Polycystic liver disease

Polycystic liver disease

    * Introduction
    * Signs and symptoms
    * Treatment
Polycystic liver disease is an inherited disorder in which many cysts form in the liver of various sizes associated with congenital hepatic fibrosis.
Both have in common pathological conditions increase the number of malformed bile ducts.
Polycystic liver disease is a genetic disease manifestation of PCOS. The first event is kidney damage, liver polichistoza is the most common extrarenal manifestation, is developing the later than the kidney, especially in women.
The tendency to develop multiple cysts are probably present at birth but usually does not become large cysts cause problems, not only in adult life. Cysts may be very small, no bigger than a needle and pins but can reach a size of 10 cm in diameter. The liver can remain the same size, or may become enlarged in volume. The body maintains its functions, the disease is not considered that would shorten life.
Polycystic liver disease is more common with age once but it's a rare condition affecting 1% of the population. Women tend to present more numerous cysts and large, a condition associated with increased levels of estrogen.
Polycystic liver disease begins at puberty, the symptoms become more obvious to the age of 30 years. Most infected people are diagnosed between 40 and 50 years. Distended abdomen, hepatomegaly, are often painful and associated with the disease. Often the disease is discovered accidentally or during the diagnosis of polycystic kidney. Almost all people with polycystic kidney cysts are present and liver.
Laboratory tests such as liver and kidney function are the first investigation performed. Liver function tests are useful for highlighting a liver destructions, malfunctii or hepatitis. They measure the levels of certain blood enzymes and proteins that can change when the liver is affected. It is still used and imaging tests such as ultrasound, magnetic resonance and computer tomography.
Cysts rarely require treatment. If indicators can be used too much of laparoscopic enucleation. In rare cases with multiple cysts, when the liver is excessively increased volume and painful liver transplantation is indicated. Treatment is chosen depending on the presence or absence of involvement of polycystic kidney.
Pathogenesis and causes
Polycystic liver disease is caused by mutations occurring in the PKD genes. PKD1 gene encodes a protein synthesis-policistina which is expressed on the plasma membrane of renal tubular cells, pancreatic and bile duct epithelium cells, and skin cells. Another gene shows some similarities with PKD1 PKD2. PKD 1 and 2 genes interact with each other and are interdependent.
Polycystic liver disease is part of the ductal plate maformatiile therefore associated with congenital hepatic fibrosis and Caroli syndrome. The disease can be associated with Peutz-Jeghers syndrome.
Liver cysts are derived from biliary epithelium and secretin-dependent. Hamartroamele derive from bile and may have initial communication with the biliary tree.