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Amyloidosis - The Heart and Vascular Disease PDF Print E-mail
Written by Ruai Pharmaceuticals   
Monday, 15 August 2011 15:25

Amyloidosis

 

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Medical Author: William C. Shiel Jr., MD, FACP, FACR

  • What is amyloidosis?
  • What are symptoms of amyloidosis?
  • How is amyloidosis diagnosed?
  • How is amyloidosis treated?
  • Amyloidosis At A Glance
  • Patient Discussions: Amyloidosis

What is amyloidosis?

Amyloidosis is a group of diseases that result from the abnormal deposition of a particular protein, called amyloid, in various tissues of the body. Amyloid protein can be deposited in a localized area and may not be harmful or only affect a single tissue of the body. This form of amyloidosis is called localized amyloidosis. Amyloidosis that affects tissues throughout the body is referred to as systemic amyloidosis. Systemic amyloidosis can cause serious changes in virtually any organ of the body.

Amyloidosis can occur as its own entity or "secondarily" as a result of another illness, including multiple myeloma, chronic infections (such as tuberculosis or osteomyelitis), or chronic inflammatory diseases (such as rheumatoid arthritis and ankylosing spondylitis). Amyloidosis can also be localized to a specific body area from aging. This localized form of amyloidosis does not have systemic implications for the rest of the body. The protein that deposits in the brain of patients with Alzheimer's disease is a form of amyloid.

Systemic amyloidosis has been classified into three major types that are very different from each other. These are distinguished by a two-letter code that begins with an A (for amyloid). The second letter of the code stands for the protein that accumulates in the tissues in that particular type of amyloidosis. The types of systemic amyloidosis are currently categorized as primary (AL), secondary (AA), and hereditary (ATTR).

In addition, other forms of amyloidosis include beta-2 microglobulin amyloidosis and localized amyloidoses.

Primary amyloidosis

Primary amyloidosis, or AL, occurs when a specialized cell in the bone marrow (plasma cell) spontaneously overproduces a particular protein portion of an antibody called the light chain. (This is why it is coded as AL.) The deposits in the tissues of people with primary amyloidosis are AL proteins. Primary amyloidosis can occur with a bone marrow cancer of plasma cells called multiple myeloma. Primary amyloid is not associated with any other diseases but is a disease entity of its own, conventionally requiring chemotherapy treatment. Researchers at the Mayo Clinic in Rochester, Minnesota, and Boston University in Boston, Massachusetts, have demonstrated benefits from stem-cell transplantation, harvesting patients' own stem cells to treat primary amyloidosis.

Secondary amyloidosis

When amyloidosis occurs "secondarily" as a result of another illness, such as multiple myeloma, chronic infections (for example, tuberculosis or osteomyelitis), or chronic inflammatory diseases (for example, rheumatoid arthritis and ankylosing spondylitis), the condition is referred to as secondary amyloidosis or AA. The amyloid tissue deposits in secondary amyloidosis are AA proteins. The treatment of patients' secondary amyloidosis is directed at treating the underlying illness in that particular patient.

Familial amyloidosis

Familial amyloidosis, or ATTR, is a rare form of inherited amyloidosis. The amyloid deposits in familial amyloidosis are composed of the protein transthyretin, or TTR, which is made in the liver. Familial amyloidosis is an inherited autosomal dominant in genetics terminology. This means that for the offspring of a person with the condition, there is a 50% chance of inheriting it.

Beta-2 microglobulin amyloidosis

Beta-2 microglobulin amyloidosis occurs when amyloid deposits develop in patients on dialysis with longstanding kidney failure. The amyloid deposits are composed of beta-2 microglobulin protein and are often found around joints.

Localized amyloidoses

The many forms of localized amyloidosis are a result of amyloid deposits in specific areas of the body and are distinct from systemic forms of amyloidosis that deposit amyloid throughout the body. Localized amyloid deposits occur in the brain from Alzheimer's disease. In various tissues, often with aging, amyloid can be locally produced and deposited to cause tissue injury.

What are symptoms of amyloidosis?

Symptoms in patients with amyloidosis result from abnormal functioning of the particular organs involved. The heart, kidneys, liver, bowels, skin, nerves, joints, and lungs can be affected. As a result, symptoms are vague and can include fatigue, shortness of breath, weight loss, lack of appetite, numbness, tingling, weakness, enlarged tongue, and swelling. Amyloidosis affecting the kidney leads to "nephrotic syndrome," which is characterized by severe loss of protein in the urine and swelling of the extremities.

How is amyloidosis diagnosed?

The diagnosis of amyloidosis is made by detecting the characteristic amyloid protein in a biopsy specimen of involved tissue (such as mouth, rectum, fat, kidney, heart, or liver). A needle aspiration biopsy of fat just under the skin of the belly (fat pad aspiration), originally developed at Boston University, offers a simple and less invasive method to diagnose systemic amyloidosis. Pathologists can see the protein in the biopsy specimen when it is coated with a special dye, called Congo red stain.

How is amyloidosis treated?

Initial treatment of amyloidosis involves correcting organ failure and treating any underlying illness (such as myeloma, infection, or inflammation). The disease is frequently discovered after significant organ damage has already occurred. Therefore, stabilization of organ function is an initial target of treatment. The most frequent cause of death in systemic amyloidosis is kidney failure.

Sephardic Jews and Turks inherit a genetic disease called Familial Mediterranean Fever, which is associated with amyloidosis and characterized by episodes of "attacks" of fever, joint, and abdominal pains. These attacks can be prevented with the medication colchicine. Armenians and Ashkenazi Jews also have a higher incidence of Familial Mediterranean Fever attacks but do not suffer amyloid deposition disease. Other reports of amyloidosis in families are extremely rare.

Researchers are currently enrolling patients with primary amyloidosis in clinical trials using a cancer chemotherapy medication (melphalan [Alkeran]), in conjunction with bone-marrow stem-cells transplantation. The results have been promising, and this combination treatment is offered to eradicate the amyloidosis in selected patients, provided that the underlying medical condition of the patient is adequate. These aggressive treatment options with stem-cell transplantation and high doses of chemotherapy are a true breakthrough in the treatment of these patients.

Familial amyloidosis can now be cured with liver transplantation. This option requires an accurate diagnosis of the specific protein that causes the disease.

 
Amyloidosis At A Glance
  • Amyloidosis is a disorder resulting from abnormal protein (amyloid) deposits in body tissues.
  • Amyloidosis can occur as an isolated disease or as a result of another illness.
  • Symptoms in patients with amyloidosis result from abnormal functioning of the particular organs involved.
  • Diagnosis of amyloidosis is made with a biopsy of involved tissue.
  • Treatment options for amyloidosis depends on the type of amyloidosis and involves correcting organ failure and treating any underlying conditions.


Antinuclear Antibody Test
(ANA)

Medical Author: William C. Shiel Jr., MD, FACP, FACR

  • What are antinuclear antibodies?
  • How is the ANA test designed? What is it for?
  • What are autoimmune diseases?
  • What other conditions cause ANAs to be produced?
  • Can medications cause ANAs to be produced?
  • ANAs are defined as having patterns. What does this mean?
  • Are ANAs always associated with illness?
  • Patient Discussions: Antinuclear Antibody Test

What are antinuclear antibodies?

We normally have antibodies in our blood that repel invaders into our body, such as virus and bacteria microbes. Antinuclear antibodies (ANAs) are unusual antibodies, detectable in the blood, that have the capability of binding to certain structures within the nucleus of the cells. The nucleus is the innermost core within the body's cells and contains the DNA, the primary genetic material. ANAs are found in patients whose immune system may be predisposed to cause inflammation against their own body tissues. Antibodies that are directed against one's own tissues are referred to as auto-antibodies. The propensity for the immune system to work against its own body is referred to as autoimmunity. ANAs indicate the possible presence of autoimmunity and provide, therefore, an indication for doctors to consider the possibility of autoimmune illness.

How is the ANA test designed? What is it for?

The ANA test was designed by Dr. George Friou in 1957. The ANA test is performed using a blood sample. The antibodies in the serum of the blood are exposed in the laboratory to cells. It is then determined whether or not antibodies are present that react to various parts of the nucleus of cells. Thus, the term anti-"nuclear" antibody. Fluorescence techniques are frequently used to actually detect the antibodies in the cells, thus ANA testing is sometimes referred to as fluorescent antinuclear antibody test (FANA). The ANA test is a sensitive screening test used to detect autoimmune diseases.

What are autoimmune diseases?

Autoimmune diseases are conditions in which there is a disorder of the immune system characterized by the abnormal production of antibodies (auto-antibodies) directed against the tissues of the body. Autoimmune diseases typically feature inflammation of various tissues of the body. ANAs are found in patients with a number of different autoimmune diseases, such as systemic lupus erythematosus, Sjogren's syndrome, rheumatoid arthritis, polymyositis, scleroderma, Hashimoto's thyroiditis, juvenile diabetes mellitus, Addison disease, vitiligo, pernicious anemia, glomerulonephritis, and pulmonary fibrosis. ANAs can also be found in patients with conditions that are not considered classic autoimmune diseases, such as chronic infections and cancer.

What other conditions cause ANAs to be produced?

ANAs can be produced in patients with infections (virus or bacteria), lung diseases (primary pulmonary fibrosis, pulmonary hypertension), gastrointestinal diseases (ulcerative colitis, Crohn's disease, primary biliary cirrhosis, alcoholic liver disease), hormonal diseases (Hashimoto's autoimmune thyroiditis, Grave's disease), blood diseases (idiopathic thrombocytopenic purpura, hemolytic anemia), cancers (melanoma, breast, lung, kidney, ovarian and others), skin diseases (psoriasis, pemphigus), as well as in the elderly and those people with a family history of rheumatic diseases.

Can medications cause ANAs to be produced?

Many medications can sometimes stimulate the production of ANAs, including procainamide (Procan SR), hydralazine, and dilantin. ANAs that are stimulated by medication are referred to as drug-induced ANAs. This does not necessary mean that any disease is present when these ANAs are "induced." Sometimes diseases are associated with these ANAs, and they are referred to as drug-induced diseases.

 

ANAs are defined in certain patterns. What does this mean?

ANAs present different "patterns" depending on the staining of the cell nucleus in the laboratory: homogeneous or diffuse; speckled; nucleolar; and peripheral or rim. While these patterns are not specific for any one illness, certain illnesses can more frequently be associated with one pattern or another. The patterns then can sometimes give the doctor further clues as to types of illnesses to look for in evaluating a patient. For example, the nucleolar pattern is more commonly seen in the disease scleroderma. The speckled pattern is seen in many conditions and in people who do not have any autoimmune disease.

Are ANAs always associated with illness?

No. ANAs can be found in approximately 5% of the normal population, usually in low titers (low levels). These people usually have no disease. Titers of lower than 1:80 are less likely to be significant. (ANA titers of less than or equal to 1:40 are considered negative.) Even higher titers are often insignificant in patients over 60 years of age. Ultimately, the ANA result must be interpreted in the specific context of an individual patient's symptoms and other test results. It may or may not be significant in a given individual.


Anabolic Steroid Abuse

 

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Medical Author: Benjamin C. Wedro, MD, FACEP, FAAEM
Medical Editor: William C. Shiel Jr., MD, FACP, FACR

  • What are anabolic steroids?
  • Why do people abuse anabolic steroids?
  • How do people abuse anabolic steroids?
  • Are anabolic steroids addictive?
  • What are the psychological and physical side effects of anabolic steroid abuse?
  • How are anabolic steroid abuse and addiction diagnosed?
  • What is the treatment for anabolic steroid abuse and addiction?
  • Can anabolic steroid abuse and addiction be prevented?
  • Where can I get more information on anabolic steroid abuse?
  • Anabolic Steroid Abuse At A Glance

What are anabolic steroids?

Performance-enhancing drugs are no longer just for bodybuilders or pro athletes who are willing to try illegal and potentially dangerous means to improve their body's function. These drugs are being used every day by people of all ages, from middle-school, high-school, and college students to older recreational athletes.

Anabolic steroids are one type of performance-enhancing drug. They mimic testosterone in the body to enhance performance by making muscle cells larger and by allowing the body to recover more quickly from the stress of exercise. Slang for anabolic steroids is "roids."

There are two types of steroids that are naturally produced in the body:

Catabolic steroids or glucocorticoids are part of the body's response to stress. The word catabolic comes from the Greek word meaning to "throw down," and these steroids help break down large chemicals into smaller ones. For example, cortisol helps glycogen, a large molecule that is stored in the liver, metabolize into glucose, a small molecule that can be used for energy by the body. Cortisol is manufactured in the adrenal glands and is required for many of the basic functions of the body, including glucose metabolism, the immune response to infection, and protein, fat, and carbohydrate metabolism. Prednisone, hydrocortisone, and dexamethasone are examples of steroids that are used to treat a range of illnesses from asthma and COPD to rheumatoid arthritis and allergic reactions.

Anabolic androgenic steroids are steroids that mimic testosterone in the body. Anabolic refers to the properties of these drugs to increase production of proteins that act as building blocks for muscle cells, bone, and other tissues within the body. The androgenic response (andro=male + genic=formation) describes the increased male features (secondary sexual characteristics) that occur as a result of androgenic steroids, including facial, body, and pubic hair, deepened voice, and increased sex drive or libido. The few medical indications for prescribing these medications are due to a lack of testosterone production in the body, either because of inborn errors of metabolism or because of illnesses that decrease production. Some physicians believe that decreased testosterone that occurs normally with aging is an indication for replacement therapy with anabolic steroids, but their use in otherwise healthy older patients is still controversial because of the potential serious side effects.

There are many anabolic steroids that have been manufactured over the past decades. The goal of the manufacturing chemists is to promote the anabolic effect of the drug while decreasing the androgenic effects that are most often associated with potential side effects that can be life-threatening. Examples of anabolic steroids include the following:

  • Testosterone
  • Androstenedione
  • Stanozolol
  • Durabolin
  • Dianabol
  • Nandrolone

Why do people abuse anabolic steroids?

People abuse steroids for a variety of reasons, but most do it to increase body performance and appearance. There may be peer pressure to use anabolic steroids in some sports because of a fear that normal training will not be enough to succeed as well as the perception that everybody else is using them and gaining an unfair advantage.

People who use steroids to enhance their appearance by increasing muscle and decreasing fat may suffer from muscle dysmorphia or abnormal perception of their own body. Males may think that they are perpetually too small and weak, and females may think themselves fat, even though their perception may not be actually true.

Body builders and weight lifters are not the only athletes to think that anabolic steroid use is their road to success. Athletes, from strength sports like football and throwing the discus to speed sports like track sprinters and speed skaters, have attempted to use steroids to enhance performance and increase the efficiency of their training.

How do people abuse anabolic steroids?

Though most anabolic steroids need to be injected into the body to be effective, some may be taken by orally and others used as a cream or gel and applied to the skin. The user will try to take enough anabolic steroid to increase the ability to exercise and allow muscles to grow while minimizing the risk of side effects and the potential of being caught. Usually steroids are taken in cycles with regular injections followed by periods of rest. Numerous books and web sites discuss the benefits and risks of different techniques to maximize the effect of a variety of steroids on the body.

Cycling, stacking, and pyramiding are three common ways that anabolic steroid abusers take their drugs. Cycling refers to taking a steroid for a period of time, stopping for the body to rest, and then restarting again. If more than one type of steroid is used at a time, this is called stacking. There is the belief that using two or more steroids at a time increases the effectiveness of each. Pyramiding combines the cycling and stacking. One or more steroids are begun in a low dose and the dose gradually increased until halfway through the cycle where the amount is maximized and it is then tapered to zero by the end of the cycle.

Exercise programs are tailored so that more can be done as the effect of the steroid increases. The cycles of steroid use are usually six to 12 weeks long and may be followed by a rest period.

In athletes who use anabolic steroids, a cat and mouse game exists. They try to time their steroid injections, so that the drug is out of their system if and when they are drug tested. Sometimes masking drugs are also taken to try to "beat" the test by making the test negative. Designer steroids are being continually developed to again prevent their use from being detected.

Are anabolic steroids addictive?

The common signs of addiction include cravings for the drug, requiring more drug to get the same effect, and withdrawal symptoms should the drug be stopped. These are all behaviors that can apply to anabolic steroids. The following is a statement from the National Institute of Drug Abuse concerning anabolic steroids:

"An undetermined percentage of steroid abusers may become addicted to the drugs, as evidenced by their continued abuse despite physical problems and negative effects on social relations. Also, steroid abusers typically spend large amounts of time and money obtaining the drugs, which is another indication that they may be addicted. Individuals who abuse steroids can experience withdrawal symptoms when they stop taking steroids, such as mood swings, fatigue, restlessness, loss of appetite, insomnia, reduced sex drive, and steroid cravings. The most dangerous of the withdrawal symptoms is depression, because it sometimes leads to suicide attempts. If left untreated, some depressive symptoms associated with anabolic steroid withdrawal have been known to persist for a year or more after the abuser stops taking the drugs."

What are the psychological and physical side effects of anabolic steroid abuse?

Anabolic steroids are used as performance-enhancing drugs to increase the ability to do work and exercise by abnormally stimulating muscle growth, power, and aerobic capacity. This increased function comes with a cost of potentially life-threatening side effects.

The complications of anabolic steroid abuse are a result of excess testosterone affecting almost all the organ systems in the body. Some of the effects are reversible and decrease when the drug use stops while others are permanent and irreversible.

In males, the excess steroid suppresses the normal testosterone production in the body and can lead to shrunken testicles and decreased sperm count, baldness, and breast development (gynecomastia).

In females, steroids will lead to masculinization with loss of body fat and breast size, swelling of the clitoris, deepening of the voice, and the development of facial and body hair.

Life-threatening side effects include heart attack and stroke, the risk of forming blood clots (deep vein thrombosis and pulmonary embolus), liver cancer, and liver failure.

The skin is often affected by excess steroid use and the issues are similar to the adolescent male going through puberty with its testosterone spike. Acne is often present along with cyst formation. Hair can also become oily.

Infections are a common side effect of steroid abuse because of needle sharing and unsanitary techniques used when injecting the drugs into the skin. These are similar risks to IV drug abusers with a risk for blood-borne infections such as hepatitis and HIV/AIDS. Skin abscesses may occur at injection sites and may spread to other organs of the body. Endocarditis or an infection of the heart valves is not uncommon.

Psychiatric and psychologic complications include manic behavior and psychosis including hallucinations and delusions. Aggressive behavior is common and is often known as "roid rage"

Because the muscle growth can occur quickly, it can cause stress on the tendons that attach the muscle to bone and anabolic steroid abusers are at risk for tendon rupture.

Anabolic steroids can increase bone production, especially in the skull and face. Teeth can splay apart as the maxilla and mandible grow and there can be overgrowth of the forehead giving an "Incredible Hulk" appearance. If adolescent teenagers abuse steroids before they have finished growing, these drugs can prematurely close bone growth plates leading to short stature.

How are anabolic steroid abuse and addiction diagnosed?

The diagnosis of anabolic steroid abuse in high school, college, and professional athletes may occur when they fail a drug test, but many people who abuse these drugs are never randomly tested. The diagnosis is often made when they present with one of the side effects of steroid use.

Once the potential diagnosis of drug abuse is considered, it is important that the care provide opportunity for the patient to consider drug treatment options, just like any other addictive drug. However, the first step in diagnosis and treatment must be taken by the patient to admit there is a potential for abuse and their willingness to consider intervention and treatment.

What is the treatment for anabolic steroid abuse and addiction?

Counseling is the mainstay of therapy for anabolic steroid abuse. The patient and their support group, family and friends, need to appreciate that the approach to this addiction may be similar to addiction to other drugs and alcohol.

Depression and suicidal thoughts may occur when the steroids are stopped, and this potential must be monitored closely.

Withdrawal symptoms vary with each patient and the health-care practitioner may need to prescribe short courses of medications to help with headaches, muscle aches, and insomnia.

Can anabolic steroid abuse and addiction be prevented?

Prevention of steroid abuse begins at a young age. There is pressure even at middle school to take drugs to increase performance on the playing field and in the gym. As well, personal appearance and perception begin early on. Unrealistic expectations can drive adolescent boys and girls to look like models on fashion magazines and athletes in the gym.

Counseling and guidance that continues through high school and beyond has been shown to be effective in decreasing steroid use in the younger population.

Where can I get more information on anabolic steroid abuse?

