Patient information: Celiac disease in children

CELIAC DISEASE OVERVIEW

Celiac disease is a condition in which the immune system responds abnormally to a proteins found in wheat, rye, barley, and a multitude of prepared foods . These proteins are called gluten. When ingested, gluten can cause damage to the lining of the small intestine. Celiac disease is also known as gluten sensitive enteropathy (pronounced "enter-AH-pathy"), celiac sprue, and nontropical sprue.

Celiac disease is different from wheat allergy, in which a different part of the immune system is activated by wheat, causing allergic symptoms such as hives and wheezing. (See "Patient information: Food allergy symptoms and diagnosis".)

The small intestine is responsible for absorbing food and nutrients. Thus, damage to the lining of the small intestines, caused by the abnormal immune response, can lead to difficulty absorbing necessary nutrients; this problem is referred to as malabsorption. Although celiac disease cannot be cured, avoiding gluten usually stops the damage to the intestinal lining and the malabsorption that results. Celiac disease can occur in people of any age and it affects both genders.

Celiac disease in adults is discussed separately. (See "Patient information: Celiac disease in adults".)

CELIAC DISEASE CAUSES

Celiac disease occurs as a result of a child's genetics and exposure to a trigger. A child who develops celiac disease probably inherits the risk from one or both parents and is then develops the disease when exposed to the trigger, gluten.

CELIAC DISEASE SYMPTOMS

The symptoms of celiac disease can vary from mild to severe. In some cases, the child does not have any of the more common gastrointestinal symptoms (usually diarrhea, but sometimes constipation), but does have problems with growth, iron deficiency anemia, skin rash, tooth changes, bone disease, or other mild symptoms.

Common symptoms — The most common symptoms of celiac disease in children include chronic diarrhea, poor appetite, abdominal bloating or abdominal pain, and weight loss or difficulty gaining weight.

These symptoms can begin during infancy (between six and 24 months), after the child begins to eat gluten-containing foods. More commonly, the symptoms do not develop until later in childhood, adolescence, or adulthood. Celiac disease is often diagnosed later in people with milder symptoms.

In older children, symptoms may include constipation or diarrhea, oily stools that float, abdominal bloating, and gas. Older children may also be shorter than normal for their age, have difficulty gaining weight, or have other problems (anemia, bone thinning).

Skin symptoms — A skin problem, known as dermatitis herpetiformis, is common in adults with celiac disease. This condition is uncommon before puberty in children with celiac disease. A person may have skin symptoms with few or no gastrointestinal symptoms.

The symptoms of dermatitis herpetiformis include intensely itchy, raised, fluid-filled blisters on the skin, usually located on the elbows, knees, buttocks, lower back, face, neck, trunk and occasionally within the mouth (picture 1).

The most bothersome symptoms are itching and burning. This feeling is quickly relieved when the blister ruptures. Scratching causes the blister to rupture and dry up, but this leaves an area of darkened skin and scarring. A skin biopsy may be taken to see if the rash is dermatitis herpetiformis. The condition will improve after eliminating gluten from the diet, although it may take several weeks to see significant improvement.

Tooth problems — Children with celiac disease commonly develop changes in the permanent (adult) teeth. This may include cream-colored, yellow, or brown discoloration, grooves, or pits in the teeth (picture 2). Between 38 and 96 percent of people with celiac develop these changes.

Bone thinning — Children with celiac disease are at risk for weakening of the bones caused by poor absorption of vitamin D. Treatment of celiac disease by eating a gluten-free diet can resolve the problem in most cases. Sometimes, vitamin supplements are also recommended. (See "Patient information: Vitamin D deficiency".)

Mild symptoms — Celiac disease can cause very mild, non-specific symptoms such as fatigue, borderline iron deficiency anemia, or being shorter than normal for age. Once a child is confirmed as having celiac disease with a skin or intestinal biopsy, treatment (avoiding gluten) is important, as discussed below. (See 'Celiac disease treatment' below.)

WHICH CHILDREN NEED TESTING FOR CELIAC DISEASE?

If the child has symptoms — All children do not need to be tested for celiac disease. If you are concerned that your child has symptoms that might be caused by celiac disease, ask your child's doctor if the child should be tested.

