• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

相似文献

1
Home Albumin Infusion Therapy, Another Alternative Treatment in Patients With Congenital Nephrotic Syndrome of the Finnish Type.家庭白蛋白输注疗法,芬兰型先天性肾病综合征患者的另一种替代治疗方法。
Front Pediatr. 2021 Jan 14;8:614535. doi: 10.3389/fped.2020.614535. eCollection 2020.
2
Unilateral nephrectomy for young infants with congenital nephrotic syndrome of the Finnish type.单侧肾切除术治疗芬兰型先天性肾病综合征的婴儿。
Clin Exp Nephrol. 2022 Feb;26(2):162-169. doi: 10.1007/s10157-021-02141-5. Epub 2021 Sep 28.
3
Recurrence of nephrotic syndrome in kidney grafts of patients with congenital nephrotic syndrome of the Finnish type: role of nephrin.芬兰型先天性肾病综合征患者肾移植中肾病综合征的复发:nephrin的作用
Transplantation. 2002 Feb 15;73(3):394-403. doi: 10.1097/00007890-200202150-00013.
4
Genotype/phenotype correlations of NPHS1 and NPHS2 mutations in nephrotic syndrome advocate a functional inter-relationship in glomerular filtration.肾病综合征中NPHS1和NPHS2突变的基因型/表型相关性表明肾小球滤过存在功能上的相互关系。
Hum Mol Genet. 2002 Feb 15;11(4):379-88. doi: 10.1093/hmg/11.4.379.
5
Congenital nephrotic syndrome (NPHS1): features resulting from different mutations in Finnish patients.先天性肾病综合征(NPHS1):芬兰患者不同突变导致的特征
Kidney Int. 2000 Sep;58(3):972-80. doi: 10.1046/j.1523-1755.2000.00254.x.
6
Recurrence of proteinuria following renal transplantation in congenital nephrotic syndrome of the Finnish type.芬兰型先天性肾病综合征肾移植后蛋白尿复发
Pediatr Nephrol. 2006 May;21(5):711-8. doi: 10.1007/s00467-006-0047-0. Epub 2006 Mar 4.
7
Treatment and outcome of congenital nephrotic syndrome.先天性肾病综合征的治疗和结局。
Nephrol Dial Transplant. 2019 Mar 1;34(3):458-467. doi: 10.1093/ndt/gfy015.
8
Detailed clinical manifestations at onset and prognosis of neonatal-onset Denys-Drash syndrome and congenital nephrotic syndrome of the Finnish type.新生儿期发病的迪尼斯-德拉斯综合征及芬兰型先天性肾病综合征起病时的详细临床表现及预后
Clin Exp Nephrol. 2019 Aug;23(8):1058-1065. doi: 10.1007/s10157-019-01732-7. Epub 2019 Apr 8.
9
Management of congenital nephrotic syndrome of the Finnish type.芬兰型先天性肾病综合征的管理
Pediatr Nephrol. 1995 Feb;9(1):87-93. doi: 10.1007/BF00858984.
10
Muscular dystonia and athetosis in six patients with congenital nephrotic syndrome of the Finnish type (NPHS1).6例芬兰型先天性肾病综合征(NPHS1)患者出现肌张力障碍和手足徐动症。
Pediatr Nephrol. 2006 Feb;21(2):182-9. doi: 10.1007/s00467-005-2116-1. Epub 2005 Dec 17.

引用本文的文献

1
Commentary on "Pediatric Idiopathic Steroid-sensitive Nephrotic Syndrome Diagnosis and Therapy - Short version of the updated German Best Practice Guideline (S2e)".《小儿特发性激素敏感性肾病综合征的诊断与治疗——德国最新最佳实践指南(S2e)简版》述评
Pediatr Nephrol. 2021 Oct;36(10):2961-2966. doi: 10.1007/s00467-021-05136-2. Epub 2021 Jun 5.

