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as promised disorders in more detail

alcohol

When you drink alcohol it is quickly absorbed directly into the blood stream from the stomach and upper part of the gut (small intestine). The absorbed alcohol then passes through the liver and subsequently into the blood stream where it reaches all organs in the body. Although most tissues are capable of breaking down alcohol, this is mainly carried out by the liver, where alcohol is eventually converted into water and carbon dioxide, which is removed through the lungs.

Since the liver sees the highest concentrations of alcohol, it is one of the organs in the body most prone to developing alcohol related problems. However, alcohol also causes toxic effects on other organs in the body including the brain, heart, muscles and pancreas.

Almost all excessive drinkers will develop the first stage of alcoholic liver disease fatty liver. This is a ‘side-effect’ of the liver breaking down alcohol into carbon dioxide and water.  Fatty liver disappears when patients stop drinking excessively. If patients continue drinking excessively then a proportion (around 20-30%) will develop the next stage of alcoholic liver disease - alcoholic hepatitis. In this condition, the liver becomes inflamed and in its extreme form, patients can die of liver failure.

An even smaller proportion of patients (around 10%) will develop a permanently scarred and damaged liver (cirrhosis), if they continue to drink excessively.

Why certain heavy drinkers remain at the stage of fatty liver and others progress to alcoholic hepatitis and cirrhosis is not known at present, although undoubtedly, the more you drink and the greater the frequency and duration of heavy drinking, the more likely you are to develop the more advanced forms of disease. Recent evidence suggests that being overweight increases the risk of developing serious alcoholic liver disease and as yet largely unidentified genetic (inherited) factors may also be important.

Other harm caused by alcohol

Excessive drinking can also cause:

  • stomach disorders

  • pancreatitis leading to diabetes

  • high blood pressure

  • heart muscle damage leading to heart failure

  • strokes

  • cardiac rhythm disturbances

  • sudden cardiac death

  • vitamin deficiencies

  • sexual difficulties

  • problems with the brain

  • depression

  • problems with nerves in the limb

  • cancer of the liver, mouth, throat, gullet, large bowel and breast.

Hepatitis means inflammation of the liver.

An inflamed liver can be caused by:

  • Drinking too much alcohol

  • The side effects of some medicines and chemicals

  • Viruses

  • Some drugs (including certain herbal remedies)

  • Other causes (such as some inherited metabolic diseases).

Autoimmune hepatitis

 often referred to as AIH, is one cause of chronic hepatitis and can be, if untreated, one of the most severe forms. For a long time AIH was known as chronic active hepatitis. AIH is just one of the causes of chronic active hepatitis but it is now recognised as a specific disease.

The cause is not well understood, but it is believed to be the result of the immune system attacking the liver, as if the liver did not belong to the body. The cells that do the damage are circulating blood cells known as lymphocytes. They behave as though the hepatocytes (the liver cells) are foreign and start to destroy them. This leads to a chronic hepatitis which, if untreated will progress to cirrhosis and eventually to liver failure.

Fortunately autoimmune hepatitis is relatively uncommon. It occurs in both men and women. Between the ages of 15 and 25, it is women who are mostly affected.  Around the ages of 45 and 55, both men and women are affected.

 Budd Chiari

Budd-Chiari Syndrome (BCS), identified by Budd and Chiari in the mid 1890's, results from the obstruction of hepatic venous outflow; the most common cause in western countries being thrombosis (in Oriental and Middle Eastern countries, mass lesions and webs in the major hepatic veins are more characteristic)

With an average through-flow of 1.7litres per minute, the liver normally receives 70%-80% of its blood from the portal vein and the remainder from the hepatic artery. The blood mixes together and is collected in small venules within the liver lobes from where it is circulated to progressively larger veins, which ultimately become the major hepatic veins.

Most individuals have two groups of hepatic veins:

  • An upper group consisting of 3 large veins.
  • A lower group of generally smaller veins.

Between them, these veins drain the blood from the liver via the inferior vena cava (which is the large central vein passing through the liver on course from the abdomen back to the heart).

BCS is an interruption of this blood flow from the liver involving a blockage in one or more veins in either the upper or lower group.Where thrombosis occurs it may be partial or affect only some of the hepatic veins, which allows for compensated liver function and an absence of symptoms.

BCS may present as an acute condition which progresses rapidly with abdominal pain (in the upper right quadrant) ascites (fluid in the abdomen) and hepatomegaly (an enlarged and distended liver due to severe venous congestion). Alternatively, BCS may develop gedually over a period of months.

Ascites is present in 70%-90% of patients as result of the sudden development of portal hypertension but jaundice is not normally apparent.

For people who have no symptoms, BCS may be diagnosed as an incidental finding on a hepatic ultra sound.

Diagnosis can be made by liver biopsy, ultra sound, CT and MRI scans.

Hepatic venography can assist in identifying the exact location of the obstruction and may also be used as ameans of decompressing the liver, ideally by recanalising the hepatic veins. When this is not possible, shunts, including TIPPS (Transjugular Intrahepatic Portal Systemic Shunt), side to side portacaval shunts and mesoatrial shunts are used to re-direct the blood from the liver back to the heart thus converting the portal vein from an infow to an outflow vessel.

Stents may be inserted to hold veins/shunts open and ensure they remain "patent" (open)

Liver transplantation may be an option where severe fibrosis and other signs implying chronic liver disease are present.

BCS often occurs in patients with an underlying predisposition to thrombosis (such as myeloproliferative disorders) and those who use oral contrceptives, but a variety of other causes are also linked. Long-term anticoagulant medication is required to prevent a recurrence of the thrombosis.

The objectives of treatment for BCS are to:

  • Prevent propagation (increase) of thrombosis
  • Relieve hepatic congestion
  • Manage the ascites (e.g low sodium diet, diuretics)
  • Prevent further damage to the liver and allow the cells to regenerate.

