CEN Case Reports最新文献

{"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":null,"pages":null},"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}

{"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":null,"pages":null},"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}

{"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":null,"pages":null},"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}

{"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":null,"pages":null},"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}

{"title":"Multilocular cystic nephroma in an adult: a diagnostic quandary.","authors":"Ritu Verma, Jyoti Verma, Neelima Gupta","doi":"10.1007/s13730-024-00852-8","DOIUrl":"10.1007/s13730-024-00852-8","url":null,"abstract":"<p><p>Multilocular cystic nephroma (MLCN) is an unusual, benign slow-growing renal cystic neoplasm which mimics other cystic renal lesions and has such clinical, radiological, and morphological features that causes diagnostic dilemma. MLCN lies in the spectrum of mixed epithelial and stromal tumor (MEST) family of kidney. According to World Health Organization (WHO 2016 classification), MEST encompasses spectrum of tumors ranging from predominantly cystic tumors, adult cystic nephroma (ACN) to tumors that are variably solid (MEST), thus creating diagnostic dilemma. Moreover, it has several benign and malignant differentials due to its several overlapping histomorphological features which when not cautiously dealt with may result in misdiagnosing it as malignant lesion. We hereby present a case of a woman in late twenties who presented with left flank swelling and pain since 6 months which was misdiagnosed as renal cell carcinoma on radiology which turned out to be ACN on histology and further verified on immunohistochemistry.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139982486","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A case of non-lupus full-house nephropathy diagnosed by kidney biopsy but observed IgA nephropathy on second biopsy.","authors":"Yoichi Iwafuchi, Tetsuo Morioka, Yuko Oyama, Ichiei Narita","doi":"10.1007/s13730-024-00860-8","DOIUrl":"10.1007/s13730-024-00860-8","url":null,"abstract":"<p><p>We describe a case of full-house nephropathy without any underlying disease, including systemic lupus erythematosus. A 40-year-old woman was referred to our hospital with mild proteinuria and microscopic hematuria. The patient was diagnosed with immune complex-mediated glomerulonephritis with a predominant mesangioproliferative pattern based on renal histopathological results using full-house immunofluorescence staining. She showed no clinical criteria for the diagnosis of systemic lupus erythematosus, except for kidney disorders, and tested negative for antinuclear antibodies throughout her clinical course. However, in the second kidney biopsy, no C1q or C4 were detected in the immunofluorescence study, suggesting an immunoglobulin A nephropathy-like pattern. The patient responded favorably to corticosteroid treatment. We found a heterozygous CFHR3-CFHR1 deletion. The association between full-house nephropathy and CFHR3-CFHR1 deletion is unknown, but its influence on the histological pattern in our case is suspected. This indicates the diversity in the pathogenesis of non-lupus full-house nephropathy and warrants further investigation.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442713/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139989440","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effective calcineurin inhibitor treatment in adult-onset steroid-resistant nephrotic syndrome with a novel splice donor site variant of TRPC6: a case report.","authors":"Tomoki Nagasaka, Kiyotaka Uchiyama, Eriko Yoshida Hama, Daiki Kojima, Kenji Kaneko, Norifumi Yoshimoto, Itaru Yasuda, Mamiko Yamada, Fuyuki Miya, Hisato Suzuki, Takaya Tajima, Shintaro Yamaguchi, Kaori Hayashi, Takeshi Kanda, Akinori Hashiguchi, Kenjiro Kosaki, Hiroshi Itoh","doi":"10.1007/s13730-024-00935-6","DOIUrl":"https://doi.org/10.1007/s13730-024-00935-6","url":null,"abstract":"<p><p>Transient receptor potential canonical 6 (TRPC6) variants, which were initially detected in adult-onset familial focal segmental glomerulosclerosis (FSGS), were also identified in pediatric-onset one. Here, we present a patient with adult-onset steroid-resistant nephrotic syndrome (SRNS) who harbored a likely pathogenic TRPC6 variant and partially responded to calcineurin inhibitors (CNIs). A 44-year-old woman with stable rheumatoid arthritis, systemic lupus erythematosus, and Sjögren's syndrome was presented with nephrotic syndrome. Her renal biopsy results showed minor glomerular abnormalities. Upon admission, she was treated with steroids for around 4 weeks, but it was ineffective. After 1-2 weeks of cyclosporine A (CyA) administration, urine output increased, renal function improved without a decrease in proteinuria, and she was discharged. Her renal function was maintained for 2 months, but after a CyA dose reduction, she was again admitted to the hospital due to relapsing edema, decreased urine output, and worsening renal function. CyA was replaced by tacrolimus (TAC). A second renal biopsy showed nearly the same findings as the first except for tubulointerstitial lesions. After 1-2 weeks of TAC administration, urine output increased, and renal function improved. However, urinary protein levels did not decrease as before. After discharge, a whole exome analysis revealed a heterozygous splice donor site variant NM_004621.6;c.2644 + 1G > A in TRPC6. Genetic testing identified a novel splice donor site variant of TRPC6 in a patient with adult-onset SRNS, which prevented unnecessary steroid continuation. The safety and efficacy of CNI in TRPC6 glomerulopathy must be evaluated in future larger studies with longer follow-up.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342437","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}

