Veno-occlusive Disease of the Liver

Veno-occlusive disease of the liver is blockage of the very small (microscopic) veins in the liver.

• Fluid tends to accumulate in the abdomen, the spleen may enlarge, and severe bleeding may occur in the esophagus.
• The skin and whites of the eyes may turn yellow, and the abdomen may enlarge.
• Doctors base the diagnosis on symptoms and results of Doppler ultrasonography.
• If possible, the cause is corrected or eliminated, and symptoms are treated.

Veno-occlusive disease is similar to Budd-Chiari syndrome except that blood flow is blocked within (rather than outside of) the liver. That is, blockages do not affect the large hepatic veins and the inferior vena cava (the large vein that carries blood from the lower parts of the body, including the liver, to the heart).

Veno-occlusive disease may occur at any age. It is more common among people who are malnourished.

Because flow out of the liver is blocked, blood backs up in the liver. This backup (congestion) then reduces the amount of blood entering the liver. Liver cells are damaged because they do not get enough blood (ischemia). The congestion causes the liver to become engorged and enlarged. Such outflow obstruction also is transmitted across the liver, increasing pressure in the portal vein (portal hypertension). Portal hypertension can result in dilated, twisted (varicose) veins in the esophagus (esophageal varices). The elevated pressures in the portal vein and the liver congestion lead to fluid accumulating in the abdomen—a disorder called ascites. The spleen also tends to enlarge.

Such congestion reduces blood flowing into the liver. The resulting liver damage leads eventually to severe scarring (cirrhosis).

Causes

Common causes include the following:

• Ingestion of pyrrolizidine alkaloids, which are found in crotalaria and senecio plants (used in Jamaica to make herbal tea) and in other herbs, such as comfrey
• Use of certain drugs that occasionally have toxic effects on the liver, including cyclophosphamide and azathioprine Some Trade Names
  IMURAN
  (used to suppress the immune system)
• Radiation therapy (used to suppress the immune system before bone marrow or stem cell transplantation)
• A reaction after bone marrow or stem cell transplantation (graft-versus-host disease)

In graft-versus-host disease, white blood cells in the transplanted tissue attack the recipient’s tissues. This reaction tends to occur about 3 weeks after transplantation.

Symptoms

Symptoms may begin suddenly. The liver enlarges and becomes tender. The abdomen may swell because of fluid accumulating there. The skin and the whites of the eyes may become yellow—a condition called jaundice.

Varicose veins in the esophagus may rupture and bleed, sometimes profusely, causing people to vomit blood and often go into shock. The blood may pass through the digestive tract, making stools black, tarry, and foul-smelling (called melena). When bleeding is severe, shock ensues. A few may experience liver failure with deterioration of brain function (hepatic encephalopathy), resulting in confusion and coma.

Others develop cirrhosis with time, likely over months, depending upon the cause and repeated exposure to toxic agents.

Diagnosis

Doctors suspect veno-occlusive disease based on symptoms or blood test results that suggest liver dysfunction, particularly if people have ingested substances or have conditions (particularly following bone marrow transplantation) that may cause the disease. Blood tests evaluate the liver and blood clotting.

Doppler ultrasonography often confirms the diagnosis. Occasionally, invasive tests may be necessary. Liver biopsy or blood pressure measurements of the hepatic veins and portal veins rarely are necessary. These are done by inserting a catheter into a vein in the neck (jugular vein) and threading it to the hepatic veins. A biopsy of the liver can be taken at the same time.

Prognosis

The prognosis depends on how extensive the damage is and whether the condition causing it recurs or continues—for example, when people continue to drink senecio tea.

Overall, about one fourth of people with veno-occlusive disease die of liver or other organ failure within 3 months. When the cause is graft-versus-host disease after bone marrow transplantation, veno-occlusive disease often resolves on its own within a few weeks. Increasing the dose of drugs used to suppress the immune system can also cause graft-versus-host disease to resolve. If the cause is an ingested substance, stopping its use helps prevent further liver damage.

Treatment

There is no specific treatment for the blockage. If possible, the cause should be eliminated.

Ursodeoxycholic acid helps prevent veno-occlusive disease from developing after bone marrow or stem cell transplantation.

Problems resulting from the blocked vessels are treated. For example, a low-salt (low-sodium) diet and diuretics help keep fluid from accumulating in the abdomen.

An alternate route for blood flow, bypassing the liver, may be created by directly connecting the portal vein to the inferior vena cava. A catheter is inserted in a neck vein (jugular vein), threaded to the portal vein, and used to make this connection (shunt). Then a wire mesh tube (called a transjugular intrahepatic stent) is inserted to keep the shunt open. The effectiveness of such shunts is unclear.

Liver transplantation may be necessary in extreme cases.

Adapted from: Merck & Co., Inc.

Posted on December 13, 2009 | Filed under 9 | Permalink

Congestive Hepatomegaly , Ischemic Cholangiopathy

Congestive Hepatomegaly

Congestive hepatomegaly is a backup of blood in the liver, resulting from heart failure.

Severe heart failure causes blood to back up from the heart into the inferior vena cava (the large vein that carries blood from the lower parts of the body to the heart). Such congestion increases pressure in this vein and other veins that carry blood to it, including the hepatic veins (which drain blood from the liver). If this pressure is high enough, the liver becomes engorged (congested) with blood and malfunctions.

In most people, the congested liver causes only mild abdominal discomfort. The liver (in the upper right part of the abdomen) is tender and enlarged. In severe cases, the skin and whites of the eyes may turn yellow—a disorder called jaundice. Fluid may accumulate in the abdomen—a disorder called ascites. The spleen also tends to enlarge. If congestion is severe and chronic, liver damage or even severe scarring (cirrhosis) develops.

Doctors suspect the disorder in people with heart failure who have typical symptoms and abnormal results on blood tests done to evaluate the liver.

Management focuses on treating the heart failure. Such treatment may restore normal liver function.

Ischemic Cholangiopathy

Ischemic cholangiopathy is damage to one or more bile ducts caused by inadequate blood flow.

Bile ducts (such as the hepatic ducts and the common bile duct), unlike the liver, are supplied with blood from only one major blood vessel, the hepatic artery. Thus, disruption of blood flow through the hepatic artery can prevent the bile ducts from obtaining enough oxygen. Consequently, the cells lining the ducts are damaged or die—a disorder called ischemic cholangiopathy. Blood flow can be disrupted by the following:

• Surgical trauma during liver transplantation or removal of the gallbladder by laparoscopy
• Injury from radiation therapy
• A disorder that makes blood more likely to clot (blood clotting disorder)
• A procedure to block a bleeding blood vessel that causes clot formation (embolization)

Ischemic cholangiopathy is mostly commonly seen in those who have had a liver transplant.

Symptoms and Diagnosis

The damaged bile duct narrows (causing a stricture) so that the flow of bile slows or is blocked. As a result, bile pigment (bilirubin) is retained, the skin and the whites of the eyes turn yellow (called jaundice) and the urine becomes dark. As bile (containing pigment such as bilirubin) does not enter the small intestine, the stools become pale. Itching (termed pruritus) is common, often beginning in the hands and feet but usually affecting the whole body. Itching is especially worse at night. Bile duct infection (cholangitis) may also occur, producing abdominal pain, chills and fever.

The diagnosis comes from the symptoms and abnormal blood test results, especially in people who have conditions that make ischemic cholangiopathy more likely (eg, liver transplant recipients). Ultrasonography helps doctors visualize the ducts, but the results may be inconclusive. Better definition often requires magnetic resonance imaging of the bile ducts (a procedure called magnetic resonance cholangiopancreatography or MRCP) or endoscopic retrograde cholangiopancreatography (ERCP). ERCP involves inserting a flexible viewing tube (endoscope) through the mouth and into the small intestine and injecting dye into the bile duct system.

Treatment

In addition to detecting the narrowing of the bile ducts, ERCP can be used in treatment. A wire with a deflated balloon at its end is introduced through the endoscope; doctors inflate the balloon to widen (dilate) the narrowing. A mesh tube (stent) then keeps the duct open.

People who have had a liver transplant on occasion may require another transplant.

Adapted from: Merck & Co., Inc.

Posted on December 13, 2009 | Filed under 9 | Permalink

Ischemic Cholangiopathy, Ischemic Hepatitis

Ischemic Cholangiopathy

Ischemic cholangiopathy is damage to one or more bile ducts caused by inadequate blood flow.

Bile ducts (such as the hepatic ducts and the common bile duct), unlike the liver, are supplied with blood from only one major blood vessel, the hepatic artery. Thus, disruption of blood flow through the hepatic artery can prevent the bile ducts from obtaining enough oxygen. Consequently, the cells lining the ducts are damaged or die—a disorder called ischemic cholangiopathy. Blood flow can be disrupted by the following:

• Surgical trauma during liver transplantation or removal of the gallbladder by laparoscopy
• Injury from radiation therapy
• A disorder that makes blood more likely to clot (blood clotting disorder)
• A procedure to block a bleeding blood vessel that causes clot formation (embolization)

Ischemic cholangiopathy is mostly commonly seen in those who have had a liver transplant.

Symptoms and Diagnosis

The damaged bile duct narrows (causing a stricture) so that the flow of bile slows or is blocked. As a result, bile pigment (bilirubin) is retained, the skin and the whites of the eyes turn yellow (called jaundice) and the urine becomes dark. As bile (containing pigment such as bilirubin) does not enter the small intestine, the stools become pale. Itching (termed pruritus) is common, often beginning in the hands and feet but usually affecting the whole body. Itching is especially worse at night. Bile duct infection (cholangitis) may also occur, producing abdominal pain, chills and fever.

The diagnosis comes from the symptoms and abnormal blood test results, especially in people who have conditions that make ischemic cholangiopathy more likely (eg, liver transplant recipients). Ultrasonography helps doctors visualize the ducts, but the results may be inconclusive. Better definition often requires magnetic resonance imaging of the bile ducts (a procedure called magnetic resonance cholangiopancreatography or MRCP) or endoscopic retrograde cholangiopancreatography (ERCP). ERCP involves inserting a flexible viewing tube (endoscope) through the mouth and into the small intestine and injecting dye into the bile duct system.

Treatment

In addition to detecting the narrowing of the bile ducts, ERCP can be used in treatment. A wire with a deflated balloon at its end is introduced through the endoscope; doctors inflate the balloon to widen (dilate) the narrowing. A mesh tube (stent) then keeps the duct open.

People who have had a liver transplant on occasion may require another transplant.

Ischemic Hepatitis

Ischemic hepatitis is damage throughout the liver caused by an inadequate blood or oxygen supply.

• Heart or respiratory failure may reduce the blood flow or oxygen supply to the liver.
• People feel nauseated and vomit; the liver may be tender and enlarged.

In ischemic hepatitis, liver cells are damaged or die because the liver does not receive enough blood or oxygen.

Ischemic hepatitis differs from other types of hepatitis. Usually, “hepatitis” implies inflammation of the liver, which can have many causes, most commonly a virus (as in hepatitis A or B). In ischemic hepatitis, however, the liver is not inflamed; rather liver cell death (necrosis) occurs. It is termed hepatitis because, as in viral and other types of hepatitis, liver enzymes called aminotransferases leak from damaged liver cells into the blood.

Causes

For ischemic hepatitis to develop, the liver’s requirements for blood, oxygen or both are not being met. The most common cause for such unmet needs is when blood flow throughout the body decreases. Causes include the following:

• Heart failure
• Respiratory failure
• Shock
• Massive bleeding
• Severe dehydration

A severe infection that affects all or most of the body, such as sepsis, can increase the liver’s need for oxygen and thus contribute to ischemic hepatitis.

Because the liver receives blood from the hepatic artery and portal vein, narrowing or blockage of one of these vessels does not usually cause ischemic hepatitis. This disorder results when blood flow in both vessels is reduced or blocked. The most common cause of blocked blood vessels is a blood clot. (Blockage by a blood clot is termed thrombosis.) Blood clots in the hepatic artery can have many causes, such as the following:

• Injury of blood vessels (as occurs during liver transplantation surgery)
• Aneurysms of the hepatic artery
• Inflammation of the artery (vasculitis)
• Use of cocaine (causing spasm of the artery)
• Tumors, certain medical procedures, or heart infections (endocarditis) that cause emboli—clumps of material, such as a piece of fatty material or blood clot on the wall of an artery—to break off and travel through the bloodstream and become lodged in a blood vessel

Disorders that make blood more likely to clot (blood clotting disorders) can cause blockages in arteries or veins. These disorders may be inherited or acquired.