While there are many web sites that teach people how to abuse anabolic steroids without getting caught, there are also many that can educate about their benefits, risks  and dangers. Examples include the following:

"Anabolic Steroid Abuse," National Institute on Drug Abuse
http://www.steroidabuse.gov/

"NIDA InfoFacts: Steroids (Anabolic-Androgenic)," National Institute on Drug Abuse
http://www.drugabuse.gov/infofacts/
steroids.html

"Research Report Series - Anabolic Steroid Abuse," National Institute on Drug Abuse
http://www.nida.nih.gov/ResearchReports/
Steroids/anabolicsteroids5.html#addictive

 

Anabolic Steroid Abuse At A Glance
  • Anabolic steroids are used illegally to increase muscle, decrease fat, and enhance athletic performance and body appearance.
  • Anabolic steroids act by increasing the androgenic testosterone effects within the body.
  • Anabolic steroids can lead to potentially fatal side effects.
  • Anabolic steroids may be addictive, and users may go through withdrawal.
  • Prevention requires community education, as well as reviewing why adolescents may want to start using the drugs and understanding the risks involved


Anal Cancer

 

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  • What is anal cancer?
  • What are the risk factors for anal cancer?
  • What are the signs and symptoms of anal cancer?
  • How is anal cancer diagnosed?
  • What affects the prognosis?
  • How is the staging of anal cancer determined?
  • What is recurrent anal cancer?
  • What is the treatment for anal cancer?
  • How does staging affect the treatment of anal cancer?
  • What are the treatment options for recurrent anal cancer?
  • Where can I find more information about anal cancer?
  • Find a local Oncologist in your town

General Information About Anal Cancer

Anal cancer is a disease in which malignant (cancer) cells form in the tissues of the anus.

The anus is the end of the large intestine, below the rectum, through which stool (solid waste) leaves the body. The anus is formed partly from the outer skin layers of the body and partly from the intestine. Two ring-like muscles, called sphincter muscles, open and close the anal opening to let stool pass out of the body. The anal canal, the part of the anus between the rectum and the anal opening, is about 1½ inches long.

The skin around the outside of the anus is called the perianal area. Tumors in this area are skin tumors, not anal cancer.

 

Being infected with the human papillomavirus (HPV) can affect the risk of developing anal cancer.

Risk factors include the following:

  • Being over 50 years old.
  • Being infected with human papillomavirus (HPV).
  • Having many sexual partners.
  • Having receptive anal intercourse (anal sex).
  • Frequent anal redness, swelling, and soreness.
  • Having anal fistulas (abnormal openings).
  • Smoking cigarettes.
Picture of the anatomy of the lower digestive system, showing the colon and other organs.
Anatomy of the lower digestive system, showing the colon and other organs.

Possible signs of anal cancer include bleeding from the anus or rectum or a lump near the anus.

These and other symptoms may be caused by anal cancer. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Bleeding from the anus or rectum.
  • Pain or pressure in the area around the anus.
  • Itching or discharge from the anus.
  • A lump near the anus.
  • A change in bowel habits.

Tests that examine the rectum and anus are used to detect (find) and diagnose anal cancer.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
  • Digital rectal examination (DRE): An exam of the anus and rectum. The doctor or nurse inserts a lubricated, gloved finger into the lower part of the rectum to feel for lumps or anything else that seems unusual.
  • Anoscopy: An exam of the anus and lower rectum using a short, lighted tube called an anoscope.
  • Proctoscopy: An exam of the rectum using a short, lighted tube called a proctoscope.
  • Endo-anal or endorectal ultrasound: A procedure in which an ultrasound transducer (probe) is inserted into the anus or rectum and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. If an abnormal area is seen during the anoscopy, a biopsy may be done at that time.

Certain factors affect the prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) depends on the following:

  • The size of the tumor.
  • Where the tumor is in the anus.
  • Whether the cancer has spread to the lymph nodes.

The treatment options depend of the following:

  • The stage of the cancer.
  • Where the tumor is in the anus.
  • Whether the patient has human immunodeficiency virus (HIV).
  • Whether cancer remains after initial treatment or has recurred.

Stages of Anal Cancer

After anal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the anus or to other parts of the body.

The process used to find out if cancer has spread within the anus or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests may be used in the staging process:

  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. For anal cancer, a CT scan of the pelvis and abdomen may be done.
  • Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
  • Endo-anal or endorectal ultrasound: A procedure in which an ultrasound transducer (probe) is inserted into the anus or rectum and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

 

The following stages are used for anal cancer:

Stage 0 (Carcinoma in Situ)

In stage 0, cancer is found only in the innermost lining of the anus. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 cancer is also called carcinoma in situ.

Pea, peanut, walnut, and lime show tumor sizes.
Pea, peanut, walnut, and lime show tumor sizes.

 

Stage I

In stage I, cancer has formed and the tumor is 2 centimeters or smaller.

Stage II

In stage II, the tumor is larger than 2 centimeters.

Stage IIIA

In stage IIIA, the tumor may be any size and has spread to either:

  • lymph nodes near the rectum; or
  • nearby organs, such as the vagina, urethra, and bladder.

Stage IIIB

In stage IIIB, the tumor may be any size and has spread:

  • to nearby organs and to lymph nodes near the rectum; or
  • to lymph nodes on one side of the pelvis and/or groin, and may have spread to nearby organs; or
  • to lymph nodes near the rectum and in the groin, and/or to lymph nodes on both sides of the pelvis and/or groin, and may have spread to nearby organs.

Stage IV

In stage IV, the tumor may be any size and cancer may have spread to lymph nodes or nearby organs and has spread to distant parts of the body.

Recurrent Anal Cancer

Recurrent anal cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the anus or in other parts of the body.

Treatment Option Overview

There are different types of treatment for patients with anal cancer.

Different types of treatments are available for patients with anal cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Three types of standard treatment are used:

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

 

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Surgery

  • Local resection: A surgical procedure in which the tumor is cut from the anus along with some of the healthy tissue around it. Local resection may be used if the cancer is small and has not spread. This procedure may save the sphincter muscles so the patient can still control bowel movements. Tumors that develop in the lower part of the anus can often be removed with local resection.
  • Abdominoperineal resection: A surgical procedure in which the anus, the rectum, and part of the sigmoid colon are removed through an incision made in the abdomen. The doctor sews the end of the intestine to an opening, called a stoma, made in the surface of the abdomen so body waste can be collected in a disposable bag outside of the body. This is called a (colostomy. Lymph nodes that contain cancer may also be removed during this operation.
Anal cancer surgery with colostomy. The anus, rectum, and part of the colon are removed, a stoma is created, and a colostomy bag is attached to the stoma.
Anal cancer surgery with colostomy. The anus, rectum, and part of the colon are removed, a stoma is created, and a colostomy bag is attached to the stoma.

 

Having the human immunodeficiency virus can affect treatment of anal cancer.

Cancer therapy can further damage the already weakened immune systems of patients who have the human immunodeficiency virus (HIV). For this reason, patients who have anal cancer and HIV are usually treated with lower doses of anticancer drugs and radiation than patients who do not have HIV.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.

 

Radiosensitizers

Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment Options by Stage

Stage 0 Anal Cancer (Carcinoma in Situ)

Treatment of stage 0 anal cancer is usually local resection.

Stage I Anal Cancer

Treatment of stage I anal cancer may include the following:

  • Local resection.
  • External-beam radiation therapy with or without chemotherapy. If cancer remains after treatment, additional chemotherapy and radiation therapy may be given to avoid the need for a permanent colostomy.
  • Internal radiation therapy.
  • Abdominoperineal resection, if cancer remains or comes back after treatment with radiation therapy and chemotherapy.
  • Internal radiation therapy for cancer that remains after treatment with external-beam radiation therapy.

Patients who have had treatment that saves the sphincter muscles may receive follow-up exams every 3 months for the first 2 years, including rectal exams with endoscopy and biopsy, as needed.

Stage II Anal Cancer

Treatment of stage II anal cancer may include the following:

  • Local resection.
  • External-beam radiation therapy with chemotherapy. If cancer remains after treatment, additional chemotherapy and radiation therapy may be given to avoid the need for a permanent colostomy.
  • Internal radiation therapy.
  • Abdominoperineal resection, if cancer remains or comes back after treatment with radiation therapy and chemotherapy.
  • A clinical trial of new treatment options.

Patients who have had treatment that saves the sphincter muscles may receive follow-up exams every 3 months for the first 2 years, including rectal exams with endoscopy and biopsy, as needed.

Stage IIIA Anal Cancer

Treatment of stage IIIA anal cancer may include the following:

  • External-beam radiation therapy with chemotherapy. If cancer remains after treatment, additional chemotherapy and radiation therapy may be given to avoid the need for a permanent colostomy.
  • Internal beam radiation.
  • Abdominoperineal resection, if cancer remains or comes back after treatment with chemotherapy and radiation therapy.
  • A clinical trial of new treatment options.

Stage IIIB Anal Cancer

Treatment of stage IIIB anal cancer may include the following:

  • External-beam radiation therapy with chemotherapy.
  • Local resection or abdominoperineal resection, if cancer remains or comes back after treatment with chemotherapy and radiation therapy. Lymph nodes may also be removed.
  • A clinical trial of new treatment options.

Stage IV Anal Cancer

Treatment of stage IV anal cancer may include the following:

  • Surgery as palliative therapy to relieve symptoms and improve the quality of life.
  • Radiation therapy as palliative therapy.
  • Chemotherapy with radiation therapy as palliative therapy.
  • A clinical trial of new treatment options.

Treatment Options for Recurrent Anal Cancer

Treatment of recurrent anal cancer may include the following:

  • Radiation therapy and chemotherapy, for recurrence after surgery.
  • Surgery, for recurrence after radiation therapy and/or chemotherapy.
  • A clinical trial of radiation therapy with chemotherapy and/or radiosensitizers.

Get More Information From NCI

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There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.


Anal Fissure

 

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Medical Author: Thomas Sokol, MD, FACS, FASCRS
Medical Editor: Jay W. Marks, MD

  • What are anal fissures?
  • What causes anal fissures?
  • What are the symptoms of anal fissures?
  • How are anal fissures diagnosed and evaluated?
  • How are anal fissures treated?
  • Anal Fissures At A Glance
  • Patient Discussions: Anal Fissure - Diagnosis
  • Patient Discussions: Anal Fissure - Effective Treatments
  • Find a local Gastroenterologist in your town

What are anal fissures?

An anal fissure is a cut or tear occuring in the anus (the opening through which stool passes out of the body) that extends upwards into the anal canal. Fissures are a common condition of the anus and anal canal and are responsible for 6-15% of the visits to a colonic and rectal (colorectal) surgeon. They affect men and women equally and both the young and the old. Fissures usually cause pain during bowel movements that often is severe. Anal fissure is the most common cause of rectal bleeding in infancy.

Anal fissures occur in the specialized tissue that lines the anus and anal canal, called anoderm. At a line just inside the anus--referred to as the anal verge or intersphincteric groove--the skin (dermis) of the inner buttocks changes to anoderm. Unlike skin, anoderm has no hairs, sweat glands, or sebaceous (oil) glands and contains a larger number of somatic sensory nerves that sense light touch and pain. (The abundance of nerves explains why anal fissures are so painful.) The hairless, gland-less, extremely sensitive anoderm continues for the entire length of the anal canal until it meets the demarcating line for the rectum, called the dentate line. (The rectum is the distal 15 cm of the colon that lies just above the anus and rectum and just below the sigmoid colon.)

What causes anal fissures?

Anal fissures are caused by trauma to the anus and anal canal. The cause of the trauma usually is a bowel movement, and many patients can remember the exact bowel movement during which their pain began. The fissure may be caused by a hard stool or repeated episodes of diarrhea. Occasionally, the insertion of a rectal thermometer, enema tip, endoscope, or ultrasound probe (for examining the prostate gland) can result in sufficient trauma to produce a fissure. During childbirth, trauma to the perineum (the skin between the posterior vagina and the anus) may cause a tear that extends into the anoderm.

The most common location for an anal fissure in both men and women (90% of all fissures) is the midline posteriorly in the anal canal, the part of the anus nearest the spine. Fissures are more common posteriorly because of the configuration of the muscle that surrounds the anus. This muscle complex, referred to as the external and internal anal sphincters, underlies and supports the anal canal. The sphincters are oval-shaped and are best supported at their sides and weakest posteriorly. When tears occur in the anoderm, therefore, they are more likely to be posterior. In women, there also is weak support for the anterior anal canal due to the presence of the vagina anterior to the anus. For this reason, 10% of fissures in women are anterior, while only 1% are anterior in men. At the lower end of fissures a tag of skin may form, called a sentinal pile.

When fissures occur in locations other than the midline posteriorly or anteriorly, they should raise the suspicion that a problem other than trauma is the cause. Other causes of fissures are anal cancer, Crohn's disease, leukemia as well as many infectious diseases including tuberculosis, viral infections (cytomegalovirus or herpes), syphilis, gonorrhea, chlamydia , chancroid (Hemophilus ducreyi), and human immunodeficiency virus (HIV). Among patients with Crohn's disease, 4% will have an anal fissure as the first manifestation of their Crohn's disease, and half of all patients with Crohn's disease eventually will develop an anal ulceration that may look like a fissure.

Studies of the anal canal in patients with anal fissures consistently show that the muscles surrounding the anal canal are contracting too strongly (they are in spasm), thereby generating a pressure in the canal that is abnormally high. The two muscles that surround the anal canal are the external anal sphincter and the internal anal sphincter (already discussed). The external anal sphincter is a voluntary (striated) muscle, that is, it can be controlled consciously. Thus, when we need to have a bowel movement we can either tighten the external sphincter and prevent the bowel movement, or we can relax it and allow the bowel movement. On the other hand, the internal anal sphincter is an involuntary (smooth) muscle, that is, a muscle we cannot control. The internal sphincter is constantly contracted and normally prevents small amounts of stool from leaking from the rectum. When a substantial load of stool reaches the rectum, as it does just prior to a bowel movement, the internal anal sphincter relaxes automatically to let the stool pass (that is, unless the external anal sphincter is consciously tightened).

When an anal fissure is present, the internal anal sphincter is in spasm. In addition, after the sphincter finally does relax to allow a bowel movement to pass, instead of going back to its resting level of contraction and pressure, the internal anal sphincter contracts even more vigorously for a few seconds before it goes back to its elevated resting level of contraction. It is thought that the high resting pressure and the "overshoot" contraction of the internal anal sphincter following a bowel movement pull the edges of the fissure apart and prevent the fissure from healing.

The supply of blood to the anus and anal canal also may play a role in the poor healing of anal fissures. Anatomic and microscopic studies of the anal canal on cadavers found that in 85% of individuals that the posterior part of the anal canal (where most fissures occur) has less blood flowing to it than the other parts of the anal canal. Moreover, ultrasound studies that measure the flow of blood showed that the posterior anal canal had less than half of the blood flow of other parts of the canal. This relatively poor flow of blood may be a factor in preventing fissures from healing. It also is possible that the increased pressure in the anal canal due to spasm of the internal anal sphincter may compress the blood vessels of the anal canal and further reduce the flow of blood.

What are the symptoms of anal fissures?

Patients with anal fissures almost always experience anal pain that worsens with bowel movements. The pain following a bowel movement may be brief or long lasting; however, the pain usually subsides between bowel movements. The pain can be so severe that patients are unwilling to have a bowel movement, resulting in constipation and even fecal impaction. Moreover, constipation can result in the passage of a larger, harder stool that causes further trauma and makes the fissure worse. The pain also can affect urination by causing discomfort when urinating (dysuria), frequent urination, or the inability to urinate. Bleeding in small amounts, itching (pruritus ani), and a malodorous discharge may occur due to the discharge of pus from the fissure. As previously mentioned, anal fissures commonly bleed in infants.

How are anal fissures diagnosed and evaluated?

A careful history usually suggests that an anal fissure is present, and gentle inspection of the anus can confirm the presence of a fissure. If gentle eversion (pulling apart) the edges of the anus by separating the buttocks does not reveal a fissure, a more vigorous examination following the application of an anesthetic to the anus and anal canal may be necessary. An cotton-tipped swab may be inserted into the anus to gently localize the source of the pain.

An acute anal fissure looks like a linear tear. A chronic anal fissure frequently is associated with a triad of findings that includes a tag of skin at the edge of the anus (sentinel pile), thickened edges of the fissure with muscle fibers of the internal sphincter visible at the base of the fissure, and an enlarged anal papilla at the upper end of the fissure in the anal canal.

If rectal bleeding is present, an endoscopic evaluation using a rigid or flexible viewing tube is necessary to exclude the possibility of a more serious disease of the anus and rectum. A sigmoidoscopy that examines only the distal part of the colon may be reasonable in patients younger than 50 years of age who have a typical anal fissure. In patients with a family history of colon cancer or age greater than 50 (and, therefore, at higher risk for colon cancer), a colonoscopy that examines the entire colon is recommended. Atypical fissures that suggest the presence of other diseases, as discussed previously, require other diagnostic studies including colonoscopy and upper gastrointestinal (UGI) and small bowel x-rays.

How are anal fissures treated?

Thegoal of treatment for anal fissures is to break the cycle of spasm of the anal sphincter and its repeated tearing of the anoderm.

General treatment. In acute fissures, medical (nonoperative) therapy is successful in the majority of patients. Of acute fissures, 80-90% will heal with conservative measures as compared with chronic (recurrent) fissures, which show only a 40% rate of healing. Initial treatment involves adding bulk to the stool and softening the stool with psyllium or methylcellulose preparations and a high fiber diet. Additionally, patients are advised to avoid "sharp" foods that may not be well-digested (i.e., nuts, popcorn, tortilla chips), increase their liquid intake, and, at times, take stool softeners (docusate or mineral oil preparations). Sitz baths (essentially soaking in a tub of warm water) are encouraged, particularly after bowel movements, to relax the spasm, to increase the flow of blood to the anus, and to clean the anus without rubbing the irritated anoderm.

Anesthetics and steroids. Topical anesthetics (e.g., Xylocaine, lidocaine, tetracaine, pramoxine) are recommended especially prior to a bowel movement to reduce the pain of defecation. Often, a small amount of a steroid is combined in the anesthetic cream to reduce inflammation. The use of steroids should be limited to two weeks because longer use will result in thinning of the anoderm (atrophy), which makes it more susceptible to trauma. Oral medications to relax the smooth muscle of the internal sphincter have not been shown to aid healing.

 

Nitroglycerin. Because of the possibility that spasm of the internal sphincter and reduced flow of blood to the sphincter play roles in the formation and healing of anal fissures, ointments with the muscle relaxant, nitroglycerin (glyceryl trinitrate), have been tried and found to be effective in healing anal fissures. Glycerin trinitrate (nitroglycerin) has been shown to cause relaxation of the internal anal sphincter and to decrease the anal resting pressure. When ointments containing nitroglycerin are applied to the anal canal, the nitroglycerin diffuses across the anoderm and relaxes the internal sphincter and reduces the pressure in the anal canal. This relieves spasm of the muscle and also may increase the flow of blood, both of which promote healing of fissures. Unlike Nitropaste, a 2.0% concentration of nitroglycerin that is used on the skin for patients with heart disease and angina, the nitroglycerin ointment used for treating anal fissures contains a concentration of nitroglycerin of only 0.2%. One randomized, controlled trial has demonstrated the healing of anal fissures in 68% of patients with nitroglycerin as compared to 8% of patients treated with placebo (inactive treatment). Other studies have shown a 33-47% recurrence rate of fissures following treatment with nitroglycerin. The presence of a sentinel pile is associated with a lower healing rate with nitroglycerin treatment.

The dose of nitroglycerin often is limited by side effects. The usual side effects are headache (due to dilation of blood vessels in the head) or light-headedness (due to a drop in blood pressure). This author recommends that a small amount of ointment be applied to a cotton-tipped swab with the swab then inserted into the anus only for the depth of the cotton-tipped portion of the swab. Smearing ointment around the outside of the anus does not allow the ointment to reach the anoderm where its effects are important, yet the nitroglycerin will be absorbed and produce side effects.

Nitroglycerin is more rapidly absorbed if blood flow in the anoderm is high. For this reason, it is recommended that nitroglycerin not be applied within 30 minutes of a bath since the warm water of the bath enlarges (dilates) the blood vessels in the skin and anoderm and increases their flow of blood. Additionally, the first application of nitroglycerin should be at bedtime while the patient is lying down in order to prevent falls due to light-headedness.

The side effects of nitroglycerin often are self-limited, that is, they become less with repeated use. Caffeine can help reduce or prevent headaches. However, if side effects are pronounced, nitroglycerin should be discontinued. Drugs for impotence (e.g., sildenafil (Viagra)), should not be used together with nitroglycerin since they increase the risk of developing low blood pressure.