Cirrhosis

cirrhosis is the medical term to describe excessive development of scar tissue (fibrosis) within a liver, irrespective of the underlying cause.  Usually, when the liver is acutely damaged, some of the liver cells die and the organ then regenerates itself without scarring. 

If, however, a chronic or repeated disease process damages the liver, then scarring starts to develop. This process usually starts slowly and progresses over many years without causing any symptoms.  Eventually excess scar tissue builds up and this begins to interfere with some of the vital functions of the liver.  At this point, the liver is no longer able to regenerate itself sufficiently. 

Symptoms can develop at any stage, but usually occur relatively late on in the scarring process.  Many of the symptoms are caused by the complications of cirrhosis when the liver is failing.

A number of conditions can lead to cirrhosis. The list below is not intended to be complete, but highlights common causes.

  • Excessive intake of alcohol

  • Chronic hepatitis C virus infection

  • Non alcoholic fatty liver disease (sometimes called NASH or non-alcoholic steatohepatitis)

  • Autoimmune chronic active hepatitis

  • Primary biliary cirrhosis and other chronic diseases of the bile ducts such as sclerosing cholangitis, or biliary atresia in children

  • Chronic hepatitis B virus infection

  • Inherited diseases:
              Haemochromatosis - abnormal processing of iron by the body
              Wilson’s disease - abnormal processing of copper by the body
              Glycogen or lipid storage diseases

  • Prolonged exposure to some drugs and toxins

  • Diseases of blood vessels e.g. Budd Chiari syndrome

It can be seen from this list that there are many causes of cirrhosis that have nothing to do with excess alcohol use.  In everyday life, however, the term cirrhosis has tended to become linked with alcoholic cirrhosis.  This commonly held mistaken belief leads to some degree of confusion about liver disease and cirrhosis among the general public. 

cystic diseases of the liver

One of the liver’s important functions is producing and excreting bile. This yellow-green fluid flows into the intestine through the bile ducts. The bile ducts in the liver are like the branches of a tree, that come together just below the stomach. A side branch leads to an organ storing bile, the gallbladder.

Gallbladder disease is a common type of illness involving the biliary tree. Less common is cystic disease of the biliary tree. This can take several forms:

  • Cysts in the main trunk (choledochal cysts)
  • Cysts in the small branches within the liver (called Caroli's syndrome)
  • Cysts in the liver separate from the biliary tree (polycystic liver disease)

What is a cyst?

A cyst is an abnormal fluid-filled balloon-like structure (a sac). It can occur in any part of the body. Liver cysts are mostly congenital (from birth) or parasitic.

What is a choledochal cyst?

In this condition, the main trunk of the biliary tree (the common bile duct) is structurally abnormal, probably from the time of birth. Eventually (usually by age 2 or 3 but sometimes not until adolescence or adulthood) the duct forms a cyst which may prevent the bile from reaching the intestine. Bile backs up into the liver and the patient becomes jaundiced (yellow). Occasionally this accumulation of bile becomes infected, causing abdominal pain and fever. In some patients the cyst can be felt by the doctor examining the abdomen. In most patients the diagnosis can be confirmed by using ultrasound or by injecting a radioactive substance which gives an image of the abnormal duct (nuclear medicine). In most cases surgical treatment permanently corrects the disease. Rarely infection recurs in the newly constructed biliary tree. If the condition is not correctly diagnosed the blockage of bile may result in scarring of the liver. This is called cirrhosis (for further information on cirrhosis please see the British Liver Trust factsheet).

What is Caroli’s syndrome?

Caroli's syndrome is another rare congenital disease. In this syndrome, the small branches of the biliary tree in the liver are abnormal. Small cysts alternate with narrowed segments of the bile ducts. These abnormalities may be present throughout the liver, or limited to only a small area. If the bile becomes infected, the patient develops fever, abdominal pain and rarely jaundice. This complication may first appear in childhood or may not occur until middle age. Caroli’s syndrome is diagnosed by imaging the bile ducts and also by injecting dye directly into the biliary tree. This may be done by inserting a needle through the skin into the liver (percutaneous transhepatic cholangiography - PTC) or by using a tube to pass dye through the intestine up into the bile duct (endoscopic retrograde cholangiography - ERCP). A liver biopsy may also be taken.

What is congenital hepatic fibrosis?

In patients with this condition, there is abnormal growth of fibrous (scar) tissue around the small branches of the bile ducts in the liver. As a result, the liver becomes enlarged and hard, and blood can no longer flow freely through the liver. The spleen, situated under the rib cage to the left of the liver, becomes enlarged because of back pressure. This causes swelling of the veins along the gullet (oesophageal varices). These may burst and cause bleeding into the stomach and bowels. People with this condition are usually discovered in childhood, either because of a large liver or because of bleeding. However in a few the diagnosis is not made until adult life. The diagnosis is by liver biopsy and x-rays of blood vessels. There is no specific treatment but many people require re-routing of blood from the intestines (shunt operation) to prevent more intestinal bleeding.

What is polycystic liver disease?

In some patients, large cysts develop from the biliary tree within the liver but do not obstruct the ducts. In severe cases, the liver looks like a sponge. These cysts may cause pain, but do not affect liver function. In most patients, the kidneys are similarly affected with cysts, which may cause high blood pressure and kidney failure. The tendency to form cysts is probably present at birth, but usually the cysts do not enlarge and give problems until adulthood. This condition may be detected using imaging methods such as ultrasound, CAT or MRI scan and x-rays of the kidney (intravenous pyelogram). Polycystic disease is inherited and once it has been detected in one member of a family, all the patient's relatives should be tested for it.

What are simple liver cysts?

Some cysts are commonly seen in the liver on ultrasound examination. These seldom cause symptoms. Occasionally if the cysts are large they may cause pain and some require surgical drainage. This condition is not inherited and is unrelated to polycystic liver disease in which many more cysts are present.

What are hydatid cysts?

