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Expert Guidelines for the Management of Alport Syndrome and Thin Basement Membrane Nephropathy
Savige, Judy1; Gregory, Martin2; Gross, Oliver3; Kashtan, Clifford4; Ding, Jie5; Flinter, Frances6
刊名JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
2013-03-01
DOI10.1681/ASN.2012020148
24期:3页:364-375
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Urology & Nephrology
研究领域[WOS]Urology & Nephrology
关键词[WOS]GENOTYPE-PHENOTYPE CORRELATIONS ; DELAYS RENAL-FAILURE ; IV COLLAGEN ; KIDNEY-TRANSPLANTATION ; HEREDITARY NEPHRITIS ; NATURAL-HISTORY ; 195 FAMILIES ; COL4A5 GENE ; MOUSE MODEL ; MUTATIONS
英文摘要

Few prospective, randomized controlled clinical trials address the diagnosis and management of patients with Alport syndrome or thin basement membrane nephropathy. Adult and pediatric nephrologists and geneticists from four continents whose clinical practice focuses on these conditions have developed the following guidelines. The 18 recommendations are based on Level D (Expert opinion without explicit critical appraisal, or based on physiology, bench research, or first principles-National Health Service category) or Level III (Opinions of respected authorities, based on clinical experience, descriptive studies, or reports of expert committees-U.S. Preventive Services Task Force) evidence. The recommendations include the use of genetic testing as the gold standard for the diagnosis of Alport syndrome and the demonstration of its mode of inheritance; the need to identify and follow all affected members of a family with X-linked Alport syndrome, including most mothers of affected males; the treatment of males with X-linked Alport syndrome and individuals with autosomal recessive disease with renin-angiotensin system blockade, possibly even before the onset of proteinuria; discouraging the affected mothers of males with X-linked Alport syndrome from renal donation because of their own risk of kidney failure; and consideration of genetic testing to exclude X-linked Alport syndrome in some individuals with thin basement membrane nephropathy. The authors recognize that as evidence emerges, including data from patient registries, these guidelines will evolve further. J Am Soc Nephrol 24: 364-375, 2013. doi: 10.1681/ASN.2012020148

语种英语
WOS记录号WOS:000315472600007
Citation statistics
Cited Times:77[WOS]   [WOS Record]     [Related Records in WOS]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/54959
Collection北京大学第一临床医学院_儿科
作者单位1.Univ Med Goettingen, Dept Nephrol & Rheumatol, Gottingen, Germany
2.Univ Melbourne, Dept Med Northern Hlth, Melbourne, Vic, Australia
3.Univ Utah, Sch Med, Div Nephrol, Salt Lake City, UT USA
4.Univ Minnesota, Sch Med, Dept Pediat, Minneapolis, MN 55455 USA
5.Peking Univ, Hosp 1, Dept Pediat, Beijing 100871, Peoples R China
6.Guys & St Thomas NHS Fdn Trust, Dept Clin Genet, London, England
Recommended Citation
GB/T 7714
Savige, Judy,Gregory, Martin,Gross, Oliver,et al. Expert Guidelines for the Management of Alport Syndrome and Thin Basement Membrane Nephropathy[J]. JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY,2013,24(3):364-375.
APA Savige, Judy,Gregory, Martin,Gross, Oliver,Kashtan, Clifford,Ding, Jie,&Flinter, Frances.(2013).Expert Guidelines for the Management of Alport Syndrome and Thin Basement Membrane Nephropathy.JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY,24(3),364-375.
MLA Savige, Judy,et al."Expert Guidelines for the Management of Alport Syndrome and Thin Basement Membrane Nephropathy".JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY 24.3(2013):364-375.
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