihiro Sato, Haruo Ohtani, Yutaka Tsutsumi, Masato Nose, Masaki Kobayashi","doi":"10.1007/s13730-024-00950-7","DOIUrl":"10.1007/s13730-024-00950-7","url":null,"abstract":"Visceral disseminated varicella zoster virus (VZV) infection is a severe complication, characterized by a notably high mortality rate. Herein, we present a case of a 36-year-old-man involving visceral disseminated VZV infection that emerged during remission induction therapy involving high-dose prednisolone (PSL), mycophenolate mofetil (MMF), and hydroxychloroquine for lupus nephritis. Two months after starting the immunosuppressive therapy, he experienced a rapid onset of severe upper abdominal pain. The following day, clinical manifestations and laboratory abnormalities rapidly deteriorated. Hyperferritinemia and hypertriglyceridemia, indicative of hemophagocytic lymphohistiocytosis (HLH), emerged, along with escalating liver and renal impairment and newly appeared disseminated intravascular coagulation, and multiple organ failure is suggested. The patient developed widespread blistering predominantly on the trunk and face, patient's condition failed to ameliorate, ultimately culminating in his demise a few hours later. At autopsy, the cutaneous lesions with blisters revealed positive immunostaining with anti-VZV antibody, and similar findings were detected in multiple organs. HLH was confirmed in lymph nodes. It is crucial to emphasize the awareness of visceral disseminated VZV, particularly in cases patients are undergoing concurrent PSL therapy alongside MMF for SLE. The progression of this fatal condition usually begins with abdominal pain, followed by a skin rash a few days later. The present case is the first to show evidence of HLH occurring as a result of visceral disseminated VZV infection. This disease is extremely rare but extremely serious, therefore, VZV-DNA should be measured in cases where you suspect this disease for early diagnosis and treatment.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"477-485"},"PeriodicalIF":1.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12126378/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142812006","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}

引用次数: 0

A case of acute appendicitis in a patient with minimal change disease. 急性阑尾炎伴微小病变1例。

IF 1

CEN Case Reports Pub Date : 2025-06-01 Epub Date: 2025-03-23 DOI: 10.1007/s13730-025-00986-3

Fumiya Sato, Shingo Nakayama, Takuo Hirose, Akari Endo, Yu Kasakura, Yoshitaka Kanomata, Ayaka Kamada, Ikuko Oba-Yabana, Tomoyoshi Kimura, Wako Yumura, Takefumi Mori

{"title":"A case of acute appendicitis in a patient with minimal change disease.","authors":"Fumiya Sato, Shingo Nakayama, Takuo Hirose, Akari Endo, Yu Kasakura, Yoshitaka Kanomata, Ayaka Kamada, Ikuko Oba-Yabana, Tomoyoshi Kimura, Wako Yumura, Takefumi Mori","doi":"10.1007/s13730-025-00986-3","DOIUrl":"10.1007/s13730-025-00986-3","url":null,"abstract":"Minimal change disease (MCD) is a common cause of idiopathic nephrotic syndrome (NS). MCD patients are complicated by acute kidney injury (AKI). Gastrointestinal disorders also occur during the course of NS; however, acute appendicitis after the development of NS has not been reported previously in patients with MCD. We report the case of a 54-year-old Japanese man with MCD who was diagnosed with acute appendicitis after developing NS. The patient visited a nearby medical clinic with abdominal distension, decreased urine volume, and edema of the face and lower limbs. As the symptoms did not improve and he developed abdominal pain, he was referred to the Division of Gastroenterology at our hospital. Hypoalbuminemia and proteinuria were detected, and he was introduced to our division and admitted for the evaluation and treatment of NS. After admission, right lower quadrant abdominal pain and rebound tenderness occurred, and an enlarged appendix and increased fat tissue density around the appendix were observed on abdominal and pelvic computed tomography. The patient underwent laparoscopic appendectomy for suspected acute perforated appendicitis and peritonitis. Although the patient required temporary hemodialysis due to oliguric AKI, the renal function and proteinuria improved with steroid therapy. We performed a renal biopsy, which revealed MCD with acute tubular injury. Since severe gastrointestinal disorders can occur in patients with MCD, these patients should be followed-up with carefully for acute abdominal pain. The prompt management of gastrointestinal disorders is important when acute abdominal pain occurs in patients with MCD.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"455-460"},"PeriodicalIF":1.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12126365/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691274","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}

