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CART for ascites accumulation and dialysis difficulty after hepatic cyst fenestration and bilateral nephrectomy in ADPKD: a case report. 肝囊肿开窗及双侧肾切除术后腹水积存及透析困难的CART治疗1例报告。
IF 1
CEN Case Reports Pub Date : 2026-04-07 DOI: 10.1007/s13730-026-01113-6
Saori Sawada, Sho Sugahara, Anna Sumii, Tomonori Sakae, Aki Yamada, Yoshimi Imamura-Uehara, Nahomi Ishimoto, Shogo Kuwagata, Kosuke Yamahara, Mako Yasuda-Yamahara, Yuki Tanaka-Sasaki, Masami Chin-Kanasaki, Shinji Kume
{"title":"CART for ascites accumulation and dialysis difficulty after hepatic cyst fenestration and bilateral nephrectomy in ADPKD: a case report.","authors":"Saori Sawada, Sho Sugahara, Anna Sumii, Tomonori Sakae, Aki Yamada, Yoshimi Imamura-Uehara, Nahomi Ishimoto, Shogo Kuwagata, Kosuke Yamahara, Mako Yasuda-Yamahara, Yuki Tanaka-Sasaki, Masami Chin-Kanasaki, Shinji Kume","doi":"10.1007/s13730-026-01113-6","DOIUrl":"10.1007/s13730-026-01113-6","url":null,"abstract":"<p><p>Hepatomegaly with hepatic cysts constitutes a common extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD), often resulting in mass effects and occasionally necessitating cyst fenestration to alleviate abdominal distension. Nonetheless, persistent cyst fluid leakage as a postoperative complication frequently leads to refractory ascites, for which treatment options are limited. In this report, we present a 62-year-old female patient with ADPKD undergoing maintenance hemodialysis. She received bilateral nephrectomy and hepatic cyst fenestration to mitigate abdominal distension. Postoperatively, she developed ascites accumulation and recurrent intradialytic hypotension, impairing effective ultrafiltration. The ascites proved unresponsive to conservative management, and analysis indicated hepatic cyst fluid leakage, with elevated CA19-9 levels in the ascitic fluid. Repeated ultrafiltration and abdominal paracentesis failed to produce sustained benefits. Subsequently, she underwent two sessions of cell-free and concentrated ascites reinfusion therapy (CART), which successfully reduced her ascites. Over the subsequent four months, her serum albumin levels increased, intradialytic hypotension resolved, and the ascites subsided without recurrence, thus permitting the repair of an inguinal hernia identified at referral. This case exemplifies that CART constitutes an effective therapeutic modality for managing ascites accumulation resulting from hepatic cyst leakage following fenestration in patients with ADPKD.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13057055/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147626901","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
Response to "Before taurine is recommended for stroke-like episodes in m.3243A > G carriers, its efficacy must be demonstrated by appropriate studies". 对“在推荐牛磺酸治疗m.3243A > G携带者的卒中样发作之前,必须通过适当的研究证明其有效性”的回应。
IF 1
CEN Case Reports Pub Date : 2026-04-07 DOI: 10.1007/s13730-026-01112-7
Ryuto Yoshida, Ryunosuke Mitsuno, Tatsuhiko Azegami, Shintaro Yamaguchi, Kaori Hayashi
{"title":"Response to \"Before taurine is recommended for stroke-like episodes in m.3243A > G carriers, its efficacy must be demonstrated by appropriate studies\".","authors":"Ryuto Yoshida, Ryunosuke Mitsuno, Tatsuhiko Azegami, Shintaro Yamaguchi, Kaori Hayashi","doi":"10.1007/s13730-026-01112-7","DOIUrl":"10.1007/s13730-026-01112-7","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13057071/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147627095","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
Living donor kidney transplantation after living donor liver transplantation from the same donor in a patient with autosomal dominant polycystic kidney disease: a case report. 常染色体显性多囊肾病患者活体肝移植后活体肾移植1例
IF 1
CEN Case Reports Pub Date : 2026-04-07 DOI: 10.1007/s13730-026-01104-7
Yatsumu Saito, Izumi Yamamoto, Mayuko Kawabe, Yutaro Ohki, Ayaka Hayashi, Go Kanzaki, Akimitsu Kobayashi, Kei Matsumoto, Takafumi Yanagisawa, Shunsuke Tsuzuki, Koichiro Haruki, Hiroyuki Ueda, Nanae Matsuo, Jun Miki, Yudo Tanno, Nobuo Tsuboi, Takahiro Kimura, Toru Ikegami, Hiroyasu Yamamoto, Takashi Yokoo
