계명대학교 의학도서관 Repository

Factors Associated With the Development and Severity of Polycystic Liver in Patients With Autosomal Dominant Polycystic Kidney Disease

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Author(s)
Yaerim KimHayne Cho ParkHyunjin RyuYong Chul KimCurie AhnKyu-Beck LeeYeong Hoon KimSeungyeup HanEun Hui BaeKyungjo JeongJungmin ChoiKook-Hwan OhYun Kyu Oh
Keimyung Author(s)
Kim, Yae RimHan, Seung Yeup
Department
Dept. of Internal Medicine (내과학)
Journal Title
J Korean Med Sci
Issued Date
2023
Volume
38
Issue
38
Keyword
Polycystic KidneyAutosomal DominantPolycystic Liver DiseaseRisk FactorsGenotype
Abstract
Background:
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: < 1,000 mL/m (Gr1), 1,000-1,800 mL/m (Gr2), and > 1,800 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 PLD. The patients with PLD showed female predominance and a higher frequency of other ADPKD-related complications. The genetic variants with truncating mutation of PKD1 (PKD1-protein-truncating [PT]) or PKD2 commonly affected the development and severity of PLD. An older age, female sex, and higher kidney volume with Mayo classification 1C-1E was significantly associated with the development of PLD, but not with the severity of PLD. On the other hand, higher body mass index, lower hemoglobin, and higher alkaline phosphatase (ALP) were the significant risk factors of severe PLD (≥ Gr2).

Conclusion:
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.

Trial registration:
Clinical Research Information Service Identifier: KCT0005580.
Keimyung Author(s)(Kor)
김예림
한승엽
Publisher
School of Medicine (의과대학)
Type
Article
ISSN
1598-6357
Source
https://jkms.org/DOIx.php?id=10.3346/jkms.2023.38.e296
DOI
10.3346/jkms.2023.38.e296
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/45079
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Internal Medicine (내과학)
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