celiac disease


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Related to celiac disease: dermatitis herpetiformis, Gluten free diet

Celiac Disease

 

Definition

Celiac disease is a disease of the digestive system that damages the small intestine and interferes with the absorption of nutrients from food.

Description

Celiac disease occurs when the body reacts abnormally to gluten, a protein found in wheat, rye, barley, and possibly oats. When someone with celiac disease eats foods containing gluten, that person's immune system causes an inflammatory response in the small intestine, which damages the tissues and results in impaired ability to absorb nutrients from foods. The inflammation and malabsorption create wide-ranging problems in many systems of the body. Since the body's own immune system causes the damage, celiac disease is classified as an "autoimmune" disorder. Celiac disease may also be called sprue, nontropical sprue, gluten sensitive enteropathy, celiac sprue, and adult celiac disease.
Celiac disease may be discovered at any age, from infancy through adulthood. The disorder is more commonly found among white Europeans or in people of European descent. It is very unusual to find celiac disease in African or Asian people. The exact incidence of the disease is uncertain. Estimates vary from one in 5000, to as many as one in every 300 individuals with this background. The prevalence of celiac disease seems to be different from one European country to another, and between Europe and the United States. This may be due to differences in diet and/or unrecognized disease. A recent study of random blood samples tested for celiac disease in the US showed one in 250 testing positive. It is clearly under-diagnosed, probably due to the symptoms being attributed to another problem, or lack of knowledge about celiac disease by physicians and laboratories. Because of the known genetic component, relatives of patients with celiac disease are considered at higher risk for the disorder.
Because celiac disease has a hereditary influence, close relatives (especially first degree relatives, such as children, siblings, and parents) have a higher risk of being affected with the condition. The chance that a first degree relative of someone with celiac disease will have the disease is about 10%.
As more is learned about celiac disease, it becomes evident that it has many variations which may not produce typical symptoms. It may even be clinically "silent," where no obvious problems related to the disease are apparent.

Causes and symptoms

Celiac disease can run in families and has a genetic basis, although the pattern of inheritance is complicated. The type of inheritance pattern that celiac disease follows is called multifactorial (caused by many factors, both genetic and environmental). Researchers think that several factors must exist in order for the disease to occur. The patient must have a genetic predisposition to develop the disorder. Then, something in their environment acts as a stimulus, or "trigger," to their immune system, causing the disease to become active for the first time. For conditions with multifactorial inheritance, people without the genetic predisposition are less likely to develop the condition with exposure to the same triggers. Or, they may require more exposure to the stimulus before developing the disease than someone with a genetic predisposition. Some of the things which may provoke a reaction include surgery, especially gastrointestinal surgery; a change to a low fat diet, which has an increased number of wheat-based foods; pregnancy; childbirth; severe emotional stress; or a viral infection. This combination of genetic susceptibility and an outside agent leads to celiac disease.
Each person with celiac disease is affected differently. When food containing gluten reaches the small intestine, the immune system begins to attack a substance called gliadin, which is found in the gluten. The resulting inflammation causes damage to the delicate finger-like structures in the intestine, called villi, where food absorption actually takes place. The patient may experience a number of symptoms related to the inflammation and the chemicals it releases, and/or the lack of ability to absorb nutrients from food, which can cause malnutrition.
The most commonly recognized symptoms of celiac disease relate to the improper absorption of food in the gastrointestinal system. Many patients with gastrointestinal symptoms will have diarrhea and fatty, greasy, unusually foul-smelling stools. The patient may complain of excessive gas (flatulence), distended abdomen, weight loss, and generalized weakness. Not all people have digestive system complications; some people only have irritability or depression. Irritability is one of the most common symptoms in children with celiac disease.
Not all patients have these problems. Unrecognized and therefore untreated celiac disease may cause or contribute to a variety of other conditions. The decreased ability to digest, absorb, and utilize food properly (malabsorption) may cause anemia (low red blood count) from iron deficiency or easy bruising from a lack of vitamin K. Poor mineral absorption may result in osteoporosis, or "brittle bones," which may lead to bone fractures. Vitamin D levels may be insufficient and bring about a "softening" of bones (osteomalacia), which produces pain and bony deformities, such as flattening or bending. Defects in the tooth enamel, characteristic of celiac disease, may be recognized by dentists. Celiac disease may be discovered during medical tests performed to investigate failure to thrive in infants, or lack of proper growth in children and adolescents. People with celiac disease may also experience lactose intolerance because they don't produce enough of the enzyme lactase, which breaks down the sugar in milk into a form the body can absorb. Other symptoms can include muscle cramps, fatigue, delayed growth, tingling or numbness in the legs (from nerve damage), pale sores in the mouth (called aphthus ulcers), tooth discoloration, or missed menstrual periods (due to severe weight loss).
A distinctive, painful skin rash, called dermatitis herpetiformis, may be the first sign of celiac disease. Approximately 10% of patients with celiac disease have this rash, but it is estimated that 85% or more of patients with the rash have the disease.
Many disorders are associated with celiac disease, though the nature of the connection is unclear. One type of epilepsy is linked to celiac disease. Once their celiac disease is successfully treated, a significant number of these patients have fewer or no seizures. Patients with alopecia areata, a condition where hair loss occurs in sharply defined areas, have been shown to have a higher risk of celiac disease than the general population. There appears to be a higher percentage of celiac disease among people with Down syndrome, but the link between the conditions is unknown.
Several conditions attributed to a disorder of the immune system have been associated with celiac disease. People with insulin dependent diabetes (type I) have a much higher incidence of celiac disease. One source estimates that as many as one in 20 insulin-dependent diabetics may have celiac disease. Patients with other conditions where celiac disease may be more commonly found include those with juvenile chronic arthritis, some thyroid diseases, and IgA deficiency.
There is an increased risk of intestinal lymphoma, a type of cancer, in individuals with celiac disease. Successful treatment of the celiac disease seems to decrease the chance of developing lymphoma.