 

Calcium channel blocking drugs. As is the case with nitroglycerin, ointments containing calcium channel blocking drugs (e.g., nifedipine (Adalat) or diltiazem (Cardizem)) relax the muscles of the internal sphincter. They also expand the blood vessels of the anoderm and increase the flow of blood. Nifedipine ointment (2%) is applied in a manner similar to nitroglycerin ointment, but seems to produce fewer side effects. Although healing of chronic fissures has been reported in up to 67% of patients treated with calcium channel blockers, they are most effective with acute fissures.

 

Botulinum toxin. Botulinum toxin (Botox) relaxes (actually paralyzes) muscles by preventing the release of acetylcholine from the nerves that normally causes muscle cells to contract. It has been used successfully to treat a variety of disorders in which there is spasm of muscles, including anal fissures. The toxin is injected into the external sphincter, the internal sphincter, the intersphincteric groove (an indentation just inside the anus that demarcates the dividing line between external and internal sphincters), or into the fissure itself. The dose is not standardized and has varied from 2.5 to 20 units of toxin in two locations (usually on either side of the fissure). The cost of a 100 unit vial of toxin is several hundred dollars and unused toxin cannot be saved. Thus, the expense for a single injection of toxin is high. In some series of patients but not all, the frequency of healing of fissures with botulinum toxin is high. When fissures recur after treatment, they usually heal again with a second injection. One representative study found that fissures healed in 87% of patients by six months after treatment with botulinum toxin. By 12 months, however, the healing rate had fallen to 75% and by 42 months to 60%. The primary side effect of botulinum toxin is weakness of the sphincters with varying degrees of incontinence (leakage of stool) that usually is transient. Other side effects are not common.

 

There is a great variability in the medical literature with respect to the effectiveness of drugs and botulinum toxin in the healing of anal fissures. Healing may be temporary and fissures may return with a hard bowel movement. Recurrent fissures often require a change to another form of treatment. Patients need to balance the effectiveness of treatment, short and long-term side effects, convenience, and expense in choosing their treatment. When patients are intolerant or unresponsive to non-surgical treatments, surgery becomes necessary.

Surgical treatment. The Standard Task Force of the American Society of Colon and Rectal Surgeons has recommended a surgical procedure called partial lateral internal sphincterotomy as the technique of choice for the treatment of anal fissures. In this procedure, the internal anal sphincter is cut starting at its distal most end at the anal verge and extending into the anal canal for a distance equal to that of the fissure. The cut may extend to the dentate line, but not farther. The sphincter can be divided in a closed (percutaneous ) fashion by tunneling under the anoderm or in an open fashion by cutting through the anoderm. The cut is made on the left or right side of the anus, hence the name "partial lateral internal sphincterotomy." The posterior midline, where the fissure usually is located, is avoided for fear of accentuating the posterior weakness of the muscle surrounding the anal canal. (Additional weakness posteriorly can lead to what is called a keyhole deformity, so called because the resulting anal canal resembles an old fashioned skeleton key. This deformity promotes soilage and leakage of stool.)

Although many surgeons decline to cut out the fissure itself during lateral sphincterotomy, this author feels that this reluctance to remove the fissure is not always appropriate, and characteristics of the fissure itself should be taken into account. If the fissure is hard and irregular, suggesting anal cancer, the fissure should be biopsied. If the edges and base of the fissure are heavily scarred, there may be a problem after surgery with anal stenosis, a condition in which additional scarring narrows the anal canal and interferes with the passage of stool. In this case, it may be better to cut out the scarred fissure so that there is a chance for the wound to heal with less scarring and chance of stenosis. Finally, an associated large anal papilla or a large hemorrhoidal tag may interfere physically with wound healing, and removing them may promote healing.

Following surgery, 93-97% of fissures heal. In one representative study, healing following surgery occurred in 98% of patients by two months. At 42 months following surgery, 94% of patients were still healed. Recurrence rates after this type of surgery are low, 0-3%.

Failure to heal following surgery often is attributed to reluctance on the part of the surgeon to adequately divide the internal anal sphincter; however, other reasons for failure to heal, such as Crohn's disease should be considered as well. The risk of incontinence (leakage) of stool following surgery is low. It is important to distinguish between short-term and long-term incontinence. In the short-term (under six weeks), the sphincter is weakened by the surgery, so leakage of stool is not unexpected. Long-term incontinence should not occur after partial lateral internal sphincterotomy because the internal sphincter is less important than the external sphincter (which is not cut) in controlling the passage of stool. It is important to distinguish between incontinence to gas, a minimal amount of stool that, at most, stains the underwear (soiling), and loss of stool that requires an immediate change in underwear. In a large series of patients followed for a mean of five years after surgery, 6% were incontinent of gas, 8% had minor soiling, and 1% experienced loss of stool.

Anal surgical stretch. Several surgeons have described procedures that stretch and tear the anal sphincters for the treatment of anal fissures. Though anal stretching often is successful in alleviating pain and healing the fissure, it is a traumatic, uncontrolled disruption of the sphincter. Ultrasonograms of the anal sphincters following stretching demonstrate trauma that extends beyond the desired area. Because only 72% of fissures heal and there is a 20% incidence of incontinence of stool, stretching has fallen out of favor.

Anal Fissures At A Glance
  • Anal fissures are cracks or tears in the anus and anal canal. They may be acute or chronic.
  • Anal fissures are caused primarily by trauma, but several non-traumatic diseases are associated with anal fissures and should be suspected if fissures occur in unusual locations.
  • The primary symptom of anal fissures is pain during and following bowel movements. Bleeding, itching, and a malodorous discharge also may occur.
  • Anal fissures are diagnosed and evaluated by visual inspection of the anus and anal canal. Endoscopy and, less commonly, gastrointestinal x-rays may be necessary.
  • Anal fissures are initially treated conservatively by adding bulk to the stool, softening the stool, consuming a high fiber diet, avoiding "sharp" or poorly digested foods, and utilizing sitz baths.
  • Ointments containing anesthetics, steroids, nitroglycerin, and calcium channel blocking drugs are used for treating anal fissures that fail to heal with less conservative management.
  • Injections of botulinum toxin may be effective when ointments are not effective. (The cost of treatment would be substantially reduced if the toxin were packaged in smaller doses.)
  • Surgery by lateral sphincterotomy is the gold standard for curing anal fissures. Because of complications, however, it is reserved for patients who are intolerant of non-surgical treatments or in whom non-surgical treatments have proven to be ineffective.


Anal Itching
(Pruritus Ani)

 

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Medical Author: Jay W. Marks, MD
Medical Editor: William C. Shiel, Jr., MD, FACP, FACR

  • What is anal itching?
  • What causes anal itching?
  • How is anal itching treated?
  • Local anesthetics
  • Vasoconstrictors
  • Protectants
  • Astringents
  • Antiseptics
  • Keratolytics
  • Analgesics
  • Corticosteroids
  • What if anal itching persists?

What is anal itching?

Anal itching is the irritation of the skin at the exit of the rectum, known as the anus, accompanied by the desire to scratch. Although itching may be a reaction to chemicals in the stool, it often implies that there is inflammation of the anal area. The intensity of anal itching and the amount of inflammation increases from the direct trauma of scratching and the presence of moisture. At its most intense, anal itching causes intolerable discomfort that often is described as burning and soreness.

What causes anal itching?

  • Anal itching can be caused by irritating chemicals in the foods we eat, such as are found in spices, hot sauces, and peppers.

     

  • Anal itching also can be caused by the irritation of continuous moisture in the anus caused by frequent liquid stools, diarrhea, or escape of small amounts of stool (incontinence). Moisture increases the possibility of infections of the anus, especially yeast, particularly in patients with diabetes mellitus or HIV.
  • Treatment with antibiotics also can lead to a yeast infection and irritation of the anus.

     

  • Psoriasis also can irritate the anus.

     

  • Abnormal passageways (fistulas) from the small intestine or colon to the skin surrounding the anus can form as a result of disease (such as Crohn's disease), and these fistulas bring irritating fluids to the anal area.

Other problems that can cause anal itching include:

  • pinworms,
  • hemorrhoids,
  • tears of the anal skin (fissures), and

     

  • skin tags (abnormal local growth of anal skin).

How is anal itching treated?

Initial treatment of simple itching is directed toward relieving the burning and soreness. It is important to clean and dry the anus thoroughly and avoid leaving soap in the anal area. Cleaning efforts should include gentle showering without direct rubbing or irritation of the skin with either the washcloth or towel. After bowel movements, moist pads (such as baby wipes, flushable moist wipes, and flushable anal cleansing wipes) should be used for cleaning the anus instead of toilet paper. If there is constant moisture present in the anus or stool incontinence, it may be necessary to clean the anus with moist pads between bowel movements.

Many over-the-counter products are sold for the treatment of anal itching. These often contain the same drugs that are used for treating hemorrhoids. Products used for the treatment of anal itching are available as ointments, creams, gels, suppositories, foams, and pads.

Ointments, creams, and gels - when used around the anus - should be applied as a thin covering. When applied to the anal canal, these products should be inserted with a finger or a "pile pipe." Pile pipes are most efficient when they have holes on the sides as well as at the end. Pile pipes should be lubricated with ointment prior to insertion. Suppositories or foams do not have advantages over ointments, creams, and gels.

Most products contain more than one type of active ingredient. Almost all contain a protectant in addition to another ingredient. Only examples of brand-name products containing one ingredient in addition to the protectant are discussed below.

Local anesthetics

Local anesthetics temporarily relieve pain, burning, and itching by numbing the nerve endings. The use of these products should be limited to the perianal area and lower anal canal. Local anesthetics can cause allergic reactions with burning and itching. Therefore, the application of anesthetics should be discontinued if burning and itching increase.

Examples of local anesthetics include:

  • benzocaine 5% to 20% (Americaine Hemorrhoidal, Lanacane Maximum Strength, Medicone)
  • benzyl alcohol (Itch-X) 5% to 20%
  • dibucaine 0.25% to 1.0% (Nupercainal)
  • dyclonine 0.5% to 1.0%
  • lidocaine (Xylocaine) 2% to 5%
  • pramoxine 1.0% (Fleet Pain-Relief, Procto Foam Non-steroid, Tronothane Hydrochloride)
  • tetracaine 0.5% to 5.0%
 

Vasoconstrictors

Vasoconstrictors are chemicals that resemble epinephrine, a naturally occurring chemical. Applied to the anus, vasoconstrictors make the blood vessels become smaller, which may reduce swelling. They also may reduce pain and itching due to their mild anesthetic effect.

Vasoconstrictors applied to the perianal area - unlike vasoconstrictors that are taken orally or by injection - have a low likelihood of causing serious side effects, such as high blood pressure, nervousness, tremor, sleeplessness, and aggravation of diabetes or hyperthyroidism.

Examples of vasoconstrictors include:

  • ephedrine sulfate 0.1% to 1.25%
  • epinephrine 0.005% to 0.01%
  • phenylephrine 0.25% (Medicone Suppository, Preparation H, Rectocaine)

 

Protectants

Protectants prevent irritation of the perianal area by forming a physical barrier on the skin that prevents contact of the irritated skin with aggravating liquid or stool from the rectum. This barrier reduces irritation, itching, pain, and burning. There are many products that are themselves protectants or that contain a protectant in addition to other medications.

Examples of protectants include:

  • aluminum hydroxide gel
  • cocoa butter
  • glycerin
  • kaolin
  • lanolin
  • mineral oil (Balneol)
  • white petrolatum
  • starch
  • zinc oxide (Desitin) or calamine (which contains zinc oxide) in concentrations of up to 25%
  • cod liver oil or shark liver oil if the amount of vitamin A is 10,000 USP units/day.

Astringents

Astringents cause coagulation (clumping) of proteins in the cells of the perianal skin or the lining of the anal canal. This action promotes dryness of the skin, which in turn helps relieve burning, itching, and pain.

Examples of astringents include:

  • calamine 5% to 25%
  • zinc oxide 5% to 25% (Calmol 4, Nupercainal, Tronolane)
  • witch hazel 10% to 50% (Fleet Medicated, Tucks, Witch Hazel Hemorrhoidal Pads)

Antiseptics

Antiseptics inhibit the growth of bacteria and other organisms. However, it is unclear whether antiseptics are any more effective than soap and water.

Examples of antiseptics include:

  • boric acid
  • hydrastis
  • phenol
  • benzalkonium chloride
  • cetylpyridinium chloride
  • benzethonium chloride
  • resorcinol

Keratolytics

Keratolytics are chemicals that cause the outer layers of skin or other tissues to disintegrate. The rationale for their use is that the disintegration allows medications that are applied to the anus and perianal area to penetrate into the deeper tissues.

The two approved keratolytics used are:

  • aluminum chlorhydroxy allantoinate (alcloxa) 0.2% to 2.0%
  • resorcinol 1% to 3%

Analgesics

Analgesic products, like anesthetic products, relieve pain, itching, and burning by depressing receptors on pain nerves.

Examples of analgesics include:

  • menthol 0.1% to 1.0% (greater than 1.0% is not recommended)
  • camphor 0.1% to 3% (greater than 3% is not recommended)
  • juniper tar 1% to 5%

Corticosteroids

Corticosteroids reduce inflammation and can relieve itching, but their chronic use can cause permanent damage to the skin. They should not be used for more than short periods of a few days to two weeks. Only products with weak corticosteroid effects are available over-the-counter. Stronger corticosteroid products that are available by prescription should not be used for treating anal itching.

What if anal itching persists?

For persistent anal itching, efforts are directed toward identifying an underlying cause. An examination by a doctor can rapidly identify most causes of anal itching. Adjustments in diet, treatment of infections, or surgical procedures to correct the underlying cause may be required.


Urinalysis
(Urine Test)

Medical Author: Siamak Nabili, MD, MPH
Medical Editor: William C. Shiel, Jr., MD, FACP, FACR

  • What is a urinalysis?
  • What can urinalysis results show?
  • Who is involved in the interpretation of urinalysis?
  • What does urinalysis involve?
  • What is macroscopic urinalysis?
  • What is urine dipstick chemical analysis?
  • What are the pros and cons of dip sticks?
  • What is microscopic urinalysis?
  • How is microscopic urinalysis done?
  • What kind of cells can be detected?
  • What can the presence of red blood cells in the urine mean?
  • What can the presence of white blood cells in the urine mean?
  • Other than urinalysis, what are other common urine tests available?
  • Patient Discussions: Urinalysis

 

What is a urinalysis?

A urinalysis is simply an analysis of the urine. It is a very common test that can be performed in many healthcare settings including doctors' offices, urgent care facilities, laboratories, and hospitals.

It is performed by collecting a urine sample from the patient in a specimen cup. Usually only small amounts (30-60 ml's) may be required for urinalysis testing. The sample can be either analyzed in the medical clinic or sent to a laboratory to perform the tests. Urinalysis is abbreviated UA.

Urine can be evaluated by its physical appearance (color, cloudiness, odor, clarity), or macroscopic analysis. It can be also analyzed based on its chemical and molecular properties or microscopic assessment.

Urinalysis is ordered by doctors for a number of reasons, as follows:

  • Routine medical evaluation: general yearly screening, assessment before surgery (pre-operative assessment), admission to hospital, screening for kidney disease, diabetes mellitus, hypertension (high blood pressure), liver disease, etc.
  • Assessing particular symptoms: abdominal pain, painful urination, flank pain, fever, blood in the urine, or other urinary symptoms.
  • Diagnosing medical conditions: urinary tract infection, kidney infection, kidney stones, uncontrolled diabetes (high blood sugars), kidney impairment, muscle breakdown (rhabdomyolysis), protein in urine, kidney inflammation (glomerulonephritis).
  • Monitoring disease progression and response to therapy: diabetes related kidney disease, kidney impairment, lupus related kidney disease, blood pressure related kidney disease, kidney infection, protein in urine, blood in urine.

 

What can urinalysis results show?

Urinalysis can disclose evidence of diseases, even some that have not caused significant signs or symptoms. Therefore, a urinalysis is commonly a part of routine health screening.

Urinalysis is also a very useful test that may be ordered by your physician for particular reasons. Urinalysis is commonly used to diagnose a urinary tract or kidney infection, to evaluate causes of kidney failure, to screen for progression of some chronic conditions such as diabetes mellitus and high blood pressure (hypertension).

It also may be used in combination with other tests to diagnose some diseases. Examples of this include kidney stones, inflammation of the kidneys (glomerulonephritis), or muscle break breakdown (rhabdomyolysis). Additional tests and clinical assessment are often required to further investigate findings of urinalysis and ultimately diagnose the causes or specific features of underlying problems. For example, urine infection is generally diagnosed based on results of urinalysis. However, urine culture is often ordered as a follow-up test to identify the bacteria that may be causing the infection.

Who is involved in the interpretation of urinalysis?

Interpretation of urinalysis is generally based on reviewing all the components of the test and correlating it with the clinical signs and symptoms of the patient and the physical examination. The results are reviewed and interpreted by the doctor who ordered the test.

What does urinalysis involve?

Urinalysis is done by collecting a urine sample from a patient. The optimal sample tends to be an early morning urine sample because it is frequently the most concentrated urine produced in the day. Typically, no fasting is required before the collection of urine sample and routine medications can be taken before the test, unless otherwise instructed by the ordering physician.

Methods of collection are slightly different for female and male patient.

  • For females, the patient is asked to clean the area around the urethra with a special cleansing wipe, by spreading the labia of the external genitals and cleaning from front to back (toward the anus).
  • For men, the tip of the penis may be wiped with a cleansing pad prior to collection.
  • The urine is then collected in a clean urine specimen cup while the patient is urinating. It is best to avoid collecting the initial stream of urine. After the initial part of urine is disposed of in the toilet, then the urine is collected in the urine container provided. Once about 30 to 60 ml (roughly 3 to 5 tablespoons) are collected in the container for testing, the remainder of the urine may be voided in the toilet again. This is called the clean catch or the midstream urine collection.

The collected urine sample should be taken to the laboratory for analysis, typically within one hour of collection. If transportation to the lab could take more than one hour, then the sample may be refrigerated.

In some patients who are unable to void spontaneously or those who are not able to follow instructions other methods may be used, such as placing a catheter (a small rubber tube) through the outside opening to the bladder (urethra) to collect the sample directly from the bladder.

What is macroscopic urinalysis?

Macroscopic urinalysis is the direct visual observation of the urine, noting its quantity, color, clarity or cloudiness, etc.

Normal urine is typically light yellow and clear without any cloudiness. Obvious abnormalities in the color, clarity, and cloudiness may suggest possibility of:
  • an infection (cloudy urine),
  • dehydration (dark urine color) ,
  • red blood in the urine, also referred to as hematuria (red urine color),
  • liver disease (urine the color of tea), or
  • breakdown of muscle (orange or tea colored urine).

Certain medications may also change the color of urine. Very foamy urine may represent large amounts of protein in the urine (proteinuria).

 

What is urine dipstick chemical analysis?

Urine dipstick is a narrow plastic strip which has several squares of different colors attached to it. Each small square represents a component of the test used to interpret urinalysis. The entire strip is dipped in the urine sample and color changes in each square are noted. The color change takes place after several seconds to a few minutes from dipping the strip. If read too early or too long after the strip is dipped, the results may not be accurate.

Each color change on a particular square may indicate specific abnormalities in the urine sample caused by a certain chemical reaction. The reference for color changes is posted on the plastic bottle container of the urine test strips. This makes for easy and quick interpretation of the urinalysis results by placing the strip next to the container and comparing its color changes to the reference provided.

The squares on the dipstick represent the following components in the urine:

  • specific gravity (concentration of urine),
  • acidity of the urine (pH),
  • protein in the urine (mainly albumin),
  • glucose (sugar),
  • ketones (products of fat metabolism),
  • blood, leukocyte esterase (suggestive of white blood cells in urine),
  • nitrite (suggestive of bacteria in urine),
  • bilirubin (possible liver disease or red blood cell breakdown), and
  • urobilinogen (possible liver disease).

Presence or absence of each of these color changes on the strip provides important clues for your doctor to make clinical decisions based on the urinalysis results.

What are the pros and cons of dip sticks?

The main advantage of dipsticks is that they are convenient, easy to interpret, and cost-effective. They can be analyzed within minutes of urine collection in the doctor's office or in the emergency room to provide valuable information.

However, what can be learned from a dipstick is limited by the design of the dipstick. The main disadvantage is that the information may not be very accurate as the test is time-sensitive. It also provides limited information about the urine as it is qualitative test and not a quantitative test (for example, it does not give a precise measure of the quantity of abnormality). Therefore, normal and abnormal values are not reported as part of urinalysis results.

What is microscopic urinalysis?

The microscopic urinalysis is the study of the urine sample under a microscope. It requires only a relatively inexpensive light microscope. Cells and cellular debris, bacteria, and crystals in the urine can detected by this examination to provide further clinical clues.

How is microscopic urinalysis done?

