CEN Case Reports最新文献

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Clostridium perfringens-induced massive hemolysis treatment with blood purification to target toxins: a case report. 针对毒素的血液净化治疗产气荚膜梭菌诱发的大量溶血:一份病例报告。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-03-04 DOI: 10.1007/s13730-024-00857-3
I Reffo, M Domini, M Cevolani, G Del Fabro, D Rufolo, S Venturini, L Pinciroli, D Tonin, M Avolio, M Crapis, G Basaglia, M Balbi, G Nadalin
{"title":"Clostridium perfringens-induced massive hemolysis treatment with blood purification to target toxins: a case report.","authors":"I Reffo, M Domini, M Cevolani, G Del Fabro, D Rufolo, S Venturini, L Pinciroli, D Tonin, M Avolio, M Crapis, G Basaglia, M Balbi, G Nadalin","doi":"10.1007/s13730-024-00857-3","DOIUrl":"10.1007/s13730-024-00857-3","url":null,"abstract":"<p><p>Clostridium perfringens can rarely cause severe systemic infections, usually from an abdominal source, associated with massive hemolysis, which is usually fatal. Hemolytic anemia and acute renal injury resulting from toxin action are critical for the development of multiple organ dysfunction syndrome (MODs), making this condition a real emergency, requiring multispecialty skills and aggressive multimodal therapies. We herein describe a case of septic shock from acute cholecystitis with massive hemolysis caused by C. perfringens in a 55 year-old man that was successfully treated with early blood purification and continuous renal replacement therapy (CRRT) along with antibiotic therapy and surgery. The effect of the enormous amount of toxins produced by Clostridium which elicit a strong cytokine response and the damage caused by the hemolysis products are the main pathogenetic mechanisms of this rare but lethal clinical entity. The main goal of treatment is to remove toxins from plasma, block toxin action, and further production by achieving bacterial killing with antimicrobial agents and controlling the infectious focus, remove waste products and prevent or limit multiorgan damage. Blood purification techniques play an important role due to a strong pathophysiological rationale, as they can remove toxins and cytokines as well as cell-free products from plasma and also replace renal function. Although this condition is rare and robust data are lacking, blood purification techniques for C. perfringens-induced massive hemolysis are promising and should be further explored.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"391-396"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11444022/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140020998","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 seronegative microscopic polyangiitis following the diagnosis of renal pelvic carcinoma. 一例肾盂癌诊断后的血清阴性显微镜下多血管炎病例。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-03-04 DOI: 10.1007/s13730-024-00856-4
Narumichi Iwamura, Kanako Tsutsumi, Yuki Ueno, Yasuhisa Tamura, Toshiaki Nakano
{"title":"A case of seronegative microscopic polyangiitis following the diagnosis of renal pelvic carcinoma.","authors":"Narumichi Iwamura, Kanako Tsutsumi, Yuki Ueno, Yasuhisa Tamura, Toshiaki Nakano","doi":"10.1007/s13730-024-00856-4","DOIUrl":"10.1007/s13730-024-00856-4","url":null,"abstract":"<p><p>The incidence rate of malignancy in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is higher than that in the general population. Malignancy has been indicated to be a risk factor or inducer of AAV. Herein, we report the case of a healthy 84-year-old man with seronegative microscopic polyangiitis (MPA) after the diagnosis of renal pelvic carcinoma. Four weeks before admission, his estimated glomerular filtration rate (eGFR) was 85 ml/min/1.73 m<sup>2</sup>, and no hematuria or proteinuria was detected. Renal biopsy on admission revealed invasive urothelial carcinoma of the right renal pelvis. On day 15, his eGFR decreased to 30 ml/min/1.73 m<sup>2</sup> without any incitement. The renal specimen extracted via right robot-assisted nephroureterectomy indicated the presence of ANCA-associated glomerulonephritis. On day 37, urinary protein/urinary creatinine level of 6.48 g/gCre, serum albumin level of 2.1 