These are parasitic cysts from the dog tapeworm. They are not very common in the UK but may be contracted abroad where there has been contact with sheep dogs and other animals. They may respond to drug therapy (eg Albendazole) but some may require surgical treatment.

Very occasionally benign or malignant tumours (cancers) present as cysts. Also some of the above conditions are rarely complicated by a form of cancer.

fatty liver

What is fatty liver?

Fatty liver is not a liver disease as such. It simply means there is more fat in the liver than normal. A person with a fatty liver is not necessarily ill.

What causes fatty liver?

Fatty liver can be caused by certain chemical compounds and by nutritional and endocrine disorders. Drugs or poisons that can cause fatty liver include alcohol, tetracycline, cortisone, phosphorus and carbon tetrachloride. Of these alcohol is by far the most common cause.

Nutritional causes of fatty liver are starvation, obesity, protein malnutrition and intestinal bypass operations for obesity. The endocrine disorder diabetes mellitus often leads to fatty liver. In juvenile diabetes the fat may be rapidly deposited leading to tenderness in the upper right of the abdomen. In all of these conditions the fatty deposits are occasionally accompanied by some inflammatory changes and scarring of the liver – so-called non-alcoholic steatohepatitis or NASH (see NASH fact sheet).

Fatty liver of pregnancy is a serious condition occurring near term. Premature termination of pregnancy may be necessary. Delivery of the baby by Caesarean section may be a life-saving measure.

What are the symptoms?

Uncomplicated fatty liver does not usually produce symptoms because fat accumulates slowly. A doctor may be able to detect that the liver is enlarged by palpation (an examination by touch). When the fat content is increased rapidly the liver expands, stretching its covering, and pain results.

In fatty liver of pregnancy there may be nausea, vomiting, abdominal pain and jaundice.

How does fat get into the liver?

Fat enters the liver from the intestines and from the tissues. Under normal conditions, fat from the diet is metabolized by the liver and other tissues. If the amount exceeds what is required by the body it is stored. In obesity some of the fat accumulates in the liver.

Can fatty liver lead to other liver disease?

Fatty liver in people who drink too much alcohol is sometimes followed by more serious liver damage in the form of alcoholic hepatitis. Serious liver damage is less common in diabetes and obese people who don’t drink but if the fat has progressed to NASH then further progression to scarring and even cirrhosis can occur.

How is fatty liver treated?

Treatment of fatty liver is related to the cause. Underlying conditions such as diabetes require treatment. Fat is decreased by removal of any drugs or other chemical compounds thought to be responsible. Nutritional causes are treated by altering the availability of fat coming into the liver. This is accomplished by providing available carbohydrates or by adding protein to overcome a complete or large deficiency in protein needed to make lipoproteins (proteins linked to fat and not capable of being dissolved in water).

How can I avoid fatty liver?

Do not drink to excess: alcohol can decrease the rate of metabolism and secretion of fat, leading to fatty liver. Overweight patients may have fatty liver, and are also at risk for several more serious conditions such as high blood pressure, stroke, diabetes and heart disease.

It is a good idea to watch your diet: Starvation, excess dieting and protein malnutrition can also result in fatty liver.

Gallstones

Overview
 
Gallstones are lumps of solid material that form in the gallbladder. They usually look like small stones or gravel and can be as small as sand or as large as pebbles, sometimes filling the gallbladder and may take years to grow. The commonest ones are made up of cholesterol, which is a fat. Others are known as pigment stones and consist of calcium and red blood cells, which have broken down and solidified.

The gallbladder is a small pear shaped pouch about three to six inches long. It is tucked just under the liver below the right rib cage and is connected to the intestine and liver by small tubes called bile ducts.  Bile ducts carry bile, a greenish-brown fluid which is made in the liver. Bile acts like a detergent, breaking up fat from the food we eat into small droplets. It also enables the body to absorb vitamins A, D, E and K. Bile is concentrated and stored in the gallbladder ready for use and is only released when we eat food.

Approximately 1 in 10 people will develop gallstones or another gallbladder disease.  Anyone can get them, but middle aged, overweight women are particularly likely to.  It is not fully understood why some people have them and others don't but gallstones are more common in the following groups of people:

  • overweight women

  • women who have been pregnant

  • people who have recently lost weight.

Symptoms

Many people with gallstones have no symptoms and are unaware they have them until the stones show up in tests performed for another reason.  When symptoms do develop it is usually because the gallbladder wall becomes inflamed or because the stones have moved out of the gallbladder and blocked the tube connected to the intestine, causing pain, jaundice and fever.

The smaller the stone, the more able it is to travel about within the bile duct system.  Stones can become lodged in the outlet of the gallbladder and cause recurrent painful attacks known as biliary colic. Frequent attacks can cause inflammation and scarring of the gallbladder known as chronic cholecystitis.

If gallstones leave the gallbladder they may cause other complications such as jaundice and cholangitis (inflammation of the bile ducts) which occur when the flow of bile from the liver is blocked and the bile becomes infected.  Stones, which pass from the bile duct into the intestine, can also temporarily obstruct the flow of digestive juices from the pancreas resulting in pancreatitis.

Most people have no symptoms until the stones begin to cause trouble. When symptoms do occur the commonest ones are:

  • Pain can vary from mild indigestion or discomfort to severe and persistent pain felt just below the right ribs spreading to the side and shoulder blade. Sometimes it may be mistaken for a heart attack or a peptic ulcer. The pain is due to strong contractions as the gallbladder tries to expel the stone. It may begin after a fatty meal or at night, and some people find relief by walking about, which dislodges the stone.
  • Jaundice is a more serious condition. It can develop when your bile duct becomes blocked by a stone. The break down products of bile build up in the blood making you feel unwell. It is easily noticeable as the skin and whites of the eyes go yellow and eventually the urine becomes dark and stools pale.
  • Shivering attacks, a sudden chill with severe shivering and a high temperature, similar to 'flu’, are a sign that infection is building up. Once again, if this happens you should see your doctor urgently.