Symptoms and Diagnosis

Symptoms include nausea and vomiting. The liver may be tender and enlarged.

Doctors suspect ischemic hepatitis when results of liver biochemical and blood clotting tests are abnormal, especially in people who have a condition that can cause the disorder. Blockage of the hepatic artery can be detected using ultrasonography, magnetic resonance angiography, or x-rays taken after a radiopaque dye (which is visible on x-rays) is injected into an artery (arteriography).

Treatment

Doctors focus on treating the condition that is reducing blood flow to the liver. If blood flow can be restored, ischemic hepatitis commonly resolves. Liver failure can occur if people already have severe scarring of the liver (cirrhosis).

Adapted from: Merck & Co., Inc.

Posted on December 13, 2009 | Filed under 9 | Permalink

Portal Vein Thrombosis

Portal vein obstruction results from thrombosis (blood clot) or narrowing of the portal vein, which brings blood to the liver from the intestines.

• Most people have no symptoms. Fluid may accumulate in the abdomen, the spleen may enlarge, and severe bleeding may occur in the esophagus.
• Doppler ultrasonography can usually confirm the diagnosis.
• If possible, the cause is treated, and drugs may be used to prevent the clot from enlarging or to dissolve the clot.

Because the portal vein is narrowed or blocked, pressure in the portal vein increases. This increased pressure (called portal hypertension) causes the spleen to enlarge (splenomegaly). It also results in dilated, twisted (varicose) veins in the esophagus (esophageal varices) and often in the stomach (portal hypertensive gastropathy). These can bleed profusely. Fluid accumulation in the abdomen (called ascites) is not common but may develop when the blockage of the portal vein is accompanied by liver congestion or damage or when large amounts of fluids are given intravenously to treat major bleeding from ruptured varices in the esophagus or stomach. Portal vein thrombosis that develops in people with cirrhosis will cause their condition to deteriorate.

Causes

About 25% of adults with cirrhosis have portal vein thrombosis, likely from sluggish blood flow. Portal vein thrombosis also can be caused by any condition that makes blood more likely to clot. Common settings differ by age group:

• Newborns: Infection of the umbilical cord stump (at the navel)
• Older children: Appendicitis
• Adults: Excess red blood cells (polycythemia), certain cancers (liver, pancreas, kidney, or adrenal gland), surgery, and pregnancy

Often, several conditions work together to cause the blockage. The cause is unknown in about one third of people.

Symptoms

Most people do not have any symptoms. In some people, problems gradually develop, resulting from portal hypertension. If varicose veins develop in the esophagus or stomach, they may rupture and bleed, sometimes profusely. People then vomit blood. The blood may also pass through the digestive tract, making stools black, tarry, and foul-smelling (called melena). Another vascular complication of portal hypertension is the development of abnormal small veins and capillaries in the stomach (portal hypertensive gastropathy), which may result in gastrointestinal bleeding.

Diagnosis

Doctors suspect portal vein thrombosis in people who have some combination of the following:

• Bleeding from esophageal or gastric varices
• An enlarged spleen
• High-risk conditions (for example, children with umbilical cord infection or acute appendicitis)

Blood tests to evaluate the liver often are quite normal.

Doppler ultrasonography usually confirms the diagnosis. It shows that blood flow through the portal vein is reduced or absent. In some, magnetic resonance imaging (MRI) or computed tomography (CT) is necessary.

Angiography is done if a procedure to create an alternate route for blood flow is planned. For angiography, x-rays of the veins are taken after a radiopaque dye (which is visible on x-rays) is injected into the portal vein.

Treatment

If a blood clot suddenly blocks the vein, a drug that dissolves clots (such as tissue plasminogen activator) is sometimes used. The effectiveness of this treatment (called thrombolysis) is unclear.

If the disorder develops gradually, an anticoagulant, such as heparin, is sometimes used long term to help prevent clots from recurring or enlarging. Anticoagulants do not dissolve existing clots.

In newborns and children, the cause (usually an infected umbilical cord or acute appendicitis) is treated.

Problems caused by portal hypertension are also treated. Bleeding from varicose veins in the esophagus can be stopped using several techniques:

• Usually, rubber bands are inserted through a flexible viewing tube (endoscope), placed through the mouth into the esophagus. The bands are used to tie off the varicose veins.
• Antihypertensive drugs, such as beta-blockers and nitrates, reduce pressure in the portal vein and thus prevent bleeding in the esophagus. (Beta blockers also are used in portal hypertensive gastropathy.)
• Octreotide Some Trade Names
  SANDOSTATIN
  , a drug that also lowers blood flow to the liver and thus decreases blood pressure in the abdomen, may be given intravenously to help stop bleeding.

Occasionally, when these treatments are ineffective, a procedure to create an alternate route for blood flow, bypassing the liver, may be done. Here, the intent is to decompress the portal venous system through creation of a shunt (connection) to the inferior vena cava. The difficulty is creating a shunt when the portal vein is blocked. Also, shunts tend to become blocked.

For some people, liver transplantation is necessary.

Adapted from: Merck & Co., Inc.

Posted on December 13, 2009 | Filed under 9 | Permalink

Porphyria

Porphyria is a term that refers to a group of disorders—the porphyrias—that affect the nervous system or skin, or both. Each type of porphyria is due to the deficiency of one of the enzymes needed to make a substance in the body called heme. Enzymes are proteins that help chemical reactions happen in the body. Making heme involves a series of eight different enzymes, each acting in turn.

Heme is a red pigment composed of iron linked to a chemical called protoporphyrin. Heme has important functions in the body. The largest amounts of heme are in the blood and bone marrow in the form of hemoglobin within red blood cells. Hemoglobin gives blood its red color and carries oxygen from the lungs to all parts of the body. In the liver, heme is a component of proteins that have many functions, including breaking down hormones, drugs, and other chemicals and generating high-energy compounds that keep liver cells alive and functioning normally.

The body makes heme mainly in the bone marrow and the liver. The process of making heme is called the heme biosynthetic pathway. Each step of the process is controlled by one of eight enzymes. If any one of the enzymes is deficient, the process is disrupted. As a result, porphyrin or its precursors—chemicals formed at earlier steps of the process—may build up in body tissues and cause illness.

What are the types of porphyria?

The table below lists each type of porphyria and the deficient enzyme responsible for the disorder. Porphyrias are often classified as acute or cutaneous. Acute types of porphyria affect the nervous system, whereas cutaneous types mainly affect the skin. Two forms of porphyria—hereditary coproporphyria and variegate porphyria—may be either acute or cutaneous, or both.

What are the types of porphyria?

The table besides lists each type of porphyria and the deficient enzyme responsible for the disorder. Porphyrias are often classified as acute or cutaneous. Acute types of porphyria affect the nervous system, whereas cutaneous types mainly affect the skin. Two forms of porphyria—hereditary coproporphyria and variegate porphyria—may be either acute or cutaneous, or both.

The most common type of porphyria overall is porphyria cutanea tarda. In the United States, acute intermittent porphyria is the most common acute porphyria.

What causes porphyria?

Most porphyrias are inherited disorders, meaning they are caused by abnormalities in genes passed from parents to children. Scientists have identified the genes for all eight enzymes in the heme pathway. Some forms of porphyria result from inheriting an abnormal gene from one parent. Other forms are due to inheriting two abnormal genes—one from each parent. The risk that members of an affected family will have the disease or transmit it to their children depends on the type of porphyria.

One type of porphyria—porphyria cutanea tarda—is most often an acquired disorder. It occurs when factors other than genes cause an enzyme deficiency in the liver.

Porphyria can be triggered by

• drugs such as barbiturates, tranquilizers, birth control pills, and sedatives
• chemicals
• fasting
• smoking
• drinking alcohol, especially heavy drinking
• infections
• excess iron in the body
• emotional and physical stress
• menstrual hormones
• exposure to the sun

What are the symptoms of porphyria?

People with cutaneous forms of porphyria develop blisters, itching, and swelling of their skin when it is exposed to sunlight. Symptoms of acute forms of porphyria include pain in the abdomen, chest, limbs, or back; numbness, tingling, paralysis, or cramping; vomiting; constipation; and personality changes or mental disorders. Acute attacks of porphyria can develop over hours or days and last for days or weeks.

Symptoms can vary widely in severity. Some people with gene mutations that can cause porphyria have no signs or symptoms of the disorder. These people are said to have latent porphyria.

How is porphyria diagnosed?

Doctors diagnose porphyria using blood, urine, and stool tests. Interpreting test results can be complex, and initial tests may be followed by further testing to confirm the diagnosis. Diagnosis may be delayed because the symptoms of porphyria are similar to symptoms of other disorders.

How is porphyria treated?

Each type of porphyria is treated differently. Treatment may involve avoiding triggers, receiving heme through a vein, taking medicines to relieve symptoms, or having blood drawn to reduce iron in the body. People who have severe attacks may need to be hospitalized.

Points to Remember

• Porphyria is a group of disorders—the porphyrias—that affect the nervous system or skin, or both.
• Each type of porphyria results from a deficiency of one of the enzymes needed to make heme.
• Most porphyrias are inherited disorders, but porphyria cutanea tarda is usually an acquired disorder.
• Cutaneous types of porphyria affect the skin, causing symptoms such as blistering, itching, and swelling.
• Acute types of porphyria affect the nervous system, causing symptoms such as abdominal pain, vomiting, numbness, and mental disorders.
• Each type of porphyria is treated differently.

Posted on December 13, 2009 | Filed under 9 | Permalink

Budd-Chiari Syndrome

Budd-Chiari syndrome is caused by blood clots that completely or partially block the large veins that carry blood from the liver (hepatic veins) into the inferior vena cava.

• Some people have no symptoms, but others experience fatigue, abdominal pain, nausea, and jaundice.
• Fluid may accumulate in the abdomen, the spleen may enlarge, and sometimes severe bleeding occurs in the esophagus.
• Doppler ultrasonography can detect narrowed or blocked veins.
• Drugs may be used to dissolve or decrease the size of the clot, or a connection may be made between veins to allow blood to bypass the liver.

Budd-Chiari syndrome usually occurs when a clot narrows or blocks the hepatic veins.

Because blood flow out of the liver is impeded, blood accumulates in the liver, causing it to enlarge. The spleen may also enlarge. The backup of blood (congestion) in the hepatic veins causes blood pressure in the portal vein to increase. This increased pressure, called portal hypertension, can result in dilated, twisted (varicose) veins in the esophagus (esophageal varices). Portal hypertension, plus the engorged and damaged liver, leads to fluid accumulating in the abdomen, a condition termed ascites. The kidneys contribute to the problem by causing salt and water to be retained.

The clot may extend to also block the inferior vena cava (the large vein that carries blood from the lower parts of the body, including the liver, to the heart). Varicose veins in the abdomen near the skin’s surface may develop and become visible.

Eventually, severe scarring of the liver (cirrhosis) occurs.

Causes

Usually, the cause is a disorder that makes blood more likely to clot, such as the following:

• Excess red cells (polycythemia)
• Sickle cell disease
• Inflammatory bowel disease
• Connective tissue disorders
• Injury

Sometimes Budd-Chiari syndrome begins suddenly and rather severely, typically during pregnancy. During pregnancy, the blood normally coagulates more readily. In some women, a blood clotting disorder may first become apparent during pregnancy. Other causes include disorders that develop near the hepatic veins, such as parasitic infections and liver or kidney tumors that press on or invade the veins. In Asia and South Africa, the cause is commonly a membrane (web) that blocks the inferior vena cava. Often, the cause is unknown.

Symptoms

Symptoms vary somewhat depending on whether they appear suddenly or develop more slowly.

Usually, symptoms develop gradually over weeks or months. Fatigue is common. The enlarged liver becomes tender, and people have abdominal pain.

Fluid may accumulate in the legs, causing swelling (edema), or in the abdomen, causing ascites. Varicose veins in the esophagus can rupture and bleed, sometimes profusely. People may vomit blood. Such bleeding is a medical emergency.

If cirrhosis develops, it can lead to liver failure with deterioration of brain function (hepatic encephalopathy), resulting in confusion and even coma

Sometimes symptoms begin suddenly, such as hepatic vein thrombosis during pregnancy. In this case, people feel tired, and the liver is enlarged and tender. Abdominal pain occurs in the upper abdomen. Additional symptoms include vomiting and a yellow discoloration of the skin and whites of the eyes—a condition called jaundice. Liver failure infrequently develops.