mg/dL, and eGFR of 20 ml/min/1.73 m<sup>2</sup> indicated the presence of nephrotic syndrome. His blood sputum was analyzed via chest computed tomography, which revealed alveolar hemorrhage. Although his myeloperoxidase-ANCA was negative, he was diagnosed with MPA based on the 2022 American College of Rheumatology/European League Against Rheumatism classification criteria. This is the first case report of  MPA or AAV complicated with renal pelvic carcinoma. The clinical indicators demonstrated that renal pelvic carcinoma preceded the onset of MPA. The spatial proximity of both diseases indicated that renal pelvic carcinoma had some influence on MPA development via the mechanism of inflammatory cytokines or neutrophil extracellular traps. Our report may be useful in elucidating the mechanism of MPA development.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"381-390"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442719/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140020997","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 Duchenne muscular dystrophy recovered from prolonged ischemic kidney injury which emerged with a normal creatinine level. 一例杜兴氏肌肉萎缩症患者从长期缺血性肾损伤中恢复,肌酐水平正常。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-03-04 DOI: 10.1007/s13730-024-00858-2
Kensuke Daikoku, Hinako Kondo, Masataka Kudo, Akira Sugiura
{"title":"A case of Duchenne muscular dystrophy recovered from prolonged ischemic kidney injury which emerged with a normal creatinine level.","authors":"Kensuke Daikoku, Hinako Kondo, Masataka Kudo, Akira Sugiura","doi":"10.1007/s13730-024-00858-2","DOIUrl":"10.1007/s13730-024-00858-2","url":null,"abstract":"<p><p>Duchenne muscular dystrophy (DMD) is an inherited disease characterized by progressive degeneration of the skeletal muscles. Renal dysfunction in patients with DMD has recently become more apparent as life expectancy has increased owing to advances in respiratory devices and heart failure therapies. A 23-year-old man with DMD who required nasal tube feeding was referred to our hospital with a 4-month history of renal dysfunction and anemia. The patient's serum creatinine (sCr) level was within the normal range (0.84 mg/dL), but his serum cystatin C level and estimated glomerular filtration rate calculated by cystatin C (5.90 mg/L and 7.5 mL/min/1.73 m<sup>2</sup>, respectively) indicated severe renal impairment. A urinalysis revealed elevated levels of protein and tubular markers. The patient's hemoglobin and erythropoietin levels indicated renal anemia. Hypotension, a collapsed inferior vena cava, and a poor tube feeding episode suggested that the kidney injury was due to renal ischemia, which progressed to tubulointerstitial kidney injury, an intrinsic kidney injury. The angiotensin-converting enzyme inhibitors and beta-blockers were discontinued, and extracellular fluid was infused. Thereafter, the patient's renal function recovered. Subsequently, the patient's urinary findings and anemia improved. Although advances in cardioprotective agents are expected to improve the prognosis of patients with DMD, it is important to consider that the number of patients with kidney injury due to renal ischemia may increase and that it is difficult to evaluate renal function using sCr level in patients with DMD because of decreased skeletal muscle mass.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"397-402"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11444038/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140020996","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 pseudo-Bartter/Gitelman syndrome caused by long-term laxative abuse, leading to end-stage kidney disease. 一例因长期滥用泻药导致终末期肾病的假性巴特/吉特曼综合征。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-02-02 DOI: 10.1007/s13730-024-00851-9
Atsushi Kondo, Kunihiko Yoshiya, Nana Sakakibara, China Nagano, Tomoko Horinouchi, Kandai Nozu