{"title":"Living donor kidney transplantation after living donor liver transplantation from the same donor in a patient with autosomal dominant polycystic kidney disease: a case report.","authors":"Yatsumu Saito, Izumi Yamamoto, Mayuko Kawabe, Yutaro Ohki, Ayaka Hayashi, Go Kanzaki, Akimitsu Kobayashi, Kei Matsumoto, Takafumi Yanagisawa, Shunsuke Tsuzuki, Koichiro Haruki, Hiroyuki Ueda, Nanae Matsuo, Jun Miki, Yudo Tanno, Nobuo Tsuboi, Takahiro Kimura, Toru Ikegami, Hiroyasu Yamamoto, Takashi Yokoo","doi":"10.1007/s13730-026-01104-7","DOIUrl":"10.1007/s13730-026-01104-7","url":null,"abstract":"<p><p>Sequential living donor liver transplantation (LDLT) and living donor kidney transplantation (LDKT) from the same donor are extremely rare, with fewer than 10 such cases reported worldwide. These procedures present unique surgical, ethical, and immunological considerations. A 63-year-old man with autosomal dominant polycystic kidney disease (ADPKD) underwent LDLT from his wife for massive polycystic liver disease complicated by portal hypertension. Three years later, he received LDKT from the same donor following comprehensive evaluation for end-stage renal disease due to ADPKD. Pre-implantation kidney biopsy of the donor revealed thin basement membrane nephropathy with mild arteriosclerosis but preserved glomeruli. The postoperative course was uneventful, with immediate graft function and stable liver and kidney function at the six-month follow-up after kidney transplantation. This case demonstrates the feasibility of sequential living donor liver and kidney transplantation from a single donor in an exceptionally selected clinical setting; however, definitive conclusions regarding immune tolerance cannot be drawn from a single case, and careful donor selection, rigorous ethical safeguards, and long-term donor follow-up are essential.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13057154/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147626904","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
Sequential cryoglobulinemia-associated glomerulonephritis and light-chain amyloidosis in monoclonal gammopathy of renal significance. 序贯性冷球蛋白血症相关性肾小球肾炎和轻链淀粉样变在单克隆伽玛病中的肾脏意义。
IF 1
CEN Case Reports Pub Date : 2026-04-07 DOI: 10.1007/s13730-026-01108-3
Yuki Nakayama, Makoto Harada, Koji Hashimoto, Takashi Ehara, Yuji Kamijo
{"title":"Sequential cryoglobulinemia-associated glomerulonephritis and light-chain amyloidosis in monoclonal gammopathy of renal significance.","authors":"Yuki Nakayama, Makoto Harada, Koji Hashimoto, Takashi Ehara, Yuji Kamijo","doi":"10.1007/s13730-026-01108-3","DOIUrl":"10.1007/s13730-026-01108-3","url":null,"abstract":"<p><p>Kidney disorders associated with monoclonal gammopathy are diagnostically challenging because diverse kidney lesions can occur during the disease course. Here, we report a man in his early 60s who presented with edema, proteinuria, and impaired kidney function. Kidney biopsy revealed cryoglobulinemia-associated glomerulonephritis. Although bone marrow examination showed no increase in plasma cells, the patient was diagnosed with monoclonal gammopathy of renal significance (MGRS) and treated with steroids and cryofiltration, resulting in transient improvement. Later, proteinuria worsened, and kidney function declined despite intensified steroid therapy. A repeat kidney biopsy demonstrated AL (lambda) amyloid deposition in the glomeruli, leading to a diagnosis of AL amyloidosis. This case illustrates that distinct kidney pathologies can emerge sequentially in patients with MGRS and underscores the importance of repeat kidney biopsies when clinical findings change.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13057078/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147627234","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
Multidisciplinary introduction of peritoneal dialysis enabling ICU liberation and home-based care in Ebstein's anomaly with refractory ascites and CKD: a case report. 多学科介绍腹膜透析使ICU解放和家庭护理Ebstein异常难治性腹水和CKD:一个病例报告。
IF 1
CEN Case Reports Pub Date : 2026-04-07 DOI: 10.1007/s13730-026-01111-8
Yoshio Funahashi, Sosuke Fukui, Noritoshi Kato, Tomoki Kosugi, Masashi Mizuno, Shoichi Maruyama