Diagnosis

If gallstones are suspected, your GP will feel your abdomen to see if your liver or gallbladder is tender or enlarged and will arrange for you to be seen by a specialist in hospital for further investigations. These will usually include blood tests and an ultrasound scan.

  • Ultrasound scan: most stones can be clearly seen on an ultrasound scanner, which uses sound waves to build up a picture on a screen. It is the same scan used during pregnancy to see how a baby is growing in the womb. You won't feel anything during the examination, which is simple and quick. You may be asked not to eat or drink anything for about six hours before the test. A special jelly, which may feel cold, is smeared over the area and a small probe similar to a microphone is passed over your gallbladder.

  • ERCP: sometimes doctors do an examination called ERCP, which is short for endoscopic retrograde cholangiopancreatography. It gives a detailed x-ray of your pancreas and bile ducts, which is useful when stones have passed into them, especially when jaundice has developed. You will be given a sedative injection to make you feel sleepy and a little oxygen to help you breathe easily during the test. The whole test may last from 15 to 30 minutes and most people remember nothing about it afterwards because of the sedative injection. A long, thin flexible tube with a camera at the end is passed through your mouth and into your stomach and intestine. Dye is then injected and x-rays are taken. The dye passes out of your body harmlessly when you pass water.

  • Other tests: there are other types of examinations.  One, called an oral cholecystogram, involves swallowing tablets containing dye, which is then concentrated in the gallbladder.  When an x-ray is taken it will show whether gallstones are present and indicate whether the gallbladder is working properly.

  • Another slightly different test is a HIDA scan.  An injection of a minute dose of a radioactive material is given which shows up under a special camera. The radiation dose from this type of examination is no different from having an ordinary x-ray.  Other scans that are used for the detection of gallstones include CT scans, MRCP scans (magnetic resonance cholangiopancreatography) and endoscopic ultrasound but these techniques are not routinely used in the majority of hospitals.

Treatment

If gallstones have been discovered incidentally and are not troublesome, your doctor may want to adopt and watch and see policy, which means your symptoms will be monitored to see if they progress before treatment is considered. Some people may have no symptoms, or just one mild attack of pain and no further trouble, while others have continuing problems. Waiting to see if further symptoms develop is quite common and safe. If your symptoms are causing more problems, your doctor will recommend one of the following forms of treatment:

  • Removal of your gallbladder: is the most usual treatment for gallstones that cause symptoms. Your gallbladder is not essential for life and most people notice little difference without it. It actually stops working properly when stones form, so your body has already adjusted to its loss. Nowadays the gallbladder is usually removed by keyhole surgery and the operation is called a laparoscopic cholecystectomy. A general anaesthetic is given with very small incisions needed in the abdomen, which enable the surgeon to pass through fine instruments and a tube with a camera on the end. The instruments are controlled by the doctor watching a TV screen. The gallbladder is removed through a cut in your navel.  Most people are allowed home the following day, though some are discharged later the same day. Generally people are back to normal activities within two weeks.

  • Other types of operations: sometimes it is not possible to remove the gallbladder by keyhole surgery and about 1 in 10 people need a more traditional operation, which requires a longer stay in hospital and approximately 6 weeks convalescence. A few surgeons perform an operation called a minilaparotomy cholecystectomy, which uses special instruments and requires only a small cut.

  • Via ERCP examination: sometimes stones, which have passed into the bile duct and cause infection or jaundice, can be removed during an ERCP examination. This is done by widening the opening to the bile duct with an electrically heated wire (diathermy), which you won't feel. The stones are removed or left to pass into your intestine. Sometimes a short plastic tube called a stent is left in the bile duct to help bile drain out. The stent may remain in place permanently or be removed at a later date.

  • Other treatments: other methods to remove stones such as dissolving them with drugs or breaking them up with shock wave treatment are now only used occasionally.

Gilbert's syndrome

Gilbert’s syndrome is a harmless condition but often causes anxiety before the condition is diagnosed.

Haemoglobin is a chemical in the red cells that carries oxygen to the tissues. One of the breakdown products of haemoglobin is called bilirubin. An enzyme called UDB glucuronyl transferase helps the body get rid of bilirubin. Gilbert’s syndrome is a genetic disorder which means that there is a slight deficiency of this enzyme.

When there is less enzyme than normal, levels of bilirubin increase in the blood and the person may notice jaundice (yellowing of the skin and whites of the eyes). This jaundice may become more pronounced when the person is stressed, either physically or mentally. Thus, jaundice may develop when the person becomes ill with an infection or when stressed, for example when taking an exam.

Gilbert’s is often diagnosed in the late teens and early twenties. The condition is inherited and is relatively common being estimated to affect about 1 person in 20. Men are often affected more than women. The condition usually has no symptoms although some people do complain of stomach pains.

Gilbert’s syndrome is usually detected because of mild jaundice, or because slightly abnormal liver tests are noted when blood tests are performed for unrelated reasons. It is important to make the diagnosis so that the person can be reassured and serious liver disease excluded as a possibility.

The diagnosis can often be made on the basis of simple blood tests. All the liver tests should be normal apart from the serum bilirubin, which is increased.

Different blood tests may need to be carried out, if there are other symptoms. These will look for other reasons that the levels of bilirubin might be raised, such as other undiagnosed liver diseases. It is only rarely that a liver biopsy is necessary. In some cases more specific tests may be needed to confirm the diagnosis and this should be discussed with your specialist.

Hepatitis A

Overview

Hepatitis means your liver becomes inflamed (swollen and tender). The most common cause is being infected with a virus. An inflamed liver can also be caused by:

  • drinking too much alcohol

  • the side effects of some medicines and chemicals

  • a liver disease called autoimmune hepatitis where the body’s immune system doesn’t work properly and attacks the liver.

There are several different hepatitis viruses which affect the liver - the main ones are hepatitis A, B, C, D and E.