{"title":"A case of pseudo-Bartter/Gitelman syndrome caused by long-term laxative abuse, leading to end-stage kidney disease.","authors":"Atsushi Kondo, Kunihiko Yoshiya, Nana Sakakibara, China Nagano, Tomoko Horinouchi, Kandai Nozu","doi":"10.1007/s13730-024-00851-9","DOIUrl":"10.1007/s13730-024-00851-9","url":null,"abstract":"<p><p>Pseudo-Bartter/Gitelman syndrome (PBS/PGS) is a disorder that presents with hypokalemia and metabolic alkalosis resembling Gitelman syndrome (GS) due to secondary factors, such as lifestyle and /or medicines. Notably, PBS/PGS is more likely to cause renal dysfunction than GS. We report the first case of PBS/PGS due to long-term laxative abuse leading to end-stage kidney disease (ESKD). The patient was a 49-year-old woman with a history of constipation since school, who had used excessive doses of laxatives on her own judgment for nine years at least from 22 years of age. Two years later, blood tests revealed hypokalemia (serum K 3.1 mEq/L), and nine years later, the patient's renal function began to deteriorate (Cr-eGFR 48.7 mL/min/1.73 m<sup>2</sup>). Since abuse of laxatives was suspected as the cause, it was changed to the proper dosage of laxatives. At 33 years, the patient developed acute kidney injury (AKI), due to a urinary tract infection, and required intensive treatment, including hemodialysis. Although the patient was eventually weaned off dialysis, the renal function did not recover to pre-AKI levels. In suspected GS, comprehensive genetic testing for renal disease-related genes was performed; however, no obvious pathogenic variants were identified. Thereafter, despite decreasing the laxative doses and potassium supplementation, her renal function continued to decline. At 49 years, the patient developed ESKD and was started on maintenance hemodialysis. PBS/PGS is a disease that can lead to ESKD. An early diagnosis of PBS/PGS is crucial to prevent renal function deterioration, and the underlying causes should be removed immediately.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"326-329"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442729/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139671382","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 late-onset organizing pneumonia following COVID-19 infection in a post-kidney transplant patient. 一例肾移植术后患者感染 COVID-19 后引发的迟发性组织化肺炎。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-02-17 DOI: 10.1007/s13730-023-00849-9
Kumiko Fujieda, Shoji Saito, Akihito Tanaka, Kazuhiro Furuhashi, Yosinari Yasuda, Yuta Sano, Masashi Kato, Shoichi Maruyama
{"title":"A case of late-onset organizing pneumonia following COVID-19 infection in a post-kidney transplant patient.","authors":"Kumiko Fujieda, Shoji Saito, Akihito Tanaka, Kazuhiro Furuhashi, Yosinari Yasuda, Yuta Sano, Masashi Kato, Shoichi Maruyama","doi":"10.1007/s13730-023-00849-9","DOIUrl":"10.1007/s13730-023-00849-9","url":null,"abstract":"<p><p>A 50-year-old man who had undergone a living-donor kidney transplant 12 years prior for chronic renal failure due to autosomal dominant polycystic kidney disease contracted coronavirus disease 19 (COVID-19). He had a positive antigen test, mild symptoms, sore throat, and fever of 37.9 ℃. The patient was treated with molnupiravir for 5 days, and the symptoms disappeared 5 days after onset. However, 10 days after onset, he developed a fever of approximately 37 ℃ and a non-productive cough; 27 days after onset, the patient was hospitalized for anorexia and a worsening respiratory condition. The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) antigen test results on admission were negative, and no antiviral medications were administered against SARS-CoV-2. Computed tomography revealed extensive ground-glass opacities in both lung fields. The patient was treated with steroid pulse therapy, ceftriaxone, atovaquone, azithromycin, and respiratory management using a high-flow nasal cannula. The combined therapies were successful, and the patient was managed with a nasal oxygen cannula after 3 days. Oxygen administration was discontinued after 6 days of hospitalization, and the patient was discharged after 14 days. Based on the laboratory findings, bacterial, interstitial, and Pneumocystis pneumonia were unlikely. The success of the steroid pulse therapy suggested that respiratory failure was caused by pneumonia due to the immune response after COVID-19 infection.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"346-350"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442844/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139897858","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
