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POLYCYSTIC
LIVER DISEASE TREATMENTS
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WHAT IS POLYCYSTIC LIVER DISEASE? Polycystic Liver Disease is inherited as either ADPKD autosomal dominant polycystic kidney disease or ADPLD autosomal dominant polycystic liver disease. With ARPKD, autosomal recessive polycystic kidney disease, sometimes Caroli's disease of the liver is seen, dilation of the hepatic ducts. Dr. Torres and Dr. Somlo have isolated the ADPLD gene. Research for ADPLD includes a genetic study. Email for more information. The criteria for diagnosing PLD is contained in this article.
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HOW DO LIVER CYSTS DEVELOP? Liver cells are composed of hepatocytes or cholangiocytes. Liver cysts arise only from cholangiocytes cells. Cholangiocytes make up only 3% of all liver cells. These cholangiocytes are very important in that cholangiocytes produce 40% of the bile. Hepatocytes however make up 80-90% of the total liver tissue.
Cholangiocytes have somatostatin receptors on the exterior cellular surface. The interior of liver cysts contain cyclic AMP. Cyclic AMP is elevated in the interior all cyst cells.
Exciting results were published by the Mayo Clinic in the GastroEnterology Journal. Animal studies with Octreotide dramatically decreased 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. According to the article in Gastro Enterology, within liver cholangiocytes and within the serum of Polycystic rats, these contained approximately 2 times higher concentrations of cAMP than in the normal rats. Researchers are hopeful that clinical trials with Octreotide will prove useful in diminishing liver cysts in humans and also may be helpful for Caroli's Disease as well.
MEDICAL TREATMENTS PLD Avoid
hormones, caffeine, and if reflux is present a secondary benefit might be gained
from taking prilosec (and other H2 receptor blockers or proton pump inhibitors). This results in a reduction in the hormone secretin. Diminished secretin results
in liver cyst expansion and a filling with fluid.
Use caution with nexium. In the over 50 crowd long term use can increase the risk of hip fractures.
The liver converts toxic compounds to another second set of compounds, surrounding this set with a watery fluid. This second compound is often more toxic than the original compound. The liver does this so all of the products which the body has been using, the left over residues, can then be eliminated easily through the kidneys. As cysts replace liver cells this process becomes slowed and sluggish, resulting in an abundance of circulating, water-filled, toxic molecules. This triggers further cyst growth. The back up of toxic fluid affects the kidneys which are unable to eliminate all of the compounds. This may then trigger further cyst formation within the kidneys that causes destruction of kidney cells. This makes the body more acidic. This acidity affects the liver and kidneys adversely.
There are (4) surgical treatments for PLD
(1) Liver resection
(2) Liver transplant
(3) Liver artery ablation (a new procedure pioneered by Dr. Ubara in Japan)
(4) Liver cyst reduction marsuplization deroofing decortication
(5) Liver dialysis has the possibility of offering the possibility of some temporary relief.
(1) Liver Resection The greatest number
of successes from liver resection surgeries are from those performed at the Mayo Clinic in Rochester Minnesota by Dr. D M Nagorney or someone who has trained with him. In order to be a candidate for a liver resection there must be two (2) segments of the liver which remains after the resection, that is relatively cyst free. We have (8) eight. Following the liver resection, the veins of the liver must remain open allowing for a good blood flow to the liver remnant. A few have reported receiving a bloodless liver resection in North Carolina. Here are some articles about liver resection.
PLD Liver resection Chinese experience
PLD liver resection treatment of choice
PLD liver resection Alberta teaching hospital
PLD liver resection Mayo PLD liver resection Dr. Nagorney
PLD liver resection 1995 Dr. Nagorney (1) Liver cyst reduction. This is for pain relief and provides temporary relief from counter pressure upon other body organs from an enlarged cystic liver. Pain is the most common symptom we PLD'rs have. According to the following articles liver deroofing usually requires repeated surgeries and ultimately either a liver resection or liver transplant to reduce the mass effect of extensive cystic liver disease will be required.
(2) Liver transplants may be done with either a living donor or a cadaver donor. The living donor is not without risk. About 75% of individuals who need surgical intervention for liver cysts are candidates for a liver transplant. It takes a great deal of patience to become the donor of a cadaver liver. It is rare for cystic livers to fail.Time on the transplant list matters and eventually your name will come to the top. One individual received a bloodless liver transplant. Several lucky individuals have received liver transplants from Florida, from the UK, from the Netherlands and from France for polycystic liver disease. Read more on liver transplants.
(3) Liver Artery Ablation is relatively new and this is being done in by Dr. Ubara in Japan. The doctors look carefully at the blood supply to the liver and block one of the vessels which feeds a cystic section of the liver. With the blood supply lost, eventually this portion of the liver will no longer be fed. There is pain for the patient lasting perhaps about a week. In one years time there has been a 40% reduction in the liver size.