Microscopic urinalysis is done simply pouring the urine sample into a test tube and centrifuging it (spinning it down in a machine) for a few minutes. The top liquid part (the supernatant) is discarded. The solid part left in the bottom of the test tube (the urine sediment) is mixed with the remaining drop of urine in the test tube and one drop is analyzed under a microscope.

First, the sediment is examined through the microscope under low-power to identify what are called casts, crystals, squamous (flat) cells, and other large objects.

Examination is then performed through the microscope at high power to further identify any cells, bacteria and clumps of cells or debris called casts.

What kind of cells can be detected?

Epithelial (flat cells) and red and white blood cells may be seen in the urine.

Sometimes cells, cellular debris, and casts are seen in the microscopic urinalysis. Epithelial cells (cells in the lining of the bladder or urethra) may suggest inflammation within the bladder, but they also may originate form the skin and could be contamination.

Casts and cellular debris originate from higher up in the urinary tract, such as in the kidneys. These are material shed from kidney cell lining due to injury or inflammation and travel down through the urinary tubes. These usually suggest an injury to the kidney from an inflammation or lack of blood flow to the kidneys. Rarely, tumor cells can be in the urine suggesting a urinary tract cancer.

What can the presence of red blood cells in the urine mean?

Red blood cells can enter the urine from the vagina in menstruation or from the trauma of bladder catheterization.

A high count of red blood cells in the urine can indicate infection, trauma, tumors, or kidney stones. If red blood cells seen under microscopy look distorted, they suggest kidney as the possible source and may arise due to kidney inflammation (glomerulonephritis). Small amounts of red blood cells in the urine are sometimes seen young healthy people and usually are not indicative of any disease.

What can the presence of white blood cells in the urine mean?

Urine is a generally thought of as a sterile body fluid, therefore, evidence of white blood cells or bacteria in the urine is considered abnormal and may suggest a urinary tract infection such as, bladder infection (cystitis), infection of kidney (pyelonephritis). White blood cells may be detected in the urine through a microscopic examination (pyuria or leukocytes in the blood). They can be seen under high power field and the number of cells are recorded (quantitative).

White cells from the vagina or the opening of the urethra (in males, too) can contaminate a urine sample. Such contamination aside, the presence of abnormal numbers of white blood cells in the urine is significant.

Other than urinalysis, what are other common urine tests available?

Other commonly performed urine tests are drug tests, pregnancy tests, specific chemicals and proteins in the body, which are not a part of routine urinalysis.

Urine drug screen is done routinely to check for drugs or their byproducts in the urine. There are many purposes for these tests including athletic screening, emergency rooms settings, drug detoxification programs, school and employment screening. This test detects the presence of commonly used drugs such as:

  • cocaine,
  • amphetamines,
  • metamphetamines,
  • marijuana,
  • phencyclidine,
  • barbiturates,
  • benzodiazepines, and
  • opiates.
 

Urine pregnancy test is very common and it measures a hormone in the urine associated with pregnancy (beta-HCG or beta- human chorionic gonadotropin). This test can be done in medical settings, but numerous kits are available for home use.

Other urine tests can also be used in evaluation of many medical conditions. Examples include:

  • urine culture (in determining the bacterial cause of urine infection)
  • urine creatinine (in assessing kidney disease)
  • urine total protein and albumin (in assessing kidney disease and protein loss from kidney)
  • urine cytology (in evaluating for possible bladder or other urinary tumors)
  • urine calcium (in evaluating elevated blood calcium levels)
  • 24 hour urine collection for proteins (in diagnosing causes of kidney impairment, diabetic related kidney disease, lupus related kidney disease)
  • 24 hour urine collection for protein electrophoresis (for measuring different components of proteins in urine in evaluating multiple myeloma, kidney inflammation with increased protein loss)
  • 24 hour urine collection of catecholamine metabolites (in evaluating adrenal gland disease, difficult to treat high blood pressure)


Henoch-Schonlein Purpura (HSP)
or
Anaphylactoid Purpura

 

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Medical Author: William C. Shiel Jr., MD, FACP, FACR
Medical Editors: Dennis Lee, MD, and Melissa Conrad Stöppler, MD

  • What is Henoch-Schonlein purpura (HSP)?
  • What causes HSP?
  • What are symptoms of HSP?
  • How is HSP diagnosed?
  • What is the treatment for HSP?
  • What is the prognosis for patients with HSP?
  • Henoch-Schonlein Purpura At A Glance
  • Patient Discussions: Henoch-Schonlein Purpura - Symptoms
  • Patient Discussions: Henoch-Schonlein Purpura - Describe Your Experience

What is Henoch-Schonlein purpura (HSP)?

Henoch-Schonlein purpura (HSP) is a form of blood vessel inflammation or vasculitis. There are many different conditions that feature vasculitis. Each of the forms of vasculitis tends to involve certain characteristic blood vessels. HSP affects the small vessels called capillaries in the skin and frequently the kidneys. HSP results in skin rash (most prominent over the buttocks and behind the lower extremities) associated with joint inflammation (arthritis) and sometimes cramping pain in the abdomen. Henoch-Schonlein purpura is also referred to as anaphylactoid purpura.

What causes HSP?

HSP occurs most often in the spring and frequently follows an infection of the throat or breathing passages. HSP seems to represent an unusual reaction of the body's immune system that is in response to this infection (either bacteria or virus). Aside from infection, drugs can also trigger the condition. HSP occurs most commonly in children, but people of all age groups can be affected.

What are symptoms of HSP?

Classically, HSP causes skin rash, pain in the abdomen, and joint inflammation (arthritis). Not all features need be present for the diagnosis. The rash of skin lesions appears in gravity-dependent areas, such as the legs. The joints most frequently affected with pain and swelling are the ankles and the knees. Patients with HSP can develop fever. Inflammation of the blood vessels in the kidneys can cause blood and/or protein in the urine. Serious kidney complications are infrequent but can occur.

Symptoms usually last approximately a month. Recurrences are not frequent but do occur.

How is HSP diagnosed?

HSP is usually diagnosed based on the typical skin, joint, and kidney findings. Throat culture, urinalysis, and blood tests for inflammation and kidney function are used to suggest the diagnosis. A biopsy of the skin, and less commonly kidneys, can be used to demonstrate vasculitis. Special staining techniques (direct immunofluorescence) of the biopsy specimen can be used to document antibody deposits of IgA in the blood vessels of involved tissue.

What is the treatment for HSP?

While HSP is generally a mild illness that resolves spontaneously, it can cause serious problems in the kidneys and bowels. The rash can be very prominent, especially on the lower extremities.

The treatment of HSP is directed toward the most significant area of involvement. Joint pain can be relieved by antiinflammatory medications such as aspirin or ibuprofen (Motrin). Some patients can require cortisone medications, such as prednisone or prednisolone, especially those with significant abdominal pain or kidney disease. With more severe kidney disease, involvement called glomerulonephritis or nephritis, cyclophosphamide (Cytoxan), azathioprine (Imuran), or mycophenolate mofetil (Cellcept) have been used to suppress the immune system. Infection, if present, can require antibiotics.

 

What is the prognosis for patients with HSP?

The prognosis for patients with HSP is generally excellent. Nearly all patients have no long-term problems. The kidney is the most serious organ involved when it is affected. Rarely, patients can have serious long-term kidney damage or an abnormal bowel folding called intussusception. Some patients have recurrences of symptoms, particularly skin rash, for months to a year after the onset of the illness.

Recent data show that HSP in adults is generally more severe than in children. Adults have more severe kidney involvement and can require more aggressive treatment. The ultimate outcome, however, is usually very good for both adults and children.

Henoch-Schonlein Purpura At A Glance
  • Henoch-Schonlein purpura is a particular form of blood vessel inflammation called vasculitis.
  • Henoch-Schonlein purpura frequently follows an infection of the throat or breathing passages, but it can be induced by certain medications.
  • Henoch-Schonlein purpura causes skin rash, pain in the abdomen, and joint inflammation (arthritis).
  • The treatment of Henoch-Schonlein purpura is directed toward the most significant area of involvement.
  • The prognosis for patients with Henoch-Schonlein purpura is generally excellent.


Anaphylaxis
(Severe Allergic Reaction)

 

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Revising Medical Author: Jerry R. Balentine, DO, FACEP
Revising Medical Editor: Melissa Conrad Stöppler, MD

  • Introduction to anaphylaxis
  • What does anaphylaxis mean?
  • How common is anaphylaxis?
  • What are common causes of anaphylaxis?
  • What are the signs of anaphylaxis?
  • What are anaphylaxis symptoms?
  • What happens after the symptoms begin?
  • Are there any disorders that appear similar to anaphylaxis?
  • How is anaphylaxis diagnosed?
  • How do we manage anaphylaxis?
  • What are emergency measures for anaphylaxis?
  • Can anaphylaxis be prevented?
  • Anaphylaxis At A Glance
  • Patient Discussions: Anaphylaxis - Symptoms and Signs
  • Patient Discussions: Anaphylaxis - Describe Your Experience
  • Find a local Doctor in your town

Introduction to anaphylaxis

Anaphylaxis refers to a rapidly developing and serious allergic reaction that affects a number of different areas of the body at one time. Severe anaphylactic reactions can be fatal. Most people experience allergy symptoms only as a minor annoyance. However, a small number of people are susceptible to a reaction that can lead to shock or even death.

Anaphylaxis is often triggered by substances that are injected or ingested and thereby gain access into the blood stream. An explosive reaction involving the skin, lungs, nose, throat, and gastrointestinal tract can then result. Although severe cases of anaphylaxis can occur within seconds or minutes of exposure and be fatal if untreated, many reactions are milder and can be ended with prompt medical therapy.

What does anaphylaxis mean?

To fully understand this term, we need to go back almost 100 years. The story begins on a cruise aboard Prince Albert I of Monaco's yacht. The Prince had invited two Parisian scientists to perform studies on the toxin produced by the tentacles of a local jellyfish, the Portuguese Man of War. Charles Richet and Paul Portier were able to isolate the toxin and tried to vaccinate dogs in the hope of obtaining protection, or "prophylaxis," against the toxin. They were horrified to find that subsequent very small doses of the toxin unexpectedly resulted in a new dramatic illness that involved the rapid onset of breathing difficulty and resulted in death within 30 minutes. Richet and Portier termed this "anaphylaxis" or "against protection." They rightly concluded that the immune system first becomes sensitized to the allergen over several weeks and upon re-exposure to the same allergen may result in a severe reaction. An allergen is a substance that is foreign to the body and can cause an allergic reaction in certain people.

Allergy Facts
  • The first documented case of presumed anaphylaxis occurred in 2641 B.C. when Menes, an Egyptian pharaoh, died mysteriously following a wasp or hornet sting. Later, in Babylonian times, there are two distinct references to deaths due to wasp stings.
  • Charles Richet was awarded the Nobel Prize in 1913 for his work on anaphylaxis.

Richet went on to suggest that the allergen must result in the production of a substance, which then sensitized the dogs to react in such a way upon re-exposure. This substance turned out to be IgE.

In the first part of the 20th century, anaphylactic reactions were most commonly caused by tetanus diphtheria vaccinations made from horse serum. Today, human serum is used for tetanus prevention, and the most common causes of anaphylaxis are now penicillin and other antibiotics, insect stings, and certain foods.

Allergy Fact

In 1956, Mary Hewitt Loveless showed that the injection of wasp venoms could cause anaphylaxis in individuals allergic to wasps. She subsequently used wasp extracts to successfully immunize such individuals.

How common is anaphylaxis?

The exact prevalence of anaphylaxis is unknown. The available statistics probably underestimate the true frequency because reactions are not always reported. Milder reactions may be attributed to an asthma attack or a sudden episode of hives. More serious, fatal episodes might be reported as a heart attack since the indicative signs of hives, swollen throat, and asthma can fade quickly. Thus, it is quite possible that even the true incidence of fatalities due to anaphylaxis is both under- recognized and under-reported. The importance of awareness, early recognition, and prompt treatment of this disorder must be stressed.

 

What are common causes of anaphylaxis?

The causes of anaphylaxis are divided into two major groups:

  • IgE mediated: This form is the true anaphylaxis that requires an initial sensitizing exposure, the coating of mast cells and basophils (cells in the blood and tissue that secrete the substances that cause allergic reactions, known as mediators) by IgE, and the explosive release of chemical mediators upon re-exposure.
  • Non-IgE mediated: These reactions, the so called "anaphylactoid" reactions, are similar to those of true anaphylaxis, but do not require an IgE immune reaction. They are usually caused by the direct stimulation of the mast cells and basophils. The same mediators as occur with true anaphylaxis are released and the same effects are produced. This reaction can happen, and often does, on initial as well as subsequent exposures, since no sensitization is required.

 

The terms anaphylaxis and anaphylactoid (meaning "like anaphylaxis") are both used to describe this severe, allergic reaction. Anaphylaxis is used to describe reactions that are initiated by IgE and anaphylactoid is used in reference to reactions that are not caused by IgE. The effects of the reactions are the same, however, and are generally treated in the same manner. Often, they can not be distinguished initially.

Although it may appear that IgE mediated anaphylaxis occurs upon a first exposure to a food, drug, or insect sting, there must have been a prior, and probably unwitting, sensitization from a previous exposure. You may not remember an uneventful sting or be aware of "hidden" allergens in foods.

What are the signs of anaphylaxis?

It is worth mentioning a few general observations regarding the features of anaphylactic reactions. Be aware, however, that these guidelines are not always consistent or reliable for a particular individual.

  • The severity of the reaction varies from person to person.
  • Subsequent reactions to the same trigger are typically similar in nature.
  • The more rapid the onset of symptoms, the more severe the reaction is likely to be.
  • A history of allergic disease (rhinitis, eczema, asthma) does not increase the risk of developing IgE mediated anaphylaxis, but it does incline the person to a non-IgE mediated reaction.
  • Underlying asthma may result in a more severe reaction and can be more difficult to treat.
  • The risk of anaphylaxis may diminish over time if there are no repeated exposures or reactions. However, a person at risk should always expect the worst and be prepared.

 

What are anaphylaxis symptoms?

The symptoms of an anaphylactic reaction may occur within seconds of exposure, or be delayed 15 to 30 minutes, or even an hour or more after exposure (typical of reactions to aspirin and similar drugs). Early symptoms are often related to the skin and include:

 
  • Flushing (warmth and redness of the skin),
  • itching (often in the groin or armpits), and
  • hives.

These symptoms are often accompanied by:

  • a feeling of "impending doom,"
  • anxiety, and
  • sometimes a rapid, irregular pulse.

Frequently following the above symptoms, throat and tongue swelling results in hoarseness, difficulty swallowing, and difficulty breathing.

Symptoms of rhinitis (hay fever) or asthma may occur causing:

  • a runny nose,
  • sneezing, and wheezing, which may worsen the breathing difficulty,
  • vomiting, diarrhea, and stomach cramps may develop.

About 25% of the time, the mediators flooding the blood stream cause a generalized opening of capillaries (tiny blood vessels) which results in a drop in blood pressure, lightheadedness, or even loss of consciousness. These are the typical features of anaphylactic shock.

What happens after the symptoms begin?

There are three possible outcomes:

  1. The signs and symptoms may be mild and fade spontaneously or be quickly ended by administering emergency medication. In this outcome, the symptoms do not subsequently recur from this particular exposure.

  2. After initial improvement, the symptoms may recur within 4 to 12 hours (late phase reaction) and require additional treatment and close observation. Recent evidence suggests that a late phase reaction occurs in fewer than 10% of cases.

  3. Lastly, the reaction may be persistent and more severe, thus requiring intensive medical treatment and hospitalization. This may occur up to 20% of the time with certain exposures.

Epinephrine, which is also known as "adrenaline," is a drug that acts immediately to cause the blood vessels to contract, thereby preventing fluid leakage. It is one of the medications frequently used to treat anaphylaxis. Epinephrine also helps relax the bronchial tubes, thus relieving breathing difficulty. It also lessens stomach cramps and stops itching and hives. More importantly, epinephrine helps prevent the release of more mediators of the allergic reaction. In addition to epinephrine, other medications and IV fluids and oxygen will probably be administered as well. The choice of interventions will depend on the severity of the reaction the patient experiences.

 

Are there any disorders that appear similar to anaphylaxis?

Several disorders may appear similar to anaphylaxis. Fainting (vaso-vagal reaction) is the reaction that is most likely to be confused with anaphylaxis. The key differences are that in a fainting episode, the affected person has a slow pulse, cool and pale skin, and no hives or difficulty breathing. Other conditions, such as heart attacks, blood clots to the lungs, septic shock, and panic attacks can also be confused with anaphylaxis.

How is anaphylaxis diagnosed?

Once you think that you might have had an anaphylactic reaction, the first order of business is to seek emergency care. Once the acute reaction has been treated you should follow-up with your doctor who will probably recommend seeing an allergist. The allergist will assess whether or not the reaction was indeed allergic in nature. Usually, a careful and detailed medical history and selected blood or skin tests can identify the cause. Be prepared to recall your activities before the event, the food and medications you ingested, and whether or not you had any contact with rubber products.

Table 1: The Common Causes of Anaphylaxis

Causes - IgE Mediated Examples
Medications Penicillin, Cephalosporin, Anesthetics, Streptokinase, Others
Insect Stings Hornet, Wasp, Yellow Jacket, Honey Bee, Fire Ant
Foods Peanuts, Treenuts, Fish, Shellfish, Eggs, Milk, Soy, Wheat
Vaccines Allergy Shots, Egg and Gelatin based vaccines
Hormones Insulin, Possibly Progesterone
Latex Rubber Products
Animal/Human Proteins Horse Serum (used in some snake anti-venoms)
Causes - Non IgE Mediated Examples
Medication Non-steroidal Anti-inflammatories (Aspirin, Motrin, etc.), Morphine, Muscle Relaxants (Robaxin, Norflex, and others), Gamma Globulin
X-ray Dye
Preservatives Sulfites
Physical Exercise, Heat-Induced Urticaria (Hives), Cold- Induced Urticaria
Idiopathic Unknown Cause

How do we manage anaphylaxis?

The optimal management of anaphylaxis saves lives. An affected or at-risk person must be aware of possible triggers and early warning signs. If you are prone to these reactions, you must be familiar with the use of emergency anaphylaxis treatment kits and always have them with you. Emergency measures and prevention are central to management. As always, allergic diseases are best treated by avoidance measures, which will be reviewed in detail below.

What are emergency measures for anaphylaxis?

If you suspect that you or someone you are with is having an anaphylactic reaction, the following are important first aid measures. In general, try to perform these in the order that they are presented.

  • Call emergency services or 911 IMMEDIATELY.
  • If the patient has an epi-pen, inject epinephrine immediately. The shot is given into the outer thigh and can be administered through light fabric. Rub the site to improve absorption of the drug.
  • Place a conscious person lying down and elevate the feet if possible.
  • Stay with the person until help arrives.
  • If trained, begin CPR if the person stops breathing or doesn't have a pulse.

 

Allergy Assist

Shots of epinephrine can be given through light clothing such as trousers, skirts, or stockings. Heavy garments may have to be removed prior to injecting. Only inject epinephrine if the patient has a history of anaphylactic reactions or under guidance of a healthcare provider.

After 10 to 15 minutes, if the symptoms are still significant, you can inject another dose of epinephrine if available. Even after the reaction subsides you need to go to an emergency department immediately. Other treatments may be given, such as oxygen, intravenous fluids, breathing medications, and possibly more epinephrine. Steroids and antihistamines may be given but these are often not helpful initially and do not take the place of epinephrine. However, they may be more useful in preventing a recurrent delayed reaction.

Do not be surprised if epinephrine makes you feel shaky and causes a rapid, pounding pulse. These are normal side effects and are not dangerous except for those with severe heart problems.

Two situations deserve special attention at this point since they are not covered elsewhere but are particularly interesting.

  1. In the 1970's, it was noted that exercise could cause anaphylaxis. Exercise-induced anaphylaxis (EIA) usually occurs with prolonged, strenuous exercise. Conditioned athletes such as marathon runners are frequently affected. The reaction may occur while exercising shortly after eating a meal, after eating specific foods (for example, lettuce, shellfish, or celery) or after taking aspirin. It appears as though food or aspirin loads the gun and exercise pulls the trigger. Early symptoms are usually flushing and itching, which may progress to other typical symptoms of anaphylaxis if the exercise continues. Pre-medication with antihistamines or other drugs does not consistently prevent EIA. Exercise avoidance is the most effective treatment. If this is not feasible, exercising with a "buddy" and carrying emergency kits is mandatory.

