法布里病与膜性肾病并存:病例报告

IF 0.8 4区 医学 Q4 UROLOGY & NEPHROLOGY Iranian journal of kidney diseases Pub Date : 2024-08-20
Yan Jin, Li Pen, Lei Lan, Jun Jiang
{"title":"法布里病与膜性肾病并存:病例报告","authors":"Yan Jin, Li Pen, Lei Lan, Jun Jiang","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>Fabry disease (FD) is a rare X-linked genetic disease that can coexist with multiple glomerulopathies. We report a 32-year-old female patient of FD coexisting with stage II membranous nephropathy (MN), who presented with proteinuria, normal renal function, and hypo-hidrosis as the only symptom. The renal biopsy manifested a subepithelial immunocomplex deposit in the glomeruli along with basement membrane thickening on light microscopy. Electron microscopy revealed myeloid bodies in some podocytes, which suggested the patient possibly coexistence with Fabry disease. The low activity of α-galactosidase A and one pathogenic heterozygous mutation (c.335G > Ap.Arg112His) in the α-galactosidase A gene confirmed the diagnosis of Fabry disease. This patient's son had the same gene mutation as his mother but without any symptoms at the time. Treatment with ramipril turned urine protein negative. The proteinuria had reoccurred, as shown by the presence of foamy urine, a protein to creatinine ratio of 1.54 g/g, and a blood albumin level of 34.4g/L. The patient was being treated with allisartan isoproxil. However, at the time, the urine protein did not turn negative.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"18 4","pages":"239-243"},"PeriodicalIF":0.8000,"publicationDate":"2024-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Coexistence of Fabry Disease and Membranous Nephropathy: A Case Report.\",\"authors\":\"Yan Jin, Li Pen, Lei Lan, Jun Jiang\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Fabry disease (FD) is a rare X-linked genetic disease that can coexist with multiple glomerulopathies. We report a 32-year-old female patient of FD coexisting with stage II membranous nephropathy (MN), who presented with proteinuria, normal renal function, and hypo-hidrosis as the only symptom. The renal biopsy manifested a subepithelial immunocomplex deposit in the glomeruli along with basement membrane thickening on light microscopy. Electron microscopy revealed myeloid bodies in some podocytes, which suggested the patient possibly coexistence with Fabry disease. The low activity of α-galactosidase A and one pathogenic heterozygous mutation (c.335G > Ap.Arg112His) in the α-galactosidase A gene confirmed the diagnosis of Fabry disease. This patient's son had the same gene mutation as his mother but without any symptoms at the time. Treatment with ramipril turned urine protein negative. The proteinuria had reoccurred, as shown by the presence of foamy urine, a protein to creatinine ratio of 1.54 g/g, and a blood albumin level of 34.4g/L. The patient was being treated with allisartan isoproxil. However, at the time, the urine protein did not turn negative.</p>\",\"PeriodicalId\":14610,\"journal\":{\"name\":\"Iranian journal of kidney diseases\",\"volume\":\"18 4\",\"pages\":\"239-243\"},\"PeriodicalIF\":0.8000,\"publicationDate\":\"2024-08-20\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Iranian journal of kidney diseases\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"UROLOGY & NEPHROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Iranian journal of kidney diseases","FirstCategoryId":"3","ListUrlMain":"","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0

摘要

法布里病(FD)是一种罕见的 X 连锁遗传病,可与多种肾小球疾病并存。我们报告了一名 32 岁的女性法布里病并发 II 期膜性肾病(MN)患者,她出现蛋白尿,肾功能正常,唯一的症状是低热。肾活检显示,肾小球上皮下免疫复合物沉积,光镜下基底膜增厚。电子显微镜检查发现一些荚膜细胞中存在髓样小体,这表明患者可能同时患有法布里病。α-半乳糖苷酶 A 的低活性和α-半乳糖苷酶 A 基因的一个致病性杂合突变(c.335G > Ap.Arg112His)证实了法布里病的诊断。该患者的儿子与母亲一样也有基因突变,但当时没有任何症状。使用雷米普利治疗后,尿蛋白转为阴性。蛋白尿再次出现,表现为尿液呈泡沫状,蛋白与肌酐的比率为 1.54 克/克,血白蛋白水平为 34.4 克/升。患者正在接受阿利沙坦异丙嗪治疗。然而,当时尿蛋白并未转阴。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
Coexistence of Fabry Disease and Membranous Nephropathy: A Case Report.

Fabry disease (FD) is a rare X-linked genetic disease that can coexist with multiple glomerulopathies. We report a 32-year-old female patient of FD coexisting with stage II membranous nephropathy (MN), who presented with proteinuria, normal renal function, and hypo-hidrosis as the only symptom. The renal biopsy manifested a subepithelial immunocomplex deposit in the glomeruli along with basement membrane thickening on light microscopy. Electron microscopy revealed myeloid bodies in some podocytes, which suggested the patient possibly coexistence with Fabry disease. The low activity of α-galactosidase A and one pathogenic heterozygous mutation (c.335G > Ap.Arg112His) in the α-galactosidase A gene confirmed the diagnosis of Fabry disease. This patient's son had the same gene mutation as his mother but without any symptoms at the time. Treatment with ramipril turned urine protein negative. The proteinuria had reoccurred, as shown by the presence of foamy urine, a protein to creatinine ratio of 1.54 g/g, and a blood albumin level of 34.4g/L. The patient was being treated with allisartan isoproxil. However, at the time, the urine protein did not turn negative.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Iranian journal of kidney diseases
Iranian journal of kidney diseases UROLOGY & NEPHROLOGY-
CiteScore
2.50
自引率
0.00%
发文量
43
审稿时长
6-12 weeks
期刊介绍: The Iranian Journal of Kidney Diseases (IJKD), a peer-reviewed journal in English, is the official publication of the Iranian Society of Nephrology. The aim of the IJKD is the worldwide reflection of the knowledge produced by the scientists and clinicians in nephrology. Published quarterly, the IJKD provides a new platform for advancement of the field. The journal’s objective is to serve as a focal point for debates and exchange of knowledge and experience among researchers in a global context. Original papers, case reports, and invited reviews on all aspects of the kidney diseases, hypertension, dialysis, and transplantation will be covered by the IJKD. Research on the basic science, clinical practice, and socio-economics of renal health are all welcomed by the editors of the journal.
期刊最新文献
Early Prediction of Acute Kidney Injury in Living Donor Liver Transplantation by Serum Cystatin C Concentration at the End of the Surgery. Erratum:Trends in Research on Acute Kidney Injury: A Bibliometric Analysis of Academic Journals Published Between the Years 2000 and 2022. Establishing a Risk Model for Diabetic Nephropathy and Addressing the Therapeutic Effect of  Combined Epalrestat- Dapagliflozin Regimen. Exploring the Key Pathogenesis and Potential Intervention Targets of Sulforaphane in Acute Kidney Injury in Sepsis Based on Bioinformatics. New Onset Systemic Lupus Erythematosus with Unusual Presentation and Multi-Organ Involvement after Covid-19 Vaccination in a Pediatric Patient: A Case Report.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1