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    Celiac disease - sprue

    Sprue; Nontropical sprue; Gluten intolerance; Gluten-sensitive enteropathy

    Celiac disease is a condition that creates inflammation and damages the lining of the small intestine. This prevents absorbing components of food that are important for staying healthy. The damage is due to a reaction to eating gluten, which is found in wheat, barley, rye, and possibly oats.

    Causes

    The exact cause of celiac disease is unknown. The lining of the intestinesis covered byvilli, which help absorb nutrients. When people with celiac disease eat foods or use products that contain gluten, their immune system reacts by damaging these villi.

    This damage affects the ability to absorb nutrients properly. A person becomes malnourished, no matter how much food he or she eats.

    The disease can develop at any point in life, from infancy to late adulthood.

    People who have a family member with celiac disease are at greater risk for developing the disease. The disorder is most common in Caucasians and persons of European ancestry. Women are affected more often than men.

    People with celiac disease are more likely to have:

    • Autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus, and Sjogren syndrome
    • Addison's disease
    • Down syndrome
    • Intestinal cancer
    • Intestinal lymphoma
    • Lactose intolerance
    • Thyroid disease
    • Type 1 diabetes

    Symptoms

    The symptoms of celiac disease can be different from person to person. This is part of the reason why the diagnosis is not always made right away. For example, one person may have constipation, a second may have diarrhea, and a third may have no problem with stools.

    Gastrointestinal symptoms include:

    • Abdominal pain, bloating, gas, or indigestion
    • Constipation
    • Decreased appetite (may also be increased or unchanged)
    • Diarrhea, either constant or off and on
    • Lactose intolerance (common when the person is diagnosed, usually goes away after treatment)
    • Nausea and vomiting
    • Stools that are foul smelling, oily, or and stick to the toilet when flushed
    • Unexplained weight loss (although people can be overweight or of normal weight)

    Because the intestines do not absorb many important vitamins, minerals, and other parts of food, the following symptoms may start over time:

    • Bruising easily
    • Depression or anxiety
    • Fatigue
    • Growth delay in children
    • Hair loss
    • Itchy skin with a rash (dermatitis herpetiformis)
    • Missed menstrual periods
    • Mouth ulcers
    • Muscle cramps and joint pain
    • Nosebleeds
    • Seizures
    • Tingling or numbness in the hands or feet
    • Unexplained short height

    Children with celiac disease may have:

    • Defects in the tooth enamel and changes in tooth color
    • Delayed puberty
    • Diarrhea, constipation, fatty or foul-smelling stools, nausea, or vomiting
    • Irritable and fussy behavior
    • Poor weight gain
    • Slowed growth and shorter than normal height for their age

    Exams and Tests

    • Bone density
    • Cholesterol (may be low)
    • Complete blood count (CBC - test for anemia)
    • Comprehensive metabolic panel
    • Folate level (serum)
    • Iron level (serum)
    • Prothrombin time
    • Vitamin B12 level (serum)
    • Vitamin D level

    Blood tests can detect several special antibodies, called antitissue transglutaminase antibodies (tTGA) or anti-endomysial antibodies (EMA). The health care provider will order these antibody tests if celiac disease is suspected.

    If the tests are positive, upper endoscopy is usually performed to sample a piece of tissue (biopsy) from the first part of the small intestine (duodenum). The biopsy may show a flattening of the villi in the parts of the intestine below the duodenum.

    Genetic testing of the blood is also available to help determine who may be at risk for celiac disease.

    A follow-up biopsy or blood test may be ordered several months after the diagnosis and treatment. These tests evaluate your response to treatment. Normal results mean that you have responded to treatment, which confirms the diagnosis. However, this does not mean that the disease has been cured.

    Treatment

    Celiac disease cannot be cured. However, your symptoms will go away and the villi in the lining of the intestines will heal if you follow a lifelong gluten-free diet. Do not eat foods, beverages, and medications that contain wheat, barley, rye, and possibly oats.

    You must read food and medication labels carefully to look for hidden sources of these grains and ingredients related to them. Because wheat and barley grains are common in the American diet, sticking with this diet is challenging. With education and planning, you will heal.

    You should NOT begin the gluten-free diet before you are diagnosed. Starting the diet will affect testing for the disease.

    The health care provider may prescribe vitamin and mineral supplements to correct nutritional deficiencies. Occasionally, corticosteroids (such as prednisone) may also be prescribed for short-term use or if you have sprue that does not respond to treatment. Following a well-balanced, gluten-free diet is generally the only treatment you need to stay well.

    When you are diagnosed, get help from a registered dietitian who specializes in celiac disease and the gluten-free diet. A support group may also help you cope with the disease and diet.

    Support Groups

    For additional information and support, see the organizations listed in celiac disease resources.

    Outlook (Prognosis)

    Following a gluten-free diet heals the damage to the intestines and prevents further damage. This healing most often occurs within 3 - 6 months in children, but it may take 2 - 3 years in adults.

    Rarely, long-term damage will be done to the lining of the intestines before the diagnosis is made.

    Some problems caused by celiac disease may not improve, such as a shorter than expected height and damage to the teeth.

    You must carefully continue to follow the gluten-free diet. When untreated, the disease can cause life-threatening complications.

    Delaying diagnosis or not following the diet puts you at risk for related conditions such as:

    • Autoimmune disorders
    • Bone disease (osteoporosis, kyphoscoliosis, fractures)
    • Certain types of intestinal cancer
    • Low blood count (anemia)
    • Infertility or repeated miscarriage
    • Liver disease

    When to Contact a Medical Professional

    Call your health care provider if you have symptoms of celiac disease.

    Prevention

    Because the exact cause is unknown, there is no known way to prevent the development of celiac disease. However, being aware of the risk factors (such as having a family member with the disorder) may increase your chances of early diagnosis, treatment, and a long, healthy life.

    References

    Green PH, Cellier C. Celiac disease. N Engl J Med. 2007;357:1731-1743.

    Kagnoff MF. AGA Institute medical position statement on the diagnosis and management of celiac disease.Gastroenterology 2006;131(6): 1977-1980.

    Semrad CE. Approach to the patient with diarrhea and malabsorption. In: Goldman L, Shafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 142.

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          Tests for Celiac disease - sprue

            Review Date: 2/19/2012

            Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, and the A.D.A.M. Editorial team.

            The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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