{"title":"Successful treatment of severe renal failure caused by malignant hypertension using a combination of renin-angiotensin-aldosterone system inhibitors: a case report.","authors":"Wataru Harada, Yujiro Maeoka, Akira Takahashi, Mahoko Yoshida, Yosuke Osaki, Naoki Ishiuchi, Kensuke Sasaki, Takao Masaki","doi":"10.1007/s13730-024-00934-7","DOIUrl":"https://doi.org/10.1007/s13730-024-00934-7","url":null,"abstract":"<p><p>Marked activation of the renin-angiotensin-aldosterone system (RAAS) plays an important role in malignant hypertension (MHT) by worsening hypertension and renal function. The rates of readmission for severe hypertension and cardiovascular disease in such emergencies are high, suggesting that suppression of the RAAS may be inadequate during the acute phase in some cases. This report presents a case of MHT complicated with renal insufficiency (creatinine 3.93 mg/dL) and massive proteinuria, in which antihypertensive therapy, including an angiotensin receptor blocker, aliskiren, and spironolactone, normalized blood pressure (BP) and preserved renal function. Plasma renin activity was extremely high (131.9 ng/mL/h) on admission but normalized within almost 2 weeks. Although aliskiren and spironolactone were discontinued before discharge, BP was well controlled and renal function was further improved (creatinine 1.14 mg/dL) at follow-up 24 months later. This case of renal failure induced by MHT was successfully treated with a combination of RAAS inhibitors during the acute phase. The controlled BP and improved renal function in this patient suggest that adequate suppression of the RAAS cascade during the acute phase is potentially effective in terms of breaking the vicious cycle of MHT with hyperreninemia.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342438","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}

{"title":"Multiple relapses of sigmoid volvulus in a patient on automated peritoneal dialysis: the role of an elongated sigmoid colon and peritoneal dialysis as potential predisposing factors.","authors":"Keitaro Uehara, Shunsuke Yamada, Yusuke Mizuuchi, Takanari Kitazono, Toshiaki Nakano","doi":"10.1007/s13730-024-00930-x","DOIUrl":"https://doi.org/10.1007/s13730-024-00930-x","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280881","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}

{"title":"MPO-ANCA-positive rapidly progressive glomerulonephritis after COVID-19 vaccination during treatment of plaque psoriasis with bimekizumab.","authors":"Takuya Sugiura, Tomohito Doke, Akihito Tanaka, Yuka Sato, Kayaho Maeda, Kazuhiro Furuhashi, Noritoshi Kato, Tomoki Kosugi, Shoichi Maruyama","doi":"10.1007/s13730-024-00927-6","DOIUrl":"https://doi.org/10.1007/s13730-024-00927-6","url":null,"abstract":"<p><p>A 75-year-old man presented with MPO-ANCA-positive rapidly progressive glomerulonephritis after COVID-19 vaccination during the treatment of plaque psoriasis vulgaris with bimekizumab. Bimekizumab, an anti-IL17 monoclonal antibody, was regularly administered to control the activity of plaque psoriasis. After receiving the sixth COVID-19 vaccine, his kidney function rapidly declined over the course of weeks. Urinalysis showed microscopic hematuria and proteinuria with deformed red blood cells and granular cast. The immunology test was positive for MPO-ANCA. The patient was clinically diagnosed with MPO-ANCA-associated glomerulonephritis. As the patient lost his appetite and developed lower extremity edema with low eGFR (< 15 ml/min/1.73m²) on admission day, hemodialysis induction was initiated along with methylprednisolone pulse, followed by oral prednisolone. The kidney function and urine volume were improved in response to immunosuppressive therapy, and withdrawal from hemodialysis was considered. However, the patient developed a catheter infection due to methicillin-sensitive Staphylococcus aureus 2 weeks after the initial prednisolone treatment, causing a decline in kidney function. Antibiotics treatment for the catheter infection was effective, but kidney function remained low, resulting in dependence on regular hemodialysis. COVID-19 vaccination provides significant improvement in overall prognosis; however, there have been reports of kidney function decline and exacerbation of hematuria in patients with IgA nephropathy following vaccination. The incidence of MPO-ANCA-associated glomerulonephritis after COVID-19 vaccination was rare. Data accumulation is warranted to understand the risk factors for secondary MPO-ANCA glomerulonephritis after COVID-19 vaccination. Regular monitoring of urinalysis and kidney function after COVID-19 vaccination is recommended in patients with psoriasis vulgaris treated with IL17 monoclonal antibodies.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280880","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}