The viruses are different from each other in:

  • how they are passed from person to person

  • the way they cause liver damage

  • the effects they can have on health.

Hepatitis A, sometimes called hep A or HAV, is a liver disease caused by the hepatitis A virus. You can prevent illness by having a vaccination that will protect you from being infected for up to 10 years.

Hepatitis A is common in places where water supplies and sewage disposal are of a poor standard, and where personal and food hygiene standards are poor. Southern and Eastern Europe, Africa and parts of the Middle and Far East are high-risk areas.

Hepatitis A is passed from person to person by eating food or drinking water contaminated (infected) with the virus. The illness can spread easily within families and where people live closely together.

The virus is passed out in the bowel motions of an infected person, which is why it is important to wash your hands after going to the toilet. Drinking water can be contaminated with the virus.

Fruit, vegetables and uncooked food washed in contaminated water can cause infection, especially in hot countries. Shellfish can be infected if it comes from sea contaminated with sewage. Cooked food is safe, but can be contaminated if it has been handled by someone with the virus.

Infection is not very common in the UK. But the true number of people affected is unknown as people who only have mild symptoms may not go to a doctor.

Hepatitis B

overview

Hepatitis means your liver becomes inflamed (swollen and tender). The most common cause is being infected with a virus.

An inflamed liver can also be caused by:

  • drinking too much alcohol

  • the side effects of some medicines and chemicals

  • a liver disease called autoimmune hepatitis where the body’s immune system doesn’t work properly and attacks the liver.

There are several different hepatitis viruses which affect the liver - the main ones are hepatitis A, B, C, D and E.

The viruses are different from each other in:

  • how they are passed from person to person

  • the way they cause liver damage

  • the effects they can have on health.

Hepatitis B, sometimes called hep B or HBV, is a liver disease caused by the hepatitis B virus. You can prevent illness by having a vaccination that will give you protection from the virus.

Hepatitis B is common in south-east Asia, the Middle and Far East, southern Europe and Africa. The World Health Organisation estimates that one third of the world’s population has been infected at some time and that there are approximately 350 million people who are infected long term. In Europe, there are estimated to be one million people infected every year. In the UK, approximately 1 in 1000 people are thought to have the virus. In some inner-city areas, where there is a high percentage of people from parts of the world where the virus is common, as many as 1 in 50 pregnant women may be infected.

The virus is present in body fluids such as blood, saliva, semen and vaginal fluid. In the UK, Europe and North America, hepatitis B is mainly passed from person to person by having unprotected sex. In the rest of the world the most common way of getting infected is from infected mothers to their children, or from child to child.

Blood

A tiny amount of blood from someone who has the virus will pass on the infection if it gets into your bloodstream, e.g. through an open wound, cut or scratch, or from a contaminated needle.

People who use drugs and share any injecting equipment have a high risk of infection.

The virus can also be passed on from medical and dental treatment in countries where equipment is not sterilised properly.

All blood donations in the UK are tested for hepatitis B, but before testing was introduced it was possible to become infected by receiving blood or blood products from an infected person.

In countries where blood is not tested, blood transfusions may still be a cause of infection.

Sex

You can become infected by having unprotected sex (without a condom) with an infected person. Sexually active young adults have a high risk of getting hepatitis B.

Mother to baby

Infected mothers can pass the virus to their babies possibly during pregnancy, when giving birth or during breastfeeding. Since April 2000, all pregnant women in the UK are tested for hepatitis B. If they are infected, the baby is given a course of injections of antibodies called immunoglobulin and vaccinated immediately after birth.

Acupuncture, tattoos, body piercing

A few people have become infected by unsterile needles being used for ear and body piercing, acupuncture and tattooing. The best way to protect yourself is to ensure disposable needles are used and that they come straight out of a sterile packet.

Other body fluids

The virus may be present in other body fluids, such as saliva and vaginal fluid, particularly if it is contaminated with blood, which can pass on the infection.

People who are most at risk of being infected are:

  • injecting drug users

  • babies born to infected mothers

  • family members and partners of an infected person

  • healthcare workers who have direct contact with blood, e.g. doctors, dentists, nurses and midwives

  • people who live and work in accommodation for people with severe learning difficulties

  • prisoners

  • people travelling and working in countries where the virus is common

  • people who have unprotected sex (without a condom) with people who may be infected.

Hepatitis B can be very infectious. Some people pass on the virus more easily than others because they have more of the virus in their bloodstream.


Hepatitis C

Overview

Hepatitis means inflammation of the liver. The commonest cause is infection with a virus, but inflammation can be caused by drinking too much alcohol, the side effects of some drugs and chemicals, and a liver disease called autoimmune hepatitis, in which the body’s immune system malfunctions and attacks the liver. There are several different viruses that affect the liver, primarily hepatitis A, B, C, D and E. The main difference between the viruses is how they are spread, the way they cause liver damage and the effects they can have on your health.

Hepatitis C, sometimes referred to as hep C or HCV, is a liver disease caused by the hepatitis C virus.

Hepatitis C is passed on by:

Contact with blood

A tiny amount of blood - too small to be visible to the naked eye - from someone who has the virus, will transmit the infection if it gets into someone else’s bloodstream, for example, through an open wound, cut or scratch.

Blood donations in the United Kingdom have been screened for hepatitis C since September 1991. Some people who received blood or blood products before then may be infected, e.g. haemophiliacs (and pregnant women who received anti D as a treatment for rhesus incompatibility in the Republic of Ireland).

Blood and blood products may not be screened for the virus in some overseas countries.

Sex

Sexual transmission of hepatitis C is thought to be unusual, but probably does occasionally occur. Doctors are unsure whether the infection that occurs between partners is because of sexual exposure or because of other reasons, for example, sharing a personal item such as a toothbrush or razor.

People with more than one sexual partner are advised to use condoms, as there is some evidence that those with many sexual partners have an increased risk of being infected. Condoms may reduce the risk of infection, and all other sexually transmitted infections.