Hypereosinophilia-associated acute intradialytic hypotension: a report of three cases and literature review. 嗜铬细胞增多症相关急性肾内低血压:三例病例报告和文献综述。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-01-26 DOI: 10.1007/s13730-024-00850-w
Awaisshafiq Fukumi, Mari Tanaka, Akane Sugae, Yuki Ishida, Hiroko Yamamoto, Tomoka Watanabe, Chiho Fukushima, Miho Miyauchi, Mariko Teragaki, Kotaro Maeda, Yohtaro Takami, Sachio Iwanari, Masaki Ikeda, Hiroya Takeoka
{"title":"Hypereosinophilia-associated acute intradialytic hypotension: a report of three cases and literature review.","authors":"Awaisshafiq Fukumi, Mari Tanaka, Akane Sugae, Yuki Ishida, Hiroko Yamamoto, Tomoka Watanabe, Chiho Fukushima, Miho Miyauchi, Mariko Teragaki, Kotaro Maeda, Yohtaro Takami, Sachio Iwanari, Masaki Ikeda, Hiroya Takeoka","doi":"10.1007/s13730-024-00850-w","DOIUrl":"10.1007/s13730-024-00850-w","url":null,"abstract":"<p><p>Occasionally, patients undergoing dialysis develop acute severe hypotension that requires interruption of dialysis within minutes of initiating every dialysis session. Although the underlying causes of recurrent intradialytic hypotension are evaluated extensively, including dialysis-associated allergic reactions or other possible causes, the definitive cause is sometimes missed. Dialysis is a life-sustaining procedure; therefore, prompt identification and management of the underlying cause of dialysis intolerance are crucial. Herein, we report three cases of patients undergoing dialysis who presented with hypereosinophilia-associated acute intradialytic hypotension. All three patients developed acute severe hypotension within minutes after the start of every dialysis session. The prescriptions for dialysis were changed, but episodes of intradialytic hypotension persisted. Pretreatment with methylprednisolone given intravenously before the dialysis session was also ineffective. All patients had hypereosinophilia (> 1500/μL) of different etiology. Eosinophil-lowering therapy with 0.5 mg/kg of prednisolone given orally daily was initiated, and all of them could restart dialysis without any hypotensive episodes within a few days. Our case report and literature review indicated that hypereosinophilia, regardless of its etiology, could result in severe acute hypotension shortly after the start of dialysis session. The oral administration of prednisolone daily was highly effective on hypereosinophilia-associated intradialytic hypotension, while pretreatment with intravenous corticosteroid therapy just before dialysis had no effect. Hypereosinophilia-associated acute intradialytic hypotension is an under-recognized condition; therefore, clinicians need to be aware of this clinical entity and initiate effective treatment strategies. We also provide a brief summary of previously published cases.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"311-317"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442880/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139563652","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
Hemodialysis treatment of vancomycin-induced drug reaction with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome in a patient undergoing peritoneal dialysis. 一名接受腹膜透析的患者因万古霉素诱发的药物反应伴有嗜酸性粒细胞增多和全身症状/药物过敏综合征而接受血液透析治疗。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-02-10 DOI: 10.1007/s13730-023-00847-x
Ryunosuke Mitsuno, Takashin Nakayama, Kiyotaka Uchiyama, Norifumi Yoshimoto, Ei Kusahana, Kohkichi Morimoto, Jun Yoshino, Tadashi Yoshida, Takeshi Kanda, Shintaro Yamaguchi, Kaori Hayashi