Children who have the following symptoms should be tested:

• Shortness or underweight for age, particularly if the child's growth percentiles have decreased significantly over time or if the child is significantly smaller than other family members (see "Patient information: Poor weight gain in infants and children".
• Diarrhea for more than a few weeks
• Chronic constipation, recurrent abdominal pain, or vomiting
• Certain tooth problems (see 'Tooth problems' above)
• Children who have not begun puberty at the expected time (see "Normal puberty")
• Iron deficiency anemia that has not improved with treatment

High-risk groups — Testing may be recommended for certain children (older than three years) who are at increased risk of developing celiac disease, even if the child has no symptoms.

Children who are at increased risk of developing celiac disease include the following:

• First-degree relatives (children, siblings) of a person with celiac disease
• Down syndrome
• Type 1 diabetes
• Selective IgA deficiency
• Turner syndrome
• Williams syndrome
• Autoimmune thyroiditis

CELIAC DISEASE TESTING

Several tests are done to diagnose celiac disease. The child should continue to eat gluten-containing foods while being tested. Avoiding gluten before testing is completed can cause the tests to be inaccurate. (See "Clinical manifestations and diagnosis of celiac disease in children".)

Blood test — The first test for celiac disease is usually a blood test. A general pediatric or family healthcare provider can do this test. The blood test determines if the child has an increased level of antibodies (proteins) called tissue transglutaminase (anti-tTG). The level of these antibodies is usually elevated in people with celiac disease (as long as their diet contains gluten), but is almost never elevated in people without celiac disease. If the test is positive for tTG, a small intestine biopsy is usually recommended to confirm the diagnosis of celiac disease.

Small intestine biopsy — If the blood test shows high levels of tTG antibodies, the diagnosis must be confirmed by examining a sample of the lining of the small intestine with a microscope.

The sample (called a biopsy) is usually collected during an upper endoscopy, a test that involves swallowing a small flexible instrument called an endoscope, which has a camera at the end. A pediatric gastroenterologist performs this test while the child is sedated. The camera allows the physician to examine the upper part of the gastrointestinal system and remove a small piece (biopsy) of the small intestinal lining through the endoscope. The biopsy is not painful. (See "Patient information: Upper endoscopy".)

In people with celiac disease, the lining of the small intestine has a unique appearance when viewed with a microscope. Normally, the lining has distinct finger-like structures, which are called villi. Villi allow the small intestine to absorb nutrients efficiently. The villi become flattened in people with celiac disease, which interferes with absorption. Once the child stops eating gluten, the villi revert back to normal.

CELIAC DISEASE TREATMENT

The most effective treatment for children with celiac disease is complete avoidance of all foods and drinks that contain gluten. If the child is malnourished because of the disease, nutritional supplements (high calorie drinks and/or vitamins) may also be needed.

Gluten avoidance is recommended for children who have an abnormal antibody testing AND:

• An abnormal small intestine biopsy and symptoms of celiac disease
• An abnormal small intestine biopsy and are in a high-risk group (even if there are no symptoms)
• Dermatitis herpetiformis confirmed with a skin biopsy (see 'Skin symptoms' above)

If the results of the antibody testing and biopsy disagree or are equivocal, or if the child does not have symptoms of celiac disease, additional testing may be needed. (See "Clinical manifestations and diagnosis of celiac disease in children".)

How do we stop eating gluten? — Strict avoidance of gluten is recommended because eating even small amounts of gluten can aggravate the disease. Eating a gluten free diet can be a difficult because it requires major lifestyle adjustments for both parents and children. (See "Management of celiac disease in children".)

Meeting with a dietitian who is experienced in treating celiac disease can help parents and children to better understand what foods are safe, which are not, and how to know the difference. In addition, a number of helpful resources are available. (See 'Where to get more information' below.)

Fortunately, life on a gluten free diet has become easier due to the increased availability of gluten free foods. Excellent gluten free substitute foods are now widely available (table 1).

What foods contain gluten? — The most commonly consumed grains in the Western world (wheat, rye, and barley) contain gluten, and gluten is included as an additive in a large number of prepared foods .