Naturally, couples are often worried about infecting each other, but when one partner is positive and the other negative after many years, it seems reasonable to advise that it is unnecessary to change to using condoms for protection.

Penetrative sex during a period is best avoided when a woman is hepatitis C positive.

Normal social contact

Infection is not acquired through normal social contact, for example, from a cup or by touching an infected person. However, it is a sensible precaution if you are HCV positive to use your own personal items such as a nailbrush, scissors or razor and to be meticulous about cleaning up any blood, for example from cuts or scratches. Undiluted household bleach should be used to clean up blood from floors and work surfaces. Scratches, cuts and wounds should be carefully cleaned and covered with a waterproof dressing or plaster.

Injecting drug use

Current evidence suggests that 50% - 80% of past and present users may be infected with hepatitis C. They become infected by sharing any equipment used in the process of injecting because it is likely to have invisible contamination with blood. Only one occasion of sharing injecting equipment is needed to be exposed to the virus - and that may have been years previously. An increasing number of people who injected a few times many years ago, are now being diagnosed as having hepatitis C.

Recently there has been some concern about sharing straws for snorting cocaine because the practice may be a route of infection through nosebleeds.

For more information on injecting drug use, please ask for a copy of our booklet Injecting Drug Use and Hepatitis C.

Acupuncture, tattoos, body piercing

A few people have become infected by unsterile needles being used for ear and body piercing, acupuncture and tattooing. The best way to protect yourself is to ensure disposable needles are used and that they come straight out of a sterile packet.

Saliva

The virus has been detected in saliva, but it is unlikely that it can be transmitted by kissing. It is advisable to reduce the possibility of infection by not using someone else’s toothbrush and by maintaining good oral hygiene.

Mother to baby

The risk of a mother with hepatitis C infecting her baby during pregnancy or during the birth is about 6%. When it does occur, it is not known whether this is in the womb, during delivery or immediately after the birth, but it is not during conception.

Babies are sometimes found to have antibodies to the virus, but these usually disappear by the time the baby is 12 to 18 months old, which shows that the antibodies are acquired from the mother and that the baby has never been infected with the virus. Since the usual test is for antibodies, it may not be possible to determine if a baby is infected until it is over a year old, but there is a test for the virus itself (“PCR test”) that may detect it in the first few months. Doctors do not yet know if the disease that occurs when a baby is infected will go on to become serious in all cases.

Infected mothers are often concerned about passing the virus on to their other children. As long as all the precautions listed below are taken, household transmission is a low risk. Kissing and cuddling a child is safe.

Breastfeeding

Most doctors consider breastfeeding to be safe if the mother has no symptoms. In theory infection could be possible if a mother has cracked nipples that bleed and the baby has a cut in the mouth.

Unknown

In some cases doctors don’t know how people became infected.

Hepatitis D

overview

Hepatitis D virus (HDV) requires the presence of Hepatitis B virus (HBV) to reproduce it. You can acquire Hepatitis D infection at the same time as you are infected with Hepatitis B, this is called co-infection.

Infection may also occur if you already have chronic Hepatitis B and it has not cleared from your body, this is called super-infection.

The combination of Hepatitis D and Hepatitis B can be more serious than Hepatitis B alone and is more likely to cause chronic hepatitis and  cirrhosis

Hepatitis D is seen mainly in Central Africa, the Middle East and central South America. Low rates of infection are present in most of Europe, the United States and Australia.

Hepatitis D is spread through anyone coming into contact with infected blood, contaminated needles or by having unprotected sex with a Hepatitis D infected person. The risk factors are similar to those for Hepatitis B infection.

The symptoms of Hepatitis D infection are similar to Hepatitis B infection, many people who are infected have no symptoms and others have flu-like symptoms or even jaundice (yellowing of the skin and whites of the eyes). The virus has an incubation period of between 3 and 12 weeks, it is diagnosed by an antibody blood test.

There is no vaccine for Hepatitis D, however vaccination against Hepatitis B will prevent Hepatitis D infection. Those people with Hepatitis B cannot prevent infection with Hepatitis D.

Currently Interferon alpha is used to treat patients with chronic Hepatitis B who also have Hepatitis D infection.

Hepatitis E

overview

Hepatitis E virus (HEV) causes acute hepatitis but does not lead to long term liver damage.

It can however cause a serious infection in women who are in the last three months of pregnancy, possibly being fatal in 1 in 5 cases. Hepatitis E may also cause a miscarriage at any stage of pregnancy.

Hepatitis E is similar to Hepatitis A in that it occurs mainly by contamination of food and water. It is common in India, Asia, Africa, Middle East and Central America.

The way in which Hepatitis E is spread is similar to Hepatitis A virus. The virus is passed from person to person by eating food or drinking water contaminated (infected) with the virus. The virus is passed out in the bowel motions of an infected person. (This is why it is important to wash your hands after going to the toilet).

It is not transmitted through blood, needles, or other body fluids or through sexual contact.

Epidemics happen when water supplies are contaminated with sewage after monsoons and flooding.

The symptoms of Hepatitis E are tiredness, aches and pains, nausea (feeling sick), sickness, stomach ache and/or diarrhoea. Jaundice (yellowing of the skin and whites of the eyes) may develop, urine may become dark and bowel motions may go pale.

Hepatitis E has an incubation period of between 2 and 12 weeks.

Infected persons may be infectious for up to 2 weeks after symptoms appear.

If Hepatitis E is suspected (i.e.travellers returning from areas where HEV is common or a recent epidemic) it can be confirmed by a blood test.

At present there is no current vaccine for Hepatitis E

There is no specific treatment for Hepatitis E, many people feel tired and need more rest than usual, they can eat and drink, as they are able. It is best to avoid alcohol and a lot of exercise. Pregnant women are advised to see a specialist as a matter of urgency.