引用次数: 0

A case of IgG-type heavy chain amyloidosis with membranous nephropathy-like changes with long-term survival. igg型重链淀粉样变性伴膜性肾病样改变1例。

IF 1

CEN Case Reports Pub Date : 2025-06-01 Epub Date: 2025-02-28 DOI: 10.1007/s13730-025-00982-7

Kei Kono, Naoki Sawa, Yuki Oba, Koichi Kikuchi, Hiroki Mizuno, Masayuki Yamanouchi, Eiko Hasegawa, Tatsuya Suwabe, Atsushi Wake, Yukako Shintani-Domoto, Kenichi Ohashi, Fuyuki Kametani, Masahide Yazaki, Takehiko Wada, Yoshifumi Ubara

{"title":"A case of IgG-type heavy chain amyloidosis with membranous nephropathy-like changes with long-term survival.","authors":"Kei Kono, Naoki Sawa, Yuki Oba, Koichi Kikuchi, Hiroki Mizuno, Masayuki Yamanouchi, Eiko Hasegawa, Tatsuya Suwabe, Atsushi Wake, Yukako Shintani-Domoto, Kenichi Ohashi, Fuyuki Kametani, Masahide Yazaki, Takehiko Wada, Yoshifumi Ubara","doi":"10.1007/s13730-025-00982-7","DOIUrl":"10.1007/s13730-025-00982-7","url":null,"abstract":"We performed a kidney biopsy on a 68-year-old man with 2.6 g/day proteinuria. Immunofluorescence (IF) study showed a positive finding of IgG (IgG1) along the glomerular capillary wall, suggesting membranous nephropathy. However, electron microscopy showed no subepithelial electron dense deposits and amorphous deposits of 8-12 nm fibrillar structure, and positive DFS/Congo red staining confirmed the diagnosis of amyloidosis. Amyloid deposits were localized only in the glomeruli. No overt extrarenal amyloid lesions were identified. Serum and urine were positive for monoclonal IgG-lambda, but IF was negative for kappa and lambda, ruling out AL-amyloidosis. At 75 years of age, he underwent a second kidney biopsy, and the IgG-positive findings at IF became more pronounced, with progressive glomerular sclerosing lesions. A proteomic analysis was performed focusing on the positive IgG finding in IF. The amyloid was proven to be composed of fragments of a heavy chain variable region sequence. AH-amyloidosis was the final diagnosis. He was treated according to the treatment for AL-amyloidosis, but hemodialysis was started at the age of 81, and died at the age of 86. We report a valuable case of AH-amyloidosis with an observed long-term prognosis.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"434-441"},"PeriodicalIF":1.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12126444/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143531333","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}

引用次数: 0

Tonsillectomy in Immunoglobulin A vasculitis with nephritis: case series. 扁桃体切除术治疗免疫球蛋白A血管炎合并肾炎：病例系列。

IF 1

CEN Case Reports Pub Date : 2025-06-01 Epub Date: 2025-02-17 DOI: 10.1007/s13730-025-00975-6

Eisuke Kubo, Kotaro Haruhara, Hirokazu Marumoto, Takaya Sasaki, Masahiro Okabe, Shinya Yokote, Akihiro Shimizu, Hiroyuki Ueda, Nobuo Tsuboi, Takashi Yokoo