Diagnosis

Doctors suspect the Budd-Chiari syndrome in people with either of the following:

• An enlarged liver, ascites, liver failure, or cirrhosis when there is no obvious cause, even after testing
• Abnormal results of blood tests done to evaluate liver function plus conditions that increase the risk of blood clots

If results of liver function tests are abnormal, an imaging test, typically Doppler ultrasonography, is done. If results are unclear, magnetic resonance imaging of blood vessels (magnetic resonance angiography) or computed tomography (CT) is done.

If surgery is planned, venography is necessary. For this procedure, x-rays of the veins are taken after a radiopaque dye (which is visible on x-rays) is injected into a vein in the groin.

A liver biopsy may be done to confirm the diagnosis and identify if cirrhosis has developed.

Prognosis

When the vein remains completely blocked, most people, if untreated, die of liver failure within 3 years. When the blockage is incomplete, life expectancy is longer but varies.

Treatment

Treatment depends on how rapidly the disorder has developed and how severe it is.

When symptoms begin suddenly and the cause is a clot, fibrinolytic (thrombolytic) drugs, which dissolve clots, help. On a more long-term basis, anticoagulant drugs prevent clots from enlarging or recurring.

If a vein is narrowed or blocked by a web, angioplasty may be done to widen it. For this procedure (called percutaneous transluminal angioplasty), a catheter with a deflated balloon at its tip is inserted through the skin into a blood vessel (such as the femoral vein in the groin) and threaded to the blocked vein. The balloon is inflated, widening the vein. A wire mesh tube (stent) is then inserted and left in place to keep the vein open.

Another solution is to create an alternate route for blood flow, bypassing the liver. This procedure, called transjugular intrahepatic portal-systemic shunting (TIPS), reduces pressure in the portal vein. For the procedure, a local anesthetic is used to numb the neck, and a catheter with a cutting needle is inserted into a vein in the neck (jugular vein). The catheter is threaded through the inferior vena cava to the hepatic vein. The needle is used to create a connection (called a shunt) between two veins, usually a branch of the hepatic vein and the portal vein, so that blood can bypass the liver. Then, an expandable metal tube (stent) is threaded to and placed in the shunt to keep it open. The shunt enables blood to bypass the liver, flowing from the portal vein (which normally brings blood to the liver) directly to the hepatic veins (which drain blood away from the liver). The blood returns to the heart through the inferior vena cava. However, such shunts increase the risk of hepatic encephalopathy (deterioration of brain function due to liver dysfunction). Also, shunts occasionally become blocked, especially in people who have a tendency to form blood clots.

Liver transplantation can be life-saving, particularly for people with severe liver failure.

Problems resulting from the disorder are also treated:

• Bleeding from varicose veins in the esophagus: Several techniques can be used to stop the bleeding. Usually, rubber bands are inserted through a flexible viewing tube (endoscope), placed through the mouth into the esophagus. The bands are used to tie off the varicose veins (termed ligation).
• Fluid accumulation in the abdomen: A low-salt (sodium) diet and diuretics can help prevent too much fluid from accumulating in the abdomen.

Most people need to take anticoagulants indefinitely to prevent new blockages from developing.

Adapted from: Merck & Co., Inc.

Posted on December 13, 2009 | Filed under 9 | Permalink

Cirrhosis of the Liver

Cirrhosis refers to scarring of the liver. Scar tissue forms because of injury or long-term disease. It replaces healthy tissue.

Scar tissue cannot do what healthy liver tissue does—make protein, help fight infections, clean the blood, help digest food, and store energy for when you need it. Scar tissue also blocks the normal flow of blood through the liver. Too much scar tissue means that your liver cannot work properly. To live, you need a liver that works.

Cirrhosis can be life-threatening, but it can also be controlled if treated early.

What are the symptoms of cirrhosis?

You may have no symptoms at all in the early stages. As cirrhosis progresses you may

• feel tired or weak
• lose your appetite
• feel sick to your stomach
• lose weight

Cirrhosis can also lead to other problems.

• You may bruise or bleed easily, or have nosebleeds.
• Bloating or swelling may occur as fluid builds up in the abdomen or legs. Fluid build up in the abdomen is called ascites (ah-SI-teez) and in the legs is called edema.
• Medications may have a stronger effect on you because your liver does not break them down as quickly.
• Waste materials from food may build up in the blood or brain and may cause confusion or difficulty thinking. For example, protein that you eat breaks down into chemicals like ammonia. When red blood cells get old, they break down and leave a substance called bilirubin (bil-ih-ROO-bun). A healthy liver removes these byproducts, but a diseased liver leaves them in the body.
• Blood pressure may increase in the vein entering the liver, a condition called portal hypertension.
• Enlarged veins, called varices (VARE-ah-seez), may develop in the esophagus and stomach. Varices can bleed suddenly, causing vomiting of blood or passing of blood in a bowel movement.
• The kidneys may not work properly or may fail.

As cirrhosis progresses, your skin and the whites of your eyes may turn yellow, a condition called jaundice (JON-diss). You may also develop severe itching or gallstones.

In the early stages, cirrhosis causes your liver to swell. Then, as more scar tissue replaces normal tissue, the liver shrinks.

About 5 percent of patients with cirrhosis also get cancer of the liver.

What causes cirrhosis?

Cirrhosis has many causes, including

• alcohol abuse (alcoholic liver disease)
• chronic viral hepatitis (hepatitis B, C, or D)
• autoimmune hepatitis, which is destruction of liver cells by the body’s immune system
• nonalcoholic fatty liver disease or nonalcoholic steatohepatitis (NASH), which is fat deposits and inflammation in the liver
• some drugs, toxins, and infections
• blocked bile ducts, the tubes that carry bile from the liver
• some inherited diseases such as
  • hemochromatosis (HEE-moh-KROH-muh-TOH-sus), a disease that occurs when the body absorbs too much iron and stores the excess iron in the liver, pancreas, and other organs
  • Wilson disease, which is caused by the buildup of too much copper in the liver
  • protoporphyria (PROH-toh-pour-FEAR-ee-uh), a disorder that affects the skin, bone marrow, and liver

Sometimes the cause of cirrhosis remains unknown even after a thorough medical examination.

How is cirrhosis diagnosed?

Your symptoms, a physical examination, and certain tests can help your doctor diagnose cirrhosis. Some tests are

• Blood tests to see whether your liver is working properly. Routine blood tests may be normal in cirrhosis. However, with advanced cirrhosis, blood tests may reveal abnormal levels of bilirubin and other substances.
• X rays, magnetic resonance imaging, or ultrasound images, which are pictures developed from sound waves, may show an enlarged or shrunken liver.
• Liver biopsy, an examination of a piece of your liver under a microscope, to look for scar tissue. This is the most accurate way to diagnose cirrhosis.

How is cirrhosis treated?

Once you have cirrhosis, nothing can make the scar tissue go away completely. However, treating the cause will keep cirrhosis from getting worse. For example, if cirrhosis is due to alcoholic liver disease, the treatment is to completely stop drinking alcohol. If cirrhosis is caused by hepatitis C, then that disease may be treated with medication.

Your doctor will suggest treatment based on the cause of your cirrhosis and your symptoms. Early diagnosis and carefully following an appropriate treatment plan can help many people with cirrhosis. In very advanced cirrhosis, however, certain treatments may not be possible. In that situation, your doctors will work with you to prevent or manage the complications that cirrhosis can cause.

How can I prevent cirrhosis if I already have liver disease?

• See your doctor for treatment of your liver disease. Many of the causes of cirrhosis are treatable, and early treatment may prevent cirrhosis.
• Follow a healthy lifestyle, eat a healthy diet, and stay active.
• Try to keep your weight in the normal range. Being overweight can make several liver diseases worse.
• Do not drink alcohol. Alcohol can harm liver cells, and chronic alcohol use is one of the major causes of cirrhosis.
• Stay away from illegal (street) drugs, which can increase your chances of getting hepatitis B or hepatitis C.
• See your doctor if you have chronic viral hepatitis. Effective treatments for both hepatitis B and hepatitis C are available. If you are on treatment, follow your treatment directions exactly.
• If you have autoimmune hepatitis, take medications and have regular check-ups as recommended by your doctor or a liver specialist (hepatologist).

What can I do to keep cirrhosis from getting worse?

• Stop drinking alcohol completely.
• Do not take any medications, including those you can buy without a prescription such as vitamins and herbal supplements, without discussing them with your doctor. Cirrhosis makes your liver sensitive to certain medications.
• Get vaccinated against hepatitis A and hepatitis B. These forms of liver disease are preventable. Also, ask your doctor about getting a flu shot and being vaccinated against pneumonia.
• Avoid eating raw oysters or other raw shellfish. Raw shellfish can harbor bacteria (Vibrio vulnificus) that cause severe infections in people with cirrhosis.

Adapted from: National Institute of Diabetes and Digestive and Kidney Diseases

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Fatty Liver

What is fatty liver?

Fatty liver is just what its name suggests: the build-up of excess fat in the liver cells. It is normal for your liver to contain some fat. But if fat accounts for more than 10% of your liver’s weight, then you have fatty liverand you may develop more serious complications.

Fatty liver may cause no damage, but sometimes the excess fat leads to inflammation of the liver. This condition, called steatohepatitis, does cause liver damage. Sometimes, inflammation from a fatty liver is linked to alcohol abuse; this is known as alcoholic steatohepatitis. Otherwise the condition is called nonalcoholic steatohepatitis, or NASH.

An inflamed liver may become scarred and hardened over time. This condition, called cirrhosis , is serious and often leads to liver failure.

What are the symptoms of fatty liver?

A fatty liver produces no symptoms on its own, so people often learn about their fatty liver when they have medical tests for other reasons. NASH can damage your liver for years or even decades without causing any symptoms. If the disease gets worse, you may experience fatigue, weight loss, abdominal discomfort, weakness and confusion.

What causes fatty liver?

Eating excess calories causes fat to build up in the liver. When the liver does not process and break down fats as it normally should, too much fat will accumulate. People tend to develop fatty liver if they have certain other conditions, such as obesity, diabetes, or high triglycerides. Alcohol abuse, rapid weight loss and malnutrition may also lead to fatty liver. However, some people develop fatty liver even if they have none of these conditions – so everyone should know about it.

How is fatty liver diagnosed?

Your doctor may see something unusual in your blood test or notice that your liver is slightly enlarged during a routine checkup. These could be signs of a fatty liver. To make sure you don’t have another liver disease, your doctor may ask for more blood tests, an ultrasound, a CT scan or an MRI. If other diseases are ruled out, you may be diagnosed with NASH. The only way to know for sure is to get a liver biopsy. Your doctor will remove a sample of liver tissue with a needle and check it under a microscope.

How is fatty liver treated?

There are no medical or surgical treatments for fatty liver, but there are some steps you can take that may help prevent or reverse some of the damage. In general, if you have fatty liver, and in particular if you have NASH, you should:

• Lose weight – safely! That usually means losing no more than one or two pounds a week.
• Lower your triglycerides through diet, medication or both
• Avoid alcohol
• Control your diabetes, if you have it
• Eat a balanced, healthy diet
• Increase your physical activity
• Get regular checkups from a doctor who specializes in liver care

If I’ve been diagnosed with fatty liver, what questions should I ask my doctor?

• “What is the likely cause of my fatty liver?”
• “Do I have NASH? If not, how likely am I to develop NASH?”
• “Do I have cirrhosis? If not, how likely am I to develop cirrhosis?”
• “Do I need to lose weight? How can I do so safely?”
• “Should I be taking any medication to control my triglyceride levels?”
• “What medications or other substances should I avoid to protect my liver?”

Who is at risk for fatty liver?

Most (but not all) fatty liver patients are middle-aged and overweight. The risk factors most commonly linked to fatty liver disease are:

• Overweight (body mass index of 25-30)
• Obesity (body mass index above 30)
• Diabetes
• Elevated triglyceride levels

What is the best way to prevent fatty liver?

The best way to reduce your risk of developing fatty liver is to maintain a healthy weight and normal triglyceride levels. You should also avoid excess alcohol and other substances that could harm your liver.