  1. When no cause can be found for anaphylaxis, it is termed idiopathic. Recent reports suggest that 25% of all episodes of anaphylaxis are idiopathic. Many of those affected have underlying allergy or asthma conditions. Extensive allergy testing for foods may uncover an unusual food allergy that is responsible for these reactions. For frequent episodes of anaphylaxis, your physician may recommend a combination of antihistamine, cortisone, and a medication to widen the airways of the lungs (bronchial dilator) to help reduce the severity of attacks.

Can anaphylaxis be prevented?

Preventing anaphylaxis is the ideal form of treatment. However, that may not always be easy since insect stings are frequently unanticipated and allergic foods are often hidden in a variety of different preparations. A consultation with an allergist is vital in helping you identify the trigger(s) and providing you with information and instruction on how to best avoid them. You will learn how to use emergency kits and how to become prepared for any reaction in the future.

These are three situations in which preventive treatment might be offered by the allergist.

  1. Allergy shots may be suggested to some people with wasp, yellow jacket, hornet, honey bee, or fire ant reactions. This form of treatment gives 98% protection against the first four insect reactions, though somewhat less protection against fire ant reactions.

  1. Pre-medication is most helpful in preventing anaphylaxis from x-ray dyes. Alternative dyes that are less likely to cause reactions may be available.

  1. Desensitization to problematic medications is often effective. This process is accomplished by gradually increasing the amount of the medication given under controlled conditions. Sensitivities to penicillin, sulfa drugs, and insulin have been successfully treated in this way.

Anyone known to be at risk for anaphylaxis should wear a Medic-Alert bracelet that clearly states the allergic trigger, the risk of anaphylaxis, and the availability of an epinephrine kit.

Allergy Assist

People with anaphylaxis to medications should take new medications by mouth whenever possible since the risk of anaphylaxis is higher with injections.

 

Table 2: Basic Avoidance Measures for Anaphylaxis

Trigger Avoidance Principle
Drugs/Medications
  • Advise all health care personnel of your allergies.
  • Ask your doctor whether the prescribed medication contains the drug(s) you are allergic to.
  • Take all drugs by mouth if possible.
Insect Stings
  • Avoid areas such as outdoor garbage, barbecues, and insect nests.
  • Avoid bright clothing, perfume, hair spray or lotion that might attract insects.
  • Wear long sleeved clothing, long trousers, and shoes while outdoors
Food
  • Carefully read all labels.
  • Ask what the ingredients are when eating out.
  • Avoid foods that may cross react such as bananas, kiwi fruit, and avocado.
Latex
  • Avoid all Latex products.
  • Ask if your hospital has Latex safety issues if you need to be hospitalized.

Since avoidance is not fail safe, a person at risk for an anaphylactic reaction must be adequately prepared in an emergency to handle a reaction. It is recommended that everyone at risk carry epinephrine injection kits designed for self-administration. These kits are available by prescription only and come in two forms:

  • Epi-pen is a spring-loaded automatic syringe that delivers a predetermined dose (0.3mg) when the tip is pressed hard for several seconds. An Epi-pen junior is available for children under 33 pounds and contains half of the dose.
  • Ana-kit contains a preloaded syringe and needles with two 0.3mg doses of epinephrine. These are injected under the skin or into the muscle of the thigh. An antihistamine, alcohol swab, and a tourniquet are included in the kit.

Here are some important points to remember regarding the kits:

  • Ask you doctor to explain the use of the kit carefully and practice with the demonstrator kit.
  • Check expiration dates and replace outdated kits.
  • Keep kits out of direct sunlight, which may affect the drug.
  • Additional kits should be brought to school or work.
  • Always have kits with you or readily available.
  • Make sure that your friends, relatives, exercise buddies, and co-workers are aware of your condition and know what to do in case of a reaction.
Anaphylaxis At A Glance
  • Anaphylaxis is the most severe allergic reaction and is potentially life threatening.
  • Anaphylaxis is rare. The vast majority of people will never have an anaphylactic reaction.
  • The most common causes of anaphylaxis include drugs, such as penicillin, insect stings, foods, x-ray dye, latex, and exercise.
  • The symptoms of anaphylaxis may vary from hives, tongue swelling, and vomiting, to shock.
  • If you are at risk, avoidance is the best form of treatment.
  • If you have a history of serious allergic reaction, always have an epinephrine kit available - it could save your life.


Adult Brain Tumors

  • Adult brain tumor facts*
  • What are adult brain tumors?
  • What are metastatic brain tumors?
  • What are the symptoms of an adult brain tumor?
  • What tests are used to find and diagnose adult brain tumors?
  • What is the grade of a tumor?
  • What are the types of adult brain tumors?
  • How are adult brain tumors treated?
  • Three types of standard treatment are used.
  • Other types of treatment that are being tested in clinical trials
  • Treatment options by type of adult brain tumor
  • Where can a patient get more information about adult brain tumors?
  • Find a local Oncologist in your town

Adult brain tumor facts*

*Adult brain tumor facts Medically Edited by: Charles P. Davis, MD, PhD

  • The brain is a soft mass of tissue that has three major parts, the cerebrum, cerebellum and the brain stem, all of which are effectively surrounded and protected by the bones of the skull; the brain is the tissue that controls people's voluntary and involuntary actions.
  • Cancer is the unregulated growth of abnormal cells in the body (cancer cells are also termed malignant cells).
  • Malignant brain tumors contain cancer cells; benign brain tumors do not contain cancer cells but do contain abnormally replicating cells that do not metastasize (spread to other organs) but may still cause problems, often because of their size and are regulated to grow in a specific area.
  • Primary brain tumors are composed of abnormal types of brain cells with unregulated growth; the most common type is termed gliomas that arise from brain glial cells, but there are many other types (for example, astrocytomas, ependymomas, medulloblastomas and others).
  • Secondary brain tumors are tumors that have spread to the brain tissue, but are composed of cancer cells from other organs (for example, breast, lung).
  • The exact cause of brain tumors is unknown; however, people at higher risk for them are children and the elderly, white males, people with family members that have brain tumors, radiation exposure, and exposures to many different chemicals.
  • Symptoms of brain tumors, many of which are non-specific and occur in other diseases, may include headaches, nausea, vomiting, speech, hearing or vision changes, memory problems, personality changes and paresthesias (an abnormal sensation of the skin such as numbness, tingling, prickling, burning, or creeping on the skin that has no objective cause).
  • Brain tumors are diagnosed preliminarily by many methods including detailed physical exam, CT and/or MRI exams, angiograms, and X-rays; definitive diagnosis is by removing tissue from the tumor (tumor biopsy) and examining the cells microscopically.
  • Treatment choices for a brain tumor depends on joint decisions made by the patient and the patient's physician team (team members may include oncologists, surgeons, therapists and others the patient may choose, including other doctors who may give a second opinion); treatment methods are based on the individual's disease and may consist of surgery, chemotherapy, radiation therapy, combinations of these methods or no treatment.
  • Side effects of treatments are common and numerous but vary from patient to patient depending on the disease, method(s) used and the effectiveness of medications and other methods to reduce them; some of the most common side effects are weakness, nausea, edema, skin changes and hair loss but may include more serious problems such as infections, seizures, disabilities such as speech problems, mental changes and occasionally, death.
  • Rehabilitation is frequently included in the treatment plan; specialists like physical, occupational and speech therapists can help the patient improve.
  • Follow-up appointments are part of the treatment plan for brain tumors to catch any recurrent disease and to help with rehabilitation treatments.
  • Support groups are available to patients with brain tumors and to patients who have and are undergoing treatments; for example, the American Cancer Society, American Brain Tumor Society.

 

What are adult brain tumors?

Adult brain tumors are diseases in which cancer (malignant) cells begin to grow in the tissues of the brain. The brain controls memory and learning, senses (hearing, sight, smell, taste, and touch), and emotion. It also controls other parts of the body, including muscles, organs, and blood vessels. Tumors that start in the brain are called primary brain tumors.

What are metastatic brain tumors?

Often, tumors found in the brain have started somewhere else in the body and spread (metastasized) to the brain. These are called metastatic brain tumors.

What are the symptoms of an adult brain tumor?

A doctor should be seen if the following symptoms appear:

  • Frequent headaches.
  • Vomiting.
  • Loss of appetite.
  • Changes in mood and personality.
  • Changes in ability to think and learn.
  • Seizures.

What tests are used to find and diagnose adult brain tumors?

Tests that examine the brain and spinal cord are used to detect (find) adult brain tumor. The following tests and procedures may be used:

  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the brain and spinal cord. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).

Adult brain tumor is diagnosed and removed in surgery. If a brain tumor is suspected, a biopsy is done by removing part of the skull and using a needle to remove a sample of the brain tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery. An MRI may then be done to determine if any cancer cells remain after surgery. Tests are also done to find out the grade of the tumor.

What is the grade of a tumor?

The grade of a tumor refers to how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. The pathologist determines the grade of the tumor using tissue removed for biopsy. The following grading system may be used for adult brain tumors:

Grade I

The tumor grows slowly, has cells that look similar to normal cells, and rarely spreads into nearby tissues. It may be possible to remove the entire tumor by surgery.

Grade II

The tumor grows slowly, but may spread into nearby tissue and may become a higher-grade tumor.

Grade III

The tumor grows quickly, is likely to spread into nearby tissue, and the tumor cells look very different from normal cells.

Grade IV

The tumor grows very aggressively, has cells that look very different from normal cells, and is difficult to treat successfully.

The chance of recovery (prognosis) and choice of treatment depend on the type, grade, and location of the tumor and whether cancer cells remain after surgery and/or have spread to other parts of the brain.

Types of Adult Brain Tumors

The extent or spread of cancer is usually described as stages. There is no standard staging system for brain tumors. Primary brain tumors may spread within the central nervous system (brain and spinal cord), but they rarely spread to other parts of the body. For treatment, brain tumors are classified by the type of cell in which the tumor began, the location of the tumor in the central nervous system, and the grade of the tumor.

Types of adult brain tumors include the following:

Brain Stem Gliomas

These are tumors that form in the brain stem, the part of the brain connected to the spinal cord. They are often high-grade. Brain stem gliomas that are high-grade or spread widely throughout the brain stem are difficult to treat successfully. To prevent damage to healthy brain tissue, brain stem glioma is usually diagnosed without a biopsy.

Pineal Astrocytic Tumor

Pineal tumors form in or near the pineal gland. The pineal gland is a tiny organ in the brain that produces the hormone melatonin, a substance that helps control our sleeping and waking cycle. There are several kinds of pineal tumors. Pineal astrocytic tumors are astrocytomas that occur in the pineal region and may be any grade.

Pilocytic Astrocytoma (grade I)

Astrocytomas are tumors that start in brain cells called astrocytes. Pilocytic astrocytomas grow slowly and rarely spread into the tissues around them. These tumors occur most often in children and young adults. They usually can be treated successfully.

Diffuse Astrocytoma (grade II)

Diffuse astrocytomas grow slowly, but they often spread into nearby tissues. Some of them progress to a higher grade. They occur most often in young adults.

Anaplastic Astrocytoma (grade III)

Anaplastic astrocytomas are also called malignant astrocytomas. They grow rapidly and spread into nearby tissues. The tumor cells look different from normal cells. The average age of patients developing anaplastic astrocytomas is 41 years.

Glioblastoma (grade IV)

Glioblastomas are malignant astrocytomas that grow and spread aggressively. The cells look very different from normal cells. Glioblastoma is also called glioblastoma multiforme or grade IV astrocytoma. They occur most often in adults between the ages of 45 and 70 years.

Oligodendroglial Tumors

Oligodendroglial tumors begin in the brain cells called oligodendrocytes, which support and nourish nerve cells. Grades of oligodendroglial tumors include the following:

  • Oligodendroglioma (grade II): Oligodendrogliomas are slow-growing tumors with cells that look very much like normal cells. These tumors occur most often in patients between the ages of 40 and 60 years.
  • Anaplastic oligodendroglioma (grade III): Anaplastic oligodendrogliomas grow quickly and the cells look very different from normal cells.

Mixed Gliomas

Mixed gliomas are brain tumors that contain more than one type of cell. The prognosis is affected by the cell type with the highest grade present in the tumor.

  • Oligoastrocytoma (grade II): Oligoastrocytomas are slow-growing tumors composed of cells that look like astrocytes and oligodendrocytes.
  • Anaplastic oligoastrocytoma (grade III): These are higher-grade oligoastrocytomas. The average age of patients developing anaplastic oligoastrocytomas is 45 years.

Ependymal Tumors

Ependymal tumors usually begin in cells that line the spaces in the brain and around the spinal cord. These spaces contain cerebrospinal fluid, a liquid that cushions and protects the brain and spinal cord. Grades of ependymal tumors include the following:

  • Grade I and grade II ependymomas: These ependymomas grow slowly and have cells that look very much like normal cells. They can often be removed completely by surgery.
  • Anaplastic ependymoma (grade III): Anaplastic ependymomas grow very quickly.

Medulloblastoma (grade IV)

Medulloblastomas are brain tumors that begin in the lower back of the brain. They are formed from abnormal brain cells at a very early stage in development. Medulloblastomas are usually found in children or young adults between the ages of 21 and 40 years. This type of cancer may spread from the brain to the spine through the cerebrospinal fluid.

Pineal Parenchymal Tumors

Pineal parenchymal tumors form from parenchymal cells or pinocytes, the cells that make up most of the pineal gland. These differ from pineal astrocytic tumors, which are astrocytomas that form in tissue that supports the pineal gland. Grades of pineal parenchymal tumors include the following:

  • Pineocytomas (grade II): Pineocytomas are slow-growing pineal tumors that occur most often in adults aged 25 to 35.
  • Pineoblastomas (grade IV): Pineoblastomas are rare and highly malignant. They usually occur in children.

Meningeal Tumors

Meningeal tumors form in the meninges, thin layers of tissue that cover the brain and spinal cord. Types of meningeal tumors include the following:

  • Grade I meningioma: Meningiomas are the most common meningeal tumor. Grade 1 meningiomas are slow-growing and benign. They are found most often in women.
  • Grade II and III meningiomas and hemangiopericytomas: These are rare malignant meningeal tumors. They grow quickly and are likely to spread within the brain and spinal cord. Grade III meningiomas are more common in men. Hemangiopericytomas often recur after treatment and most of them spread to other parts of the body.

Germ Cell Tumor

Germ cell tumors arise from germ cells, cells that are meant to form sperm in the testicles or eggs in the ovaries. These cells may travel to other parts of the body and form tumors. Types of germ cell tumors include germinomas, embryonal cell carcinomas, choriocarcinomas, and teratomas. They can occur anywhere in the body and can be either benign or malignant. In the brain, they usually form in the center, near the pineal gland, and can spread to other parts of the brain and spinal cord. Most germ cell tumors occur in children.

Craniopharyngioma (grade II)

Craniopharyngiomas occur in the sellar region of the brain, near the pituitary gland. The pituitary gland is a small organ about the size of a pea, located at the base of the brain. This gland controls many of the body's functions, especially growth. In adults, these tumors occur most often after the age of 50 years. Craniopharyngiomas can press on vital brain tissue and cause symptoms to appear. The tumors can also block fluid in the brain and cause swelling. The prognosis is good for craniopharyngiomas that are completely removed in surgery.

Pituitary tumors also occur in this region.

Recurrent Adult Brain Tumor

Recurrent adult brain tumor is a tumor that has recurred (come back) after it has been treated. Adult brain tumors often recur, sometimes many years after the first tumor. The tumor may come back in the brain or in other parts of the body.

Metastatic Brain Tumors

The types of cancer that commonly spread to the brain are cancers of the lung, breast, unknown primary site, melanoma, and colon. About half of metastatic spinal cord tumors are caused by lung cancer.

Prognosis depends on the following:

  • Whether the patient is younger than 60 years.
  • Whether there are fewer than 3 tumors in the brain and/or spinal cord.
  • The location of the tumors in the brain and/or spinal cord.
  • How well the tumor responds to treatment.
  • Whether the primary tumor continues to grow or spread.

The prognosis is better for brain metastases from breast cancer than from other types of primary cancer.

The prognosis is worse for brain metastases from colon cancer.

How are adult brain tumors treated?

Different types of treatment are available for patients with adult brain tumor. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Clinical trials are taking place in many parts of the country. To learn more about clinical trials, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Three types of standard treatment are used.

  1. Surgery: Surgery is used, when possible, to treat adult brain tumor, as described in the Description section of this summary.
  2. Radiation therapy: Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
  3. Chemotherapy: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). A dissolving wafer may be used to deliver an anticancer drug directly into the brain tumor site after the tumor has been removed by surgery. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Other types of treatment are being tested in clinical trials.

New methods of delivering radiation therapy

  • Radiosensitizers: Drugs that make tumor cells more sensitive to radiation. Combining radiation with radiosensitizers may kill more tumor cells.
  • Hyperfractionation: Radiation therapy given in smaller-than-usual doses two or three times a day instead of once a day.
  • Stereotactic radiosurgery: A radiation therapy technique that delivers radiation directly to the tumor with less damage to healthy tissue. The doctor uses a CT scan or MRI to find the exact location of the tumor. A rigid head frame is attached to the skull and high-dose radiation is directed to the tumor through openings in the head frame, reducing the amount of radiation given to normal brain tissue. This procedure does not involve surgery. This is also called stereotaxic radiosurgery and gamma knife therapy.

Hyperthermia therapy

Hyperthermia therapy is a treatment in which body tissue is exposed to high temperatures to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation and certain anticancer drugs.

Biologic therapy

Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

How are metastatic brain tumors treated?

Tumors that have spread to the brain from somewhere else in the body are usually treated with radiation therapy and/or surgery. Chemotherapy may be used if the primary tumor is the kind that responds well to chemotherapy. Clinical trials are under way to study new treatments

Treatment Options by Type of Adult Brain Tumor

Brain Stem Gliomas

Treatment of brain stem gliomas may include the following:

  1. Hyperfractionated radiation therapy.
  2. A clinical trial of new anticancer drugs and/or biologic therapy.

Pineal Astrocytic Tumors

Treatment of pineal astrocytic tumors may include the following:

  1. Surgery and radiation therapy, with or without chemotherapy.
  2. A clinical trial of external radiation therapy plus hyperthermia therapy or new methods of delivering radiation therapy.
  3. A clinical trial of new anticancer drugs and biologic therapy following radiation therapy.

Pilocytic Astrocytomas

Treatment of pilocytic astrocytoma is usually surgery with or without radiation therapy.

Diffuse Astrocytomas

Treatment of diffuse astrocytoma may include the following:

  1. Surgery, usually with radiation therapy.
  2. A clinical trial of surgery and radiation therapy with or without chemotherapy for tumors that cannot be completely removed by surgery.
  3. A clinical trial of radiation therapy delayed until the tumor progresses.
  4. A clinical trial comparing high-dose and low-dose radiation therapy.

Anaplastic Astrocytomas

Treatment of anaplastic astrocytoma may include the following:

  1. Surgery plus radiation therapy, with or without chemotherapy.
  2. A clinical trial of external radiation therapy plus hyperthermia therapy or new methods of delivering radiation therapy.
  3. A clinical trial of new anticancer drugs and biologic therapy following radiation therapy.
  4. A clinical trial of chemotherapy combined with different methods of delivering radiation therapy.

Glioblastoma

Treatment of glioblastoma may include the following:

  1. Surgery plus radiation therapy, with or without chemotherapy.
  2. A clinical trial of chemotherapy placed into the brain during surgery.
  3. A clinical trial of radiation and concurrent chemotherapy.
  4. A clinical trial of external radiation therapy plus hyperthermia therapy or new methods of delivering radiation therapy.
  5. A clinical trial of new anticancer drugs and biologic therapy following radiation therapy.
  6. A clinical trial of chemotherapy and new methods of delivering radiation therapy.
  7. Clinical trials of new treatments.

Oligodendroglial Tumors

Treatment of oligodendrogliomas may include the following:

  1. Surgery, usually with radiation therapy.
  2. A clinical trial of surgery and radiation therapy with or without chemotherapy for tumors that cannot be completely removed by surgery.
  3. A clinical trial of chemotherapy using one or more drugs.

Treatment of anaplastic oligodendroglioma may include the following:

  1. Surgery plus radiation therapy with or without chemotherapy.
  2. Chemotherapy using more than one drug.
  3. Radiation therapy with or without chemotherapy using more than one drug.
  4. Clinical trials of new treatments.

Mixed Gliomas

Treatment of mixed gliomas may include the following:

  1. Surgery plus radiation therapy with or without chemotherapy.
  2. A clinical trial of external radiation therapy plus hyperthermia therapy or new methods of delivering radiation therapy.
  3. A clinical trial of new anticancer drugs or biologic therapy following radiation therapy.

Ependymal Tumors

Treatment of grade I and grade II ependymomas is usually surgery with or without radiation therapy.