{"title":"Tonsillectomy in Immunoglobulin A vasculitis with nephritis: case series.","authors":"Eisuke Kubo, Kotaro Haruhara, Hirokazu Marumoto, Takaya Sasaki, Masahiro Okabe, Shinya Yokote, Akihiro Shimizu, Hiroyuki Ueda, Nobuo Tsuboi, Takashi Yokoo","doi":"10.1007/s13730-025-00975-6","DOIUrl":"10.1007/s13730-025-00975-6","url":null,"abstract":"There is no consensus-based treatment for adult-onset immunoglobulin A vasculitis with nephritis (IgAV nephritis). Tonsillectomy is a treatment option for primary IgA nephropathy, which has similar histopathological features and pathogenesis to IgAV nephritis. The present case series aimed to describe the clinical course of patients with IgAV nephritis who underwent tonsillectomy in our institution. Adult patients with biopsy-proven IgAV nephritis who received tonsillectomy from 2015 to 2022 were systematically reviewed at six hospitals in Japan. Hematuria, proteinuria and slope of the estimated glomerular filtration rate (eGFR) were evaluated before and after tonsillectomy. Patients with IgAV nephritis who underwent tonsillectomy was identified in 12 of 2626 kidney biopsies performed during the study period. The median observation periods before and after tonsillectomy were 20.7 and 48.6 months, respectively. The following drugs were administered concurrent with tonsillectomy: corticosteroids (n = 8), mizoribin (n = 1), and rituximab (n = 1). Three patients were not treated with corticosteroids or immunosuppressants. During post-tonsillectomy observation, 5 patients showed remission of hematuria. Of the 10 patients whose proteinuria was not at a remission level prior to tonsillectomy, 7 showed remission of proteinuria after tonsillectomy. The eGFR slope was attenuated in 9 patients after tonsillectomy relative to before tonsillectomy. This study suggests that some patients may benefit from tonsillectomy in the treatment of IgAV nephritis. The efficacy of tonsillectomy or combination therapy with immunosuppression for IgAV nephritis requires further case series to clarify the clinicopathologic picture of patients associated with a response to tonsillectomy.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"381-389"},"PeriodicalIF":1.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12126448/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440026","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}

引用次数: 0

Ceftriaxone encephalopathy in a very elderly dialysis patient. 头孢曲松脑病在老年透析患者中的应用。

IF 1

CEN Case Reports Pub Date : 2025-06-01 Epub Date: 2024-12-09 DOI: 10.1007/s13730-024-00953-4

Yumiko Nakamura, Ryo Yamamoto, Yuya Shioda, Takatsugu Iwashita, Taisuke Shimizu, Akito Maeshima, Hajime Hasegawa, Tomonari Ogawa

{"title":"Ceftriaxone encephalopathy in a very elderly dialysis patient.","authors":"Yumiko Nakamura, Ryo Yamamoto, Yuya Shioda, Takatsugu Iwashita, Taisuke Shimizu, Akito Maeshima, Hajime Hasegawa, Tomonari Ogawa","doi":"10.1007/s13730-024-00953-4","DOIUrl":"10.1007/s13730-024-00953-4","url":null,"abstract":"Ceftriaxone is widely used clinically but it can potentially cause ceftriaxone encephalopathy in individuals who are on dialysis. We describe ceftriaxone encephalopathy in a dialysis patient. The 87-year-old Japanese woman had a 9-year dialysis history. She was admitted to our department with right subcostal pain and lower back pain and was diagnosed with renal cyst hemorrhage and infection. Post-admission, we treated her with ceftriaxone (CTRX) 2 g/day and bed rest, which improved her symptoms. On the 7th hospitalization day, she began to exhibit incoherent speech and unresponsiveness; by day 10, her consciousness level had decreased to grade III and myoclonus appeared. Brain MRI and a cerebrospinal fluid examination ruled out cerebrovascular disease and encephalitis. EEG showed triphasic waves predominantly in the frontal lobe. Suspecting ceftriaxone encephalopathy, we switched the antibiotic to cefazolin 1 g/day. By day 12, the myoclonus decreased and the patient was able to communicate. By day 14, her consciousness level had improved to her admission level. We treated ceftriaxone encephalopathy in a dialysis patient. Although CTRX is generally thought to not require dose reduction in patients with renal failure (due to its hepatic excretion), caution is necessary as overdosage in dialysis patients can lead to myoclonus and encephalopathy.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"312-317"},"PeriodicalIF":1.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12126401/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142799422","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}

引用次数: 0

IgA nephropathy and IgA vasculitis in a pediatric Crohn's disease patient with early IgA deposition in vascular walls of intestines. 儿童克罗恩病伴早期IgA血管壁沉积的IgA肾病和IgA血管炎