Adapted from: American Liver Foundation

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Liver Cysts and Tumors

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Biliary Atresia (BA)

Biliary atresia is a disease of the bile ducts that affects only infants. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the small intestine, where it helps digest fats.

In biliary atresia, the bile ducts become inflamed and blocked soon after birth. This causes bile to remain in the liver, where it starts to destroy liver cells rapidly and cause cirrhosis, or scarring of the liver.

What causes biliary atresia?

The cause of this disease is not known. In some infants, the condition is most likely congenital, meaning present from birth. About one in 10 babies with biliary atresia have other congenital defects. Some research indicates that an early viral infection may be linked to biliary atresia.

Scientists do know that biliary atresia is not hereditary; parents do not pass it on to their child. It is also not contagious, and it is not preventable. It is also not caused by anything an expectant mother did or did not do.

What are the symptoms of biliary atresia?

Symptoms of biliary atresia usually appear between two and six weeks after birth. The baby will appear jaundiced, with yellowing of the skin and whites of the eyes. The liver may harden and the abdomen may become swollen. Stools appear pale grey and the urine may appear dark. Some babies may develop intense itching.

How is biliary atresia diagnosed?

Since other conditions lead to symptoms similar to those of biliary atresia, doctors must perform many tests before a conclusive diagnosis can be made. These tests may include blood and liver tests, an ultrasound examination, X-rays and a liver biopsy, in which a small amount of liver tissue is removed with a needle for examination in a laboratory.

How is biliary atresia treated?

Unfortunately, there is no cure for biliary atresia. The only treatment is a surgical procedure in which the blocked bile ducts outside the liver are replaced with a length of the baby’s own intestine, which acts as a new duct. This surgery is called the Kasai procedure after Dr. Morio Kasai, the Japanese surgeon who developed it.

The aim of the Kasai procedure is to allow drainage of bile from the liver into the intestine through the new duct. The operation is completely or partially successful about 80 percent of the time if performed early (before 3 months of age). In babies who respond well, jaundice and other symptoms usually disappear after several weeks.

In cases where the Kasai procedure does not work, the problem often lies in the fact that blocked bile ducts are “intrahepatic,” or inside the liver, as well as extrahepatic, or outside the liver. No procedure, except for liver transplant, has been developed to replace blocked intrahepatic ducts.

The Kasai procedure is most successful in babies younger than 3-months-old, so early diagnosis is important.

If the Kasai procedure is not successful, the only other option is a liver transplant. However, a suitable donor organ must be found quickly, before damage to the liver from the backed-up bile becomes deadly.

What happens after surgery?

The aim of treatment after surgery is to encourage normal growth and development. If bile flow is good, the child is given a regular diet. If tests show that bile flow is reduced, a low-fat diet and vitamin supplements will be required, since the absorption of fats and vitamins is impaired.

What is the outlook for babies with biliary atresia?

Without successful treatment, few children with biliary atresia live beyond age two. In some cases, where the Kasai procedure is completely successful, the child may recover and live a normal life. However, in most cases, even when surgery is successful, patients will suffer gradual damage to the liver. These children will need specialized medical care throughout their lives, and many will ultimately need a liver transplant.

Is a liver transplant the solution?

In children with biliary atresia, a liver transplant is generally not attempted unless the Kasai procedure has been tried. In cases where that operation is unsuccessful, the child may become a candidate for a liver transplant. Success depends on the timely availability of a matching donor liver. Fortunately, survival rates for liver transplant recipients have increased dramatically with improved surgical techniques and the development of drugs that help overcome the problem of organ rejection.

What can the family do?

Watching a newborn suffer from biliary atresia is a heart-breaking experience. Parents are frustrated because so little is known about the disease and treatment options are so limited. Many parents find comfort in support groups because it allows them to talk to other parents in similar situations. Finding out that others feel the way they do, and learning how other parents are coping with a child’s disease, is often a great comfort.

Adapted from: American Liver Foundation

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Cholestasis

Cholestasis is reduction or stoppage of bile flow.

• Disorders of the liver, bile duct, or pancreas can cause cholestasis.
• The skin and whites of the eyes look yellow, the skin itches, urine is dark, and stools may become light-colored and smell foul.
• Laboratory and often imaging tests are needed to identify the cause.
• Treatment depends on the cause, but drugs can help relieve itching.

With cholestasis the flow of bile (the digestive fluid produced by the liver) is impaired at some point between the liver cells and the duodenum (the first segment of the small intestine). When bile flow is stopped, the pigment bilirubin (a waste product formed when old or damaged red blood cells are broken down) escapes into the bloodstream and accumulates.

Causes

The causes of cholestasis are divided into two groups: those originating within the liver and those originating outside the liver.

Within the Liver: Causes include acute hepatitis, alcoholic liver disease, primary biliary cirrhosis with inflammation and scarring of the bile ducts, cirrhosis due to viral hepatitis B or C (also with inflammation and scarring of the bile ducts), drugs, hormonal effects on bile flow during pregnancy (a condition called cholestasis of pregnancy (see ), and cancer that has spread to the liver.

Outside the Liver: Causes include a stone in a bile duct, narrowing (stricture) of a bile duct, cancer of a bile duct, cancer of the pancreas, and inflammation of the pancreas (pancreatitis).

Symptoms

Jaundice, dark urine, light-colored stools, and generalized itchiness are characteristic symptoms of cholestasis. Jaundice results from excess bilirubin deposited in the skin, and dark urine results from excess bilirubin excreted by the kidneys. Retention of bile products in the skin may cause itching, with subsequent scratching and skin damage. Stools may become light-colored because the passage of bilirubin into the intestine is blocked. Stools may contain too much fat (a condition called steatorrhea) because bile cannot enter the intestine to help digest fat in foods. Fatty stools may be foul-smelling. The lack of bile in the intestine also means that calcium and vitamin D are poorly absorbed. If cholestasis persists, a deficiency of these nutrients can cause loss of bone tissue. Vitamin K, which is needed for blood clotting, is also poorly absorbed from the intestine, causing a tendency to bleed easily.

Prolonged jaundice due to cholestasis produces a muddy skin color and fatty yellow deposits in the skin. Whether the person has other symptoms, such as abdominal pain, loss of appetite, vomiting, or fever, depends on the cause of cholestasis.

Diagnosis

A doctor tries to determine whether the cause is within or outside the liver on the basis of symptoms and the results of a physical examination.

Recent use of drugs that can cause cholestasis suggests a cause within the liver. Small, spiderlike blood vessels visible in the skin, an enlarged spleen, and fluid in the abdominal cavity (ascites), which are signs of chronic liver disease, also suggest a cause within the liver.

Findings that suggest a cause outside the liver include certain kinds of abdominal pain (such as intermittent pain in the upper right side of the abdomen and sometimes also in the right shoulder ) and an enlarged gallbladder (felt during the physical examination or detected by imaging studies).

Some findings do not indicate whether the cause is within or outside the liver. They include heavy alcohol intake, loss of appetite, nausea, and vomiting.

Typically, the blood levels of two enzymes, alkaline phosphatase and gamma-glutamyl transpeptidase, are very high in people with cholestasis. A blood test that measures the level of bilirubin indicates the severity of the cholestasis but not its cause. An imaging study, usually ultrasonography, is almost always done if blood test results are abnormal. Computed tomography (CT) or sometimes magnetic resonance imaging (MRI) may be done in addition to or instead of ultrasonography. If the cause appears to be within the liver, a liver biopsy may be done and usually establishes the diagnosis. If the cause appears to be blockage of the bile ducts, more precise images of these ducts are usually needed. Typically, either endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP) is done. MRCP uses magnetic resonance imaging. In ERCP, a contrast agent is injected and x-rays are taken.

Treatment

A blockage of the bile ducts can usually be treated with surgery or endoscopy (using a flexible viewing tube with surgical instruments attached). A blockage within the liver may be treated in various ways depending on the cause. If a drug is the suspected cause, the doctor stops its use. If acute hepatitis is the cause, cholestasis and jaundice usually disappear when hepatitis has run its course. A person with cholestasis is advised to avoid or stop using any substance that is toxic to the liver, such as alcohol and certain drugs.

Cholestyramine, taken by mouth, can be used to treat itchiness. This drug binds with certain bile products in the intestine, so they cannot be reabsorbed to irritate the skin. Unless the liver is severely damaged, taking vitamin K can improve blood clotting. Supplements of calcium and vitamin D are often taken if the cholestasis persists, but they are not very effective in preventing loss of bone tissue.

Adapted from: Merck & Co., Inc.

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Alagille Syndrome

Alagille syndrome is a rare, inherited disorder in which bile flow from the liver is decreased and the there are fewer than normal bile ducts.

Alagille syndrome is a disorder that often affects the liver and other organs, including the heart, eyes, spine and kidneys.

Often, patients with alagille syndrome have distinctive facial appearances. A small number of patients (about 15%) go on to develop severe liver disease and require liver transplantation.

This disorder is usually present at birth. Alagille syndrome is one of the most common inherited disorders that causes reduced bile flow within the liver. It occurs about once in every 100,000 births.

What are the causes of alagille syndrome?

Recent studies have discovered that defects of a specific gene cause alagille syndrome. The disease usually is inherited from one parent.

A single copy of the diseased gene (received from either the mother or father) “dominates” the other normal gene and results in this disease.

There is a 50% chance a parent with alagille syndrome will transmit this disorder to their child. Family members with Alagille syndrome may be affected very differently by the disorder.

Bile is produced in the liver and then travels through the many small bile ducts in the liver into one large bile duct, and eventually into the intestines. In patients with alagille syndrome, the lack of small bile ducts inside the liver slows down the flow of bile from the liver to the intestines.

Bile is composed of bilirubin, bile salts, cholesterol and metals such as copper. Bile acids are necessary to absorb fat and certain vitamins. When the bile flow is decreased, each of its components builds up in the body, and the body is not able to properly absorb fat and vitamins. This causes a wide range of symptoms.

What are the signs and symptoms of alagille syndrome?

Symptoms of alagille syndrome range from mild to severe. Symptoms within the first three months of life often include:

• Jaundice, usually present at birth (yellowing of the skin)
• Severe itching (caused by the buildup of bile salt in the body)
• Pale, loose or clay-colored stools (this happens because there is little or no bile reaching the intestine to color the bowel movements)
• Poor weight gain (due to a lack of bile needed to digest and absorb fat)
• Poor growth
• Deficiency of vitamins A, D, E and K which depends on bile acids for absorption; this may result in vision changes, rickets, difficulty with balance and poor clotting of the blood
• Heart murmurs, due to narrowing of the blood vessels in the lung (peripheral pulmonary artery stenosis)

Other symptoms that may develop later include:

• Jaundice that will not go away
• Continued growth and development problems in early childhood
• Enlarged liver (feels larger and harder than normal)
• Hard, whitish nodules (called xanthonmas) that develop in the skin due to cholesterol and fatty deposits; the nodules usually occur in spots of repeated trauma in young children, such as knees and elbows
• Urine appears very dark yellow or brown (due to high levels of bilirubin in the bloodstream which pass to the kidneys)
• Persistent itching

How is alagille syndrome diagnosed?

A diagnosis of alagille syndrome is made based on either tests or a physical examination. While there are specific findings that indicate alagille syndrome, all patients do not demonstrate all of them.

Reduced bile flow is one finding that indicates alagille symdrome. Symptoms of reduced bile flow include jaundice, itching and cholesterol deposits in the skin.

A liver biopsy may be done to show examine the bile ducts and degree of scarring in the liver. Liver function tests are blood tests used to assess the general state of the liver or biliary system.

A nuclear scan may be done to show how much bile is flowing from the liver. Genetic testing may be available through research laboratoraties but as of 2006, it is not routinely available.

Other findings that may indicate alagille syndrome include:

• Heart murmur (due to narrowing of the blood vessel connecting the heart to the lungs)
• Bone defects (shape of the bones in the spinal column may look like a butterfly’s wings)
• Kidney problems or kidney failure
• Distinct physical features, such as a broad forehead, straight nose, deep set eyes, a small, pointed chin, and fingers that are shorter than normal
• Problems with the eye — an extra thickening of a line, known as the Schwalbe’s line on the surface of the eye

What is the treatment?

There is no cure for alagille syndrome. Management of the disorder is aimed at preventing complications, increasing the flow of bile from the liver, maintaining normal growth and development and reducing blood cholesterol levels.

