Volume progression in polycystic kidney disease
Volume progression in polycystic kidney disease
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DOI：
10.1056/nejmoa054341
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发表时间：
2006-05-18
影响因子：
158.5
通讯作者：
Miller, JP
中科院分区：
文献类型：
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作者：
Grantham, JJ;Torres, VE;Miller, JP
背景资料：常染色体显性遗传性多囊肾病（ADPKD）的特点是进行性增大的囊肿填充kidney.Methods：在一项为期三年的研究中，我们测量了总肾脏体积，总囊肿体积的变化率，碘酞酸盐的清除率与ADPKD患者。在总共241名患者中，232名基线时年龄为15至46岁的无氮质血症患者中，我们使用磁共振成像将总肾脏体积和总囊肿体积与碘酞酸盐清除率相关联。统计方法包括方差分析，Pearson相关，多元回归analysis.Results：总肾体积和总囊肿体积呈指数增长，结果与依赖于增长的扩张过程相一致。214例患者的平均（+/-SD）肾脏总体积在基线时为1060 +/-642 ml，在3年内平均增加了204 +/-246 ml（每年5.27 +/-3.92%，P <0.001）。同期210例患者的总囊肿体积增加了218 ± 263 ml（P <0.001）。基线总肾脏体积预测随后的体积增加率，与年龄无关。51例患者的基线总肾脏体积超过1500 ml与肾小球滤过率下降相关（每年每分钟4.33 ± 8.07 ml，P <0.001）。135例PKD1突变患者的肾脏总体积（245 ± 268 ml）比28例PKD2突变患者的肾脏总体积（136 ± 100 ml）增加更多（P = 0.03）。结论：ADPKD患者囊肿扩张导致的肾脏增大是持续的、可量化的，与肾功能下降相关。肾脏增大的速率越高，肾功能下降的速度越快。
Background: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive enlargement of cyst-filled kidneys.Methods: In a three-year study, we measured the rates of change in total kidney volume, total cyst volume, and iothalamate clearance in patients with ADPKD. Of a total of 241 patients, in 232 patients without azotemia who were 15 to 46 years old at baseline we used magnetic-resonance imaging to correlate the total kidney volume and total cyst volume with iothalamate clearance. Statistical methods included analysis of variance, Pearson correlation, and multivariate regression analysis.Results: Total kidney volume and total cyst volume increased exponentially, a result consistent with an expansion process dependent on growth. The mean (+/-SD) total kidney volume was 1060+/-642 ml at baseline and increased by a mean of 204+/-246 ml (5.27+/-3.92 percent per year, P < 0.001) over a three-year period among 214 patients. Total cyst volume increased by 218+/-263 ml (P < 0.001) during the same period among 210 patients. The baseline total kidney volume predicted the subsequent rate of increase in volume, independently of age. A baseline total kidney volume above 1500 ml in 51 patients was associated with a declining glomerular filtration rate (by 4.33+/-8.07 ml per minute per year, P < 0.001). Total kidney volume increased more in 135 patients with PKD1 mutations (by 245+/-268 ml) than in 28 patients with PKD2 mutations (by 136+/-100 ml, P=0.03).Conclusions: Kidney enlargement resulting from the expansion of cysts in patients with ADPKD is continuous and quantifiable and is associated with the decline of renal function. Higher rates of kidney enlargement are associated with a more rapid decrease in renal function.