{"title":"Multidisciplinary introduction of peritoneal dialysis enabling ICU liberation and home-based care in Ebstein's anomaly with refractory ascites and CKD: a case report.","authors":"Yoshio Funahashi, Sosuke Fukui, Noritoshi Kato, Tomoki Kosugi, Masashi Mizuno, Shoichi Maruyama","doi":"10.1007/s13730-026-01111-8","DOIUrl":"10.1007/s13730-026-01111-8","url":null,"abstract":"<p><p>Patients with refractory hepatic ascites and chronic kidney disease (CKD) secondary to right-sided heart failure pose unique therapeutic challenges when renal replacement therapy (RRT) is required in the intensive care unit (ICU). Conventional hemodialysis often worsens hypotension and venous congestion in these settings. Peritoneal dialysis (PD), by contrast, offers slow and continuous ultrafiltration that preserves hemodynamic stability, yet its use during vasopressor-dependent shock in the ICU is rarely described. We report the case of a 50-year-old woman with Ebstein's anomaly-related right heart failure, oliguria, CKD, and refractory ascites, admitted to the ICU with norepinephrine-dependent hypotension complicated by acute kidney injury (AKI). Repeated ascites drainage and attempts to taper vasopressor support led to recurrent oliguria, worsening renal function, and fluid overload. After multidisciplinary evaluation by nephrology, cardiology, critical care, anesthesia, and surgery, PD was identified as the only feasible strategy aligned with both the patient's hemodynamic status and her goal of home-based care. PD was initiated in the ICU beginning with low-volume exchanges and titrated to achieve effective ultrafiltration. PD enabled norepinephrine cessation, improvement in volume status, and eventual discharge home on ambulatory PD. This case demonstrates the utility of PD as a capable RRT modality to achieve both ICU liberation and home dialysis initiation in complex hepato-cardio-renal syndrome. Successful outcomes required cross-specialty collaboration and PD expertise within critical care. Broader recognition of PD's role in such settings may help expand therapeutic options for patients who are otherwise considered unsuitable for conventional RRT in the ICU.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13057097/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147627065","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
Post-streptococcal glomerulonephritis in Xakriabá indigenous children: case reports. xakriab<e:1>土著儿童链球菌感染后肾小球肾炎病例报告。
IF 1
CEN Case Reports Pub Date : 2026-04-07 DOI: 10.1007/s13730-026-01101-w
Dilceu Silveira Tolentino Júnior, Hilana Danielle Honorato Veloso, Maelso Bispo de Sousa, Maiele Bispo de Sousa, Roberto Carlos de Oliveira, Eliseu Miranda de Assis
{"title":"Post-streptococcal glomerulonephritis in Xakriabá indigenous children: case reports.","authors":"Dilceu Silveira Tolentino Júnior, Hilana Danielle Honorato Veloso, Maelso Bispo de Sousa, Maiele Bispo de Sousa, Roberto Carlos de Oliveira, Eliseu Miranda de Assis","doi":"10.1007/s13730-026-01101-w","DOIUrl":"10.1007/s13730-026-01101-w","url":null,"abstract":"<p><p>Post-streptococcal glomerulonephritis (PSGN) is a serious public health problem that mainly affects children. Although rare, the disease affected two children from the Xakriabá indigenous community, being the most common cause of acute nephritis in this age group and potentially leading to irreversible kidney damage if not treated promptly. We report two cases of PSGN in indigenous children that occurred in the village of Brejo, municipality of São João das Missões, Minas Gerais, Brazil. The cases were identified between August and December 2024. All cases were observed in male rural residents, aged between 8 and 10 years. The cause of the disease was a previous cutaneous infection by Streptococcus pyogenes, confirmed by titration of type O antistreptolysin. The predominant symptoms were hypertension, facial and lower limb edema, microscopic hematuria, and abdominal pain. All cases were treated in a hospital setting and recovered without complications. We recommend that health teams encourage regular hygiene practices and that the government invest in improvements in environmental sanitation to protect the kidney health of this vulnerable indigenous population.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 3","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13057157/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147627016","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