本文引用的文献

1
Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases.先天性肾病综合征婴儿的预后与其他肾脏疾病婴儿相当。
Pediatr Nephrol. 2019 Apr;34(4):649-655. doi: 10.1007/s00467-018-4122-0. Epub 2018 Oct 29.
2
Management of children with congenital nephrotic syndrome: challenging treatment paradigms.儿童先天性肾病综合征的管理:具有挑战性的治疗模式。
Nephrol Dial Transplant. 2019 Aug 1;34(8):1369-1377. doi: 10.1093/ndt/gfy165.
3
Catheter-Directed Thrombolysis With a Continuous Infusion of Low-Dose Alteplase for Subacute Proximal Venous Thrombosis: Efficacy and Safety Compared to Urokinase.持续输注低剂量阿替普酶进行导管定向溶栓治疗亚急性近端静脉血栓形成:与尿激酶相比的疗效和安全性
Clin Appl Thromb Hemost. 2018 Nov;24(8):1333-1339. doi: 10.1177/1076029618775514. Epub 2018 May 16.
4
Genetic testing in steroid-resistant nephrotic syndrome: when and how?激素抵抗型肾病综合征的基因检测:时机与方法?
Nephrol Dial Transplant. 2016 Nov;31(11):1802-1813. doi: 10.1093/ndt/gfv355. Epub 2015 Oct 27.
5
Domiciliary administration of intravenous albumin in congenital nephrotic syndrome.先天性肾病综合征的静脉注射白蛋白居家给药
Pediatr Nephrol. 2015 Nov;30(11):2045-50. doi: 10.1007/s00467-015-3177-4. Epub 2015 Aug 7.
6
Home intravenous therapy: Accessibility for Canadian children and youth.家庭静脉治疗:加拿大儿童和青少年的可及性
Paediatr Child Health. 2011 Feb;16(2):105-14. doi: 10.1093/pch/16.2.105.
7
Educational paper: the podocytopathies.教育论文:足细胞病。
Eur J Pediatr. 2012 Aug;171(8):1151-60. doi: 10.1007/s00431-011-1668-2. Epub 2012 Jan 13.
8
[Nutritional support in the home-based hospitalization setting].[居家住院环境下的营养支持]
Nutr Hosp. 2009 Jan-Feb;24(1):93-6.
9
Congenital nephrotic syndrome.先天性肾病综合征
Pediatr Nephrol. 2009 Nov;24(11):2121-8. doi: 10.1007/s00467-007-0633-9. Epub 2007 Oct 30.
10
Nephrotic syndrome in the first year of life: two thirds of cases are caused by mutations in 4 genes (NPHS1, NPHS2, WT1, and LAMB2).一岁以内的肾病综合征:三分之二的病例由4个基因(NPHS1、NPHS2、WT1和LAMB2)的突变引起。
Pediatrics. 2007 Apr;119(4):e907-19. doi: 10.1542/peds.2006-2164. Epub 2007 Mar 19.

家庭白蛋白输注疗法,芬兰型先天性肾病综合征患者的另一种替代治疗方法。

Home Albumin Infusion Therapy, Another Alternative Treatment in Patients With Congenital Nephrotic Syndrome of the Finnish Type.

作者信息

Serramontmany Eugènia, Muñoz Marina, Fernández-Polo Aurora, Morillo María, Gómez-Ganda Laura, Cañete-Ramírez Carme, Ariceta Gema

机构信息

Pharmacy Department, Vall d'Hebron University Hospital, Barcelona, Spain.

Pediatric Nephrology Department, Vall d'Hebron University Hospital, Barcelona, Spain.

出版信息

Front Pediatr. 2021 Jan 14;8:614535. doi: 10.3389/fped.2020.614535. eCollection 2020.

DOI:10.3389/fped.2020.614535
PMID:33520897
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7841340/
Abstract

Congenital nephrotic syndrome of the Finnish type (CNF) is a rare, severe glomerular disease caused by mutations in the NPHS1 gene, which codes for nephrin. It is characterised by massive proteinuria and severe edoema. Progression to end-stage kidney failure occurs during early childhood and the only curative treatment is kidney transplantation. Nowadays, patients need aggressive medical treatment, which includes daily albumin infusions (for months) until they get clinical stability to receive transplant. In our paediatric hospital, we implemented a multidisciplinary program for the home infusion of albumin with outpatient follow-up. The aim of the study was to assess the safety and efficacy of this program for the first four years of its implementation. Retrospective observational study of CNF paediatric patients treated with home albumin infusion therapy from March 2014 to July 2018 at a tertiary care paediatric hospital. Information on albumin administration was obtained from the electronic prescription assistance program and details on clinical and care-related variables from the hospital's electronic information systems. Four patients with CNF received albumin infusions for 18, 21, 22 months, and 3 years. The treatment was safe, and the complication rates were to be expected considering the severity of disease. Patients required a median of two hospital admissions a year (19 in total); 47% due to catheter-related complications, but there were just three catheter infections. In our experience, home albumin infusion therapy is safe and effective and helps to improve children health and quality of life.

摘要

芬兰型先天性肾病综合征(CNF)是一种罕见的严重肾小球疾病,由编码nephrin的NPHS1基因突变引起。其特征为大量蛋白尿和严重水肿。在儿童早期会进展为终末期肾衰竭,唯一的治愈性治疗方法是肾脏移植。如今,患者需要积极的医学治疗,包括每日输注白蛋白(持续数月),直至临床稳定以接受移植。在我们的儿科医院,我们实施了一项多学科计划,进行白蛋白家庭输注并门诊随访。本研究的目的是评估该计划实施头四年的安全性和有效性。对2014年3月至2018年7月在一家三级儿科医院接受白蛋白家庭输注治疗的CNF儿科患者进行回顾性观察研究。白蛋白给药信息来自电子处方辅助程序,临床和护理相关变量的详细信息来自医院的电子信息系统。4例CNF患者接受白蛋白输注的时间分别为18个月、21个月、22个月和3年。治疗是安全的,考虑到疾病的严重程度,并发症发生率在意料之中。患者每年平均住院两次(共19次);47%是由于导管相关并发症,但只有3例导管感染。根据我们的经验,白蛋白家庭输注治疗是安全有效的,有助于改善儿童健康和生活质量。