Bazojoo Vahid, Davoudi Vahid, Blumenfeld Jon D, Zhu Chenglin, Malha Line, Lo Grace C, Chevalier James M, Shimonov Daniil, Sharbatdaran Arman, Dev Hreedi, Raza Syed I, Hu Zhongxiu, He Xinzi, RoyChoudhury Arindam, Prince Martin R
Department of Radiology, Weill Cornell Medicine, New York, NY 10022, USA.
Department of Medicine, Division of Nephrology and Hypertension, Weill Cornell Medical College, New York, NY 10021, USA.
J Clin Med. 2025 May 24;14(11):3688. doi: 10.3390/jcm14113688.
: Polycystic liver disease (PLD) is the most common extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). PLD is more prevalent in women, and women have larger liver cysts, possibly due to estrogen-related mechanisms. Maternal estrogen levels normally increase during pregnancy. Thus, we investigated the pregnancy-associated increase in liver volume, liver cyst volume, total kidney volume (TKV), and kidney cyst growth rates in ADPKD patients. : Kidney, liver, and cyst volumes were measured in 16 ADPKD patients by magnetic resonance imaging (MRI) at multiple timepoints before and after pregnancy. The log-transformed TKV, liver volume, and cyst volume growth rates during a period with pregnancy were compared to a period without pregnancy. : In ADPKD patients, a higher annualized liver cyst growth rate was observed during a period with pregnancy compared to a period without pregnancy (34 ± 16%/yr vs. 23 ± 17%/yr; -value = 0.005). Liver volume growth was also higher during a period with pregnancy, 6 [2, 7]%/yr vs. 0.3 [-0.4, 2]%/yr (-value = 0.04). In addition, the mean kidney cyst growth rate was higher (12 ± 11%/yr vs. 4 ± 9%/yr; -value = 0.05), and there was a trend toward a pregnancy-associated increase in the TKV growth rate (6 [4, 8]%/yr vs. 3 [0.8, 5]%/yr, (-value = 0.14) during a period with pregnancy. : In patients with ADPKD, the liver volume and cyst volume growth rates increased during pregnancy. This supports the hypothesis that the estrogen-mediated stimulation of liver cyst growth may contribute to the severe polycystic liver disease that is more prevalent in women than men with ADPKD. Further studies with larger populations are needed to explore the mechanisms and long-term implications of these findings.
多囊肝病（PLD）是常染色体显性多囊肾病（ADPKD）最常见的肾外表现。PLD在女性中更为普遍，且女性的肝囊肿更大，这可能与雌激素相关机制有关。孕期母体雌激素水平通常会升高。因此，我们研究了ADPKD患者孕期肝脏体积、肝囊肿体积、总肾体积（TKV）及肾囊肿生长速率的增加情况。
通过磁共振成像（MRI）在16例ADPKD患者孕期前后的多个时间点测量肾脏、肝脏及囊肿体积。将孕期期间对数转换后的TKV、肝脏体积及囊肿体积生长速率与非孕期进行比较。
在ADPKD患者中，与非孕期相比，孕期期间观察到更高的年化肝囊肿生长速率（34±16%/年 vs. 23±17%/年；P值 = 0.005）。孕期期间肝脏体积生长也更高，为6[2, 7]%/年 vs. 0.3[-0.4, 2]%/年（P值 = 0.04）。此外，平均肾囊肿生长速率更高（12±11%/年 vs. 4±9%/年；P值 = 0.05），且孕期期间TKV生长速率有与妊娠相关增加的趋势（6[4, 8]%/年 vs. 3[0.8, 5]%/年，（P值 = 0.14）。
在ADPKD患者中，孕期肝脏体积和囊肿体积生长速率增加。这支持了以下假设：雌激素介导的肝囊肿生长刺激可能导致在患有ADPKD的女性中比男性更普遍的严重多囊肝病。需要对更多人群进行进一步研究以探索这些发现的机制和长期影响。