Post-streptococcal infection-related glomerulonephritis presenting with acute kidney injury and severe hypoglycaemia in a patient with diabetes. 1例糖尿病患者链球菌感染后并发急性肾损伤和严重低血糖的肾小球肾炎
IF 1
CEN Case Reports Pub Date : 2026-03-27 DOI: 10.1007/s13730-026-01102-9
Takuya Sugiura, Marina Yamamoto Kawase, Shun Ito, Reina Ueda, Toshikazu Ozeki, Yuki Yokoe, Kaoru Yasuda, Shoichi Maruyama
{"title":"Post-streptococcal infection-related glomerulonephritis presenting with acute kidney injury and severe hypoglycaemia in a patient with diabetes.","authors":"Takuya Sugiura, Marina Yamamoto Kawase, Shun Ito, Reina Ueda, Toshikazu Ozeki, Yuki Yokoe, Kaoru Yasuda, Shoichi Maruyama","doi":"10.1007/s13730-026-01102-9","DOIUrl":"10.1007/s13730-026-01102-9","url":null,"abstract":"<p><p>Infection-related glomerulonephritis (IRGN) in adults often arises in individuals with diabetes mellitus and can present with severe acute kidney injury (AKI). Because AKI impairs renal drug clearance, sulfonylureas (SU) may accumulate and precipitate profound hypoglycaemia. A 67 year-old man with type 2 diabetes mellitus was found unconscious and presented with severe hypoglycaemia (28 mg/dL) and marked AKI (serum creatinine 10.89 mg/dL) accompanied by oliguria and volume overload. Laboratory findings showed heavy proteinuria, haematuria, low complement C3, and elevated anti-streptolysin O titre, consistent with post-streptococcal IRGN. Haemodialysis was initiated for persistent oliguria. Renal biopsy was deferred because of a newly detected chronic subdural haematoma and deep vein thrombosis. Renal function gradually improved, and dialysis was discontinued by day 12. At 1 year, kidney function remained stable. This case illustrates that preceding infectious symptoms in diabetic patients may be subtle, delaying recognition of IRGN. The resulting AKI likely contributed to reduced SU clearance and severe hypoglycaemia at presentation. This case underscores the importance of considering IRGN as a cause of acute kidney injury in adults with recent or unrecognised infections, particularly in those with diabetes. When AKI occurs, clinicians should be vigilant regarding altered drug clearance and the potential for severe adverse events such as hypoglycaemia. Early evaluation of infection history, renal function, and medication safety is essential for timely diagnosis and management.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 2","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13031485/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147527032","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
Correction: A case of membranoproliferative glomerulonephritis-type IgA nephropathy modified by nintedanib. 更正:1例膜增生性肾小球肾炎型IgA肾病经尼达尼布治疗。
IF 1
CEN Case Reports Pub Date : 2026-03-24 DOI: 10.1007/s13730-026-01106-5
Yasushi Kunisho, Tadashi Sofue, Hirona Nagano, Masato Higashitani, Takafumi Shiga, Aiko Shiraishi, Yuhei Aoki, Keisuke Onishi, Emi Ibuki, Tetsuo Minamino
{"title":"Correction: A case of membranoproliferative glomerulonephritis-type IgA nephropathy modified by nintedanib.","authors":"Yasushi Kunisho, Tadashi Sofue, Hirona Nagano, Masato Higashitani, Takafumi Shiga, Aiko Shiraishi, Yuhei Aoki, Keisuke Onishi, Emi Ibuki, Tetsuo Minamino","doi":"10.1007/s13730-026-01106-5","DOIUrl":"10.1007/s13730-026-01106-5","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 2","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13013878/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147509743","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
Early recognition of atypical hemolytic uremic syndrome to prevent irreversible kidney injury: cardiac failure and refractory hypertension as critical clues in young patients. 早期识别非典型溶血性尿毒症综合征预防不可逆肾损伤:年轻患者心衰和难治性高血压是关键线索。
IF 1
CEN Case Reports Pub Date : 2026-03-24 DOI: 10.1007/s13730-026-01100-x
Nikolaos Tsiamis, Christo Kole, Eleni Stefanou, Christos Tountas, Hiras Theodoros, George Liapis, Anastasia Kitsiou