IF 1

CEN Case Reports Pub Date : 2025-06-01 Epub Date: 2025-02-26 DOI: 10.1007/s13730-025-00970-x

Junqian Tang, Lan Wang, Wei Zhou, Youying Mao, Chenxing Zhang, Jiayao Shen, Minzhi Yin, Lei Yin

{"title":"IgA nephropathy and IgA vasculitis in a pediatric Crohn's disease patient with early IgA deposition in vascular walls of intestines.","authors":"Junqian Tang, Lan Wang, Wei Zhou, Youying Mao, Chenxing Zhang, Jiayao Shen, Minzhi Yin, Lei Yin","doi":"10.1007/s13730-025-00970-x","DOIUrl":"10.1007/s13730-025-00970-x","url":null,"abstract":"Patients with inflammatory bowel disease may present with extraintestinal manifestations. Crohn's disease complicated with IgA nephropathy or IgA vasculitis is relatively rare. In this case, an 11-year-old girl was diagnosed with Crohn's disease and infliximab was administered. 1 year after treatment, she presented with asymptomatic but persistent microscopic hematuria. The child was diagnosed with IgA vasculitis and IgA nephropathy at the fourth year of follow-up. To explore the association between Crohn's disease and IgA associated diseases, immunostaining for IgA and GdIgA1 deposition was retrospectively conducted in intestinal biopsy tissues obtained at the time of initiation and relapse of Crohn's disease. GdIgA1 deposition in intestinal tissues was found not only at the time of relapse of Crohn's disease, but also at the beginning of Crohn's disease when patient had neither exposure to any drug nor any symptom of IgA vasculitis or IgA nephropathy. The early appearance of GdIgA1 deposition indicated that Crohn's disease played a greater role in its formation than infliximab induction and the child might be prone to IgA associated diseases. Patients with Crohn's disease, especially those who receive tumor necrosis factor-alpha inhibitors are recommended to receive regular kidney examinations.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"335-344"},"PeriodicalIF":1.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12126441/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143514792","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}

引用次数: 0

A case of PLA2R-positive membranous nephropathy with subsequent development of IgG4-related disease. 一例 PLA2R 阳性膜性肾病，随后发展为 IgG4 相关疾病。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-11-08 DOI: 10.1007/s13730-024-00941-8

Fumiaki Tanemoto, Imari Mimura, Hiroyuki Abe, Masaomi Nangaku

{"title":"A case of PLA2R-positive membranous nephropathy with subsequent development of IgG4-related disease.","authors":"Fumiaki Tanemoto, Imari Mimura, Hiroyuki Abe, Masaomi Nangaku","doi":"10.1007/s13730-024-00941-8","DOIUrl":"10.1007/s13730-024-00941-8","url":null,"abstract":"Membranous nephropathy (MN) is a common cause of adult-onset nephrotic syndrome. It is also known as a minor but established renal manifestation of Immunoglobulin G4-related disease (IgG4-RD). Previous reports suggest that MN can also be an initial manifestation of IgG4-RD, all of which are phospholipase A2 receptor (PLA2R)-negative MN. We describe a case of PLA2R-positive MN that subsequently developed other manifestations of IgG4-RD. A 60-year-old male with nephrotic syndrome was diagnosed as primary MN with positive staining for PLA2R on the initial renal biopsy, which remained in partial remission with supportive therapy using angiotensin II receptor blocker (ARB) without steroid. About 1 year later, a renal mass was detected during an annual checkup, and contrast-enhanced computed tomography revealed low-density masses in bilateral kidneys and the head of the pancreas. The findings of endoscopic biopsy of the pancreatic mass were consistent with autoimmune pancreatitis (AIP) and the second renal biopsy showed the findings of MN with tubulointerstitial nephritis, both of which led to a diagnosis of IgG4-RD. The second renal biopsy also showed positive PLA2R. The patient received oral glucocorticoid therapy for IgG4-RD, which improved IgG4-related AIP and renal masses and also resulted in complete remission of MN. To our knowledge, this is the first reported case of PLA2R-positive MN with subsequent development of IgG4-RD. It is sometimes difficult to determine whether PLA2R-positive MN occurring with IgG4-RD is primary MN or secondary MN associated with IgG4-RD. The possibility of developing IgG4-RD should be considered even when preceding MN is PLA2R-positive, suggesting of primary MN.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"280-290"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958855/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142602295","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}