Medications. Medicine may be used to increase bile flow and to relieve severe itching caused by buildup of bile in the blood and skin. These same medicines may be used to treat high cholesterol levels that cause the hard, whitish nodules that develop in the skin in alagille syndrome patients.

Vitamin supplements. Reduced bile flow can lead to difficulty digesting fat and vitamins from a child’s diet. Fat-soluble vitamin supplements (A, D, E, and K) may be used.
Baby formula rich in triglyceride. Baby formula rich in a type of fat that can be digested despite reduced bile flow may be used to help with fat absorption in infants.

High-calorie diet. Alagille syndrome patients often have difficulty absorbing the calories they eat. To prevent malnutrition and growth failure, a high-calorie diet with a lot of protein may be recommended. A feeding tube that delivers large quantities of nutrients overnight may be used.

Liver transplantation. A small percentage of patients will develop cirrhosis (severe damage to the liver). Liver transplantation is the only option for these patients.

A liver transplant also may be the last resort for patients with severe itching, portal hypertension (high blood pressure) and severe growth failure that is not responding to medication. Liver transplantation has been used successfully in these cases.

What is the long-term prognosis for alagille syndrome patients?

Prognosis depends on the severity of the bile flow obstruction and scarring of the liver, and the severity of other problems that may develop — such as heart or lung problems or problems related to poor nutrition.

A typical course of liver disease with alagille syndrome patients is worsening cholestasis (decreasing bile flow) for several years, and then some improvement after that time.
Children with alagille syndrome generally have a better outcome than children with other liver disorders at the same age. Many adults with alagille syndrome lead normal lives

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Alpha-1 Antitrypsin Deficiency

Alpha-1 antitrypsin deficiency (Alpha-1) is a hereditary genetic disorder which may lead to the development of lung and/or liver disease. It is the most common genetic cause of liver disease in children. Adults can also be affected by Alpha-1 and may develop lung conditions such as emphysema as well as liver problems. Fortunately, many persons diagnosed with Alpha-1 never develop any of the associated diseases.

What are the symptoms of Alpha-1 Antitrypsin Deficiency?

In newborns, the typical symptoms of Alpha-1 are jaundice, swelling of the abdomen, and poor feeding. Alpha-1 may also appear in late childhood or adulthood and be detected because of fatigue, poor appetite, swelling of the abdomen and legs or abnormal liver tests.

What causes Alpha-1 Antitrypsin Deficiency?

Alpha-1 antitrypsin is a protein made in the liver which plays an important role in preventing the breakdown of enzymes in various organs. Individuals with Alpha-1 have a genetic disorder that prevents their body from creating enough of this protein. This trait must be inherited from both parents (who are, by definition, carriers) in order to cause the disorder.

How is Alpha-1 Antitrypsin Deficiency diagnosed?

The diagnosis of Alpha-1 is made by blood tests showing the low levels of alpha-1 antitrypsin and abnormal liver tests. Other tests such as ultrasound imaging or tests using specialized X-ray techniques may be necessary. A liver biopsy may be performed to check for damage to the liver.

How is Alpha-1 Antitrypsin Deficiency treated?

Currently, there is no cure for Alpha-1. Treatment is designed to maintain normal nutrition, to provide the liver and the body with essential nutrients, and to identify complications early in order to treat them better. Multiple vitamins and vitamins E, D, and K are often given. Certain abnormalities can be treated or controlled. Jaundice, severe itching, and fluid retention can all be dealt with through medication.

If I or my child has been diagnosed with Alpha-1 Antitrypsin Deficiency, what should I ask my doctor?

Speak with your doctor about the particular symptoms you are experiencing and the best methods for treating them. Also discuss the best ways to check for future progression of the disease.

Who is at risk for Alpha-1 Antitrypsin Deficiency?

Worldwide, approximately 1 in 2,500 individuals has Alpha-1 Antitrypsin Deficiency. This disorder is found in all ethnic groups; however, it occurs most frequently in whites of European ancestry.

Adapted from: American Liver Foundation

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Ascites

Ascites is the accumulation of protein-containing (ascitic) fluid in the abdominal cavity.

• Many disorders can cause ascites, but cirrhosis is the most common.
• If large amounts of fluid accumulate, the abdomen becomes very large, sometimes making people lose their appetite and feel short of breath.
• Analysis of the fluid can help determine the cause.
• Usually, bed rest, a low-salt diet, and diuretics help eliminate excess fluid.

Ascites tends to occur in long-standing (chronic) rather than in short-lived (acute) disorders. It occurs most commonly in cirrhosis (severe scarring of the liver), especially in cirrhosis caused by alcoholism or viral hepatitis. It may occur in other liver disorders, such as severe alcoholic hepatitis without cirrhosis, chronic hepatitis, and obstruction of the hepatic vein (Budd-Chiari syndrome). Ascites can also occur in disorders unrelated to the liver, such as cancer, heart failure, kidney failure, inflammation of the pancreas (pancreatitis), and tuberculosis affecting the lining of the abdominal cavity.

In people with a liver disorder, ascitic fluid leaks from the surface of the liver and intestine. A combination of factors is responsible. They include portal hypertension, decreased ability of the blood vessels to retain fluid, fluid retention by the kidneys, and alterations in various hormones and chemicals that regulate bodily fluids.

Symptoms and Diagnosis

Small amounts of fluid in the abdominal cavity usually produce no symptoms, but massive amounts may cause abdominal swelling (distention) and discomfort. Pressure on the stomach from the swollen abdomen may lead to loss of appetite, and pressure on the lungs may lead to shortness of breath. When a doctor taps (percusses) the abdomen, the fluid makes a dull sound. When the abdominal cavity contains large amounts of fluid, the abdomen is taut, and the navel is flat or even pushed out. In some people with ascites, the ankles swell with excess fluid (edema). However, a doctor may not be able to detect ascitic fluid unless the volume is about a quart or more.

If the presence of ascites or its cause is not clear, the doctor may use ultrasonography. In addition, a small sample of ascitic fluid can be withdrawn by inserting a needle through the abdominal wall—a procedure called diagnostic paracentesis. Laboratory analysis of the fluid can help determine the cause.

Treatment

The basic treatment for ascites is bed rest and a salt-restricted diet, usually combined with drugs called diuretics, which make the kidneys excrete more water into the urine. If ascites makes breathing or eating difficult, the fluid may be removed through a needle inserted into the abdomen—a procedure called therapeutic paracentesis. The fluid tends to reaccumulate unless the person also restricts salt consumption and takes a diuretic. Because a large amount of albumin (the major protein in plasma) is usually lost from the blood into the abdominal fluid, albumin may be administered intravenously.

An infection called spontaneous bacterial peritonitis occasionally develops in ascitic fluid for no apparent reason, especially in people with alcoholic cirrhosis. Untreated, this infection can be fatal. Survival depends on early vigorous treatment with antibiotics.

Adapted from: Merck & Co., Inc.

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Dyspepsia

Dyspepsia, also called indigestion, is a problem that most adults experience at least once in their lifetime. Upper abdominal discomfort, nausea, vomiting, and belching are all symptoms of dyspepsia. Indigestion is often related to consumption of too much food, especially heavy or greasy foods, eating late at night, smoking, alcohol consumption, or life stress. For some individuals, dyspepsia occurs regularly and becomes a health and quality-of-life issue. The April 5, 2006, issue of JAMA includes an article about dyspepsia.

SYMPTOMS

· Upper abdominal discomfort

· Sensation of bloating

· Nausea with or without vomiting

· Belching

· Abdominal gas

· Feeling full after eating small amounts (called early satiety

WHEN TO SEE YOUR DOCTOR

Because symptoms similar to those of dyspepsia may indicate a serious medical condition, you should see your doctor if you have indigestion on a regular basis. If you have bloody stools or vomit blood you should see a doctor immediately. Medical problems that have similar symptoms include gastroesophageal reflux disease (known as GERD), peptic ulcer disease, stomach cancer, pancreatic cancer, pancreatitis, gallbladder disease, irritable bowel syndrome, and even heart disease (upper abdominal pain may be due to angina or heart attack).

TESTING

Because dyspepsia can be a symptom of an underlying medical problem, your doctor may prescribe tests to look for these other conditions. These tests may include a barium swallow (an x-ray test in which the patient swallows a barium-containing solution to outline the gastrointestinal tract), upper gastrointestinal endoscopy (a lighted tube is passed through the esophagus into the stomach to look for abnormalities), or computed tomography (CT scan).

TREATMENT AND PREVENTION

• Avoid foods that trigger your dyspepsia symptoms.
• Avoid factors that make indigestion worse, such as eating large meals, especially heavy or greasy foods, eating late at night, eating quickly, or eating before exercise.
• Do not smoke.
• Avoid alcohol, if it produces symptoms of dyspepsia.
• Maintain a healthy weight, which helps to reduce symptoms of dyspepsia.
• Exercise regularly (at least 30 minutes of moderate to vigorous physical activity on most days of the week) to reduce stress, control weight, and improve well-being, all of which may ease dyspepsia.
• Follow your doctor’s recommendations regarding medications for treating dyspepsia. These may include antacids, proton pump inhibitors, histamine-2 receptor blockers, and motility drugs (to help the stomach empty). Other medications may be prescribed for individuals with other medical problems that influence the symptoms of indigestion.
  

Adapted from: JAMA Patient Page

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Helicobacter Pylori

This unusual name identifies a specific bacteria that can cause infection of the stomach. This infection can contribute to the development of diseases, such as dyspepsia (heartburn, bloating and nausea), gastritis (inflammation of the stomach), and ulcers in the stomach and duodenum. It will be useful to know some things about the upper digestive tract to understand how and where Helicobacter pylori infection can occur. When food is swallowed, it passes through the esophagus (the tube that connects the throat to the stomach). It then enters the larger upper part of the stomach. A strong acid that helps to break down the food is secreted in the stomach. The narrower, lower part of the stomach is called the antrum. The antrum contracts frequently and vigorously, grinding up the food and squirting it into the small intestine.

The duodenum is the first part of the small intestine, just beyond the stomach. The stomach, including the antrum, is covered by a layer of mucous that protects it from the strong stomach acid. It is known that alcohol, aspirin, and arthritis drugs such as ibuprofen can disrupt the protective mucous layer. This allows the strong stomach acid to injure underlying stomach cells. In some people, corticosteroids, smoking, and stress appear to contribute in some way. Until the mid 1980s, it was felt that one or more of these factors working together led to the development of gastritis and ulcers. Since that time, evidence has been mounting that Helicobacter pylori (H. pylori) has a major role in causing these diseases.

The Infection

H. pylori is a fragile bacteria that has found an ideal home in the protective mucous layer of the stomach. These bacteria have long threads protruding from them that attach to the underlying stomach cells. The mucous layer that protects the stomach cells from acid also protects H. pylori. These bacteria do not actually invade the stomach cells as certain other bacteria can. The infection, however, is very real and it does cause the body to react. Infection-fighting white blood cells move into the area, and the body even develops H. pylori antibodies in the blood. H. pylori infection probably occurs when an individual swallows the bacteria in food, fluid, or perhaps from contaminated utensils. The infection is likely one of the most common worldwide. The rate of infection increases with age, so it occurs more often in older people. It also occurs frequently in young people in the developing countries of the world, since the infection tends to be more common where sanitation is poor or living quarters are cramped. In many cases it does not produce symptoms. In other words, the infection can occur without the person knowing it. The infection remains localized to the gastric area, and probably persists unless specific treatment is given.

How is H. pylori Infection Diagnosed?

There are currently three ways to diagnose H. pylori infection. During endoscopy (a visual exam of the stomach through a thin, lighted, flexible tube), the physician can remove small bits of tissue through the tube. The tissue is then tested for the bacteria. A breath test is now available. In this test, a substance called urea is given by mouth. A strong enzyme in the bacteria breaks down the urea into carbon dioxide, which is then exhaled and can be measured. Finally, there is a blood test that measures the protein antibodies against these bacteria that are present in the blood. This antibody can mean the infection is present, or that it was present in the past and is now cleared. In other words, a person can have a positive blood test but no infection.

Gastritis and Dyspepsia

The symptoms are discomfort, bloating, nausea and perhaps vomiting. The person may also have symptoms that suggest ulcers such as burning or pain in the upper abdomen, usually occurring about an hour or so after meals or even during the night. The symptoms are often relieved temporarily by antacids, milk, or medications that reduce stomach acidity. Yet, the physician does not find an ulcer when the patient is tested by x-ray or endoscopy. When H. pylori is found in the stomach, it is tempting to believe that it is the cause of the symptoms, although this connection is not yet clear cut. The physician will usually prescribe antibiotic therapy to see if clearing the infection relieves symptoms.

