Hepatic Medicine: Evidence and Research (Sep 2022)
Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment
Abstract
Luiz Fernando Norcia,1 Erika Mayumi Watanabe,2 Pedro Tadao Hamamoto Filho,3 Claudia Nishida Hasimoto,1 Leonardo Pelafsky,1 Walmar Kerche de Oliveira,1 Ligia Yukie Sassaki4 1Department of Surgery, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil; 2Department of Radiology, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil; 3Department of Neurology, Psychology and Psychiatry, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil; 4Department of Internal Medicine, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, BrazilCorrespondence: Luiz Fernando Norcia, Department of Surgery, São Paulo State University (UNESP), Medical School, 783 Pedro Delmanto Street, Botucatu, São Paulo, 18610-303, Brazil, Tel +55 19982840542, Email [email protected]: Polycystic liver disease (PLD) is a clinical condition characterized by the presence of more than 10 cysts in the liver. It is a rare disease Of genetic etiology that presents as an isolated disease or assoc\iated with polycystic kidney disease. Ductal plate malformation, ciliary dysfunction, and changes in cell signaling are the main factors involved in its pathogenesis. Most patients with PLD are asymptomatic, but in 2– 5% of cases the disease has disabling symptoms and a significant reduction in quality of life. The diagnosis is based on family history of hepatic and/or renal polycystic disease, clinical manifestations, patient age, and polycystic liver phenotype shown on imaging examinations. PLD treatment has evolved considerably in the last decades. Somatostatin analogues hold promise in controlling disease progression, but liver transplantation remains a unique curative treatment modality.Keywords: liver, polycystic liver disease, hepatomegaly, liver cysts, therapeutics