Treatment of anaplastic ependymoma may include the following:

  1. Surgery plus radiation therapy.
  2. A clinical trial of surgery followed by chemotherapy before, during, and after radiation therapy.
  3. A clinical trial of chemotherapy and/or biologic therapy.

Medulloblastoma

Treatment of medulloblastomas may include the following:

  1. Surgery plus radiation therapy to the brain and spine.
  2. A clinical trial of surgery and radiation therapy to the brain and spine for tumors that are more difficult to treat successfully.
  3. A clinical trial of chemotherapy.

Pineal Parenchymal Tumors

Treatment of pineal parenchymal tumors may include the following:

  1. Surgery plus radiation therapy with or without chemotherapy.
  2. A clinical trial of external radiation therapy plus hyperthermia therapy or new methods of delivering radiation therapy.
  3. A clinical trial of new anticancer drugs and biologic therapy following radiation therapy.

Meningeal Tumors

Treatment of meningiomas may include the following:

  1. Surgery with or without radiation therapy.
  2. Radiation therapy for tumors that cannot be removed by surgery.

Treatment of malignant meningioma may include the following:

  1. Surgery plus radiation therapy.
  2. A clinical trial of external radiation therapy plus hyperthermia therapy or new methods of delivering radiation therapy.
  3. A clinical trial of new anticancer drugs and/or biologic therapy following radiation therapy.

Germ Cell Tumors

Treatment of central nervous system germ cell tumors depends on the type of cancer cells, the location of the tumor, whether the cancer can be removed in an operation, and other factors.

Craniopharyngioma

Treatment of craniopharyngiomas may include the following:

  1. Surgery to remove the entire tumor.
  2. Surgery to remove as much of the tumor as possible, followed by radiation therapy.

Recurrent Adult Brain Tumor

Treatment of recurrent adult brain tumors may include the following:

  1. Surgery with or without chemotherapy.
  2. Radiation therapy, if not used during previous treatment, with or without chemotherapy.
  3. Internal radiation therapy.
  4. Chemotherapy.
  5. A clinical trial of new anticancer drugs.
  6. A clinical trial of chemotherapy placed into the brain during surgery.
  7. A clinical trial of biologic therapy.

Metastatic Brain Tumors

Treatment of a single metastatic brain tumor is usually surgery followed by radiation therapy to the brain.

Treatment of more than one metastatic brain tumor may include the following:

  1. Radiation therapy to the brain.
  2. Surgery, for large tumors that are pressing on areas of the brain and causing symptoms.

To Learn More

Call

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

Web sites and Organizations

The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. There are also many other places where people can get materials and information about cancer treatment and services. Local hospitals may have information on local and regional agencies that offer information about finances, getting to and from treatment, receiving care at home, and dealing with problems associated with cancer treatment.

Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.


Anemia

 

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  • What is anemia?
  • What causes anemia?
  • Can not enough iron cause anemia?
  • What about acute (sudden) blood loss as a cause of anemia?
  • What are other causes of anemia?
  • Can anemia be hereditary?
  • What are the symptoms of anemia?
  • How is anemia diagnosed?
  • What is a complete blood cell (CBC) count?
  • How is blood collected for a complete blood cell (CBC)?
  • What is the red blood cell (RBC) count?
  • What is hemoglobin?
  • What does a low hemoglobin level mean?
  • What is the hematocrit?
  • How is hematocrit determined?
  • How is anemia treated?
  • What are the complications of anemia?
  • What is the outlook (prognosis) for anemia?
  • Patient Discussions: Anemia - Treatments
  • Patient Discussions: Anemia - Symptoms Experienced
  • Find a local Hematologist in your town

What is anemia?

Anemia is a medical condition in which the red blood cell count or hemoglobin is less than normal. The normal level of hemoglobin is generally different in males and females. For men, anemia is typically defined as hemoglobin level of less than 13.5 gram/100ml and in women as hemoglobin of less than 12.0 gram/100ml. These definitions may vary slightly depending on the source and the laboratory reference used.

 

What causes anemia?

 

Any process that can disrupt the normal life span of a red blood cell may cause anemia. Normal life span of a red blood cell is typically around 120 days. Red blood cells are made in the bone marrow.

Anemia is caused essentially through two basic pathways. Anemia is either caused:

  1. by a decrease in production of red blood cell or hemoglobin, or
  2. by a loss or destruction of blood.

As more common classifications of anemia (low hemoglobin) is based on the MCV, or the volume of individual red blood cells.

  1. If the MCV is low (less than 80), the anemia is categorized as microcytic anemia (low cell volume).
  2. If the MCV is in the normal range (80-100), it is called a normocytic anemia (normal cell volume).
  3. If the MCV is high, then it is called a macrocytic anemia (large cell volume).

Looking at each of the components of a complete blood count (CBC), especially the MCV, a physician can gather clues as what may be the most common reason for anemia.

 

Picture of Red Blood Cells

Can not enough iron cause anemia?

Absolutely! This is because iron is major component of hemoglobin and essential for its proper function. Chronic blood loss due to any reason is the main cause of low iron level in the body as it depletes the body's iron stores to compensate for the ongoing loss of iron. Anemia that is due to low iron levels is called iron deficiency anemia. Iron deficiency it is a very common cause of anemia.

Women are more likely than men to have iron deficiency anemia because of the loss of blood each month through normal menstruation. This is generally without any major symptoms as the blood loss is relatively small and temporary.

Iron deficiency anemia can also be due to small repeated l bleeding, for instance from colon cancer or from stomach ulcers. Stomach ulcer bleeding that may or may no be induced by medications even very common over-the-counter drugs as aspirin and ibuprofen (Advil, Motrin). In infants and young children, iron deficiency anemia is most often due to a diet lacking iron.

Interpretation of CBC may lead to clues to suggest this type of anemia. For instance, iron deficiency anemia usually presents with low mean corpuscular volume (microcytic anemia) in addition to low hemoglobin.

What about acute (sudden) blood loss as a cause of anemia?

Acute blood loss from internal bleeding (as from a bleeding ulcer) or external bleeding (as from trauma) can produce anemia in an amazingly short span of time. This type of anemia could result in severe symptoms and consequences if not addressed promptly.

What are other causes of anemia?

Some of the most common causes include:

  • Vitamin B12 deficiency may cause pernicious anemia. This type of anemia could happen in people who are unable to absorb vitamin B12 from their intestines due to a number of reasons:
    • strict vegetarians who may not be taking adequate vitamin supplements, or
    • long-term alcoholics.

     

This typically causes macrocytic (large cell volume) anemia. Vitamin B12, along with folate, is a involved in making the heme molecule that is an integral part of hemoglobin. Folate deficiency can be the culprit of anemia. This may also be caused by inadequate absorption, under-consumption of green, leafy vegetables, and also long-term heavy alcohol use.

  • There can be rupture of red blood cells (hemolytic anemia) due to antibodies clinging to the surface of the red cells (for example, hemolytic disease of the newborn and in many other conditions).
  • A wide assortment of bone marrow diseases can cause anemia.

     

    • For example, cancers that spread (metastasize) to the bone marrow, or cancers of the bone marrow (such as leukemia or multiple myeloma) can cause the bone marrow to inadequately produce red blood cells, resulting in anemia.
    • Certain chemotherapy for cancers can also cause damage to the bone marrow and decrease red blood cell production, resulting in anemia.
    • Certain infections may involve the bone marrow and result in bone marrow impairment and anemia.
    • Finally, patients with kidney failure may lack the hormone necessary to stimulate normal red blood cell production by the bone marrow.
  • Another common cause of anemia is called anemia of chronic disease. This could typically occur in individuals with long-standing chronic diseases.
  • Some medications can cause anemia in a variety of ways.
  • Human immunodeficiency virus (HIV) and acquired immune deficiency syndrome (AIDS) can cause anemia.

Can anemia be hereditary?

 

Yes, anemia may be genetic. Hereditary disorders can shorten the life-span of the red blood cell and lead to anemia (for example, sickle cell anemia). Hereditary disorders can also cause anemia by impairing the production of hemoglobin (for example, alpha thalassemia and beta thalassemia).

Depending on the degree of the genetic abnormality, hereditary anemias may cause mild, moderate, or severe anemia. In fact, some may be too severe to be compatible with life and may result in death of the fetus (unborn infant). On the other hand, some of these anemias are so mild that they are not noticeable and are incidentally revealed during a routine blood work.

What are the symptoms of anemia?

Some patients with anemia have no symptoms. Others with anemia may feel:

  • tired,
  • fatigue easily,
  • appear pale,
  • develop palpitations (feeling of heart racing), and
  • become short of breath.

Additional symptoms may include:

  • hair loss,
  • malaise (general sense of feeling unwell), and
  • worsening of heart problems.

It is worth noting that if anemia is longstanding (chronic anemia), the body may adjust to low oxygen levels and the individual may not feel different unless the anemia becomes severe. On the other hand, if the anemia occurs rapidly (acute anemia), the patient may experience significant symptoms relatively quickly.

How is anemia diagnosed?

Anemia is usually detected or at least confirmed by a complete blood cell (CBC) count. CBC test may be ordered by a physician as a part of routine general check-up and screening or based on clinical signs and symptoms that may suggest anemia or other blood abnormalities.

What is a complete blood cell (CBC) count?

A CBC is a test for counting and examining the different types of cells in the blood. Traditionally, CBC analysis was performed by a physician or a laboratory technician by viewing a glass slide prepared from a blood sample under a microscope. Today, much of this work is often automated and done by machines. Six component measurements make up a CBC test:

  1. Red blood cell (RBC) count
  2. Hematocrit
  3. Hemoglobin
  4. White blood cell (WBC) count
  5. Differential blood count (the "diff")
  6. Platelet count

Only the first three of these tests: the red blood cell (RBC) count, the hematocrit, and the hemoglobin, are relevant to the diagnosis of anemia.

Additionally, mean corpuscular volume (MCV) is also often reported in a CBC, which basically measures the average volume of red blood cells in a blood sample. This is important in distinguishing the causes of anemia. Units of MCV are reported in femtoliters, a fraction of one millionth of a liter.

Other useful clues to causes of anemia that are reported in a CBC are the size, shape, and color of red blood cells.

Picture of Red Blood Cells

How is blood collected for a CBC?

Blood is collected by venipuncture (using a needle to draw blood from a vein) in a lab, hospital, or physician's office. Typically, blood is collected in a special sterile tube from an arm vein. The tube has some preservatives to prevent clotting of the blood. Results may be available in an hour or longer depending on the setting.

In some instances, a quick in office test called hemoglobin rapid test may be performed using a few drops of blood from a finger prick. The advantage of this quick test is that results may be obtained in a few minutes and only a few drops of blood may be required.

What is the red blood cell (RBC) count?

The red blood cells (RBCs or erythrocytes) are the most common type of cells in the blood. We each have millions and millions of these little disc-shaped cells. The RBC count is done to determine if the number of red blood cells is low (anemia) or high (polycythemia).

In an RBC count, the number and size of the RBCs are determined. This is usually reported as number of RBCs per a specified volume, typically in millions of RBC's in micro-liters (one one-thousandth of an ml) of whole blood. The shape of the red blood cells is also evaluated under a microscope. All of this information, the number, size and shape of the RBCs, is useful in the diagnosis of anemia. Further, the specific type of anemia may be determined by this information.

What is hemoglobin?

Hemoglobin is a red pigment that imparts the familiar red color to red blood cells and to blood. Functionally, hemoglobin is the key chemical compound that combines with oxygen from the lungs and carries the oxygen from the lungs to cells throughout the body. Oxygen is essential for all cells in the body to produce energy.

The blood also transports carbon dioxide, which is the waste product of this energy production process, back to the lungs from which it is exhaled into the air. The transport of the carbon dioxide back to the lung is also achieved by hemoglobin. The carbon dioxide bound to hemoglobin is unloaded in the lungs in exchange for oxygen to be transported to the tissues of the body.

What does a low hemoglobin level mean?

Low hemoglobin is called anemia. When there is a low hemoglobin level, there is often a low red blood cell count and a low hematocrit, too. Reference ranges are slightly different from one source to another, but typically hemoglobin of less than 13.5 is abnormal in men less than 12.0 in women.

What is the hematocrit?

The hematocrit is specifically a measure of how much of the blood is made of red cells. The hematocrit is a very convenient way to determine whether the red blood cell count is too high, too low, or normal. The hematocrit is a measure of the proportion of blood that is composed of the red blood cells.

How is hematocrit determined?

 

The RBCs in the sample of blood are packed down by spinning the tube in a centrifuge under prescribed conditions. The proportion of the tube that consists of RBCs is then measured. Let's say that it is 45%. The hematocrit is 45.

How is anemia treated?

The treatment of the anemia varies greatly. First, the underlying cause of the anemia should be identified and corrected. For example, anemia as a result of blood loss from a stomach ulcer should begin with medications to heal the ulcer. Likewise, surgery is often necessary to remove a colon cancer that is causing chronic blood loss and anemia.

Sometimes iron supplements will also be needed to correct iron deficiency. In severe anemia, blood transfusions may be necessary. Vitamin B12 injections will be necessary for patients suffering from pernicious anemia or other causes of B12 deficiency.

In certain patients with bone marrow disease (or bone marrow damage from chemotherapy) or patients with kidney failure, epoetin alfa (Procrit, Epogen) may be used to stimulate bone marrow red blood cell production.

 

If a medication is thought to be the culprit, then it should be discontinued under the direction of the prescribing doctor.

What are the complications of anemia?

As mentioned earlier, hemoglobin has the important role of delivering oxygen to all parts of the body for consumption and carries back carbon dioxide back to the lung to exhale it out of the body. If hemoglobin level is too low, this process may be impaired, resulting in body having low oxygen level (hypoxia).

What is the outlook (prognosis) for anemia?

 

Anemia generally has a very good prognosis and it may be curable in many instances. The overall prognosis depends on the underlying cause of anemia, its severity, and the overall health of the patient.

 

Additional resources from WebMD Boots UK on Anemia


Spina Bifida
(Neural Tube Defect)

 

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  • Introduction to spina bifida
  • What is spina bifida?
  • What are the different types of spina bifida?
  • What causes spina bifida?
  • What are the signs and symptoms of spina bifida?
  • What are the complications of spina bifida?
  • How is spina bifida diagnosed?
  • How is spina bifida treated?
  • Can spina bifida be prevented?
  • What is the prognosis for spina bifida?
  • What research is being done on spina bifida?
  • Where can I get more information?

Introduction to spina bifida

The human nervous system develops from a small, specialized plate of cells along the back of an embryo. Early in development, the edges of this plate begin to curl up toward each other, creating the neural tube—a narrow sheath that closes to form the brain and spinal cord of the embryo. As development progresses, the top of the tube becomes the brain and the remainder becomes the spinal cord. This process is usually complete by the 28th day of pregnancy. But if problems occur during this process, the result can be brain disorders called neural tube defects, including spina bifida.

What is spina bifida?

Spina bifida, which literally means "cleft spine," is characterized by the incomplete development of the brain, spinal cord, and/or meninges (the protective covering around the brain and spinal cord). It is the most common neural tube defect in the United States - affecting 1,500 to 2,000 of the more than 4 million babies born in the country each year.

What are the different types of spina bifida?

There are four types of spina bifida:

  1. occulta,
  2. closed neural tube defects,
  3. meningocele, and
  4. myelomeningocele.

Occulta is the mildest and most common form in which one or more vertebrae are malformed. The name "occulta," which means "hidden," indicates that the malformation, or opening in the spine, is covered by a layer of skin. This form of spina bifida rarely causes disability or symptoms.

Closed neural tube defects make up the second type of spina bifida. This form consists of a diverse group of spinal defects in which the spinal cord is marked by a malformation of fat, bone, or membranes. In some patients there are few or no symptoms; in others the malformation causes incomplete paralysis with urinary and bowel dysfunction.

In the third type, meningocele, the meninges protrude from the spinal opening, and the malformation may or may not be covered by a layer of skin. Some patients with meningocele may have few or no symptoms while others may experience symptoms similar to closed neural tube defects.

Myelomeningocele, the fourth form, is the most severe and occurs when the spinal cord is exposed through the opening in the spine, resulting in partial or complete paralysis of the parts of the body below the spinal opening. The paralysis may be so severe that the affected individual is unable to walk and may have urinary and bowel dysfunction.

What causes spina bifida?

The exact cause of spina bifida remains a mystery. No one knows what disrupts complete closure of the neural tube, causing a malformation to develop. Scientists suspect genetic, nutritional, and environmental factors play a role. Research studies indicate that insufficient intake of folic acid - a common B vitamin - in the mother's diet is a key factor in causing spina bifida and other neural tube defects. Prenatal vitamins that are prescribed for the pregnant mother typically contain folic acid as well as other vitamins.

What are the signs and symptoms of spina bifida?

The symptoms of spina bifida vary from person to person, depending on the type. Often, individuals with occulta have no outward signs of the disorder. Closed neural tube defects are often recognized early in life due to an abnormal tuft or clump of hair or a small dimple or birthmark on the skin at the site of the spinal malformation.

Meningocele and myelomeningocele generally involve a fluid-filled sac—visible on the back—protruding from the spinal cord. In meningocele, the sac may be covered by a thin layer of skin, whereas in most cases of myelomeningocele, there is no layer of skin covering the sac and a section of spinal cord tissue usually is exposed.

What are the complications of spina bifida?

Complications of spina bifida can range from minor physical problems to severe physical and mental disabilities. It is important to note, however, that most people with spina bifida are of normal intelligence. Severity is determined by the size and location of the malformation, whether or not skin covers it, whether or not spinal nerves protrude from it, and which spinal nerves are involved. Generally all nerves located below the malformation are affected. Therefore, the higher the malformation occurs on the back, the greater the amount of nerve damage and loss of muscle function and sensation.

In addition to loss of sensation and paralysis, another neurological complication associated with spina bifida is Chiari II malformation—a rare condition (but common in children with myelomeningocele) in which the brainstem and the cerebellum, or rear portion of the brain, protrude downward into the spinal canal or neck area. This condition can lead to compression of the spinal cord and cause a variety of symptoms including difficulties with feeding, swallowing, and breathing; choking; and arm stiffness.

Chiari II malformation may also result in a blockage of cerebrospinal fluid, causing a condition called hydrocephalus, which is an abnormal buildup of cerebrospinal fluid in the brain. Cerebrospinal fluid is a clear liquid that surrounds the brain and spinal cord. The buildup of fluid puts damaging pressure on the brain. Hydrocephalus is commonly treated by surgically implanting a shunt—a hollow tube—in the brain to drain the excess fluid into the abdomen.

Some newborns with myelomeningocele may develop meningitis, an infection in the meninges. Meningitis may cause brain injury and can be life-threatening.

Children with both myelomeningocele and hydrocephalus may have learning disabilities, including difficulty paying attention, problems with language and reading comprehension, and trouble learning math.

Additional problems such as latex allergies, skin problems, gastrointestinal conditions, and depression may occur as children with spina bifida get older.

How is spina bifida diagnosed?

In most cases, spina bifida is diagnosed prenatally, or before birth. However, some mild cases may go unnoticed until after birth, or postnatally. Very mild cases, in which there are no symptoms, may never be detected.

Prenatal Diagnosis

The most common screening methods used to look for spina bifida during pregnancy are second trimester maternal serum alpha fetoprotein (MSAFP) screening and fetal ultrasound. The MSAFP screen measures the level of a protein called alpha-fetoprotein (AFP), which is made naturally by the fetus and placenta. During pregnancy, a small amount of AFP normally crosses the placenta and enters the mother's bloodstream. But if abnormally high levels of this protein appear in the mother's bloodstream it may indicate that the fetus has a neural tube defect. The MSAFP test, however, is not specific for spina bifida, and the test cannot definitively determine that there is a problem with the fetus. If a high level of AFP is detected, the doctor may request additional testing, such as an ultrasound or amniocentesis to help determine the cause.

The second trimester MSAFP screen described above may be performed alone or as part of a larger, multiple-marker screen. Multiple-marker screens look not only for neural tube defects, but also for other birth defects, including Down syndrome and other chromosomal abnormalities. First trimester screens for chromosomal abnormalities also exist but signs of spina bifida are not evident until the second trimester when the MSAFP screening is performed.

Amniocentesis—an exam in which the doctor removes samples of fluid from the amniotic sac that surrounds the fetus—may also be used to diagnose spina bifida. Although amniocentesis cannot reveal the severity of spina bifida, finding high levels of AFP may indicate that the disorder is present.

Postnatal Diagnosis

Mild cases of spina bifida not diagnosed during prenatal testing may be detected postnatally by X-ray during a routine examination. Doctors may use magnetic resonance imaging (MRI) or a computed tomography (CT) scan to get a clearer view of the spine and vertebrae. Individuals with the more severe forms of spina bifida often have muscle weakness in their feet, hips, and legs. If hydrocephalus is suspected, the doctor may request a CT scan and/or X-ray of the skull to look for extra fluid inside the brain.

