92% of PLD'rs have no symptoms.
These lucky 92% have polycystic liver disease but they do not need to be concerned about it. Without experiencing any symptoms it is very likely that there is no need for treatment. Polycystic livers can go from a texture similar to a smooth peach to that resembling a bumpy fluid filled raspberry. Polycystic liver disease is inherited, either with cysts limited to the liver (ADPLD) or with cysts involving both the liver and kidneys (ADPKD). Another possibility is a spontaneous occurrence. No one else in your family has cystic organs but you do. From this point on, passing on cystic organs is exactly like inherited cystic organs. Increased occurrence for this risk can possibly happen in utero (this is not known). Maintaining the integrity of ones own personal DNA can squelch the second hit mechanism that causes an increase in cyst numbers and symptoms.
An individual in their 20's or 30's having only 2-3 liver cysts, will rarely develop massive symptoms from their polycystic liver. If an individual is in their 20's or 30's and having difficulties, enlarged liver size, then this person should see a specialist in the treatment of PLD. One such specialist is Dr. Vicente Torres at the Mayo Clinic in Rochester, Minnesota.
Interestingly a greater percentage of females have difficulties, almost 8:1. Several studies have looked at liver cyst growth in relation to pregnancy and estrogen. Hormones, caffeine, and liver toxins cause liver cyst growth. Women who went on to use HRT hormonal replacement therapy following menopause experienced greater cystic liver growth. Liver cysts can occur as fine small grape like clusters or they can occur as very large cysts or a mixture of both types. In general, liver cysts are a disease of aging. If we live long enough, eventually all develop a liver cyst.
The Other 8%Only a few of us, about 8%, develop severe PLD Polycystic Liver Disease. We will require some type of de-bulking procedure to decrease the size of our huge cystic liver. Severe PLD liver can reach 40 pounds or more. It is capable of compressing the vena cava, the heart, the uterus, the pancreas and many more of the internal organs. This happens to only a few with PLD, to those with severe PLD.
How is ADPLD Passed Onto Children?
ADPLD has only liver cysts present; there are no kidney cysts. ADPKD can have both kidney and liver cysts. Each child of a parent with ADPLD has a 50% chance of inheriting the disease, because the gene for ADPLD is dominant. The prospects are similar to a flip of a coin. So, although 1 in every 2 children has a chance of inheriting ADPLD, sometimes both or neither will be affected. This form of inheritance is called Autosomal Dominant inheritance.
All in our environment can influence the expression of PLD symptoms within us. Researchers have given a few early answers; to decrease PLD symptoms try to diminish exposure:
Avoiding these are thought to possibly help diminish symptoms.
PLD DifficultiesAt least 90% of ADPKD individuals have liver cysts. It has been postulated that perhaps 100% of individuals with ADPKD have some liver cysts especially if they can be imaged with a fine slice MRI machine. 100 CRISP study participants, were imaged showing that most, if not all, had liver cysts. However, only a very small percentage go on to develop difficulties related to their liver cysts.
Definition of PLD
If someone has one large liver cysts or even up to three liver cysts, these are simple cysts and not necessarily autosomal dominant polycystic liver disease or autosomal dominant polycystic kidney disease with liver cysts. The definition of PLD is 20 or more liver cysts.
Hormones & PLD
Hormones, particularly estrogen, rapidly spurs the growth of liver cysts. Caffeine, bleach, plastic, ammonia, dairy, false estrogens, soy and flaxseed also increase liver cyst pain and growth. As the liver becomes filled with cysts, the metabolism of estrogen within the liver diminishes. A saliva hormonal profile test can document these changes, but many are reluctant to have costly lab tests performed when it seemingly does not alter the treatment of PLD.
Listen to a PLD lectureIt is by Dr. Torres or watch a PLD webinar by by Dr. Marie Hogan.