(4) A liver resection can be curative (in the best of surgical hands). Deroofing or sclerosing or marsupilization is done for pain relief. This is a temporary procedure for pain relief only. A review of the literature shows that this procedure can take as long a time period to perform as a liver resection and oftentimes it has to be repeated. If the doctor has had experience with polycystic liver cysts, he will tell you that eventually you will need either a liver transplant or a liver resection. Not everyone with liver cysts needs a surgical procedure, only those with severe polycystic liver disease require surgical intervention.
Deroofing Ethanol exophytic liver cysts NJ
Deroofing 2006 RSNA recurs in 75%
Deroofing UK liver cyst experience.
Laparoscopic deroofing Colorado symptomatic relief liver cysts was undertaken in eight patients, with one conversion to open technique because of bleeding from a superficial hepatic vein.
Laparoscopic nephrectomy 35 page report
Laparoscopic deroofing of hepatic cyst Sultan Qaboos University
Laparoscopic deroofing China fenestration of simple cysts
Laparoscopic deroofing Hungary 2006 Laparoscopic deroofing was indicated when a single liver cyst was larger than 5 cm (the general size of cysts was 6.9 cm) and caused complaints and was in a superficial position.
Laparoscopic deroofing Mexico 2004 laparoscopic deroofing is reserved for type I polycystic liver disease. There are no prospective randomized studies that show benefits of laparoscopic over open surgery.
Laparoscopic deroofing Greece 2005 Despite the reported morbidity, aggressive and meticulous deroofing of type II PLD is possible.
Laparoscopic deroofing Poland 2001 Fenestration (deroofing) with excision of the liver cyst wall is increasing.
Open deroofing Poland 2001 Open surgery is safe and effective for symptomatic liver cyst and complication rate is low. Deroofing 2000 Albert Einstein Med Ctr USA For simple liver cysts, percutaneous aspiration invariably leads to recurrence; laparoscopic deroofing is usually curative.
Deroofing Edinburg Scotland 1998 no symptomatic recurrences after 14 hepatic resections liver cysts.
Deroofing 2003 UCI California. Cyst decortication of 29 polycystic kidney patients One patient had a >20% increase in creatinine clearance; six patients had worsening hypertension. Every detectable cyst within 2 mm of the renal surface was treated. About half of the patients noted a 50% decrease in their pain.
(5) Liver Dialysis - The only difficulty with liver dialysis, is polycystic livers rarely fail. Liver dialysis would be helpful should a cystic liver fail. Jan-Feb 2004 article has shown that liver dialysis
has now become available on a limited basis. According to Hepatitis central
it has received FDA approval:
Sites include:
• Tulane
• University of Tennessee-Bowld
• University of Texas- San Antonio
• Loyola University
• University of Iowa
• California Pacific Medical Center
• University of California at San Diego
• New York University
• Oklahoma City
• Mayo Arizona, Jacksonville, Rochester and more
It is only for use short term according to this article.
I am wondering if it might be useful when coupled with a liver resection until
the liver remnant kicks and starts functioning?
We are each very unique in how our liver cysts grow and how we respond to treatments. I do not have the experience you have with having undergone such an extensive deroofing procedure. It has been going on near to 10 years that I have been gleaning information from many individuals around the world who have had several different procedures done for their liver cysts. I can only hope that others are able to find such a fine surgeon as you have found. Unfortunately for most, surgeons familiar with liver cysts are few. Many of us are from around the globe: UK, Netherlands, Indonesia, India, Germany, Japan, Australia, France, Ireland, Scotland, Croatia, Philippines and more. Most have access possibly to liver transplants. Not many are even aware that a liver resection is available. In the hands of surgeons not as experienced as Dr. Nagorney, liver resection leads eventually to a liver transplant. In the hands of surgeons not as experienced as Dr. Imagawa or Dr. Clayman, a cyst deroofing provides little if any relief of pain.
Lyn who founded a support group for PLD opted for a liver cyst deroofing. She found no pain relief and the liver cysts that were deroofed grew back with a vengeance according to Lyn. Another from Germany received pain relief for about 3 months. Another lasted a year. And there are others who have the deroofing repeated every 3 years. We each have our experiences.
I have heard from someone in Japan who had the hepatic artery ablation with Dr. Ubara. She said she did not notice much but was assured by her MRI that there was a 40% reduction in her liver size.
I am reporting the experiences of others as they write to me. I think Dr. Nagorney has magic hands if one qualifies for a liver resection and can manage to get to Rochester Minnesota USA this liver resection can be curative.