How is spina bifida treated?

There is no cure for spina bifida. The nerve tissue that is damaged or lost cannot be repaired or replaced, nor can function be restored to the damaged nerves. Treatment depends on the type and severity of the disorder. Generally, children with the mild form need no treatment, although some may require surgery as they grow.

The key priorities for treating myelomeningocele are to prevent infection from developing through the exposed nerves and tissue of the defect on the spine, and to protect the exposed nerves and structures from additional trauma. Typically, a child born with spina bifida will have surgery to close the defect and prevent infection or further trauma within the first few days of life.

Doctors have recently begun performing fetal surgery for treatment of myelomeningocele. Fetal surgery—which is performed in utero (within the uterus)—involves opening the mother's abdomen and uterus and sewing shut the opening over the developing baby's spinal cord. Some doctors believe the earlier the defect is corrected, the better the outcome is for the baby. Although the procedure cannot restore lost neurological function, it may prevent additional loss from occurring. However, the surgery is considered experimental and there are risks to the fetus as well as to the mother.

The major risks to the fetus are those that might occur if the surgery stimulates premature delivery such as organ immaturity, brain hemorrhage, and death. Risks to the mother include infection, blood loss leading to the need for transfusion, gestational diabetes, and weight gain due to bed rest.

Still, the benefits of fetal surgery are promising, and include less exposure of the vulnerable spinal nerve tissue and bones to the intrauterine environment, in particular the amniotic fluid, which is considered toxic. As an added benefit, doctors have discovered that the procedure affects the way the brain develops in the uterus, allowing certain complications—such as Chiari II with associated hydrocephalus—to correct themselves, thus, reducing or, in some cases, eliminating the need for surgery to implant a shunt.

Many children with myelomeningocele develop a condition called progressive tethering, or tethered cord syndrome, in which their spinal cords become fastened to an immovable structure—such as overlying membranes and vertebrae—causing the spinal cord to become abnormally stretched and the vertebrae elongated with growth and movement. This condition can cause loss of muscle function to the legs, bowel, and bladder. Early surgery on the spinal cord may allow the child to regain a normal level of functioning and prevent further neurological deterioration.

Some children will need subsequent surgeries to manage problems with the feet, hips, or spine. Individuals with hydrocephalus generally will require additional surgeries to replace the shunt, which can be outgrown or become clogged.

Some individuals with spina bifida require assistive devices such as braces, crutches, or wheelchairs. The location of the malformation on the spine often indicates the type of assistive devices needed. Children with a defect high on the spine and more extensive paralysis will often require a wheelchair, while those with a defect lower on the spine may be able to use crutches, bladder catherizations, leg braces, or walkers.

Treatment for paralysis and bladder and bowel problems typically begins soon after birth, and may include special exercises for the legs and feet to help prepare the child for walking with braces or crutches when he or she is older.

Can spina bifida be prevented?

Folic acid, also called folate, is an important vitamin in the development of a healthy fetus. Although taking this vitamin cannot guarantee having a healthy baby, it can help. Recent studies have shown that by adding folic acid to their diets, women of childbearing age significantly reduce the risk of having a child with a neural tube defect, such as spina bifida. Therefore, it is recommended that all women of childbearing age consume 400 micrograms of folic acid daily. Foods high in folic acid include dark green vegetables, egg yolks, and some fruits. Many foods—such as some breakfast cereals, enriched breads, flours, pastas, rice, and other grain products—are now fortified with folic acid. A lot of multivitamins contain the recommended dosage of folic acid as well.

Women who have a child with spina bifida, have spina bifida themselves, or have already had a pregnancy affected by any neural tube defect are at greater risk of having a child with spina bifida or another neural tube defect. These women may require more folic acid before they become pregnant.

What is the prognosis for spina bifida?

Children with spina bifida can lead relatively active lives. Prognosis depends on the number and severity of abnormalities and associated complications. Most children with the disorder have normal intelligence and can walk, usually with assistive devices. If learning problems develop, early educational intervention is helpful.

What research is being done on spina bifida?

Within the Federal Government, the National Institute of Neurological Disorders and Stroke (NINDS), a component of the National Institutes of Health (NIH), supports and conducts research on brain and nervous system disorders, including spina bifida. NINDS conducts research in its laboratories at the NIH in Bethesda, Maryland, and supports research through grants to major medical institutions across the country.

In one study supported by NINDS, scientists are looking at the hereditary basis of neural tube defects. The goal of this research is to find the genetic factors that make some children more susceptible to neural tube defects than others. Lessons learned from this research will fill in gaps of knowledge about the causes of neural tube defects and may lead to ways to prevent these disorders. These researchers are also studying gene expression during the process of neural tube closure, which will provide information on the human nervous system during development.

In addition, NINDS-supported scientists are working to identify, characterize, and evaluate genes for neural tube defects. The goal is to understand the genetics of neural tube closure, and to develop information that will translate into improved clinical care, treatment, and genetic counseling.

Other scientists are studying genetic risk factors for spina bifida, especially those that diminish or lessen the function of folic acid in the mother during pregnancy, possibly leading to spina bifida in the fetus. This study will shed light on how folic acid prevents spina bifida and may lead to improved forms of folate supplements.

NINDS also supports and conducts a wide range of basic research studies to understand how the brain and nervous system develop. These studies contribute to a greater understanding of neural tube defects, such as spina bifida, and offer hope for new avenues of treatment for and prevention of these disorders as well as other birth defects.

Another component of the NIH, the National Institute of Child Health and Human Development (NICHD), is conducting a large 5-year study to determine if fetal surgery to correct spina bifida in the womb is safer and more effective than the traditional surgery—which takes place a few days after birth. Researchers hope this study, called the Management of Myelomeningocele Study, or MOMS, will better establish which procedure, prenatal or postnatal, is best for the baby.

Where can I get more information?

For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke,


The Heart and Vascular Disease

 

  • Peripheral artery disease
  • Aneurysm
  • Renal (kidney) artery disease
  • Raynaud's Phenomenon
  • Buerger's Disease
  • Peripheral venous disease
  • Varicose veins
  • Blood clots in the veins
  • Blood clotting disorders
  • Lymphedema
  • Find a local Cardiologist in your town

Vascular disease includes any condition that affects the circulatory system. As the heart beats, it pumps blood through a system of blood vessels called the circulatory system. The vessels are elastic tubes that carry blood to every part of the body. Arteries carry blood away from the heart while veins return it.

Vascular disease ranges from diseases of your arteries, veins, and lymph vessels to blood disorders that affect circulation. The following are conditions that fall under the category of vascular disease.

 

Peripheral Artery Disease

Like the blood vessels of the heart (coronary arteries), your peripheral arteries (blood vessels outside your heart) also may develop atherosclerosis, the build-up of fat and cholesterol deposits, called plaque, on the inside walls. Over time, the build-up narrows the artery. Eventually the narrowed artery causes less blood to flow and a condition called "ischemia" can occur. Ischemia is inadequate blood flow to the body's tissue.

  • A blockage in the coronary arteries can cause symptoms of chest pain (angina) or a heart attack.
  • A blockage in the carotid arteries (the arteries supplying the brain) can lead to a transient ischemic attack (TIA) or stroke.
  • A blockage in the legs can lead to leg pain or cramps with activity (a condition called claudication), changes in skin color, sores or ulcers, and feeling tired in the legs. Total loss of circulation can lead to gangrene and loss of a limb.
  • A blockage in the renal arteries (arteries supplying the kidneys) can cause renal artery disease (stenosis). The symptoms include uncontrolled hypertension (high blood pressure), heart failure, and abnormal kidney function.

Aneurysm

An aneurysm is an abnormal bulge in the wall of a blood vessel. They can form in any blood vessel, but they occur most commonly in the aorta (aortic aneurysm) which is the main blood vessel leaving the heart. The two types of aortic aneurysm are:

  • Thoracic aortic aneurysm (part of aorta in the chest)
  • Abdominal aortic aneurysm

Small aneurysms generally pose no threat. However, one is at increased risk for:

  • Atherosclerotic plaque (fat and calcium deposits) formation at the site of the aneurysm.
  • A clot (thrombus) may form at the site and dislodge.
  • Increase in the aneurysm size, causing it to press on other organs, causing pain.
  • Aneurysm rupture -- because the artery wall thins at this spot, it is fragile and may burst under stress. A sudden rupture of an aortic aneurysm may be life threatening.

Renal (Kidney) Artery Disease

Renal artery disease is most commonly caused by atherosclerosis of the renal arteries (see above). It occurs in people with generalized vascular disease. Less often, renal artery disease can be caused by a congenital (present at birth) abnormal development of the tissue that makes up the renal arteries. This type of renal artery disease occurs in younger age groups.

Raynaud's Phenomenon (Also Called Raynaud's Disease or Raynaud's Syndrome)

Raynaud's phenomenon consists of spasms of the small arteries of the fingers and sometimes the toes, brought on by exposure to cold or excitement. Certain occupational exposures bring on Raynaud's. The episodes produce temporary lack of blood supply to the area, causing the skin to appear white or bluish and cold or numb. In some cases, the symptoms of Raynaud's may be related to underlying diseases (ie, lupus, rheumatoid arthritis, scleroderma).

Buerger's Disease

Buerger's disease most commonly affects the small and medium sized arteries, veins, and nerves. Although the cause is unknown, there is a strong association with tobacco use or exposure. The arteries of the arms and legs become narrowed or blocked, causing lack of blood supply (ischemia) to the fingers, hands, toes, and feet. Pain occurs in the arms, hands and, more frequently, the legs and feet, even when at rest. With severe blockages, the tissue may die (gangrene), requiring amputation of the fingers and toes.

Superficial vein inflammation and symptoms of Raynaud's occur commonly in patients with Buerger's disease.

Peripheral Venous Disease

Veins are flexible, hollow tubes with flaps inside called valves. When your muscles contract, the valves open and blood moves through the veins. When your muscles relax, the valves close, keeping blood flowing in one direction through the veins.

If the valves inside your veins become damaged, the valves may not close completely. This allows blood to flow in both directions. When your muscles relax, the valves inside the damaged vein(s) will not be able to hold the blood. This can cause pooling of blood or swelling in the veins. The veins bulge and appear as ropes under the skin. The blood begins to move more slowly through the veins, it may stick to the sides of the vessel walls and blood clots can form.

 

Varicose Veins

Varicose veins are bulging, swollen, purple, ropy veins, seen just under your skin, caused by damaged valves within the veins. They are more common in women than men and often run in families. They can also be caused by pregnancy, being severely overweight, or by standing for long periods of time. The symptoms of varicose veins include:

  • Bulging, swollen, purple, ropy, veins seen under the skin.
  • Spider veins -- small red or purple bursts on your knees, calves, or thighs, caused by swollen capillaries (small blood vessels).
  • Aching, stinging, or swelling of the legs at the end of the day.

Blood Clots In the Veins

Blood clots in the veins are usually caused by:

  • Long bed rest and/or immobility.
  • Damage to veins from injury or infection.
  • Damage to the valves in the vein, causing pooling near the valve flaps.
  • Pregnancy and hormones (such as estrogen or birth control pills).
  • Genetic disorders.
  • Conditions causing slowed blood flow or thicker blood, such as congestive heart failure (CHF), or certain tumors.

There are many types of blood clots that can occur in the veins:

  • Deep vein thrombosis (DVT) is a blood clot occurring in a deep vein.
  • Pulmonary embolism is a blood clot that breaks loose from a vein and travels to the lungs.
  • Chronic venous insufficiency isn't a blood clot, but a condition that occurs when damaged vein valves or a DVT causes long-term pooling of blood and swelling in the legs. If uncontrolled, fluid will leak into the surrounding tissues in the ankles and feet, and may eventually cause skin breakdown and ulceration.

Blood Clotting Disorders

Blood clotting disorders are conditions that make the blood more likely to form blood clots in the arteries and veins. These conditions may be inherited (congenital, occurring at birth) or acquired during life and include:

  • Elevated levels of factors in the blood which cause blood to clot (fibrinogen, factor 8, prothrombin).
  • Deficiency of natural anticoagulant (blood-thinning) proteins (antithrombin, protein C, protein S).
  • Elevated blood counts.
  • Abnormal fibrinolysis (the breakdown of fibrin).
  • Abnormal changes in the lining of the blood vessels (endothelium).

Lymphedema

The lymphatic system is a circulatory system that includes an extensive network of lymph vessels and lymph nodes. The lymphatic system helps coordinate the immune system's function to protect the body from foreign substances.

Lymphedema is an abnormal build-up of fluid that causes swelling, most often in the arms or legs. Lymphedema develops when lymph vessels or lymph nodes are missing, impaired, damaged, or removed.

Primary lymphedema is rare and is caused by the absence of certain lymph vessels at birth, or it may be caused by abnormalities in the lymphatic vessels.

Secondary lymphedema occurs as a result of a blockage or interruption that alters the lymphatic system. Secondary lymphedema can develop from an infection, malignancy, surgery, scar tissue formation, trauma, deep vein thrombosis (DVT), radiation, or other cancer treatment.


Brain Aneurysm
(Cerebral Aneurysm)

 

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Medical Author: Benjamin C. Wedro, MD, FAAEM
Medical Editor: Melissa Conrad Stöppler, MD

  • What is a brain aneurysm and what causes a brain aneurysm?
  • What are the symptoms of brain aneurysm?
  • How is brain aneurysm diagnosed?
  • What is the treatment for brain aneurysm?
  • What is the outcome of brain aneurysm?
  • What are future directions for the treatment of brain aneurysm?
  • Patient Discussions: Aneurysm (Brain) - Symptoms and Signs
  • Patient Discussions: Brain Aneurysm - Describe Your Experience

What is a brain aneurysm and what causes a brain aneurysm?

The brain gets its blood supply from arteries known as the Circle of Willis. It is located at the base of the brain and is a loop of arteries that join in a circle then send branches out to all parts of the brain. These arteries deliver nutrition (glucose and oxygen) to the brain cells.

The junctions where these arteries come together can form weak spots. These weak spots can balloon out and fill with blood, creating the outpouchings of blood vessels known as aneurysms. These sac-like areas may leak or rupture spilling blood into surrounding tissues. While the prevailing thought has been that aneurysms are congenital (meaning that people are born with them), it is now thought that they are due to microscopic damage to the artery walls caused by abnormal flow at the junction points where the arteries come together.

There are other rare causes of aneurysms. Mycotic aneurysms are cause by infections of the artery wall. Tumors and trauma can also cause aneurysms to form. Drug abuse, especially cocaine, can cause the artery walls to inflame and weaken.

Brain aneurysms (aneurysms within arteries in the brain) are a common occurrence. At autopsy, incidental asymptomatic aneurysms are found in more than 1% of people. Most aneurysms remain small and never become an issue or are diagnosed. Some, however, may gradually get larger and exert pressure on surrounding brain tissue and nerves and may be diagnosed because of facial symptoms such as:

  • headache,
  • numbness, or weakness of one side of the face,
  • a dilated pupil, or
  • change in vision.

The greater concern is a brain aneurysm that leaks or ruptures, and potentially causes stroke or death. Bleeding leaks into one of the membranes that covers the brain and spinal canal and is known as a subarachnoid hemorrhage (sub= beneath + arachnoid=one of the brain coverings + hemorrhage=bleeding).

What are the symptoms of brain aneurysm?

The headache associated with a leaking aneurysm is severe. Blood is very irritating to the brain and causes significant pain. Patients may describe the "worst headache of their life," and the healthcare provider needs to have an appreciation of brain aneurysm as a potential cause of this type of pain. The headache may be associated with nausea, vomiting, and change in vision. A subarachnoid hemorrhage also causes pain and stiffness of the neck.

How is brain aneurysm diagnosed?

The diagnosis of brain aneurysm begins with a high index of suspicion by the healthcare provider. The history of the headache, an acute onset of the worst headache of the patient's life, associated with a stiff neck and an ill-appearing patient on physical examination, typically lead the healthcare provider to order a CT (computerized tomography) scan of the head. This will show a subarachnoid hemorrhage in more than 90% of cases of ruptured aneurysm. In the few cases that are not recognized by CT, the healthcare provider may consider performing a lumbar puncture (LP, or spinal tap) to identify blood in the cerebrospinal fluid that runs in the subarachnoid space.

If the CT or the LP reveals the presence of blood, angiography is performed to identify where the aneurysm is located and to plan treatment. Angiography (angio=artery +graphy= picture) is a procedure in which a small flexible tube is threaded into one of the brain's arteries, and dye is injected while pictures are taken. Newer technology allows angiography to be done in association with CT or magnetic resonance imaging (MRI).

Though the symptoms may suggest a brain aneurysm, other diagnoses may need to be considered. Migraine headache, meningitis, tumor, and stroke all may cause neurologic symptoms. Based on the patient's presentation, the healthcare provider will need to decide which tests and studies to use to establish the correct diagnosis.

What is the treatment for brain aneurysm?

Treatment for a symptomatic aneurysm is to repair the blood vessels. Clipping and coiling are two treatment options.

  • Clipping: A neurosurgeon can operate on the brain by cutting open the skull, identifying the damaged blood vessel and putting a clip across the aneurysm. This prevents blood from entering the aneurysm and causing further growth or blood leakage.
  • Coiling: An interventional radiologist can thread a tube through the arteries, as with an angiogram, identify the aneurysm, and fill it with coils of platinum wire or with latex. This prevents further blood from entering the aneurysm.

Both these options have the risk of damaging the blood vessel and causing more bleeding, damaging nearby brain tissue, and causing the surrounding blood vessels to go into spasm; depriving brain tissue of blood supply and causing a stroke.

Prior, during, and after surgery, attention is paid to protect the brain and its blood vessels from potential further damage. Vitals signs are monitored frequently, and heart monitors are used to watch for abnormal heart rhythms. Medications may be used to prevent blood vessel spasm, seizure, agitation, and pain.

What is the outcome of brain aneurysm?

Brain aneurysms are deadly. About 10% of patients with a ruptured aneurysm die before receiving medical care. If untreated, another 50% will die within a month, with 25% of patients sustaining another bleeding episode within a week. Aside from the bleeding issues, there is significant risk of artery spasm leading to stroke.

Survival rates are increased by early presentation to the hospital, early aneurysm repair, and control of potential blood vessel spasm with medications.

What are future directions for the treatment of brain aneurysm?

For those who survive an initial aneurysm rupture, blood vessel spasm (vasospasm) may be the villain in causing continued brain damage. Experiments to develop new drugs to control vasospasm are ongoing. Molecules that can cause spasm are being identified, and antibodies may be able to be produced to blunt their effect.

Studies are also looking at the possibility that brain aneurysms may be hereditary, and perhaps screening of high-risk populations may be possible in the future.


Abdominal Aortic Aneurysm

 

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Medical Revising Authors: Benjamin C. Wedro, MD, FAAEM and Dennis Lee, MD
Medical Revising Editor: William C. Shiel, Jr, MD, FACP, FACR

  • What is an aneurysm?
  • What is an aortic aneurysm?
  • What are the thoracic and abdominal aorta?
  • Where do aortic aneurysms tend to develop?
  • What shape are most aortic aneurysms?
  • What's inside an aortic aneurysm?
  • Who is most likely to have an abdominal aortic aneurysm?
  • What are risk factors of aortic aneurysms?
  • What is the most common cause of aortic aneurysms?
  • What are other causes of aortic aneurysms?
  • What are the symptoms of an abdominal aortic aneurysm?
  • How is an abdominal aortic aneurysm diagnosed clinically?
  • What tests help in the diagnosis of an abdominal aortic aneurysm?
  • What is the natural history of abdominal aortic aneurysm?
  • What are the complications with an abdominal aortic aneurysm?
  • How are abdominal aortic aneurysms repaired?
  • What is done if an abdominal aortic aneurysm threatens to rupture?
  • What is the medical management (non-surgical management) of abdominal aortic aneurysm?
  • Abdominal Aortic Aneurysm At A Glance
  • Patient Discussions: Abdominal Aortic Aneurysm - Treatments
  • Patient Discussions: Abdominal Aortic Aneurysm - Describe Your Experience

What is an aneurysm?

An aneurysm is an area of a localized widening (dilation) of a blood vessel. (The word "aneurysm" is borrowed from the Greek "aneurysma" meaning "a widening").

What is an aortic aneurysm?

An aortic aneurysm involves the aorta, one of the large arteries through which blood passes from the heart to the rest of the body. The aorta bulges at the site of the aneurysm like a weak spot on an old worn tire.

What are the thoracic and abdominal aorta?

