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Porphyria Educational Services


PORPHYRIA EDUCATIONAL SERVICES Bulletin Vol. 1 No. 14 April 4, 1999
The Liver & Gallbladder

The liver is a an important focus in the hepatic types of acute porphyria as well as some of the cutaneous forms.   Before focusing on the porphyria types and the liver, let us look chiefly at the liver itself.  How much do we each know about our liver?   The largest internal organ in the human body.

The liver is the main organ that keeps you alive.
  It is said of the liver that it performs over 100 separate bodily functions.

The sheer complexity of the liver makes it susceptible to almost as many different diseases.  Many of these diseases are rare, or like some types of porphyria, not rare, but hardly common either. Of the common liver diseases some are "household names" that are all too common, including hepatitis, cirrhosis, liver disorders in children, alcohol-related disorders, and liver cancer.
  In a report published in a medical journal in 1998, it states that over 25 million people are afflicted with liver and gallbladder disease each year. Furthermore the report states that over 27,000 Americans die from cirrhosis annually.  With a mortality census like this, it makes cirrhosis of the liver the third leading cause of death for people between the ages of 25 and 59, and the seventh leading cause of death overall in the United States today.  

Viruses, hereditary defects such as porphyria, and reactions to drugs and chemicals are among the known causes of liver breakdown.
  Though few treatments are effective for life-threatening liver disease, avoiding alcohol and other substances known to cause damage can do a lot to safeguard this important organ.  Many drugs are unsafe for use in people with liver disease, so it is vital to read up on any pharmaceutical before taking it, even if your doctor has presecribed it for you.  This is especially true in porphyria.  If a drug is metabolized in the liver, you do not want to use it.   Porphyrins are manufactured in the liver.  With increased acute attacks or what some call chronic porphyria, the liver may be damaged more readily.

Avoiding drugs is imperative.  Seeking immediate Intervention Therapy is also imperative to reduce the risk of developing porphyrin crystallization in the liver.
  So where is this organ that plays such a vital role in porphyria?

The liver is located behind the lower ribs, right below the diaphragm on the right side of the abdomen.  In an average-sized man, it is about the size of a football, weighing a little over three pounds.
  The liver can be compared to an oil refinery, or a processing plant. The liver processes many chemicals necessary for the body's overall functioning.  For example, it converts carbohydrates, fats, and proteins into chemicals essential for life and growth. It manufactures and exports to other organs some of the substances they need to function properly, such as the bile used by the intestines during digestion.   By now all of us know the word "metabolizes".  This is done in the liver.

It modifies drugs taken to treat disease so that they can be used more easily by the body.  And it cleanses the blood of toxic substances either
ingested or produced by the body iself.   But that's only part of the picture.  Below are some of the liver's many other important functions:

-Regulates the blood's ability to clot
-Governs the transport of fat stores
-Stores extra vitamins, minerals, and sugars to prevent shortages
-Produces quick energy as needed
-Controls the production and excretion of cholesterol
-Breaks down alcohol Monitors and maintains the right level of numerous chemicals and drugs in the blood
-Maintains and controls hormone balance
-Helps the body resist infection by producing immune factors and cleansing bacteria from the blood
-fsStores iron

Viral hepatitis, a contagious infection of the liver, afflicts more than 70,000 Americans each year.  It is usually caused by one of three different organisms.  

Hepatitis A, formerly known as infectious hepatitis, can be contracted by consuming contaminated water or food, most notably shellfish.  Since the virus is eliminated in the stool, it also spreads through improper hand washing, especially by restaurant workers or anyone else who handles food.
Although hepatitis A is seldom serious, in one percent of the cases it can cause severe liver failure and death.  It does not cause chronic hepatitis and will not lead to cirrhosis or other long-term liver problems.
 

Hepatitis B, formerly known as serum hepatitis, is found in blood and other body fluids such as urine, tears, semen, breast milk, and vaginal secretions.  It is usually transmitted in blood, via transfusions, or through illicit injectable-drug use.  But it also can be contracted through a minor cut or abrasion, or during such everyday acts as toothbrushing, kissing, or having sex.  Infants can contract the disease from the mother at birth, or from the mother's breast milk.  Dental work, ear piercing, and tattooing are other ways people can get hepatitis B.  

