Department of Radiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
Clin J Am Soc Nephrol. 2022 Mar;17(3):374-384. doi: 10.2215/CJN.08660621. Epub 2022 Feb 25.
The progression of polycystic liver disease is not well understood. The purpose of the study is to evaluate the associations of polycystic liver progression with other disease progression variables and classify liver progression on the basis of patient's age, height-adjusted liver cystic volume, and height-adjusted liver volume.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Prospective longitudinal magnetic resonance images from 670 patients with early autosomal dominant polycystic kidney disease for up to 14 years of follow-up were evaluated to measure height-adjusted liver cystic volume and height-adjusted liver volume. Among them, 245 patients with liver cyst volume >50 ml at baseline were included in the longitudinal analysis. Linear mixed models on log-transformed height-adjusted liver cystic volume and height-adjusted liver volume were fitted to approximate mean annual rate of change for each outcome. The association of sex, body mass index, genotype, baseline height-adjusted total kidney volume, and Mayo imaging class was assessed. We calculated height-adjusted liver cystic volume ranges for each specific age and divided them into five classes on the basis of annual percentage increase in height-adjusted liver cystic volume.
The mean annual growth rate of height-adjusted liver cystic volume was 12% (95% confidence interval, 11.1% to 13.1%; <0.001), whereas that for height-adjusted liver volume was 2% (95% confidence interval, 1.9% to 2.6%; <0.001). Women had higher baseline height-adjusted liver cystic volume than men, but men had higher height-adjusted liver cystic volume growth rate than women by 2% (95% confidence interval, 0.4% to 4.5%; =0.02). Whereas the height-adjusted liver cystic volume growth rate decreased in women after menopause, no decrease was observed in men at any age. Body mass index, genotype, and baseline height-adjusted total kidney volume were not associated with the growth rate of height-adjusted liver cystic volume or height-adjusted liver volume. According to the height-adjusted liver cystic volume growth rate, patients were classified into five classes (number of women, men in each class): A (24, six); B (44, 13); C (43, 48); D (28, 17); and E (13, nine).
Compared with height-adjusted liver volume, the use of height-adjusted liver cystic volume showed greater separations in volumetric progression of polycystic liver disease. Similar to the Mayo imaging classification for the kidney, the progression of polycystic liver disease may be categorized on the basis of patient's age and height-adjusted liver cystic volume.
多囊肝的进展过程尚不清楚。本研究的目的是评估多囊肝进展与其他疾病进展变量的关系，并根据患者的年龄、肝囊肿体积身高比和肝体积身高比对肝进展进行分类。
设计、地点、参与者和测量方法：对 670 例常染色体显性遗传性多囊肾病患者的前瞻性纵向磁共振图像进行评估，随访时间长达 14 年，以测量肝囊肿体积身高比和肝体积身高比。其中，245 例基线肝囊肿体积>50ml的患者纳入纵向分析。对对数转换的肝囊肿体积身高比和肝体积身高比进行线性混合模型拟合，以近似每个结局的平均年变化率。评估了性别、体重指数、基因型、基线全肾体积身高比和 Mayo 影像学分类的相关性。我们计算了每个特定年龄的肝囊肿体积身高比范围，并根据肝囊肿体积身高比的年增长率将其分为五个等级。
肝囊肿体积身高比的年平均增长率为 12%（95%置信区间，11.1%13.1%；<0.001），而肝体积身高比的年增长率为 2%（95%置信区间，1.9%2.6%；<0.001）。女性的基线肝囊肿体积身高比高于男性，但男性的肝囊肿体积身高比增长率比女性高 2%（95%置信区间，0.4%~4.5%；=0.02）。虽然女性绝经后肝囊肿体积身高比增长率下降，但男性在任何年龄均无下降。体重指数、基因型和基线全肾体积身高比与肝囊肿体积身高比或肝体积身高比的增长率无关。根据肝囊肿体积身高比增长率，患者被分为五个等级（每个等级的女性人数、男性人数）：A（24，6）；B（44，13）；C（43，48）；D（28，17）；E（13，9）。
与肝体积身高比相比，肝囊肿体积身高比在多囊肝疾病的体积进展方面显示出更大的分离。与肾脏的 Mayo 影像学分类相似，多囊肝疾病的进展可能基于患者的年龄和肝囊肿体积身高比进行分类。