{"title":"Early recognition of atypical hemolytic uremic syndrome to prevent irreversible kidney injury: cardiac failure and refractory hypertension as critical clues in young patients.","authors":"Nikolaos Tsiamis, Christo Kole, Eleni Stefanou, Christos Tountas, Hiras Theodoros, George Liapis, Anastasia Kitsiou","doi":"10.1007/s13730-026-01100-x","DOIUrl":"10.1007/s13730-026-01100-x","url":null,"abstract":"<p><p>Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy that predominantly affects the kidneys but may cause severe extrarenal involvement, including cardiac dysfunction. Early recognition and timely complement inhibition are crucial for preventing irreversible organ injury. We report a 27-year-old man with no prior history who presented with hypertensive crisis, acute cardiogenic pulmonary edema with severe global systolic dysfunction and rapidly progressive renal failure. Despite aggressive antihypertensive therapy, diuretics, corticosteroids, hemodialysis, and seven sessions of plasmapheresis, multiorgan dysfunction persisted. Laboratory evaluation showed microangiopathic hemolytic anemia, thrombocytopenia, markedly elevated LDH, and low C3, consistent with complement-mediated TMA. Renal biopsy confirmed TMA compatible with aHUS. Eculizumab therapy was initiated, although delayed due to administrative authorization requirements, and resulted in rapid clinical stabilization and progressive improvement of cardiac function, with complete recovery at 6 months. Renal function, however, did not recover, and the patient remains dialysis-dependent. This case underscores the systemic nature of aHUS and the potential for reversible severe cardiomyopathy when complement inhibition is initiated early. Delayed treatment may permit irreversible renal injury despite full hematologic and cardiac remission, emphasizing the critical importance of timely diagnosis and intervention.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 2","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13013918/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147509749","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
PLA2R-positive primary membranous nephropathy in an adolescent with type 1 diabetes mellitus and autoimmune hypothyroidism: a case report. pla2r阳性原发性膜性肾病合并1型糖尿病和自身免疫性甲状腺功能减退1例报告
IF 1
CEN Case Reports Pub Date : 2026-03-16 DOI: 10.1007/s13730-026-01097-3
Pulla Swetha Madhuri, Mahesh Venkatachari
{"title":"PLA2R-positive primary membranous nephropathy in an adolescent with type 1 diabetes mellitus and autoimmune hypothyroidism: a case report.","authors":"Pulla Swetha Madhuri, Mahesh Venkatachari","doi":"10.1007/s13730-026-01097-3","DOIUrl":"10.1007/s13730-026-01097-3","url":null,"abstract":"<p><p>Membranous nephropathy (MN) is an uncommon cause of nephrotic syndrome in children, accounting for fewer than 5% of cases. Primary MN mediated by antibodies against the phospholipase A2 receptor (PLA2R) is particularly rare in the paediatric population. We report the case of a 14-year-old girl with type 1 diabetes mellitus and autoimmune hypothyroidism who presented with nephrotic syndrome and was subsequently diagnosed with PLA2R-positive primary MN. She presented with periorbital oedema, abdominal distension, and bilateral pitting pedal oedema. Laboratory investigations demonstrated severe hypoalbuminemia (serum albumin 1.2 g/dL), nephrotic-range proteinuria (24-hour urine protein excretion of 3.5 g/day), and preserved renal function (serum creatinine 0.3 mg/dL). Renal biopsy findings, supported by immunohistochemistry, confirmed PLA2R positivity. The patient was treated with four weekly doses of rituximab (375 mg/m2), resulting in complete clinical remission at 6 months. Serum albumin normalised to 4 g/dL, 24-hour urine protein excretion decreased to 250 mg/day, and serum PLA2R antibody levels fell below the normal range. This case highlights the importance of considering PLA2R-mediated MN in adolescents with nephrotic syndrome and the presence of multiple autoimmune comorbidities. It also underscores the diagnostic value of tissue PLA2R testing and demonstrates the efficacy of early rituximab-based B-cell-directed therapy in achieving complete remission while avoiding corticosteroid exposure.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":"15 2","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12992875/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147467085","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
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