Growth Pattern of Kidney Cyst Number and Volume in Autosomal Dominant Polycystic Kidney Disease

被引:23
作者
Bae, Kyongtae T. [1 ]
Zhou, Wen [1 ]
Shen, Chengli [2 ]
Landsittel, Douglas P. [2 ]
Wu, Zhiyuan [1 ]
Tao, Cheng [1 ]
Chapman, Arlene B. [3 ]
Torres, Vicente E. [4 ]
Yu, Alan S. L. [5 ]
Mrug, Michal [6 ,7 ]
Bennett, William M. [8 ]
Harris, Peter C. [4 ]
机构
[1] Univ Pittsburgh, Sch Med, Dept Radiol, 3362 Fifth Ave, Pittsburgh, PA 15213 USA
[2] Univ Pittsburgh, Sch Med, Dept Biomed Informat, Pittsburgh, PA USA
[3] Univ Chicago, Sch Med, Dept Internal Med, Chicago, IL 60637 USA
[4] Mayo Clin, Coll Med, Div Nephrol & Hypertens, Rochester, MN USA
[5] Univ Kansas, Med Ctr, Dept Internal Med, Kansas City, KS 66103 USA
[6] Univ Alabama Birmingham, Dept Med, Birmingham, AL 35294 USA
[7] Dept Vet Affairs Med Ctr, Birmingham, AL USA
[8] Legacy Good Samaritan Hosp, Portland, OR USA
来源
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY | 2019年 / 14卷 / 06期
基金
美国国家卫生研究院;
关键词
RENAL-FUNCTION; GENE; CONSORTIUM; ENCODES; TYPE-2;
D O I
10.2215/CJN.10360818
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:823 / 833
页数:11
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