{"title":"Hemodialysis treatment of vancomycin-induced drug reaction with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome in a patient undergoing peritoneal dialysis.","authors":"Ryunosuke Mitsuno, Takashin Nakayama, Kiyotaka Uchiyama, Norifumi Yoshimoto, Ei Kusahana, Kohkichi Morimoto, Jun Yoshino, Tadashi Yoshida, Takeshi Kanda, Shintaro Yamaguchi, Kaori Hayashi","doi":"10.1007/s13730-023-00847-x","DOIUrl":"10.1007/s13730-023-00847-x","url":null,"abstract":"<p><p>Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as drug-induced hypersensitivity syndrome (DIHS), is a severe drug-induced hypersensitivity reaction with 10% mortality. To date, there is insufficient evidence regarding the association between DRESS/DIHS and serum levels of vancomycin (VCM). Here, we report the case of a 46-year-old woman undergoing peritoneal dialysis who developed VCM-induced DRESS/DIHS. She was hospitalized for peritonitis with abdominal pain and treated with VCM. On day 10 of hospitalization, her abdominal symptoms improved; however, fever, skin rash, lymphadenopathy, eosinophilia, atypical lymphocytes, and liver and renal dysfunction developed. Based on the clinical course and laboratory findings, we diagnosed the patient with DRESS/DIHS due to VCM. Since her serum VCM concentration was high at 39.8 μg/mL, hemodialysis (HD) was performed to remove VCM, which caused her symptoms to improve. However, serum levels of VCM rebounded and the same symptoms recurred. Therefore, we re-performed HD; no further relapse occurred. This clinical course showed that increased serum VCM levels were associated with DRESS/DIHS onset and severity, suggesting that it is a blood level-dependent disease and that removal of VCM by HD is a potential therapeutic option.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"339-345"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442891/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139711593","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
Renal dysfunction caused by severe hypothyroidism diagnosed by renal biopsy: a case report. 通过肾活检确诊的严重甲状腺功能减退症导致的肾功能障碍:病例报告。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-02-28 DOI: 10.1007/s13730-024-00853-7
Hiroki Tani, Shuma Hirashio, Akihiro Tsuda, Yoshiro Tachiyama, Shigeo Hara, Takao Masaki
{"title":"Renal dysfunction caused by severe hypothyroidism diagnosed by renal biopsy: a case report.","authors":"Hiroki Tani, Shuma Hirashio, Akihiro Tsuda, Yoshiro Tachiyama, Shigeo Hara, Takao Masaki","doi":"10.1007/s13730-024-00853-7","DOIUrl":"10.1007/s13730-024-00853-7","url":null,"abstract":"<p><p>There is a close relationship between thyroid dysfunction and renal dysfunction. However, thyroid dysfunction can unfortunately result in inaccurate measurements of serum creatinine and cystatin C levels. The chronic decrease in cardiac output due to hypothyroidism can reduce renal plasma flow (RPF) resulting in renal dysfunction. We report the case of a 36-year-old male in whom renal dysfunction detected during a company health check-up was found to be caused by severe hypothyroidism. His serum creatinine levels showed poor results, but serum cystatin C levels were within the normal range. The physician thus prioritized serum cystatin C for assessing the patient's renal function, and concluded that his renal function was normal. He subsequently visited our hospital, aged 36 years, for a comprehensive examination. His serum creatinine level was 1.88 mg/dL and his serum cystatin C level was 0.75 mg/dL, indicating an unusual discrepancy between the two measurements. The patient also presented with fatigue, suggesting hypothyroidism, and we therefore evaluated his thyroid function. His free thyroxine level was below the sensitivity of the assay, while his thyroid-stimulating hormone level was > 100 μIU/mL. A renal biopsy was performed to further explore the underlying cause of his renal dysfunction, which suggested that reduced RPF could be the leading cause of his renal ischemia, with no indications of chronic glomerulonephritis or other abnormalities. His hypothyroidism and renal function improved after thyroid hormone replacement therapy. Given the limited reports of renal biopsy tissue examination during the acute phase of hypothyroidism, the current case provides important information regarding the diagnosis of renal dysfunction in patients with hypothyroidism.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"366-372"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442827/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139982487","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