I am well pleased with my liver resection. I could not ask for more. My liver cysts do expand and contract with daily exposures to liver toxins and to certain foods that contain estrogen like compounds. These minor aches and pains I treat with lemon juice, saffron tea, and milk thistle plus. I too experience a low grade constant pain that does manage to disappear most days.
From time to time I get an MRI of my liver and kidneys. Since my liver resection surgery, there has been no new liver cyst growth. My total liver cyst volume has not grown. The non cystic portions of my liver did grow back and functions normally or near normally. I still have to watch the metabolism of estrogen through my liver. One of my liver enzymes is just at the high end of normal. All the other tests are fine and have remained at a constant normal for almost 8-10 years. To clarify this, I have noticed that my liver cysts tend to swell and subside in response to daily external influences. For the most part my liver cysts remain stable.
ARTICLES PLD (to download click the underlined links)
PLD How to diagnose
PLD gene for ADPLD
PLD lecture 2004 Dr Torres
PLD researcher Dr. Somlo
PLD lecture part one Dr. Perrone 2001
PLD lecture part two Dr. Perrone 2001
PLD treatment hepatic artery ablation
PLD treatment liver resection
PLD treatment liver resection when symptomatic
PLD treatment Mini invasive approach
PLD treatment liver resection Chinese experience
PLD treatment liver resection treatment of choice
PLD treatment liver resection Alberta hospital
PLD treatment liver resection Mayo Dr. Nagorney
PLD treatment liver resection 1995 Dr. Nagorney
PLD treatment liver resection insurance helpful
PLD treatment liver resection Netherlands experience
PLD treatment Netherlands experience
PLD Transplant Liver Jacksonville 1000th transplant
PLD Transplant Liver transplants over 70
PLD Transplant man moves to Florida for liver
PLD Transplant Anti-viral CMV prior to transplant
PLD Transplant USC world's first bloodless transplant
PLD Transplant guidelines living donor transplant
PLD Transplant LA Mom dual transplant children
PLD Transplant A bloodless Coup
PLD Transplant After two organ transplants robotics
PLD Transplant Helpful insurance
PLD Transplant Traded 46 pound cystic liver
PLD Transplant Altruistic donors needed
PLD Herbs Milk Thistle useful
PLD Herbs effects on liver disease
PLD Herb Phthalates toxic beauty products
PLD Herb avoid Maca contains hormones
PLD Herb Sprouts DIMs broccoli sprouts
PLD Herb Artichoke liver protector
PLD Herb Saffron liver
PLD Herb various alkaline
PLD Herb alternative
PLD Help Tests - Interpreting Liver detox profile
PLD Help Hot Flashes black cohosh not helpful
PLD Help Hot Flashes Effexor helpful
PLD Help Hot Flashes MPA poses risk
PLD Decline 7% Breast Cancer with less HRT
PLD Help Bentonite Clay
PLD Help Proton Pump Inhibitors risk hip fractures
PLD Help H2 Blockers differences
PLD Liver harm NSAIDs
PLD Liver harm Chlorine
PLD Liver harm Chlorine Italian study Carp livers
PLD Liver harm Chlorine water genetic mutation
PLD Somatostatin Liver ascites
PLD Somatostatin analogues neuroendocrine tumors.
PLD Orthostatic Hypertension in the elderly helped
PLD Octreotide prevents portal pressure after a meal
PLD Octreotide pancreatic injuries adjunctive role
PLD Octreotide Safety efficacy long-acting ADPKD
PLD Octreotide slow the growth of renal cysts?
PLD Octreotide research update on pages 18-19
PLD Prescribing information Long Acting
PLD Prescribing short acting Octreotide
PLD Octreotide long acting form - more information.
PLD Somatostatin Octreotide used in Acromegaly.
PLD Somatostatin Prescribing more information
PAIN RELIEF
Pain Laparoscopic denervation by J F Valente MD
Pain Laparoscopic denervation by Chang MD
Pain Management Torres MD
Pain GAIT study osteoarthritis knee pain
Pain Chondroitin GAIT study knee pain
Pain deroofing Sultan Qaboos University
Pain China laparoscopic fenestration cysts
Pain Cyst aspiration fenestration recur 75%
Pain
Ethanol Hepatic Cysts alt Liver Tx
Pain splanchnic pain South Africa
Pain splanchnic pain UK
RESEARCH CLINICAL TRIALS
Research update 2006 PKD conference
Research Story of reversal of heart transplant
Research Octreotide Sandostatin PKD clinical trial
Research Octreotide Sandostatin long acting
Research Somatostatin short acting IV
Research Somatostatin Octreotide Sandostatin LAR
Research Somatostatin Grantham MD
Research Somatostatin Acromegaly
LISTEN TO LECTURES
PLD POLYCYSTIC LIVER by Dr. V. Torres listen to this exceptional taped lecture on PLD
last updated Thursday, March 6, 2008 3:29 PM