Ulcers Stomach Ulcers: With stomach ulcers, H. pylori infection is found in 60 to 80 percent of the cases. Again, it is still uncertain how the infection acts to cause the ulcer. It probably weakens the protective mucous layer of the stomach. This allows acid to seep in and injure the underlying stomach cells. However, there is still a great deal of research to be done to unravel this relationship.

Duodenal ulcers: In times past, physicians were taught “no acid, no ulcer.” The medical profession felt the single most important factor causing duodenal ulcers to form was strong stomach acid. Research has now shown that over 90% of all patients who develop duodenal ulcers have H. pylori infection in the stomach as well. Medical studies are under way to determine the relationship between the two and how an infection in the stomach can be related to a duodenal ulcer. Acid is still an important factor; patients without acid in the stomach never get duodenal ulcers. However, physicians now accept the fact that the infection is directly related to the development of duodenal ulcers. It is now rather easy to clear duodenal ulcers with the strong acid-reducing medicines available, but the ulcers will usually recur unless the H. pylori infection is also cleared from the stomach.

Stomach Cancer and Lymphoma

These two types of cancer are now known to be related to H. pylori bacteria. This does not mean that all people with H. pylori infection will develop cancer; in fact, very few do. It is likely that if the infection is present for a long time, perhaps from childhood, these cancers may then develop. This is another reason why it is important to treat H. pylori infection.

When is Treatment Necessary?

Since the infection is so common, it is sometimes recommended that no treatment be given when there are no symptoms; however, these recommendations may change as more research develops. Increasingly, physicians are treating the acute ulcer with acid-reducing medicines and treating the infection with antibiotics. Interestingly, one of these antibiotics is a bismuth compound that is available over-the-counter as Pepto-Bismol. It is also available as a generic drug called bismuth subsalicylate. The bismuth part of the medicine actually kills the bacteria; however, do not go to the drugstore and purchase a bottle of Pepto-Bismol, expecting this alone to cure the infection. H. pylori is buried deep in the stomach mucous, so it is difficult to get rid of this infection. Several antibiotic drugs are always used together to prevent the bacteria from developing resistance to any one of them. Current medical studies are being done to develop easier treatment programs for this difficult infection.

Summary

H. pylori is a very common infection of the stomach. It may be the most common infection in the world. It is now clear that the infection is directly related to the development of stomach and duodenal ulcers, and it is likely that it may be related to cancers involving the stomach. There are several diagnostic tests available, and effective treatment can prevent the recurrence of ulcers and perhaps the development of cancer

Adapted:Jackson-Siegelbaum Gastroenterology

Posted on December 13, 2009 | Filed under 9 | Permalink

Hirschsprung’s Disease

Posted on December 13, 2009 | Filed under 9 | Permalink

Pancreatitis

The pancreas is a gland that sits behind the stomach. Larger than your gallbladder, but smaller than the liver, the pancreas plays a key role in the digestive system. Its juices join bile from the liver and gallbladder to drain into the small intestine. Specifically, the pancreas:

• Secretes digestive juices (enzymes and a substance called sodium bicarbonate) into the small intestine
• Produces the hormones, including insulin and glucagon, that control your body’s ability to use sugar

The digestive substances split the fats, proteins, and carbohydrates into digestible molecules.

What is Pancreatitis?

Pancreatitis is inflammation of the pancreas. This condition usually begins at an acute stage, and in some cases, may become chronic after a severe and/or recurrent attack. When the pancreas becomes inflamed, the digestive enzymes attack the tissue that produces them. One of these enzymes, called trypsin, can cause tissue damage and bleeding, and can cause the pancreas blood cells and blood vessels to swell. With chronic pancreatitis, the pancreas may eventually stop producing the enzymes that are necessary for your body to digest and absorb nutrients. This is called exocrine failure and fat and protein are not digested or absorbed. When chronic pancreatitis is advanced, the pancreas can also lose its ability to make insulin; this is called endocrine failure.

There are two stages of pancreatitis:

Acute pancreatitis: This condition can occur suddenly, soon after the pancreas becomes damaged or irritated by its own enzymes. Although acute pancreatitis is not fully understood, its causes are usually gallstones or alcohol abuse. When gallstones pass through the bile duct, they may become stuck, causing enzymes to build up in the pancreas because they cannot drain through the duct, and damaging the pancreas. In the case of alcohol, the pancreas may be sensitive to the effects of excessive alcohol. An attack may occur anywhere from a few hours or one to two days after drinking alcohol. The amount of alcohol consumed will vary from person to person. Other less common causes of this condition are: excessive levels of fat particles in the blood, mumps, drugs, surgery, heredity, and idiopathic (unknown cause). Acute pancreatitis affects about 80,000 Americans every year.

Chronic pancreatitis: This stage of pancreatitis begins as acute pancreatitis, and becomes chronic when the pancreas becomes scarred. This condition is usually due to years of excessive alcohol consumption, but may also develop from other causes of pancreatitis.

What are the Symptoms of Pancreatitis?

The symptoms begin as those of acute pancreatitis:

• A gradual or sudden severe pain in the center part of the upper abdomen goes through to your back; this pain may get worse when you eat and builds to a persistent pain
• Nausea and vomiting
• Fever
• Jaundice (a yellowing of the skin) due to blockage of the bile duct from the inflamed pancreas
• Shock
• Weight loss
• Symptoms of diabetes mellitus

Most chronic pancreatitis is due to alcohol abuse and is already chronic at its first presentation. In rare cases this condition leads to cancer of the pancreas, an unchecked growth of abnormal cells in the pancreas.

What Should I Do If I Think I Have Pancreatitis?

If your abdominal pain lasts more than 20 minutes, call your doctor or go to the emergency room. Your doctor will take a medical history, ask about your drinking history, and draw blood to test for pancreatic enzymes. You may also need to take pancreas function tests to determine the loss of pancreatic enzymes, a fecal fat collection test for evidence of malabsorption, and an ultrasound, CT scan or other test to determine pancreas damage.

If you have unexplained weight loss that lasts more than a few weeks, call your doctor. This can be a warning sign of pancreatic cancer.

What is the Difference Between Acute and Chronic Pancreatitis?

Most cases of acute pancreatitis are mild and involve a short hospital stay to help heal the pancreas. Chronic pancreatitis is a much more persistent condition, and occurs more often in men than women.

What is the Treatment for Pancreatitis?

Your doctor will focus treatment on your nutritional and metabolic needs and on relieving your pain. Mild pain can be treated with analgesics. If the cause of acute pancreatitis is gallstones, you may have to have your gallbladder removed to prevent further attacks. If the bile duct is found to be enlarged, you may need an ERCP (endoscopic retrograde cholangiopancreatography) to drain it. An ERCP is a way your doctor can examine your pancreas, pancreatic duct, the common bile duct, and/or sphincter of Oddi. It involves passage of a long, narrow tube called an endoscope used to put X-ray contrast dye into the bile and pancreas ducts. In severe cases, surgery will be required to drain the pancreatic duct or to remove part of the pancreas.

Your doctor will also likely give you dietary guidelines to follow in order to reduce the amount of fat you eat, since your body has trouble digesting these substances. You may also need to take pancreatic enzyme supplements, which are in the form of a tablet, every time you have a meal. These supplements will help your body absorb food and help you regain some of the lost weight.

What Hope for the Future?

Most people who have chronic pancreatitis have a good prognosis if they follow the required dietary changes and take their medications and required supplements. If their condition was caused by drinking, they will have a positive outcome if they stop drinking and continue follow-up treatment.

PREVENTION OF PANCREATITIS

While pancreatitis is still not fully understood, there are some steps you can take to prevent pancreatitis from occurring again:

• If the cause is gallstones and your gallbladder has not been removed, avoid fatty foods, such as butter and fried eggs
• If the cause is alcohol, you should stop drinking altogether

Glossary

Acute pancreatitis – The initial stage of pancreatitis, characterized by gradual or sudden severe pain in the center part of the abdomen that moves around to the back, signaling a damaged or irritated pancreas.

Bile – A secretion of the liver that helps digest fats in the intestines.

Biliary system – The ducts and tubes that collect and drain bile.

Chronic pancreatitis – Occurs when the symptoms of acute pancreatitis continue to recur. Usually due to years of excessive alcohol consumption, this condition may also develop from other causes of pancreatitis.

ECRP (Endoscopic Retrograde Cholangiopancreatography) – A long, narrow tube called an endoscope used to examine the pancreas, pancreatic duct, common bile duct, and/or sphincter of Oddi. The scope can be used to put contrast dye into the pancreas to highlight stones or blockages, and can be used to remove blockages.

Diabetes mellitus – A condition where the pancreas does not produce enough insulin to use carbohydrates.

Endocrine – An internal gland secretion–directly into the blood stream.

Exocrine – The external secretion of a gland-through a duct into the intestine.

Inflammation – A response to tissue injury that causes redness, swelling, and sometimes pain.

Jaundice – The skin and/or white of the eyes turns yellow. Itching may or may not occur.

Pancreas- A gland that sits behind the stomach, and produces insulin to metabolize sugar and secretes enzymes to breakdown fats, proteins, and carbohydrates.

Pancreatic duct – Drains pancreatic enzymes into the small intestine.

Sphincter of Oddi – A circular, contracting muscle at the intestinal opening of the bile and pancreatic ducts.

Adapted from: American Gastroenterological Association

Posted on December 13, 2009 | Filed under 9 | Permalink

Esophageal Achalasia

Esophageal achalasia is a primary esophageal motility disorder of unknown etiology, characterized by absence of esophageal peristalsis and increased or normal resting pressure of the lower esophageal sphincter (LES), which fails to relax completely in response to swallowing.

Clinical Presentation

Dysphagia is the most common symptom, experienced by virtually all patients. Regurgitation is the second most common symptom, and is present in about 60% of patients. It occurs more often in the supine position, and exposes the patients to the risk of aspiration of undigested food. Chest pain occurs in about 40% of patients, and is usually experienced at the time of a meal. Heartburn is experienced by about 40% of patients. In untreated patients this symptom is usually due to stasis and fermentation of food or esophageal distension.

Diagnosis

In addition to careful symptomatic evaluation, the following tests should be routinely performed: Barium swallow usually shows narrowing at the level of the gastroesophageal junction (”bird beak”), and various degrees of esophageal dilatation. Endoscopy is important to rule out the presence of a peptic stricture or cancer, and gastroduodenal pathology. In patients older than 60 years of age, with recent onset of dysphagia and excessive weight loss, secondary or pseudo-achalasia (obstruction due to a submucosal neoplasm in the distal esophagus) should be ruled out. Because a cancer of the gastroesophageal junction is the most common cause of pseudo-achalasia, an endoscopic ultrasound or a CT scan of the gastroesophageal junction can help to establish the diagnosis. Esophageal manometry is the key test for establishing the diagnosis. The classic manometric findings are: (a) absence of esophageal peristalsis, and (b) hypertensive or normotensive LES which fails to relax completely in response to swallowing.

Treatment

Treatment is directed toward elimination of the outflow resistance at the level of the gastroesophageal junction. The following treatment modalities are available to achieve this goal:

Traditionally, pneumatic dilatation has been the first line of treatment for esophageal achalasia, while surgery was reserved for patients who had persistent dysphagia after multiple dilatations or who had suffered a perforation during dilatation. Today, minimally invasive surgery has completely changed this treatment algorithm and a laparoscopic Heller myotomy and partial fundoplication is preferred by most gastroenterologists and surgeons as the primary treatment modality. When properly performed, a Heller myotomy can be expected to result in permanent relief of dysphagia in 85-100% of patients. Critical details of the operation include a generous myotomy of the lower esophagus, extending well onto the gastric wall. Because of the lack of esophageal peristalsis, a partial (Dor or Toupet), rather than a total fundoplication is frequently added to prevent reflux. A recent prospective, randomized study demonstrated that Heller myotomy plus a partial fundoplication is superior to Heller myotomy alone in regard to the incidence of postoperative reflux as measured by 24 hour pH testing. Patients can usually eat the morning of the first postoperative day, and can be discharged home after one or two days. In the only prospective, randomized trial performed comparing balloon dilation with surgery, myotomy outperformed balloon dilation 95% to 65%.

