Growth Pattern of Kidney Cyst Number and Volume in Autosomal Dominant Polycystic Kidney Disease
Growth Pattern of Kidney Cyst Number and Volume in Autosomal Dominant Polycystic Kidney Disease
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DOI：
10.2215/cjn.10360818
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发表时间：
2019-06-07
影响因子：
9.8
通讯作者：
Harris, Peter C.
中科院分区：
文献类型：
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作者：
Bae, Kyongtae T.;Zhou, Wen;Harris, Peter C.
背景与目的通过对241例常染色体显性遗传性多囊肾病患者(基线年龄为15~46岁)进行系列磁共振成像，测量肾囊肿数和囊体积，获得最长14.23年的随访，评价常染色体显性遗传性多囊肾病患者的肾囊肿数和囊体积的生长模式与肾脏大小、人口学特征和基因分型的关系。使用重复测量和与年龄交互作用的线性混合模型(作为时间依赖的协变量)来评估随时间的变化率，分析与性别、年龄、身高调整的总肾脏体积和基因相关的生长模式。模特们也通过伊拉扎巴尔的课程进行了试穿。结果共收集到236例患者的影像和遗传学资料(至少一次访问)。平均高度调整后总囊肿数随着时间的推移呈指数增长，从基线的762个增加到最后一次临床就诊时的1715个，而平均高度调整后的总囊肿量从305个增加到770毫升。高度调整后的总肾体积、高度调整后的总囊肿数和高度调整后的总囊肿量都随着时间的推移而高度相关。女性参与者和基线时身高调整总肾体积较大的参与者在身高调整总囊数和囊体积对数中的变化率较小。在给定的年龄，PKD1与囊泡数量和体积的显著增加有关，但基因对生长速度没有显著影响。结论经高度调整的总囊数和调整高度的总囊腔体积均呈指数级增长，在14.23年的随访期间增加了一倍以上，与PKD2和未检测到突变相比，PKD1在特定年龄段具有更多的囊肿数和体积，但在生长速度上没有显著差异。
Background and objectives To evaluate the growth pattern of kidney cyst number and cyst volume in association with kidney size, demographics, and genotypes in autosomal dominant polycystic kidney disease.Design, setting, participants, & measurements Kidney cyst number and cyst volume were measured from serial magnetic resonance images, giving a maximum follow-up of 14.23 years, from 241 patients with autosomal dominant polycystic kidney disease (15-46 years old at baseline). The growth pattern was analyzed, in association with sex, age, height-adjusted total kidney volume, and genotype, using linear mixed models of repeated measurements and tests of interactions with age (as a time-dependent covariate) to assess rates of change over time. Models were also fit using Irazabal class. Genotypic groups were characterized as either (1) PKD1 truncating, PKD1nontruncating, andPKD2pluspatients withnomutation detected; or (2) in combination with PKD1 mutation strength groups.Results Imaging and genetic data were collected (at least one visit) for 236 participants. The mean heightadjusted total cyst number increased exponentially over time from a baseline value of 762 to 1715 at the last clinic visit, while the mean height-adjusted total cyst volume increased exponentially from 305 to 770 ml. Height-adjusted total kidney volume, height-adjusted total cyst number, and height-adjusted total cyst volume were all highly correlated over time. Female participants and participantswith larger height-adjusted total kidney volume at baseline showed smaller rates of change in the log of height-adjusted total cyst number and cyst volume. PKD1 was associated with significant increases in both cyst number and volume at a given age, but genotype did not significantly affect the rate of growth. Conclusions Both height-adjusted total cyst number and height-adjusted total cyst volume increased exponentially and more than doubled over 14.23 years of follow-up.Compared with PKD2 plus no mutation detected, PKD1 was associated with a greater cyst number and volume at a given age, but no significant difference in the rate of growth.