In general:

• Foods containing wheat, rye, and barley should be avoided.
• Soybean or tapioca flours, rice, corn, buckwheat (which is not related to wheat), and potatoes are safe.
• Read labels on prepared foods and condiments carefully, paying particular attention to additives such as stabilizers or emulsifiers that may contain gluten.
• Dairy products may not be well tolerated initially since many patients with celiac disease can have a temporary lactose intolerance. As a result, lactose-containing products should initially be avoided in patients whose symptoms appear to be worsened by them.
• "Wheat free" does not necessarily mean gluten free. Read the packaging or call the manufacturer if you have questions about a specific product.
• It is not clear whether oats are harmful for people with celiac disease. While pure oats are probably not harmful, oats can sometimes be contaminated with wheat during processing. Therefore, parents should be sure the package specifically indicates that the product is gluten free and was processed in a gluten free facility. Some experts suggest that children avoid eating more than two ounces of oats per day, and that those with severe or newly diagnosed disease should avoid oats altogether.

Is strict gluten avoidance really necessary? — Children who do not have bothersome symptoms of celiac disease often find it difficult to follow a strict gluten free diet. Indeed, some healthcare providers have questioned the need for a gluten free diet in this population. However, certain factors support the importance of a gluten free diet, even in children without symptoms:

• Strictly following a gluten free diet sometimes helps improve a child's energy and sense of well-being.
• Some children with celiac disease have vitamin or nutrient deficiencies, even if they feel well. These deficiencies often cause problems if gluten is not eliminated (such as anemia due to iron deficiency or bone loss due to vitamin D deficiency).

Monitoring during treatment — Most children who begin a gluten-free diet begin to feel better within two weeks.

About six months after beginning a gluten-free diet, a blood test may be recommended to test antibody levels. The antibody level should decrease, or even disappear, as the child improves and continues to avoid gluten.

A repeat biopsy is not usually necessary if the child's symptoms improve and the antibody levels decrease once a gluten-free diet is started. A repeat biopsy or other tests may be recommended if symptoms do not improve or if antibody levels remain elevated.

LIFE WITH CELIAC DISEASE

Adjusting to a gluten-free life can be hard for some people. Talking to a healthcare provider or experienced dietitian can help parents and children make the adjustment. In addition, support groups (on the internet or in the community) are another good source of information and assistance. (See 'Where to get more information' below.)

Parents of children who are newly diagnosed with celiac disease will need to speak to their child's teacher or day care provider about the condition, what foods are safe, and what to do in case of illness.

Celiac disease is a lifelong condition, and it will be necessary to avoid gluten forever. Currently there is no cure for celiac disease, although avoiding gluten probably prevents all complications of celiac disease.

Because children with celiac disease may have an increased risk of certain infections, a vaccination is recommended to reduce the risk of pneumococcal infections. The vaccine is given just once. (See "Patient information: Pneumonia prevention".)

WHERE TO GET MORE INFORMATION

Your child's healthcare provider is the best source of information for questions and concerns related to your child's medical problem. Because no two people are exactly alike and recommendations can vary from one person to another, it is important to seek guidance from a provider who is familiar with your child's situation.

This discussion will be updated as needed every four months on our web site (www.uptodate.com/patients). Additional topics as well as selected discussions written for healthcare professionals are also available for those who would like more detailed information.

Some of the most pertinent include:

Patient Level Information:
Patient information: Food allergy symptoms and diagnosis
Patient information: Celiac disease in adults
Patient information: Vitamin D deficiency
Patient information: Poor weight gain in infants and children
Patient information: Upper endoscopy
Patient information: Pneumonia prevention

Professional Level Information:
Approach to the diagnosis of chronic diarrhea in children in developed countries
Associated autoimmune diseases in children and adolescents with type 1 diabetes mellitus
Clinical manifestations and diagnosis of celiac disease in children
Diagnosis of celiac disease
Management of celiac disease in adults
Management of celiac disease in children
Pathogenesis, epidemiology, and clinical manifestations of celiac disease in adults
Normal puberty

A number of web sites have information about medical problems and treatments, although it can be difficult to know which sites are reputable. Information provided by the National Institutes of Health, national medical societies and some other well-established organizations are often reliable sources of information, although the frequency with which they are updated is variable.

• National Library of Medicine

      (www.nlm.nih.gov/medlineplus/celiacdisease.html, available in Spanish)

• The Celiac Disease Foundation

       (www.celiac.org)

• The Celiac Sprue Association

       (www.csaceliacs.org)

• The Celiac Disease and Gluten-free Diet Support Page

       (www.celiac.com)

• The Children's Digestive Health and Nutrition Foundation

      (www.cdhnf.org)

Patient Support — There are a number of online forums where patients can find information and support from other people with similar conditions.

• About.com Celiac Disease Forum

      (http://celiacdisease.about.com/forum)

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