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| POLYCYSTIC LIVER DISEASE LECTURE |
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ABOUT POLYCYSTIC LIVER DISEASE PLD Click here to listen to this lecture now by Dr. Vicente Torres given June 15, 2004 at the PKD conference. Dr. Vicente Torres past chair of scientific advisory committee for PKD Polycystic Kidney Disease. He has stepped down as the longest standing member of the scientific advisory committee for the past 12 years. Dr. Torres is a Professor of Medicine and Chair of Division of Nephrology at the Mayo Clinic Rochester MN USA. He has authored many PLD research projects. |
What is PLD Polycystic Liver Disease?
Polycystic
Liver Disease is inherited as either ADPKD autosomal dominant polycystic kidney
disease or ADPLD autosomal dominant polycystic liver disease. The good news is that most individuals with Polycystic Liver Disease never have the need for any type of treatment. I am included among the 8% of us that need some help to decrease the heavy bulk created from expanding and growing liver cysts.
Liver cysts are very unique. The liver, unlike other organs, will regenerate and grow healthy liver tissue that continues to function normally to exists alongside cystic liver tissue. This useful tool is what causes our cystic livers to enlarge greatly. Researchers measured healthy livers, cystic livers, and extensively cystic livers. In all three groups, the non-cystic smooth normal liver portions were equal. This is one reason that cystic livers rarely fail. The huge mass of a growing liver does compress other organs, blood vessels, and even bones.
There are a few functions of the liver that will remain abnormal throughout the progression of polycystic liver disease. It is the liver’s job to clear pesticides, toxins, bleach (known as false estrogens) and hormones from the body. The liver converts this to a liquid molecule that can be passed on to the kidneys and excreted. The metabolism of all hormones is greatly diminished through a cystic liver. Mine is also diminished. The problem is that liver cysts have receptors that utilize molecules that resemble hormones, even false hormone, and phytoestrogens causing the liver to expand, enlarge and increase the number of cysts.
The overall size of a cystic liver puts added weight and pressure that is constant, squeezing many organs causing difficulties. It can crush the largest blood vessel called the vena cava creating a sluggish trickle of blood that poses a danger of clot formation. Pain and discomfort is a very real issue. This constant pressure happens very slowly increasing bit by bit - a reminder of the generalized mass effect produced by cystic livers, that oftentimes we think we might be complaining or even imaging the existence of this very real pain. The never-ending slow pressure can deform bones, sometimes breaking a rib. Cystic livers displace our stomachs so nourishment becomes difficult. Our upper bodies become thin. The lower body appears fat from the fluid and edema. Back pain can result by carrying around this cystic liver that can weigh up to 40 pounds. We become increasingly tired from this slowed blood, the low platelets, low white blood cells, the weight, the pain, and more. If you experience pain and discomfort from your cystic liver, it is time to seek help for it. Continue to try and succeed at locating someone willing to help with this.
Polycystic livers can form small cysts, large dominant liver cysts or a combination of small medium and large liver cysts. It is through fine imaging studies with cross sectional MRI and CT cuts that physicians are able to view microscopic liver cysts that were previously hidden and not apparent. These flattened small cysts deep within the liver eventually expand seemingly becoming larger obvious liver cysts. Were these cysts present from birth? This is a very good question. Some researchers feel this is so.There are a few
that have both ADPKD, medullary sponge kidney and liver cysts. With ARPKD, autosomal
recessive polycystic kidney disease, sometimes Caroli's disease of the liver
is seen, a dilation of the hepatic ducts. Dr. Torres and Dr.
Somlo have isolated the gene that is inherited with ADPLD. They are looking
for volunteers to participate in genetic studies. Email The criteria for diagnosing PLD is contained in this article.
From
the CRISP study researchers looked at the liver along with the
kidneys of individuals with ADPKD and 90% of individuals had liver cysts. A
small percentage of these go on to develop difficulties with their liver cysts.
An individual in their 20's or 30's having only 2-3 cysts, this individual will rarely
develop symptoms from their liver cysts. Interestingly a greater percentage of
females will have difficulties. Several studies have looked
at liver cyst growth in relation to pregnancy and estrogen. Hormones cause a growth of liver cysts. Women who went on to use HRT hormonal replacement
therapy following menopause experienced a growth whereas individuals who did
not take HRT did not experience liver cyst growth.
The
liver cysts can occur as fine small grape like clusters of cysts or they can
occur as very large cysts. Most individuals if they were to live long
enough would eventually develop liver cysts. If someone has one large liver
cysts or even up to three liver cysts, this are simply liver cysts and not necessarily
autosomal dominant polycystic liver disease or autosomal dominant polycystic
kidney disease with liver cysts.
What is the liver?
The
liver is the only organ within the human body which has the ability to regenerate
itself. It lays protected by the ribs, tucked behind them, and weighs approximately
2-3 pounds. A muscle, the diaphragm, slides over the liver, isolating it from
the lungs. With polycystic liver disease, the liver alone can reach a weight
of 40 pounds or greater and can extend well beyond its usual place in the body. The liver
manufactures some hormones and converts many end products to a water-y compound
which can then be excreted by the kidneys or mixed with bile and through the
intestines, leaves the body. The
liver is unique in that it has dual blood vessels: the portal veins and the
hepatic veins.
What are the symptoms from PLD?
Click here to read about the symptoms.
Is there any current research ongoing with PLD?
Here are (3) different sites about PLD research:
Research projects on cystic organs
Research projects genetic ADPLD
Research projects with PLD Octreotide
last updated
Thursday, March 6, 2008 2:55 PM