Comparison of phenotypes of polycystic kidney disease types 1 and 2
Comparison of phenotypes of polycystic kidney disease types 1 and 2
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DOI：
10.1016/s0140-6736(98)03495-3
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发表时间：
1999-01-09
期刊：
影响因子：
168.9
通讯作者：
Ravine, D
中科院分区：
文献类型：
--
作者：
Hateboer, N;Dijk, MAV;Ravine, D
背景：虽然常染色体显性多囊肾病2型（PKD2）的临床表型比PKD1更轻，但在生存率方面，这两种疾病都没有与未受影响的对照人群进行比较。我们报告调查结果；一项多中心调查旨在更精确地定义；Phe存活与PKD1和PKD2的临床表达。方法将333例PKD1患者（31个家族）的临床资料与291例PKD2患者（31个家族）和398例地理匹配对照进行比较。生存分析用于比较事件年龄数据。通过逻辑回归评估并发症发生率的差异。PKD1患者死亡或终末期肾病发病时的中位年龄为53.0岁（95% CI 51.2-54.8）， PKD2患者为69.1岁（66.9-71.3），对照组为78.0岁（73.8-82.2）。女性PKD2患者的中位生存期明显长于男性（71.0年[67.4-74.8年]vs 67.3年[64.9-69.7年]），但PKD1患者的性别差异不明显。PKD2患者出现肾衰竭的年龄晚于PKD1患者（中位年龄74.0[67.2-80.8]对54.3[52.7-55.9]岁）。PKD2患者发生高血压（优势比为0.25 [95% CI 0.15-0.42]）、尿路感染史（优势比为0.50[0.31-0.83]）或血尿（优势比为0.59[0.35-0.98]）的可能性较小。虽然PKD2在临床上比PKD1更轻，但它对总体预期寿命有有害影响，不能被视为良性疾病。
Background Although autosomal dominant polycystic kidney disease type 2 (PKD2) is known to have a milder clinical phenotype than PKD1, neither disorder has been compared with an unaffected control population in terms of survival. We report the findings;of a multicentre survey that aimed to define more precisely;Phe survival and clinical expression of PKD1 and PKD2.Methods Clinical data from 333 people with PKD1 (31 families) were compared with data from 291 people with PKD2(31 families) and 398 geographically matched controls. Survival analysis was used to compare age-at-event data. Differences in the prevalence of complications were assessed by logistic regression.Findings Median age at death or onset of end-stage renal disease, was 53.0 years (95% CI 51.2-54.8) in individuals with PKD1, 69.1 years (66.9-71.3) in those with PKD2, and 78.0 years (73.8-82.2) in controls. Women with PKD2 had a significantly longer median survival than men (71.0 [67.4-74.8] vs 67.3 [64.9-69.7] years), but no sex influence was apparent in PKD1. Age at presentation with kidney failure was later in PKD2 than in PKD1 (median age 74.0 [67.2-80.8] vs 54.3 [52.7-55.9] years). PKD2 patients were less likely to have hypertension (odds ratio 0.25 [95% CI 0.15-0.42]), a history of urinary-tract infection (0.50 [0.31-0.83]), or haematuria (0.59 [0.35-0.98]).Interpretation Although PKD2 is clinically milder than PKD1, it has a deleterious impact on overall life expectancy and cannot be regarded as a benign disorder.