disease (PCLD) is an autosomal dominant genetic disorder characterized by progressive development of multiple parenchymal cysts in the liver and may also include renal cysts., Clinically, the prevalence of PCLD has been estimated to be 0.001%, which is relatively low in comparison with other liver diseases.
Abbreviations ADPKD: Autosomal dominant polycystic kidney disease ADPLD: Autosomal dominant polycystic liver
disease CLF: Chronic liver failure HA: Hepatic artery IVC: Inferior vena cava MELD: Model for end stage liver disease OLT: Orthotopic liver transplant PLD: Polycystic liver
disease PV: Portal vein RHV: Right hepatic vein.
diseases (PLDs) represent a group of genetic disorders in which cysts occur in the liver (autosomal dominant PLD) or in combination with cysts in the kidneys (autosomal dominant polycystic kidney disease).[sup], Most patients with PLD are asymptomatic and require no special treatment.
Clinical profile of autosomal dominant polycystic liver
Isolated polycystic liver
disease, which is far less common than ADPKD, is characterized by numerous hepatic cysts and an absence of renal cysts.
Patients, who have massive polycystic liver
disease as a complication of ADPKD may experience abdominal pain and altered nutrition.
He reviewed the records of 11 patients with polycystic liver
disease--10 of them women--who collectively underwent 20 laparoscopic cyst perforation procedures for relief of symptoms.
disease is found in 30% of patients with polycystic kidney disease, although the patients clinical course is usually determined by the renal involvement. Congenital cysts are outlined by an epithelial lining and probably originate secondary to an embryonic maldevelopment of bile ducts. They comprise 94% of all cysts and are the most common cysts seen in the Western world.
Thi s can take several forms: (1) cysts in the main trunk (choledochal cysts), (2) cysts (or lakes) in the small branches within the liver (Caroli's Syndrome) or (3) cysts in the liver separate from the biliary tree (polycystic liver
Autosomal Dominant Polycystic Kidney Disease with multiple extra renal complications including polycystic liver
disease, epigastric hernia and pericardial effusion.
BCS may arise secondary to hypercoagulable states infections malignancies or due to other conditions including membranous obstruction of the vena cava (MOVC) polycystic liver
disease sarcoidosis trauma to hepatic veins or inflammatory bowel disease (IBD).
Hogan's talks about Polycystic Liver
Disease can also be watched on YouTube.