引用次数: 0

Gastrointestinal calciphylaxis: a rare and devastating complication in end-stage kidney disease. 胃肠道钙化症：终末期肾病罕见的破坏性并发症。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-11-14 DOI: 10.1007/s13730-024-00944-5

Amirhossein Aarabi, Karthik Kumar

{"title":"Gastrointestinal calciphylaxis: a rare and devastating complication in end-stage kidney disease.","authors":"Amirhossein Aarabi, Karthik Kumar","doi":"10.1007/s13730-024-00944-5","DOIUrl":"10.1007/s13730-024-00944-5","url":null,"abstract":"Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare and severe condition that predominantly affects individuals with end-stage kidney disease (ESKD). It manifests through vascular calcifications that precipitate tissue necrosis, with cutaneous manifestations being common. However, gastrointestinal involvement is an infrequent but gravely serious occurrence. In this report, we present an atypical case of gastrointestinal (GI) calciphylaxis in a 66-year-old woman undergoing peritoneal dialysis. The patient's initial symptoms of abdominal pain and turbid peritoneal fluid raised suspicions of bacterial peritonitis. Despite therapeutic interventions, the patient's condition deteriorated. Computed tomography (CT) imaging revealed a perforation in the caecal wall, necessitating emergency surgical intervention. Histopathology post-surgery confirmed the diagnosis of GI calciphylaxis. The patient's postoperative trajectory was fraught with complications, ultimately leading to a fatal outcome. This case highlights the importance of vigilance for calciphylaxis in the differential diagnosis when abdominal pain develops in patients with ESKD, regardless of the presence of typical skin symptoms. Prompt detection and an integrated care approach are essential for improved prognosis. Further research is needed to find more efficacious treatments for this devastating illness.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"297-300"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958865/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142615409","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}

引用次数: 0

Identification of CUBN variants in triplets with a 20-year history of proteinuria. 在有 20 年蛋白尿病史的三胞胎中鉴定 CUBN 变体。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-08-05 DOI: 10.1007/s13730-024-00919-6

Natsumi Yamamura-Miyazaki, Nana Sakakibara, Kandai Nozu, Yuko Shima, Kenichi Satomura, Satoko Yamamoto, Minato Baba, Kaori Fujiwara, Katsusuke Yamamoto, Toshimi Michigami

{"title":"Identification of CUBN variants in triplets with a 20-year history of proteinuria.","authors":"Natsumi Yamamura-Miyazaki, Nana Sakakibara, Kandai Nozu, Yuko Shima, Kenichi Satomura, Satoko Yamamoto, Minato Baba, Kaori Fujiwara, Katsusuke Yamamoto, Toshimi Michigami","doi":"10.1007/s13730-024-00919-6","DOIUrl":"10.1007/s13730-024-00919-6","url":null,"abstract":"CUBN encodes cubilin, which plays a role in the reabsorption of glomerular-filtered albumin in the proximal tubule. CUBN-related proteinuria was recently established as a new disease concept and may be present in proteinuric cases that were previously undiagnosed either genetically or histologically. We herein report a case of triplets diagnosed with chronic benign proteinuria due to CUBN variants 20 years after its onset. The proband, the first child of triplets, tested positive for urinary protein several times during the neonatal period. A urine screening test at 3 years old was positive. Proteinuria persisted for years within a non-nephrotic range. Kidney biopsy at 8 years old revealed minor glomerular abnormalities. Renin-angiotensin system inhibitors were started for albumin-based proteinuria but were ineffective. Since the two other triplets had similar courses, analyses of the NPHS1/2 and WT1 genes were performed but revealed no abnormalities. The triplets transitioned to adult care at 15 years old. CUBN-related proteinuria was reported in 2020; therefore, we re-analyzed their DNA samples and identified compound heterozygous variants in CUBN in all three triplets. The molecular diagnosis of CUBN-related proteinuria will save patients from unnecessary treatments and concerns about renal prognosis.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"145-150"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958850/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141888563","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}

引用次数: 0