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논문분류	춘계학술대회 초록집
제목	Factors associated with the development and severity of polycystic liver in patients with autosomal-dominant polycystic kidney disease
저자	Yaerim Kim
출판정보	2023; 2023(1):
키워드
초록	Objectives: Factors related to the development and severity of polycystic liver disease (PLD) have not been well established. We aimed to evaluate the genetic and epidemiologic risk factors of PLD in patients with autosomal dominant polycystic kidney disease (ADPKD). Methods: Adult patients with inherited cystic kidney disease were enrolled from May 2019 to May 2021. Demographic, clinical, and laboratory data were collected at the initial study visit. The severity of PLD was graded based on the height-adjusted total liver volume (HtTLV): <1000 mL/m (Gr1), 1000-1800 mL/m (Gr2), and >1800 mL/m (Gr3). Targeted exome sequencing was done by a gene panel including 89 ciliopathy-related genes. We searched out the relative factors to the presence and the severity of PLD using logistic regression analysis. Results: Of 602 patients with typical ADPKD, 461 (76.6%) patients had liver cysts. The patients with liver cysts showed female predominance and a higher frequency of other ADPKD-related complications. An older age, hypertension, and genetic variants with truncating mutation of PKD1 (PKD1-PT) or PKD2 commonly affected the development and severity of PLD. Higher kidney volume with Mayo classification 1C-1E was significantly associated with the development of liver cysts, but not with the severity of PLD. On the other hand, higher body mass index, lower hemoglobin, and higher alkaline phosphokinase (ALP) were the significant risk factors of severe PLD (≥Gr 2). Conclusions: Hepatic involvement in ADPKD could be related to kidney manifestations and genetic variants including PKD1-PT or PKD2. Monitoring hemoglobin and ALP and evaluating the genetic variants might help predict severe PLD.
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