Historically, the most popular treatment for achalasia has been by forceful pneumatic dilation. The success rate of this procedure is 55-70% with a single dilation but can be increased to nearly 90% with multiple dilations. However, the risk of perforation with each dilation is at least 3-5% and has been reported as high as 12% in some series. These patients may require open surgery to close the perforation and perform a myotomy. Furthermore, when stratified by age, balloon dilation is less than 50% effective in patients younger than 40 years old and is rarely effective in adolescents.

Intrasphincteric injection of botulinum toxin (BOTOX) injection is less effective than balloon dilation and requires re-treatment to maintain an efficacy rate of 65%. Of greater concern is the fact that BOTOX injection leads to scar formation in the submucosal plane which results in a more difficult myotomy and higher mucosal perforation rate (up to 30%) during dissection. Thus, BOTOX should be reserved for the treatment of patients who are poor candidates for surgery and poor candidates for balloon dilation (dilated sigmoid esophagus) or as a bridge to surgery. An additional utility for BOTOX is in aiding in the diagnosis of patients who have equivocal findings on initial evaluation. A good response to BOTOX is usually an indication that the patient will have long-term relief following surgical myotomy.

In selected patients such as a hostile, multiply operated abdomen or following a failed abdominal myotomy, the thoracic or thoracoscopic approach may be preferred. The thoracic approach is also appropriate in managing patients with proximal esophageal motility abnormalities.

Occasionally the degree of esophageal aperistalsis is so advanced that myotomy alone will not relieve the dysphagia and the patient is better served with esophagectomy. Esophagectomy should be considered in a patient who has had a previous myotomy, with a resting LES pressure of less than 10 mmHg, and a dilated sigmoid esophagus. The need for esophagectomy for achalasia is very uncommon, even in the presence of a dilated esophagus, and should be reserved for failures after myotomy.

All patients undergoing treatment for achalasia should be followed by surveillance endoscopy, because they are at increased risk for development of both squamous and adenocarcinoma.

Risks

Aspiration of retained food in the esophagus at the time of induction of anesthesia and perforation of the esophageal mucosa are the most common operative complications. Persistent or recurrent dysphagia occurs in 5% to 10% of patients. The combination of intraoperative manometry and endoscopy can better guide the extent of the myotomy and can improve the adequacy of myotomy and are useful tools in decreasing the incidence of significant dysphagia after antireflux surgery. A complete work-up is necessary to evaluate the cause of the dysphagia in these patients, and either pneumatic dilatation or a second operation can often correct the problem. Up to 15% of patients may experience gastroesophageal reflux after myotomy, as measured by pH monitoring. In patients undergoing elective myotomy the mortality rate is less than 1%.

Expected Outcomes

About 90% of patients have long-term relief of dysphagia after a myotomy, with a low incidence of symptomatic acid reflux. There is often a poor correlation between symptoms of reflux and measurable reflux as demonstrated by pH study. All patients should be studied by postoperative pH study. Patients with demonstrated reflux by pH study or with reflux symptoms after surgery should be treated long-term with proton pump inhibitors.

Adapted from: Society for Surgery of the Alimentary Tract

Posted on December 13, 2009 | Filed under 9 | Permalink

Intussusception

Intussusception occurs when one portion of the bowel slides into the next, much like the pieces of a telescope. When this occurs, it creates an obstruction in the bowel, with the walls of the intestines pressing against one another. This, in turn, leads to swelling, inflammation, and decreased blood flow to the intestines involved.

The most common cause of intestinal obstruction in children between the ages of 3 months and 6 years, intussusception:

• occurs most often in children between 5 and 10 months of age (80% occur before a child is 24 months old)
• affects between one and four infants out of 1,000
• is three to four times more common in boys than in girls

Signs and Symptoms

Children with an intussusception have intense abdominal pain, which often begins so suddenly that it causes loud, anguished crying and causes the child to draw the knees up to the chest. The pain is usually intermittent, but recurs and becomes stronger. As the pain subsides, a child with an intussusception may stop crying and seem fine.

Other common symptoms include:

• abdominal swelling or distension
• passing stools (or poop) mixed with blood and mucus, known as currant jelly stool (60% percent of infants with an intussusception will pass currant jelly stool)
• vomiting
• vomiting up bile, a bitter-tasting fluid secreted by the liver that’s often golden-brown to greenish in color
• lethargy (i.e., drowziness or sluggishness)
• shallow breathing
• grunting

As the illness progresses, a child will become progressively weaker and may develop a fever and appear to go into shock. Symptoms of shock include lethargy, rapid heartbeat, weak pulse, low blood pressure, and rapid breathing.

Causes

In infants, the causes of intussusception are unknown, although there are some theories about why it occurs. Because intussusception is seen most often in spring and fall, this seems to suggest a possible connection to the kinds of viruses that children catch during these seasons, including upper respiratory infections.

In some cases, intussusception may follow a recent bout of gastroenteritis (sometimes called stomach flu). Gastrointestinal infections may cause swelling of the infection-fighting lymph tissue that lines the intestine, which may pull one part of the intestine into the other. Intussusception is most common around the age that infants are being introduced to solid foods. It has been suggested that the introduction of new foods may also cause some swelling of the lymph tissue in the intestines, increasing the chance of developing an instussusception.

Usually when an adult or a child older than 3 develops an intussusception, it’s often the result of enlarged lymph nodes, a tumor, or a polyp in the intestine.

Diagnosis and Treatment

The doctor will then perform a physical exam on the child, paying special attention to the abdomen. Often, the doctor can feel the part of the intestine that’s involved, which is swollen and tender and often is described as a “sausage-shaped mass.” Symptoms like pain, drawing up the legs, vomiting, lethargy, and passing bloody or currant jelly stool are meaningful in helping the doctor reach a diagnosis. In addition to doing a physical examination, the doctor will ask the parent about any concerns and symptoms their child has, the child’s past health, your family’s health, any medications the child is taking, any allergies the child may have, and other issues. This is called the medical history.

If the doctor thinks an intussusception may be the cause of the child’s pain, a pediatric surgeon will be consulted to examine the child and decide about treatment. The doctor may order an abdominal X-ray, which may or may not show an obstruction. An ultrasound examination may also help make the diagnosis. If the child appears very ill, suggesting damage to the intestine, the surgeon may opt to take the child immediately to the operating room to correct the bowel obstruction.

A barium or air enema is often used to both diagnose and treat a suspected intussusception. During a barium enema, a liquid mixture containing barium is given through a catheter tube into the child’s rectum, and special X-rays are taken. Barium outlines the bowels on the X-rays and, if an intussusception is present, shows the doctors the telescoping piece of intestine.

In many instances, the barium enema not only shows the intussusception, but the pressure from putting it in the bowel may also unfold the bowel that has been turned inside out, instantly curing the obstruction. An air enema, given rectally in a similar way as barium, can also be used to diagnosis and treat an intussusception.

The radiologist usually decides which test is most appropriate to perform. Both procedures are very safe and usually well tolerated by the child, although there is a very small risk of infection or bowel perforation. There’s a 10% risk of recurrence, which usually occurs within 72 hours following the procedure.

If the barium or air enema procedures aren’t successful or the child is too ill to attempt the enema, the child will undergo surgery. Enemas are less successful in older children, and they’re more likely to require surgery to treat intussusception. Surgeons will try to fix the obstruction but if too much damage has been done, that part of the bowel will be removed.

Some babies with intussusception may be given antibiotics to prevent infection. Babies who have been treated for intussusception will be kept in the hospital and given intravenous feedings until they’re able to eat and have normal bowel function.

Complications

If left untreated, intussusception can cause severe complications. Complications are directly related to the amount of time that passes from when the intussusception occurred until it’s treated. Most infants who are treated within the first 24 hours recover completely from an intussusception with no problems. Further delay increases the risk of complication which include irreversible tissue damage, perforation of the bowel, infection, and death.

When to Call Your Child’s Doctor

Intussusception is a medical emergency. If you’re concerned that your child has some or all of the symptoms of intussusception, such as abdominal pain, vomiting, or passing of currant jelly stool, call you child’s doctor or emergency medical services immediately.

The outcome for most infants with intussusception is very good, and with early treatment, complications are much less likely to develop. Do not delay, though — in many cases, early diagnosis can mean a child can be successfully treated without surgery

Adapted from: Nemours Foundation

Posted on December 13, 2009 | Filed under 9 | Permalink

Amebiasis, Ascariasis

Amebiasis is an intestinal illness caused by a microscopic parasite called Entamoeba histolytica. Approximately 1,000 cases are reported each year in New York State.

Who gets amebiasis?

Anyone can get amebiasis, but it is recognized more often in people arriving from tropical or subtropical areas, individuals living in institutions, and men who have sex with men.

How is amebiasis spread?

Amebiasis is contracted by consuming contaminated food or water containing the cyst stage of the parasite. It can also be spread by person-to-person contact.

What are the symptoms of amebiasis?

People exposed to this parasite may experience mild or severe symptoms or no symptoms at all. Fortunately, most exposed people do not become seriously ill. The mild form of amebiasis includes nausea, loose stools, weight loss, abdominal tenderness and occasional fever. Rarely, the parasite will invade the body beyond the intestines and cause a more serious infection, such as a liver abscess.

How soon after exposure do symptoms appear?

The symptoms may appear from a few days to a few months after exposure but usually within two to four weeks.

For how long can an infected person carry this parasite?

Some people with amebiasis may carry the parasite for weeks to years, often without symptoms.

Where are the parasites that cause amebiasis found?

The parasite lives only in humans. Fecal material from infected people may contaminate water or food, which may spread the parasites to anyone who consumes them.

How is it diagnosed?

Examination of stools under a microscope is the most common way for a doctor to diagnose amebiasis. Sometimes, several stool samples must be obtained because the number of amoeba being passed in the stool, which varies from day to day, may be too low to detect from any single sample.

What is the treatment for amebiasis?

Specific antibiotics such as metronidazole can be prescribed by a doctor to treat amebiasis.

Should an infected person be excluded from work or school?

Although people with diarrhea due to amebiasis should not attend school or go to work, it is not necessary to exclude infected persons when they feel better and stools are normal. Casual contact at work or school is unlikely to transmit the disease. Special precautions may be needed by foodhandlers or children enrolled in daycare settings. Consult your local health department for advice in such instances.

What precautions should the infected person follow?

The most important precautions are careful handwashing after each toilet visit and proper disposal of sewage. Homosexual males should refrain from intimate contact until effectively treated.

Adapted from: New York State Department of Health

Ascariasis

The disease and how it affects people

Ascariasis is an infection of the small intestine caused by Ascaris lumbricoides, a large roundworm. The eggs of the worm are found in soil contaminated by human faeces or in uncooked food contaminated by soil containing eggs of the worm. A person becomes infected after accidentally swallowing the eggs. The eggs hatch into larvae within the person’s intestine. The larvae penetrate the intestine wall and reach the lungs through the blood stream. They eventually get back to the throat and are swallowed. In the intestines, the larvae develop into adult worms. The female adult worm which can grow to over 30cm in length, lays eggs that are then passed into the faeces. If soil is polluted with human or animal faeces containing eggs the cycle begins again. Eggs develop in the soil and become infective after 2-3 weeks, but can remain infective for several months or years.

Children are infected more often than adults, the most common age group being 3-8 years. The infection is likely to be more serious if nutrition is poor. They often become infected after putting their hands to their mouths after playing in contaminated soil. Eating uncooked food grown in contaminated soil or irrigated with inadequately treated wastewater is another frequent avenue of infection.

The first sign may be the passage of a live worm, usually in the faeces. In a severe infection, intestinal blockage may cause abdominal pain, particularly in children. People may also experience cough, wheezing and difficulty in breathing, or fever.

Distribution

Ascariasis is found worldwide. Infection occurs with greatest frequency in tropical and subtropical regions, and in any areas with inadequate sanitation.

Scope of the Problem

Ascariasis is one of the most common human parasitic infections. Up to 10% of the population of the developing world is infected with intestinal worms – a large percentage of which is caused by Ascaris. Worldwide, severe Ascaris infections cause approximately 60,000 deaths per year, mainly in children.

Interventions

Health education providing the following messages reduces the number of infected people:

• avoid contact with soil that may be contaminated with human faeces;
• wash hands with soap and water before handling food;
• wash, peel or cook all raw vegetables and fruits;
• protect food from soil and wash or reheat any food that falls on the floor.