Type C hepatitis virus is the cause of a disease known as "non-A, non-B hepatitis," which is also contracted through contact with contaminated blood, or through household or sexual contact with an infected person.  It affects approximately 170,000 Americans each year.  

The problem with hepatitis B is that five to 10 percent of those who become infected with this disease become chronic carriers who can spread it to others for an indefinite period of time.  At present there are more than a million of these silent carriers in this country, and their number is growing by two to three percent annually.  Consequently, authorities recommend that all children and anyone with a high risk of exposure be vaccinated against this dangerous virus.
 

Chronic carriers usually do not develop chronic hepatitis.  If it does develop, however, cirrhosis and primary cancer of the liver can be long-term consequences.  An estimated 4,000 people in the United States die from hepatitis B-related cirrhosis annually.  Carriers are many times more likely to get liver cancer than are non-carriers.
  Treatment for acute hepatitis consists of rest and small, nourishing meals; fluids; and sometimes anti-nausea drugs such as trimethobenzamide (Tigan). Chronic cases of hepatitis B and C are now being treated with interferon, a biotech medicine derived from the human immune system.  

THE GALLBLADDER The gallbladder stores bile, a substance the liver produces to aid digestion.  The most common disorder of the gallbladder is the formation of gallstones.  These stones often get stuck in the bile ducts that lead from the gallbladder to the first part of the small intestine, causing the gallbladder to become inflamed.  Gallbladder surgery, in which the entire organ is removed, is one of the most common operations in this country. Laparoscopic cholecystectomy, a new procedure that requires only a small incision, has cut down stress and recovery time dramatically.  Chenodiol, a recently available drug that dissolves gallstones, is an alternative to surgery.  However, due to limited success and troublesome side effects, it is not widely used.  

Other Diseases of the Liver Return to top In cirrhosis, liver cells are damaged and replaced by scar tissue which, as it accumulates, hardens the liver, diminishes blood flow, and causes even more cells to die.  The loss of liver function that accompanies this degenerative condition results in gastrointestinal disturbances, jaundice, enlargement of the liver and spleen, emaciation, and accumulation of fluid in the abdomen and other tissues.  Over half of the deaths related to cirrhosis are due to alcohol abuse, hepatitis, and other viruses. Chemicals, poisons, too much iron or copper, and blockages of the bile duct also may cause the disease.
 

Treatment of cirrhosis usually consists of eliminating the underlying cause, if possible, to avoid further damage, and preventing or treating complications.  Care is mostly supportive, often including a specialized diet, diuretics (water pills), vitamins, and abstinence from alcohol.  For some patients, a liver transplant is now a feasible option.
 

Liver abscesses are caused by bacteria, such as Escherichia coli (E.  coli) or staphylococcus (staph), or by Entamoeba histolytica, the parasite that causes amebic dysentery.  In either case, the offending organisms destroy liver tissue, leaving a cavity that fills with other infectious organisms, white blood cells, and liquefied liver cells.  Common symptoms include shoulder and abdominal pain, fever, weight loss, chills, nausea, vomiting, anemia, and, if there is severe liver damage, jaundice.  If the offending organism can't be determined, liver abscesses are treated with long-term antibiotics such as aminoglycosides, cephalosporins, clindamycin, or chloramphenicol.  If E.  coli is causing the infection, treatment includes ampicillin; for Entamoeba histolytica, chloroquine (Aralen), or metronidazole (Flagyl) are included.  

Pediatric liver diseases afflict tens of thousands of children in this country annually, and kill hundreds each year.  More than 100 different liver diseases are found in infants and children.  Most of these disorders are genetic.  Among the more common are:   Biliary atresia, an inadequate bile duct, often fatal but sometimes relieved by surgery; Chronic active hepatitis, in which scar tissue forms and destroys the liver; Wilson's disease, in which an abnormally large buildup of copper in the liver is treated with vitamin B6 and d-penicillamine, or, in some cases, corticosteroids such as prednisone; Reye's syndrome, an acute, often fatal disease secondary to flu or other infections in which fat accumulates in the liver and the patient lapses into coma.

Other serious diseases of the liver, fortunately seen less frequently than these discussed above, include fatty liver, hepatic coma, and liver cancer.