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End-stage kidney disease due to Alport syndrome: outcomes in 296 consecutive Australia and New Zealand Dialysis and Transplant Registry cases
Mallett, Andrew1,2,3; Tang, Wen4; Clayton, Philip A.1,5,6; Stevenson, Sarah1,2; McDonald, Stephen P.1,7,8; Hawley, Carmel M.1,2; Badve, Sunil V.1,2; Boudville, Neil1,9; Brown, Fiona G.1,10; Campbell, Scott B.1,2; Johnson, David W.1,2
关键词Alport Syndrome Dialysis Hereditary Nephritis Outcomes Renal Transplantation
刊名NEPHROLOGY DIALYSIS TRANSPLANTATION
2014-12-01
DOI10.1093/ndt/gfu254
29期:12页:2277-2286
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Transplantation ; Urology & Nephrology
研究领域[WOS]Transplantation ; Urology & Nephrology
关键词[WOS]ANTI-ALPHA-3(IV) COLLAGEN ALLOANTIBODIES ; GENOTYPE-PHENOTYPE CORRELATIONS ; ANTI-GBM NEPHRITIS ; RENAL-TRANSPLANTATION ; NATURAL-HISTORY ; HEREDITARY NEPHRITIS ; AUTOSOMAL-DOMINANT ; CLINICAL SPECTRUM ; 195 FAMILIES ; COL4A5 GENE
英文摘要

Alport syndrome is a rare inheritable renal disease. Clinical outcomes for patients progressing to end-stage kidney disease (ESKD) are not well described.

This study aimed to investigate the characteristics and clinical outcomes of patients from Australia and New Zealand commencing renal replacement therapy (RRT) for ESKD due to Alport syndrome between 1965 and 1995 (early cohort) and between 1996 and 2010 (contemporary cohort) compared with propensity score-matched, RRT-treated, non-Alport ESKD controls.

A total of 58 422 patients started RRT during this period of which 296 (0.5%) patients had Alport ESKD. In the early cohort, Alport ESKD was associated with superior dialysis patient survival [adjusted hazard ratio (HR): 0.41, 95% confidence interval (CI): 0.20-0.83, P = 0.01], renal allograft survival (HR: 0.74, 95% CI: 0.54-1.01, P = 0.05) and renal transplant patient survival (HR: 0.43, 95% CI: 0.28-0.66, P < 0.001) compared with controls. In the contemporary cohort, no differences were observed between the two groups for dialysis patient survival (HR: 1.42, 95% CI: 0.65-3.11, P = 0.38), renal allograft survival (HR: 1.01, 95% CI: 0.57-1.79, P = 0.98) or renal transplant patient survival (HR: 0.67, 95% CI: 0.26-1.73, P = 0.41). One Alport patient (0.4%) had post-transplant anti-glomerular basement membrane (anti-GBM) disease. Four female and 41 male Alport patients became parents on RRT with generally good neonatal outcomes.

Alport syndrome patients experienced comparable dialysis and renal transplant outcomes to matched non-Alport ESKD controls in the contemporary cohort due to relatively greater improvements in outcomes for non-Alport ESKD patients over time. Post-transplant anti-GBM disease was rare.

语种英语
WOS记录号WOS:000345833500018
资助机构Baxter Healthcare Pty Ltd ; Fresenius Medical Care ; Queensland Government Health Research Fellowship ; Winston Churchill Memorial Trust Fellowship ; Amgen ; Royal Brisbane and Women&prime ; s Hospital Foundation Postgraduate Scholarship ; Roche ; Jansen Cilag
引用统计
被引频次:7[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/56744
专题北京大学第三临床医学院_肾内科
作者单位1.ANZDATA Registry, Adelaide, SA, Australia
2.Princess Alexandra Hosp, Dept Nephrol, Brisbane, Qld 4102, Australia
3.Royal Brisbane & Womens Hosp, Dept Renal Med, Brisbane, Qld, Australia
4.Royal Prince Alfred Hosp, Dept Renal Med, Sydney, NSW, Australia
5.Univ Sydney, Sch Publ Hlth, Sydney, NSW 2006, Australia
6.Monash Med Ctr, Dept Nephrol, Melbourne, Vic, Australia
7.Peking Univ, Hosp 3, Div Nephrol, Beijing 100871, Peoples R China
8.Univ Adelaide, Dept Nephrol & Transplantat Serv, Adelaide, SA, Australia
9.Cent Northern Adelaide Renal & Transplantat Serv, Adelaide, SA, Australia
10.Univ Western Australia, Sch Med & Pharmacol, Sir Charles Gairdner Hosp Unit, Perth, WA 6009, Australia
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GB/T 7714
Mallett, Andrew,Tang, Wen,Clayton, Philip A.,et al. End-stage kidney disease due to Alport syndrome: outcomes in 296 consecutive Australia and New Zealand Dialysis and Transplant Registry cases[J]. NEPHROLOGY DIALYSIS TRANSPLANTATION,2014,29(12):2277-2286.
APA Mallett, Andrew.,Tang, Wen.,Clayton, Philip A..,Stevenson, Sarah.,McDonald, Stephen P..,...&Johnson, David W..(2014).End-stage kidney disease due to Alport syndrome: outcomes in 296 consecutive Australia and New Zealand Dialysis and Transplant Registry cases.NEPHROLOGY DIALYSIS TRANSPLANTATION,29(12),2277-2286.
MLA Mallett, Andrew,et al."End-stage kidney disease due to Alport syndrome: outcomes in 296 consecutive Australia and New Zealand Dialysis and Transplant Registry cases".NEPHROLOGY DIALYSIS TRANSPLANTATION 29.12(2014):2277-2286.
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