The aorta is first called the thoracic aorta as it leaves the heart, ascends, arches, and descends through the chest until it reaches the diaphragm (the partition between the thorax and abdomen). The aorta is then called the abdominal aorta after it has passed the diaphragm and continues down the abdomen. The abdominal aorta ends where it splits to form the two iliac arteries that go to the legs.

Where do aortic aneurysms tend to develop?

Aortic aneurysms can develop anywhere along the length of the aorta. The majority, however, are located along the abdominal aorta. Most (about 90%) of abdominal aneurysms are located below the level of the renal arteries, the vessels that leave the aorta to go to the kidneys. About two-thirds of abdominal aneurysms are not limited to just the aorta but extend from the aorta into one or both of the iliac arteries.

What shape are most aortic aneurysms?

Most aortic aneurysms are fusiform. They are shaped like a spindle ("fusus" means spindle in Latin) with widening all around the circumference of the aorta. (Saccular aneurysms just involve a portion of the aortic wall with a localized out pocketing).

What's inside an aortic aneurysm?

The inside walls of aneurysms are often lined with a laminated blood clot that is layered like a piece of plywood.

Who is most likely to have an abdominal aortic aneurysm?

Aortic aneurysms are most common after 60 years of age. Males are five times more likely than females to be affected. Approximately 5% of men over age 60 develop an abdominal aortic aneurysm.

What are risk factors for aortic aneurysms?

Risk factors for aortic aneurysm include:

  • Cigarette smoking: cigarette smoking not only increases the risk of developing an abdominal aortic aneurysm, the chance of aneurysm rupture (a life-threatening complication of abdominal aneurysm) is also more common among active smokers.
  • High blood pressure
  • High serum cholesterol
  • Diabetes mellitus

What is the most common cause of aortic aneurysms?

The most common cause of aortic aneurysms is "hardening of the arteries" called arteriosclerosis. At least 80% of aortic aneurysms are from arteriosclerosis. The arteriosclerosis can weaken the aortic wall and the pressure of the blood being pumped through the aorta causes expansion at the site of weakness.

What are other causes of aortic aneurysms?

 

Other causes of aortic aneurysms include:

  • Genetic/hereditary: There is a familial tendency to developing abdominal aortic aneurysms. Individuals with first-degree relatives having abdominal aortic aneurysms have a higher risk of developing abdominal aortic aneurysm than the general population. They also tend to develop the aneurysms at younger ages and have a higher tendency to suffer aneurysm rupture than individuals without family history.
  • Genetic disease: There are also rare inheritable genetic diseases of connective tissue (tissue that make up the wall of the aorta) such as Ehlers-Danlos syndrome and Marfan's syndrome that can lead to the development of aortic aneurysms.
  • Post-trauma: After physical trauma to the aorta.
  • Arteritis: Inflammation of blood vessels as occurs in Takayasu disease, giant cell arteritis, and relapsing polychondritis.
  • Mycotic (fungal) infection: A mycotic infection that may be associated with immunodeficiency, IV drug abuse, syphilis, and heart valve surgery.

What are the symptoms of an abdominal aortic aneurysm?

Most abdominal aortic aneurysms produce no symptoms (they are asymptomatic). They are often incidentally discovered when abdominal ultrasounds and/or CT scan studies are ordered for other conditions. When they produce symptoms, the most common symptom is pain. The pain typically has a deep quality as if it is boring into the person. It is felt most prominently in the middle of the abdomen and can radiate to the back. The pain is usually steady but may be relieved by changing position. The person may also become aware of an abnormally prominent abdominal pulsation.

Abdominal aortic aneurysm can remain asymptomatic or produce mild to moderate symptoms for years. However, a rapidly expanding abdominal aneurysm can cause sudden onset of severe, steady, and worsening middle abdominal and back pain . A rapidly expanding aneurysm is also at imminent risk of rupture. Actual rupture of an abdominal aneurysm can cause sudden onset of back and abdominal pain, sometimes associated with abdominal distension, a pulsating abdominal mass, and even shock (severe low blood pressure due to massive blood loss).

How is an abdominal aortic aneurysm diagnosed clinically?

Careful palpation or feeling of the abdomen by a healthcare practitioner may reveal the abnormally wide pulsation of the abdominal aorta. This is characteristically felt on both sides of the aorta which is in the midline of the abdomen. Note that even large aneurysms can be very difficult to detect on physical examination in overweight people. Aneurysms on the verge of rupture and that are rapidly enlarging, are often tender. Listening with a stethoscope may also reveal a bruit or abnormal sound from turbulence of blood within the aneurysm.

What tests help in the diagnosis of an abdominal aortic aneurysm?

In about 90% of the cases, X-rays of the abdomen show calcium deposits in the aneurysm wall. But plain x-rays of the abdomen cannot determine the size and the extent of the aneurysm. Ultrasonography usually gives a clear picture of the size of an aneurysm. Ultrasound has about 98% accuracy in measuring the size of the aneurysm, and is safe and noninvasive. But ultrasound cannot accurately define the extent of the aneurysm and is inadequate for surgical repair planning.

Computerized tomography of the abdomen, is highly accurate in determining the size and extent of the aneurysm, and its relation to the renal arteries. However, computerized tomography uses high doses of radiation and for evaluation of blood vessels, requires intravenous dye. This carries some risk including allergic reaction to the dye and irritation of the kidneys. In patients with kidney diseases, the doctor may consider an MRA (magnetic resonance angiography), which is a study of the aorta and the other arteries using MRI scanning. Both computerized tomography and magnetic resonance imaging are effective for diagnosis. An aortogram, where dye is directly injected into the aorta to assess its anatomy, historically was the diagnostic test of choice. Presently, it's indications may be limited to use when surgery or stenting is considered (see below).

What is the natural history of abdominal aortic aneurysms?

The natural history of abdominal aortic aneurysms depends on their size and the speed of expansion. Rupture of aneurysms is uncommon when they are less than 5.5 cm wide and are expanding slowly. Rupture is far more common in aneurysms that are over 5.5 cm wide and are expanding rapidly (>0.5 cm/year). Surgical repair is therefore usually recommended for aneurysms over 5.5 cm wide.

What are the complications with an abdominal aortic aneurysm?

Rupture is a feared problem. Rupture of an abdominal aneurysm is a catastrophe. It is highly lethal and is usually preceded by excruciating pain in the lower abdomen and back, with tenderness of the aneurysm. Rupture of an abdominal aneurysm causes profuse bleeding and leads to shock. Death may rapidly follow. Half of all persons with untreated abdominal aortic aneurysms die of rupture within five years. Abdominal aortic aneurysms are the 13th leading cause of death in the U.S.

Peripheral embolization of clot within the aneurysm can occur when a piece of clot comes loose and travels further out in the arterial system. This clot fragment can lodge in a smaller artery and block the flow of blood. Infection of aneurysms can occur from turbulent blood flow from the rough inner surface of the affected aorta.

How are abdominal aortic aneurysms repaired?

The goal of surgical treatment of abdominal aortic aneurysm is to prevent aneurysm rupture. Traditionally, repair of aortic aneurysms has been surgical. The operation consists of opening the abdomen, finding the aorta and removing (excising) the aneurysm. A synthetic Dacron tube that replaces the removed piece of aorta is sewn into place.

A less invasive procedure for aortic aneurysm is endovascular surgery. This minimally invasive procedure that allows the grafts (stent) to be guided within the blood vessel itself to the site of the aneurysm without the need to cut open the abdomen. Not all aneurysms can be fixed in this manner and there may not be a long-term benefit to this type of surgery. Though the post-operative course is shorter, there is a need for closer follow-up and testing.

Picture of grafted stent to repair an abdominal aortic aneurysm

What is done if an abdominal aortic aneurysm threatens to rupture?

Threatened rupture of abdominal aneurysms is a surgical emergency. The operative risk for a ruptured aneurysm is about 50%. If kidney failure occurs after surgery, the prognosis (outlook) is particularly poor.

What is the medical management (non-surgical management) of abdominal aortic aneurysm?

 

For patients who are not surgical candidates (for example for patients with aneurysm smaller than 5 cm); medical treatment to prevent aneurysm expansion and rupture include:
  • Stopping cigarette smoking
  • Controlling high blood pressure
  • Lowering high blood cholesterol
  • Some doctors may consider medications called beta blockers, like propanolol (Inderal), atenolol (Tenormin), or metoprolol (Lopressor, Toprol XL), which block adrenaline receptors and lower pressure within the blood vessel.
  • Close monitoring of the aneurysm size with ultrasound or CT scan every 6 to 12 months (sooner in high risk patients)

 

Abdominal Aortic Aneurysm At A Glance

  • An aneurysm is an abnormal area of localized widening of a blood vessel.
  • The aorta bulges at the site of an aneurysm like a weak spot on a worn tire.
  • Aortic aneurysms are typically spindle-shaped and involve the aorta below the arteries to the kidneys.
  • Five percent of men over 60 develop an abdominal aortic aneurysms.
  • The most common cause of an aneurysm is arteriosclerosis.
  • Abdominal aortic aneurysms often do not cause symptoms. If they do, they may cause deep boring pain in the lower back or abnormally prominent abdominal pulsation.
  • X-rays of the abdomen and other radiologic tests can be used in diagnosing an aneurysm.
  • Rupture of an aortic aneurysm is a catastrophe.
  • Repair of the aneurysm can be done by surgery or endovascular stenting.


Abdominal Aortic Aneurysm

 

View the Heart Disease Slideshow

 

Heart Disease Slideshow Pictures Heart Disease Slideshow
Medical Illustrations of the Heart Image Collection Medical Illustrations of the Heart Image Collection
Take the Heart Disease Quiz! Take the Heart Disease Quiz!

Medical Revising Authors: Benjamin C. Wedro, MD, FAAEM and Dennis Lee, MD
Medical Revising Editor: William C. Shiel, Jr, MD, FACP, FACR

  • What is an aneurysm?
  • What is an aortic aneurysm?
  • What are the thoracic and abdominal aorta?
  • Where do aortic aneurysms tend to develop?
  • What shape are most aortic aneurysms?
  • What's inside an aortic aneurysm?
  • Who is most likely to have an abdominal aortic aneurysm?
  • What are risk factors of aortic aneurysms?
  • What is the most common cause of aortic aneurysms?
  • What are other causes of aortic aneurysms?
  • What are the symptoms of an abdominal aortic aneurysm?
  • How is an abdominal aortic aneurysm diagnosed clinically?
  • What tests help in the diagnosis of an abdominal aortic aneurysm?
  • What is the natural history of abdominal aortic aneurysm?
  • What are the complications with an abdominal aortic aneurysm?
  • How are abdominal aortic aneurysms repaired?
  • What is done if an abdominal aortic aneurysm threatens to rupture?
  • What is the medical management (non-surgical management) of abdominal aortic aneurysm?
  • Abdominal Aortic Aneurysm At A Glance
  • Patient Discussions: Abdominal Aortic Aneurysm - Treatments
  • Patient Discussions: Abdominal Aortic Aneurysm - Describe Your Experience

What is an aneurysm?

An aneurysm is an area of a localized widening (dilation) of a blood vessel. (The word "aneurysm" is borrowed from the Greek "aneurysma" meaning "a widening").

What is an aortic aneurysm?

An aortic aneurysm involves the aorta, one of the large arteries through which blood passes from the heart to the rest of the body. The aorta bulges at the site of the aneurysm like a weak spot on an old worn tire.

What are the thoracic and abdominal aorta?

The aorta is first called the thoracic aorta as it leaves the heart, ascends, arches, and descends through the chest until it reaches the diaphragm (the partition between the thorax and abdomen). The aorta is then called the abdominal aorta after it has passed the diaphragm and continues down the abdomen. The abdominal aorta ends where it splits to form the two iliac arteries that go to the legs.

Where do aortic aneurysms tend to develop?

Aortic aneurysms can develop anywhere along the length of the aorta. The majority, however, are located along the abdominal aorta. Most (about 90%) of abdominal aneurysms are located below the level of the renal arteries, the vessels that leave the aorta to go to the kidneys. About two-thirds of abdominal aneurysms are not limited to just the aorta but extend from the aorta into one or both of the iliac arteries.

What shape are most aortic aneurysms?

Most aortic aneurysms are fusiform. They are shaped like a spindle ("fusus" means spindle in Latin) with widening all around the circumference of the aorta. (Saccular aneurysms just involve a portion of the aortic wall with a localized out pocketing).

What's inside an aortic aneurysm?

The inside walls of aneurysms are often lined with a laminated blood clot that is layered like a piece of plywood.

Who is most likely to have an abdominal aortic aneurysm?

Aortic aneurysms are most common after 60 years of age. Males are five times more likely than females to be affected. Approximately 5% of men over age 60 develop an abdominal aortic aneurysm.

What are risk factors for aortic aneurysms?

Risk factors for aortic aneurysm include:

  • Cigarette smoking: cigarette smoking not only increases the risk of developing an abdominal aortic aneurysm, the chance of aneurysm rupture (a life-threatening complication of abdominal aneurysm) is also more common among active smokers.
  • High blood pressure
  • High serum cholesterol
  • Diabetes mellitus

What is the most common cause of aortic aneurysms?

The most common cause of aortic aneurysms is "hardening of the arteries" called arteriosclerosis. At least 80% of aortic aneurysms are from arteriosclerosis. The arteriosclerosis can weaken the aortic wall and the pressure of the blood being pumped through the aorta causes expansion at the site of weakness.

What are other causes of aortic aneurysms?

 

Other causes of aortic aneurysms include:

  • Genetic/hereditary: There is a familial tendency to developing abdominal aortic aneurysms. Individuals with first-degree relatives having abdominal aortic aneurysms have a higher risk of developing abdominal aortic aneurysm than the general population. They also tend to develop the aneurysms at younger ages and have a higher tendency to suffer aneurysm rupture than individuals without family history.
  • Genetic disease: There are also rare inheritable genetic diseases of connective tissue (tissue that make up the wall of the aorta) such as Ehlers-Danlos syndrome and Marfan's syndrome that can lead to the development of aortic aneurysms.
  • Post-trauma: After physical trauma to the aorta.
  • Arteritis: Inflammation of blood vessels as occurs in Takayasu disease, giant cell arteritis, and relapsing polychondritis.
  • Mycotic (fungal) infection: A mycotic infection that may be associated with immunodeficiency, IV drug abuse, syphilis, and heart valve surgery.

hat are the symptoms of an abdominal aortic aneurysm?

Most abdominal aortic aneurysms produce no symptoms (they are asymptomatic). They are often incidentally discovered when abdominal ultrasounds and/or CT scan studies are ordered for other conditions. When they produce symptoms, the most common symptom is pain. The pain typically has a deep quality as if it is boring into the person. It is felt most prominently in the middle of the abdomen and can radiate to the back. The pain is usually steady but may be relieved by changing position. The person may also become aware of an abnormally prominent abdominal pulsation.

Abdominal aortic aneurysm can remain asymptomatic or produce mild to moderate symptoms for years. However, a rapidly expanding abdominal aneurysm can cause sudden onset of severe, steady, and worsening middle abdominal and back pain . A rapidly expanding aneurysm is also at imminent risk of rupture. Actual rupture of an abdominal aneurysm can cause sudden onset of back and abdominal pain, sometimes associated with abdominal distension, a pulsating abdominal mass, and even shock (severe low blood pressure due to massive blood loss).

How is an abdominal aortic aneurysm diagnosed clinically?

Careful palpation or feeling of the abdomen by a healthcare practitioner may reveal the abnormally wide pulsation of the abdominal aorta. This is characteristically felt on both sides of the aorta which is in the midline of the abdomen. Note that even large aneurysms can be very difficult to detect on physical examination in overweight people. Aneurysms on the verge of rupture and that are rapidly enlarging, are often tender. Listening with a stethoscope may also reveal a bruit or abnormal sound from turbulence of blood within the aneurysm.

What tests help in the diagnosis of an abdominal aortic aneurysm?

In about 90% of the cases, X-rays of the abdomen show calcium deposits in the aneurysm wall. But plain x-rays of the abdomen cannot determine the size and the extent of the aneurysm. Ultrasonography usually gives a clear picture of the size of an aneurysm. Ultrasound has about 98% accuracy in measuring the size of the aneurysm, and is safe and noninvasive. But ultrasound cannot accurately define the extent of the aneurysm and is inadequate for surgical repair planning.

Computerized tomography of the abdomen, is highly accurate in determining the size and extent of the aneurysm, and its relation to the renal arteries. However, computerized tomography uses high doses of radiation and for evaluation of blood vessels, requires intravenous dye. This carries some risk including allergic reaction to the dye and irritation of the kidneys. In patients with kidney diseases, the doctor may consider an MRA (magnetic resonance angiography), which is a study of the aorta and the other arteries using MRI scanning. Both computerized tomography and magnetic resonance imaging are effective for diagnosis. An aortogram, where dye is directly injected into the aorta to assess its anatomy, historically was the diagnostic test of choice. Presently, it's indications may be limited to use when surgery or stenting is considered (see below).

What is the natural history of abdominal aortic aneurysms?

The natural history of abdominal aortic aneurysms depends on their size and the speed of expansion. Rupture of aneurysms is uncommon when they are less than 5.5 cm wide and are expanding slowly. Rupture is far more common in aneurysms that are over 5.5 cm wide and are expanding rapidly (>0.5 cm/year). Surgical repair is therefore usually recommended for aneurysms over 5.5 cm wide.

hat are the complications with an abdominal aortic aneurysm?

Rupture is a feared problem. Rupture of an abdominal aneurysm is a catastrophe. It is highly lethal and is usually preceded by excruciating pain in the lower abdomen and back, with tenderness of the aneurysm. Rupture of an abdominal aneurysm causes profuse bleeding and leads to shock. Death may rapidly follow. Half of all persons with untreated abdominal aortic aneurysms die of rupture within five years. Abdominal aortic aneurysms are the 13th leading cause of death in the U.S.

Peripheral embolization of clot within the aneurysm can occur when a piece of clot comes loose and travels further out in the arterial system. This clot fragment can lodge in a smaller artery and block the flow of blood. Infection of aneurysms can occur from turbulent blood flow from the rough inner surface of the affected aorta.

How are abdominal aortic aneurysms repaired?

The goal of surgical treatment of abdominal aortic aneurysm is to prevent aneurysm rupture. Traditionally, repair of aortic aneurysms has been surgical. The operation consists of opening the abdomen, finding the aorta and removing (excising) the aneurysm. A synthetic Dacron tube that replaces the removed piece of aorta is sewn into place.

A less invasive procedure for aortic aneurysm is endovascular surgery. This minimally invasive procedure that allows the grafts (stent) to be guided within the blood vessel itself to the site of the aneurysm without the need to cut open the abdomen. Not all aneurysms can be fixed in this manner and there may not be a long-term benefit to this type of surgery. Though the post-operative course is shorter, there is a need for closer follow-up and testing.

Picture of grafted stent to repair an abdominal aortic aneurysm

What is done if an abdominal aortic aneurysm threatens to rupture?

Threatened rupture of abdominal aneurysms is a surgical emergency. The operative risk for a ruptured aneurysm is about 50%. If kidney failure occurs after surgery, the prognosis (outlook) is particularly poor.

What is the medical management (non-surgical management) of abdominal aortic aneurysm?

 

For patients who are not surgical candidates (for example for patients with aneurysm smaller than 5 cm); medical treatment to prevent aneurysm expansion and rupture include:
  • Stopping cigarette smoking
  • Controlling high blood pressure
  • Lowering high blood cholesterol
  • Some doctors may consider medications called beta blockers, like propanolol (Inderal), atenolol (Tenormin), or metoprolol (Lopressor, Toprol XL), which block adrenaline receptors and lower pressure within the blood vessel.
  • Close monitoring of the aneurysm size with ultrasound or CT scan every 6 to 12 months (sooner in high risk patients)

 

Abdominal Aortic Aneurysm At A Glance

  • An aneurysm is an abnormal area of localized widening of a blood vessel.
  • The aorta bulges at the site of an aneurysm like a weak spot on a worn tire.
  • Aortic aneurysms are typically spindle-shaped and involve the aorta below the arteries to the kidneys.
  • Five percent of men over 60 develop an abdominal aortic aneurysms.
  • The most common cause of an aneurysm is arteriosclerosis.
  • Abdominal aortic aneurysms often do not cause symptoms. If they do, they may cause deep boring pain in the lower back or abnormally prominent abdominal pulsation.
  • X-rays of the abdomen and other radiologic tests can be used in diagnosing an aneurysm.
  • Rupture of an aortic aneurysm is a catastrophe.
  • Repair of the aneurysm can be done by surgery or endovascular stenting.
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Last Updated on Thursday, 13 October 2011 12:35
 
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