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Joonho Jeong 1 Article
Polycystic liver disease: an overview of clinical manifestations, diagnosis, and treatment
Joonho Jeong, Hyun Joon Park
Kosin Med J. 2023;38(2):75-86.   Published online June 28, 2023
DOI: https://doi.org/10.7180/kmj.23.128
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Abstract PDFPubReader   ePub   
Polycystic liver disease (PLD) is a hereditary disease characterized by the presence of 20 or more liver cysts. It is classified into three types: isolated autosomal dominant PLD, PLD with autosomal dominant polycystic kidney disease, and PLD with autosomal recessive polycystic kidney disease. Genetic alterations, ciliary dysfunction of the biliary epithelial cells, and aberrant cell signaling pathways are the main factors contributing to the pathophysiology of PLD; however, other complicated mechanisms are also involved. The Gigot and Schnelldorfer classifications are widely used in clinical practice. Most patients with PLD are asymptomatic; however, a few patients with advanced-stage disease may develop symptoms and complications that impair their quality of life and require treatment. The known treatment options for PLD are somatostatin analogues, aspiration with sclerotherapy, fenestration, hepatic resection, and liver transplantation. Although liver transplantation remains the only curative treatment for PLD, medical therapies are gradually being developed with the increasing knowledge of the disease’s pathophysiology. This review focuses on the clinical manifestations and diagnosis of PLD, as well as treatment strategies, to support clinicians regarding the clinical management of the disease.

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  • Predicting Safe Liver Resection Volume for Major Hepatectomy Using Artificial Intelligence
    Chol Min Kang, Hyung June Ku, Hyung Hwan Moon, Seong-Eun Kim, Ji Hoon Jo, Young Il Choi, Dong Hoon Shin
    Journal of Clinical Medicine.2024; 13(2): 381.     CrossRef

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