Inheritance of PLD
PLD, either ADPLD (without kidney cysts) or ADPKD (with kidney cysts) is inherited. It has an autosomal dominant pattern of inheritance for polycystic liver disease. This means that it is the flip of a coin or a 50% chance that a child will be affected. It does not skip a generation. However there are spontaneous occurrences meaning this maybe the very first time PLD is seen in a family. From this person on, PLD will act the same as the inherited kind. It can be passed on to children. About 10% of cases are spontaneous occurrences.
Liver cysts are a disease of aging. I have heard it said that the older we get perhaps 100% of individuals will develop a single liver cyst provided they live long enough. PLD by definition has the appearance of twenty or more cysts on the liver. From the PKD CRISP study researchers looked at participants' livers with fine sliced MRI imaging. About 99% had liver cysts, but only 8% of these go on to develop severe PLD. The other 92% have very little if any, symptoms.
How do Liver Cysts Develop?
Included among liver cells are hepatocytes and cholangiocytes. Liver cysts arise only from cholangiocytes cells, yet cholangiocytes make up only 3% of all liver cells. These cholangiocytes are very important in that they produce 40% of the bile. Hepatocytes, the other liver cells, make up 80-90% of the total liver tissue. Cholangiocytes have somatostatin receptors on their exterior cell surface. The interior of liver cysts contain cyclic AMP. Cyclic AMP is elevated in the interior of all cyst cells. Exciting results were published by the Mayo Clinic in the GastroEnterology Journal. Animal studies using Octreotide dramatically decreased both liver and kidney cyst growth. Octreotide is a somatostatin analogue. It stimulates somatostatin receptors and this decreases cyclic AMP within the cyst. Decreasing cyclic AMP diminishes cyst growth. Researchers are hopeful that clinical trials with Octreotide will prove useful in diminishing liver cysts for polycystic liver disease. Sometimes we might experience a prolonged bleeding time or a rise in billirubin, but these are scarce events. News received from time to time, is it is a rare event for a cystic liver to fail. Yet as a polycystic liver enlarges it will gradually slow down in performing some of its processes. PLD forms multiple cysts throughout the liver. Normal non-cystic liver tissue re-grows attempting to displace the cystic liver. The liver is the only human organ capable of regeneration. This regeneration can create a massively growing cystic liver. Here is more information on octreotide.
What things cause liver cysts to grow?
Caffeine and estrogen (phytoestrogens, false estrogens, xenoestrogens) all cause liver cysts to grow. Additionally as a cystic liver enlarges, the metabolism of estrogen by-products is lessened. There are several types of estrogen. Estrogen by-product is one type of estrogen. I suspect that this is very in high in pregnancy and in cystic liver disease. The liver interprets estrogen look-a-likes as true estrogen and this triggers further liver cyst growth. Even hormones produced by the body can cause liver cyst growth. Caffeine and hormones cause liver cysts to enlarge.
Individuals with massive liver cysts might consider alternatives to pregnancy. Pregnancy, hormone replacement therapy, and birth control pills all can cause cystic livers to enlarge. If one adds to this the caffeine PKD research by George and Judy Tanner that caffeine elevates PKD blood pressure and earlier PKD research on caffeine by Dr. Grantham, that caffeine also stimulates cyclic AMP causing cyst production to begin. Coffee ends up a big double whammy for individuals with PKD Polycystic Kidney Disease and PLD Polycystic Liver Disease. There are many exogenous estrogens in our environment. Some are man made, some are called natural yet main contain added estrogen to it; others are artificially added to foods, cosmetics, appearing in many unexpected places.
Liver function rarely declinesLiver functioning rarely declines, however with massive polycystic liver disease there are some abnormalities in our blood work. ADPLD is the absence of any kidney cysts. ADPKD has both kidney and liver cysts.
All of the ADPLD diagnosed individuals had:
↑ alkaline phosphatase
↑ total billirubin
|PLD Lecture: Dr. Torres||PLD Webinar Slides||PLD Webinar: Dr. Hogan|