Polycystic liver disease: a clinical review



Document title: Polycystic liver disease: a clinical review
Journal: Annals of hepatology
Database: PERIÓDICA
System number: 000417623
ISSN: 1665-2681
Authors: 1
Institutions: 1Western University, Department of Medicine, London, Ontario. Canadá
Year:
Season: Nov-Dic
Volumen: 11
Number: 6
Pages: 819-826
Country: México
Language: Inglés
Document type: Revisión bibliográfica
Approach: Analítico
English abstract Polycystic liver disease rarely occurs in isolation as part of autosomal dominant polycystic liver disease, but more commonly, it exists as an extra-renal manifestation of autosomal dominant polycystic kidney disease. The pathogenesis of polycystic liver disease involves defects in the primary cilium of the cholangiocyte, with genetic mutations that impair key proteins integral to the complex functioning of cilia. While most patients are asymptomatic and require no intervention aside from reassurance and genetic counseling, in a minority of patients, polycystic liver disease creates a myriad of symptoms from the compressive effects of enlarged cysts, and can even cause malnutrition and liver decompensation in the severest of cases. In patients with symptomatic disease, a variety of interventional radiology or surgical techniques can be considered, including aspiration with sclerotherapy of a dominant cyst, fenestration, segmental hepatic resection, and even liver transplantation. Although there are no curative medical options for polycystic liver disease, somatostatin analogs hold promise and have shown minimal efficacy in human studies. However, further research is needed to develop more efficacious medical treatments
Disciplines: Medicina
Keyword: Gastroenterología,
Genética,
Terapéutica y rehabilitación,
Enfermedad poliquística,
Hígado,
Quistes,
Epidemiología,
Patogénesis,
Manifestaciones clínicas,
Historia natural
Keyword: Gastroenterology,
Genetics,
Therapeutics and rehabilitation,
Polycystic disease,
Liver,
Cysts,
Epidemiology,
Pathogenesis,
Clinical manifestations,
Natural history
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