Lipoprotein glomerulopathy: a rare cause of steroid-resistant nephrotic syndrome in a child. 脂蛋白肾小球病:导致儿童类固醇耐受性肾病综合征的罕见病因。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-03-04 DOI: 10.1007/s13730-024-00861-7
Kinnari Vala, Kanisha Shah, Shahenaz Kapadia, Mahipal Khandelwal, Amit Jojera, Shailesh Soni, Ashka Prajapati, Anshuman Saha
{"title":"Lipoprotein glomerulopathy: a rare cause of steroid-resistant nephrotic syndrome in a child.","authors":"Kinnari Vala, Kanisha Shah, Shahenaz Kapadia, Mahipal Khandelwal, Amit Jojera, Shailesh Soni, Ashka Prajapati, Anshuman Saha","doi":"10.1007/s13730-024-00861-7","DOIUrl":"10.1007/s13730-024-00861-7","url":null,"abstract":"<p><p>Lipoprotein glomerulopathy (LPG) is a rare condition of renal lipidosis characterized by lipoprotein thrombi in glomeruli, an abnormal plasma lipoprotein profile, and a marked increase in serum apolipoprotein E (apo E) levels. It is a monogenic disorder with autosomal dominant inheritance and the average age of presentation is 32 years (4-69 years). It is rare in children. The presentation can be nephrotic syndrome, hematuria, or progressive renal failure. Here we report the first described case of LPG in an Indian 7.5-year-old boy who presented with steroid-resistant nephrotic syndrome with normal renal function. A renal biopsy was suggestive of lipoprotein glomerulopathy. The detection of a pathogenic variant in apo E, Kyoto type, by exome sequencing, confirmed the diagnosis of lipoprotein glomerulopathy. Complete response was achieved with Angiotensin-converting Enzyme inhibitor and fenofibrates.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"403-407"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442800/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140027501","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 child with TSC2/PKD1 contiguous gene deletion syndrome successfully treated with tolvaptan for rapidly enlarging renal cysts. 一名患有TSC2/PKD1连续基因缺失综合征的儿童因快速增大的肾囊肿而接受托伐普坦治疗,并获得成功。
IF 1
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-02-27 DOI: 10.1007/s13730-024-00854-6
Chika Muroga, Hiroki Yokoyama, Ryo Kinoshita, Daisuke Fujimori, Yuko Yamada, Tohru Okanishi, Naoya Morisada, Kandai Nozu, Noriyuki Namba
{"title":"A child with TSC2/PKD1 contiguous gene deletion syndrome successfully treated with tolvaptan for rapidly enlarging renal cysts.","authors":"Chika Muroga, Hiroki Yokoyama, Ryo Kinoshita, Daisuke Fujimori, Yuko Yamada, Tohru Okanishi, Naoya Morisada, Kandai Nozu, Noriyuki Namba","doi":"10.1007/s13730-024-00854-6","DOIUrl":"10.1007/s13730-024-00854-6","url":null,"abstract":"<p><p>Tolvaptan, a vasopressin receptor antagonist, has been shown to be effective in the treatment of renal cysts in ADPKD. However, tolvaptan is not indicated for pediatric patients, and reports of its use are rare, making its efficacy and adverse reactions unclear. Herein, we present the case of an 11-year-old girl who had vitiligo from birth. She was diagnosed with West syndrome at 6 months of age and tuberous sclerosis at 2 years of age. At the age of 6 years, an abdominal magnetic resonance imaging (MRI) revealed multiple bilateral renal cysts, and she was diagnosed with ADPKD. Abdominal MRI scans performed at 10 years and 11 years showed rapid renal cyst enlargement, and the renal prognosis was judged to be poor. The patient was treated with tolvaptan to delay cyst exacerbation. There were no apparent adverse events after the initiation of treatment, and the MRI performed 12 months after treatment initiation showed that renal cyst enlargement was suppressed. The results suggest that tolvaptan may be effective in pediatric patients with severe ADPKD who have rapidly enlarging renal cysts, although evaluation of renal cyst enlargement and side effects should be continued.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"351-355"},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442821/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139971099","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}
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