The availability of water for use in personal hygiene as well as proper disposal of human faeces will also reduce the number of cases. Where wastewater is used for irrigation waste stabilization ponds and some other technologies are effective in decreasing transmission due to food grown in contaminated soil.

Infected individuals (and domestic animals) should be treated with medicine to reduce disease transmission. Ascariasis can be effectively treated with mebendazole or pyrantel pamoate.

Adapted from: WHO

Posted on November 17, 2009 | Filed under 9 | Permalink

Osteogenesis Imperfecta

Steogenesis Imperfecta (OI), also known as brittle-bone disease, is a genetic (inherited) disorder characterized by bones that break easily without a specific cause. An estimated 20,000 to 50,000 people in the US have this disease.

The disorder occurs in one out of 20,000 to one out of 60,000 live births. OI can affect males and females of all races.

What causes osteogenesis imperfecta?
The cause of OI is believed to be due to a genetic defect that causes imperfectly-formed, or an inadequate amount of, bone collagen - a protein found in the connective tissue.

What are the types and symptoms of OI?
The following are the most common symptoms for osteogenesis imperfecta. However, each individual may experience symptoms differently. Although symptoms may vary, generally they are used to classify the four forms of OI, each of which represents the severity of the condition.

According to the Osteoporosis and Related Bone Diseases National Resource Center, part of the National Institutes of Health (NIH), the types of OI and their symptoms include the following:

Type I:

• most common

• bones fracture easily

• can usually be traced through the family

• near normal stature or slightly shorter

• blue sclera (the normally white area of the eye ball)

• dental problems

• hearing loss beginning in the early 20s and 30s

• most fractures occur before puberty; occasionally women will have fractures after menopause

• triangular face

• tendency toward spinal curvatures

Type II:

• newborns severely affected; frequently fatal

• usually resulting from a new gene mutation

• very small stature with extremely small chest and under developed lungs

Type III:

• tend to be isolated family incidents

• very small in stature – some only 3 feet tall

• fractures at birth very common

• x-ray may reveal healing of in utero fractures

• severe early hearing loss

• loose joints and poor muscle development in arms and legs

• barrel-shaped rib cage

Type IV:

• can frequently be traced through the family

• bones fracture easily – most before puberty

• normal or near normal colored sclera

• problems with teeth – more than Type I

• spinal curvatures

• loose joints

The symptoms of osteogenesis imperfecta may resemble other medical conditions or problems. Always consult your physician for a diagnosis.

How is osteogenesis imperfecta diagnosed?
In addition to a complete medical history and physical examination, diagnostic procedures for osteogenesis imperfecta may include a skin biopsy to evaluate the amount and structure of collagen. However, this test is complicated and not many qualified facilities are available to perform the procedure. It is not unusual for results of the biopsy to take up to six months.

Additional diagnostic tests may include:

• x-ray – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film
• an examination of the ear, nose, and throat (to detect hearing loss)

Treatment for Osteogenesis Imperfecta
Specific treatment for osteogenesis imperfecta will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• your tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

To date, there is no known treatment, medicine, or surgery that will cure osteogenesis imperfecta (OI). The goal of treatment is to prevent deformities and fractures and allow the child to function as independently as possible. Treatments for preventing or correcting symptoms may include:

• care of fractures
• surgery
• rodding – a procedure to insert a metal bar the length of a long bone to stabilize it and prevent deformity
• dental procedures
• physical therapy
• assistive devices, such as wheelchairs, braces, and other custom-made equipment

Management of Osteogenesis Imperfecta (OI)
Management of the disease includes focusing on preventing or minimizing deformities, and maximizing the individual’s functional ability at home and in the community. Management of OI is either non-surgical or surgical. Non-surgical interventions may include one or more of the following:

• physical therapy
• positioning aids (to help sit, lie, or stand)
• braces and splints (to prevent deformity and promote support or protection)
• medications
• psychological counseling

Surgical interventions may be considered to manage the following conditions:

• fractures
• bowing of bone
• scoliosis – a lateral, or sideways curvature and rotation of the back bones (vertebrae), giving the appearance that the person is leaning to one side.
• heart problems

Surgery may also be considered to maintain the ability to sit or stand. Long-Term Outlook for an Individual with Osteogenesis Imperfecta (OI)
Osteogenesis imperfecta (OI) is a progressive condition that needs life-long management to prevent deformity and complications. The interdisciplinary healthcare team helps the family to improve the functional outcomes and to provide support. The Osteogenesis Imperfecta Society can also be an important resource.

Posted on November 17, 2009 | Filed under 9 | Permalink

Paget’s Disease of the Bone

Paget’s disease of the bone is a chronic bone disorder in which bones become enlarged and deformed. Bone may become dense, but fragile, because of excessive breakdown and deformation of bone. The disease affects both genders, is rarely found in people under the age of 40, and occurs in up to 3 percent of the US population.

What causes Paget’s disease of the bone?

The exact cause of Paget’s disease of the bone is unknown, but it is suggested to be due to a slow viral infection of bone and may include a heredity factor.

What are the symptoms of Paget’s disease of the bone?

The following are the most common symptoms of Paget’s disease of the bone. However, each individual may experience symptoms differently. Symptoms may include:

• pain in the affected area
• bone deformity in the affected area
• susceptibility to fractures in the affected area
• headache (if the affected area is the skull)
• hearing loss (if the affected area is the skull)

The symptoms of Paget’s disease of the bone may resemble other bone disorders or medical problems. Always consult your physician for a diagnosis.

How is Paget’s disease of the bone diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for Paget’s disease of the bone may include the following:

• x-ray – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
• blood tests - a test that involves the alkaline phosphatase levels (an enzyme found throughout the body) in the bone being measured; any condition of bone growth or an increased activity of bone cells, including Paget’s disease, will cause alkaline phosphatase levels to rise.
• radionuclide bone scan – a nuclear imaging technique that uses a very small amount of radioactive material, which is injected into the patient’s bloodstream to be detected by a scanner. This test shows blood flow to the bone and cell activity within the bone.

Treatment for Paget’s disease of the bone:

Specific treatment for Paget’s disease of the bone will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• your tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

Treatment may include:

• medication that inhibits abnormal bone resorption
• surgery
• physical therapy
• arthroplasty of the hip (total joint replacement)
• cutting and realignment of affected bone

Adapted from: UNIVERSITY OF MARYLAND MEDICAL SYSTEM

Posted on November 17, 2009 | Filed under 9 | Permalink

Avascular Necrosis

Avascular necrosis (also called osteonecrosis, aseptic necrosis, or ischemic bone necrosis) is a disease that results from the temporary or permanent loss of blood supply to the bone. When blood supply is cut off, the bone tissue dies and the bone collapses. If avascular necrosis occurs near a joint, collapse of the joint surface may occur.

Avascular necrosis may occur in any bone, but most commonly occurs in the ends of a long bone. It may affect one bone, several bones at one time, or different bones at different times.

Although avascular necrosis may affect both genders and all age groups, it is diagnosed most often in persons in their 30s and 40s.

What causes avascular necrosis?

Avascular necrosis may be the result of the following:

• traumatic causes (including injury, fracture, or damage to blood vessels)

• non-traumatic causes (including long-term use of medications, such as corticosteroids, or excessive, long-term use of alcohol)

Other theories and associations have been suggested as risk factors.

What are the risk factors for avascular necrosis?
Suggested risk factors for avascular necrosis include the following:

• injury
• steroid use
• Gaucher disease
• alcohol use
• blood disorders, such as sickle cell anemia
• radiation treatments
• chemotherapy
• pancreatitis
• decompression disease

What are the symptoms of avascular necrosis?

The following are the most common symptoms for avascular necrosis. However, each individual may experience symptoms differently. Symptoms may include:

• minimal early joint pain
• increased joint pain as bone and joint begin to collapse
• limited range of motion due to pain

The symptoms of avascular necrosis may resemble other medical conditions or bone problems. Always consult your physician for a diagnosis.

How is avascular necrosis diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for avascular necrosis may include the following:

• imaging procedures, such as:

• x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film; to determine bone changes.

• computed tomography scan (Also called a CT or CAT scan.) – a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.

• magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.

• radionuclide bone scan – a nuclear imaging technique that uses a very small amount of radioactive material, which is injected into the patient’s bloodstream to be detected by a scanner. This test shows blood flow to the bone and cell activity within the bone.

• biopsy – a procedure in which tissue samples are removed (with a needle or during surgery) from the body for examination under a microscope; to determine if cancer or other abnormal cells are present; to remove tissue from the affected bone.

• functional evaluation of bone – tests, that usually involve surgery, to measure the pressure inside the bone.

Treatment for Avascular Necrosis

Specific treatment for avascular necrosis will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• location and amount of bone affected
• underlying cause of the disease
• your tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

The goal of treatment for avascular necrosis is to improve functionality or to stop further damage to the affected bone or joint. Treatments are necessary to keep joints from breaking down, and may include:

• medications (to control pain)

• assistive devices (to reduce weight on the bone or joint)

• core decompression – a surgical procedure in which the inner layer of bone is removed to reduce pressure, allow for increased blood flow, and slow or stop bone and/or joint destruction.

• osteotomy – a surgical procedure to reshape the bone and reduce stress on the affected area.

• bone graft – a surgical procedure in which healthy bone is transplanted from another part of the patient’s body into the affected area.

• arthroplasty (total joint replacement) – a surgical procedure to remove and replace an arthritic or damaged joint with an artificial joint (called a prosthesis); may be considered only after other treatment options have failed to provide adequate relief from pain and/or disability.

Other treatments for avascular necrosis may include electrical stimulation and combination therapies to encourage the growth of new bone.

Adapted from:UNIVERSITY OF MARYLAND MEDICAL SYSTEM

Posted on November 17, 2009 | Filed under 9 | Permalink

Fibrous Dysplasia

Fibrous Dysplasia is a chronic disorder in which bone expands due to abnormal development of fibrous tissue, often resulting in one, or more, of the following:

• uneven growth of bones
• pain
• brittle bones
• bone deformity

Any bone can be affected. More than one bone can be affected at any one time, and, when multiple bones are affected, it is not unusual for them to all be on one side of the body. However, fibrous dysplasia does not spread from one bone to another. The most commonly affected bones include the following:

• femur (thighbone)
• tibia (shin bone)
• ribs
• skull
• facial bones
• humerus (the bone of the upper arm)
• pelvis
• vertebrae in the spine (less often)

Some people develop hormonal problems and a condition called McCune-Albright syndrome. McCune-Albright syndrome, another form of fibrous dysplasia, includes different symptoms, such as early onset of puberty and skin spots, called cafe-au-lait spots.

Fibrous dysplasia usually occurs in children and young adults, and is found equally between males and females.

What causes fibrous dysplasia?

The exact cause of fibrous dysplasia is not known, but it is believed to be due to a chemical abnormality in the protein of the bone. It may be an inherited disorder.

What are the symptoms of fibrous dysplasia?

The following are the most common symptoms for fibrous dysplasia. However, each individual may experience symptoms differently. Symptoms may include:

• a waddling walk
• bone pain (as a consequence of the expanding fibrous tissue in the bone)
• bone deformity
• bone fractures
• scoliosis – a lateral, or sideways, curvature and rotation of the back bones (vertebrae), giving the appearance that the person is leaning to one side.

The symptoms of fibrous dysplasia may resemble other bone disorders or medical problems. Always consult your physician for a diagnosis.

How is fibrous dysplasia diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for fibrous dysplasia may include the following:

• x-ray – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.

• biopsy – a procedure in which tissue samples are removed (with a needle or during surgery) from the body for examination under a microscope; to determine if cancer or other abnormal cells are present; to remove tissue from the affected bone.
• computed tomography scan (Also called a CT or CAT scan.) – a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
• blood tests

Treatment for Fibrous Dysplasia

Specific treatment for fibrous dysplasia will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• your tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

Treatment may include:

• surgery, including the following:
  • removal of affected bone, followed by bone grafting (a surgical procedure in which healthy bone is transplanted from another part of the patient’s body into the affected area)
  • removal of bone wedge
  • placement of a rod down the shaft of the bone
• medication
• pain management
• physical therapy

Adapted from: University of Maryland Medical System.

Posted on November 17, 2009 | Filed under 9 | Permalink