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识别常染色体显性遗传性多囊肾病中处于终末期肾病最高风险的患者。

Identification of patients with autosomal dominant polycystic kidney disease at highest risk for end-stage renal disease.

作者信息

Johnson A M, Gabow P A

机构信息

Department of Medicine, University of Colorado Health Sciences Center, Denver 80262, USA.

出版信息

J Am Soc Nephrol. 1997 Oct;8(10):1560-7. doi: 10.1681/ASN.V8101560.

DOI:10.1681/ASN.V8101560
PMID:9335384
Abstract

To identify those potential factors that, early in the course of disease, mark a population of patients with autosomal dominant polycystic kidney disease (ADPKD) who have worse renal survival, survival analysis and risk ratio calculation for 1215 ADPKD patients were performed. Survival times were calculated as time to dialysis, transplantation, or death. Risk ratios were calculated using the Cox proportional hazards model. Three hundred eighty-eight patients entered end-stage renal disease and 205 patients died. ADPKD2 subjects had longer renal survival than ADPKD1 subjects (median survival, 68 versus 53 yr; P < 0.0005; risk ratio, 2.5). Women had significantly better renal survival than men (56 versus 52 yr; P < 0.0001; risk ratio, 1.6). Subjects who were diagnosed before age 30 and those who developed hypertension before age 35 had worse renal survival than those subjects who were diagnosed after age 30 or those who remained normotensive after age 35, respectively (age of diagnosis: 49 versus 59 yr; P < 0.0001; risk ratio, 3.2; hypertension: 51 versus 65 yr; P < 0.0001; risk ratio, 4.4). Similarly, those who had an episode of gross hematuria before age 30 had a worse renal outcome than those who did not (49 versus 59 yr; P < 0.0001; risk ratio, 2.6). We have also calculated risk ratios for a combined model. When therapeutic interventions become available for this disease, these populations with high risk ratios should be considered for such interventions.

摘要

为了确定在疾病早期那些能够区分出常染色体显性多囊肾病(ADPKD)患者中肾脏存活率较低人群的潜在因素,我们对1215例ADPKD患者进行了生存分析和风险比计算。生存时间计算为至透析、移植或死亡的时间。风险比使用Cox比例风险模型计算。388例患者进入终末期肾病阶段,205例患者死亡。ADPKD2患者的肾脏存活时间长于ADPKD1患者(中位生存期分别为68岁和53岁;P<0.0005;风险比为2.5)。女性的肾脏存活率显著高于男性(分别为56岁和52岁;P<0.0001;风险比为1.6)。30岁之前被诊断出的患者以及35岁之前出现高血压的患者,其肾脏存活率分别低于30岁之后被诊断出的患者以及35岁之后血压正常的患者(诊断年龄:49岁对59岁;P<0.0001;风险比为3.2;高血压:51岁对65岁;P<0.0001;风险比为4.4)。同样,30岁之前出现肉眼血尿的患者比未出现肉眼血尿的患者肾脏预后更差(49岁对59岁;P<0.0001;风险比为2.6)。我们还计算了一个综合模型的风险比。当针对这种疾病的治疗干预措施可用时,应该考虑对这些风险比高的人群进行此类干预。

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