Hepatitis E can be prevented by avoiding having ice cubes in drinks, drinking tap water, eating ice cream or cleaning your teeth in tap water in countries where it is common. Also by not eating poorly cooked shellfish, uncooked vegetables, salads, unpeeled fruit or unpasteurised milk in high-risk countries.

Portal hypertension and bleeding of oesophageal varices

One consequence of chronic liver disease can be portal hypertension. This is an increase in the blood pressure in the portal vein, which carries the blood from the bowel and spleen to the liver. The pressure in the portal vein may rise because there is a blockage, such as a blood clot, or because the resistance in the liver is increased because of scarring, or cirrhosis. As a result, the pressure in the portal vein rises – this is known as portal hypertension.

As the blood tries to find another way back to the heart, new blood vessels open up. Among these vessels are those that run along the wall under the lining of the upper part of the stomach and the lower end of the oesophagus (gullet). These veins protrude into the gullet and the stomach and can bleed. This bleeding may be a gentle ooze in which case anaemia is the commonest symptom. Sometimes there can be a major bleed and the person has a haemorrhage and either vomits blood or passes blood through the bowels. This blood may appear to be black, since it is often changed as it passes through the body.

There are many causes of cirrhosis, alcohol being the most common. Others include viral hepatitis, autoimmune liver disease, primary biliary cirrhosis, primary sclerosing cholangitis and some metabolic diseases. Please see our other leaflets on these specific conditions.

Portal hypertension may also arise as a result of a parasitic disease, which is common in the Middle East and parts of South America. Other conditions including clotting disorders and pancreatic disease can lead to portal hypertension.

Portal hypertension and its consequence of bleeding varices are usually seen in people with moderately advanced liver disease. There may be other features such as ascities (fluid in the stomach) and encephalopathy (disturbance of brain function as a result of disordered liver function).

Detection of varices

The dilated veins in the gullet are known as varices. Unless they bleed they do not produce any complications or symptoms. The only way they can be detected is by a process called endoscopy. During endoscopy a small flexible tube is put into the gullet and the endoscopist can see not only where the varices are present but also their size.

Prevention of bleeding

Not everyone with cirrhosis has varices and not everyone with varices will bleed. In general, small varices rarely bleed and bigger ones may bleed. Small varices however, may well develop into large varices over time.

For those people who have varices and are likely to bleed, treatment with drugs can sometimes reduce the risk of bleeding and reduce the severity of any bleed should it occur. The drug most commonly used is Propranolol. As with all drugs, not everyone is suitable and some people have side-effects. Alternative methods may sometimes be used for those who are at risk of bleeding.

Treatment

Propranolol is used both for the prevention of bleeding and also in those people who have bled. It may be used in the prevention of re-bleeding.

Treatment of bleeding varices

If you vomit blood or pass blood with your stools this is a medical emergency and you should go to hospital immediately. You should tell the doctors and nurses that you have liver disease and bleeding, since early treatment will reduce the consequences.

Initial treatment is to replace the fluid and then to identify and correct the cause of bleeding. Not everyone who has varices and who bleeds will be bleeding from varices. They may be bleeding from another area in the digestive tract.

A number of treatment options are available for the treatment and prevention of bleeding.

Drugs

Several drugs are useful in the treatment of the variceal bleed. These drugs, such as Glypressin or Octreotide, are given by injection.

Endoscopic techniques

There are two treatments that can be given at endoscopy to treat and prevent bleeding.

These are

1. Injection sclerotherapy

This is injection of a sclerosant (special chemical) material in the veins of the gullet. Usually after sedation the endoscope is passed into the gullet. A fine flexible needle is passed through this endoscope and used to inject sclerosant material into the oesophageal veins or alongside the veins. These injections cause clotting (thrombosis in the veins) and will also stimulate some scarring to reduce the risk of varices recurring.

2. Banding

With banding techniques the oesophageal varix (single varicose vein) is sucked into a ring at the end of the endoscope. A small band is placed around the base of the varix that has been sucked into the ring. After 1 or 2 days this will result in thrombosis (blood clot) of the varix, which will control the bleeding.

These two techniques are complementary and the endoscopist will use one or the other depending on the clinical situation. Both techniques have advantages and disadvantages and complications. You should discuss these with the endoscopist.

Sengstaken tube

Sometimes it is just not possible to get immediate control of the bleeding with either drugs or endoscopic techniques. In this case, a tube known as a Sengstaken tube or Lintern tube is passed through the mouth and into the stomach. The balloon is inflated and applies compression to the varices. This will achieve temporary control of the bleeding and allow time for other measures to work.

Shunts

Shunting operations involve joining two veins. Shunts may either be done surgically or by the radiologist. In a surgical shunt, the blood that would normally go into the portal vein is diverted into another vein. There are several types of shunts available. This process involves a major operation.

TIPSS

TIPSS stands for Transjugular Intrahepatic Portal Systemic Shunt. This technique is usually done by a radiologist but other clinicians also carry this out.

In this procedure a metal tube is passed across the liver to allow the blood in the portal vein to go straight into the hepatic vein and so bypass the high resistance of the liver. This procedure is usually done in the Radiology Department and may take several hours.

Both types of shunt procedure are very effective in lowering portal pressure but they do have complications.

One of these complications is encephalopathy whereby the person may get a little bit drowsy, confused or in rare cases even comatosed. This is because the blood usually clears toxins from the bowel and if these toxins bypass the liver they can affect the electrical activity of the brain.

Summary

Oesophageal varices do represent a significant complication of cirrhosis and some other types of liver damage. Current treatments do allow for early identification of those people who are at risk of variceal bleeding, and treatments can greatly reduce the risk and severity of the bleeds. For those who have bleeding oesophageal varices, this is a medical emergency but early treatment is usually highly effective. There are a variety of approaches to treat bleeding varices and the treatment used will depend on the overall condition of the individual.

Primary Biliary Cirrhosis

Overview

PBC is a disease that, little by little, can destroy some of the tubes

linking your liver to your gut. These tubes are called bile ducts.

The bile ducts carry bile – which is yellow and made in your liver –

to your gut (also known as your intestine or bowels).

When PBC damages the bile ducts, bile can no longer flow through them.

Instead it builds up in the liver, damaging the liver cells and resulting in

 inflammation and scarring.

Over the years this damage becomes wide spread.

The liver becomes less able to repair itself properly, leading to a condition known as cirrhosis

and the failure of the liver to carry out all of its jobs properly.

What is bile?

Bile plays a central role in helping the body digest fat. It acts as a detergent, breaking the fat into very small droplets so that it can be absorbed from food in your gut. It also makes it possible for the body to take up the fat-soluble vitamins A, D, E and K from the food passing through the gut.

Who is at risk of PBC?

Nine out of ten people who get PBC are women. No one knows why this is. In particular the following women are most at risk:

  • women who are middle aged or older
  • women who have a family history of PBC.

Who is at risk of PBC?

Nine out of ten people who get PBC are women. No one knows why this is. In particular the following women are most at risk:

  • women who are middle aged or older
  • women who have a family history of PBC.

Why is it called PBC?

It is called primary biliary cirrhosis as the disease attacks the bile duct (biliary), which then leads to scarring (cirrhosis) of the liver. In this case primary does not mean first, but that there is no know reason for the damage to happen.

The name PBC is a little misleading, as many people do not have cirrhosis when they are first diagnosed, or for many years afterwards. Indeed, many people with PBC never go on to get cirrhosis.

What is cirrhosis?

Cirrhosis is the result of long-term and non-stop damage to the liver. The damage leads to scarring, known as fibrosis. At the same time, irregular bumps (nodules) replace the smooth liver tissue and the liver becomes harder. Together the scarring and the nodules are called cirrhosis.

Although in the UK, alcohol abuse and hepatitis are the main cause of cirrhosis (more than half of all cases) a number of other diseases can lead to it, including PBC.

(Please read our leaflet Cirrhosis of the liver also available on this website)

Causes of PBC

PBC is thought to be a disease where the body attacks itself, known as an autoimmune disease. The body’s defence against germs and infections – the immune system – mistakes the bile ducts as ‘foreign’ and attacks them.

Why this happens is still not known. Some researchers think that something may trigger the body into making this mistake. Possible causes of this ‘trigger’ could be:

  • an infection
  • some form of poison taken in from the environment.

(Occasionally PBC comes to light during or just after pregnancy. It is not clear whether pregnancy may spark it off or, because pregnant women are under closer medical supervision, the symptoms of PBC are first noticed during pregnancy).

This external ‘trigger’ probably starts PBC only in people who are already at risk, because they have inherited certain genes from their parents.

Despite what many people think, drinking alcohol does not cause PBC.

Primary Sclerosing Cholangitis

Overview

Primary Sclerosing Cholangitis (PSC) is an uncommon chronic liver disease in which the bile ducts inside and outside the liver progressively decrease in size due to inflammation and scarring. The disease may occur alone, but frequently is associated with inflammatory diseases of the colon, especially chronic ulcerative colitis.

Bile ducts are tubes which carry bile (a greenish yellow liquid made by the liver)into the upper part of the bowel. Bile acts as a detergent breaking up fat from the food we eat into small droplets that can then be absorbed into the body.It also enables the body to absorb vitamins A,D,E and K from our diet.

As a consequence of PSC, bile that is normally carried by these ducts accumulates within the liver. This blockage to bile flow also causes damage to liver cells causing inflammation and scarring. Over many years the scarring can affect the whole liver and system of bile ducts. A damaged liver can re-grow without scarring but as with PSC in this case, the re-growth goes wrong and the healing process is incomplete. The combination of scar tissue and irregular growth is known as cirrhosis

The frequent occurence of PSC in association with inflammatory bowel disease suggests that a common cause for both diseases may exist or that the inflamed colon allows toxins or infections to be absorbed into the body and this can cause the bile duct inflammation. The disease affects both genders, although two male patients are affected for every female patient. It can affect all ages, but it is most commonly found in young adults.

WILSON’S DISEASE

Wilson's disease is an inherited disease in which too much copper builds up in the body. Copper, unlike other metals such as lead and mercury, is essential to health. In Wilson's disease the body and especially the liver is unable to get rid of the excessive amounts of copper which results in too much copper in several organs. The liver is the first organ to store copper. When its storage capacity is exhausted, the overflow passes from the liver into the bloodstream and is carried to other places, mainly the brain and the cornea of the eye.

The retention of copper begins at birth, but it may take ten to thirty years before the liver is overwhelmed and symptoms of liver disease appear. Symptoms can range from jaundice and ascites to acute liver failure.

Copper accumulation in the brain can cause psychiatric disorders such as depression, mood swings or suicidal impulses. It can also cause physical symptoms such as slurred speech, failing voice, drooling, tremors, or difficulty in swallowing. Muscular control can deteriorate until a person becomes bedridden and helpless.

The presence of both psychiatric and physical symptoms makes Wilson's disease difficult to diagnose and especially prone to misdiagnosis. During adolescence, anxiety or depression can be misinterpreted as normal for teenagers, rather than indications of a potentially fatal illness. It is possible to test for Wilson's disease with a simple blood test measuring copper and the copper binding protein known as caeruloplasmin. Another test for Wilson's disease is a ring of accumulated copper under the cornea (known as the Kayser-Fleischer ring) in the eye, which is sometimes visible to the naked eye. Although this is not always present.

Although Wilson's disease is fatal if undetected, effective treatment is available even in advanced cases. The damaging concentration of copper in the body must be removed and its reaccumulation prevented. A de-coppering agent (penicillamine is the most common) can remove excess copper from the body and prevent future accumulation. This treatment must be continued for life. As Wilson’s disease is inherited all brothers and sisters of a patient should be screened for the disease.

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