Diagnosis

Because of the variety of ways celiac disease can manifest itself, it is often not discovered promptly. Its symptoms are similar to many other conditions including irritible bowel syndrome, Crohn's disease, ulcerative colitis, diverticulosis, intestinal infections, chronic fatigue syndrome, and depression. The condition may persist without diagnosis for so long that the patient accepts a general feeling of illness as normal. This leads to further delay in identifying and treating the disorder. It is not unusual for the disease to be identified in the course of medical investigations for seemingly unrelated problems. For example, celiac disease has been discovered during testing to find the cause of infertility.
If celiac disease is suspected, a blood test can be ordered. This test looks for the antibodies to gluten (called antigliadin, anti-endomysium, and antireticulin) that the immune system produces in celiac disease. Antibodies are chemicals produced by the immune system in response to substances that the body perceives to be threatening. Some experts advocate not just evaluating patients with symptoms, but using these blood studies as a screening test for high-risk individuals, such as those with relatives (especially first degree relatives) known to have the disorder. An abnormal result points towards celiac disease, but further tests are needed to confirm the diagnosis. Because celiac disease affects the ability of the body to absorb nutrients from food, several tests may be ordered to look for nutritional deficiencies. For example, doctors may order a test of iron levels in the blood because low levels of iron (anemia) may accompany celiac disease. Doctors may also order a test for fat in the stool, since celiac disease prevents the body from absorbing fat from food.
If these tests above are suspicious for celiac disease, the next step is a biopsy (removal of a tiny piece of tissue surgically) of the small intestine. This is usually done by a gastroenterologist, a physician who specializes in diagnosing and treating bowel disorders. It is generally performed in the office, or in a hospital's outpatient department. The patient remains awake, but is sedated. A narrow tube, called an endoscope, is passed through the mouth, down through the stomach, and into the small intestine. A small sample of tissue is taken and sent to the laboratory for analysis. If it shows a pattern of tissue damage characteristic of celiac disease, the diagnosis is established.
The patient is then placed on a gluten-free diet (GFD). The physician will periodically recheck the level of antibody in the patient's blood. After several months, the small intestine is biopsied again. If the diagnosis of celiac disease was correct (and the patient followed the rigorous diet), healing of the intestine will be apparent. Most experts agree that it is necessary to follow these steps in order to be sure of an accurate diagnosis.

Treatment

The only treatment for celiac disease is a gluten-free diet. This may be easy for the doctor to prescribe, but difficult for the patient to follow. For most people, adhering to this diet will stop symptoms and prevent damage to the intestines. Damaged villi can be functional again in three to six months. This diet must be followed for life. For people whose symptoms are cured by the gluten-free diet, this is further evidence that their diagnosis is correct.
Gluten is present in any product that contains wheat, rye, barley, or oats. It helps make bread rise, and gives many foods a smooth, pleasing texture. In addition to the many obvious places gluten can be found in a normal diet, such as breads, cereals, and pasta, there are many hidden sources of gluten. These include ingredients added to foods to improve texture or enhance flavor and products used in food packaging. Gluten may even be present on surfaces used for food preparation or cooking.
Fresh foods that have not been artificially processed, such as fruits, vegetables, and meats, are permitted as part of a GFD. Gluten-free foods can be found in health food stores and in some supermarkets. Mail-order food companies often have a selection of gluten-free products. Help in dietary planning is available from dieticians (healthcare professionals specializing in food and nutrition) or from support groups for individuals with celiac disease. There are many cookbooks on the market specifically for those on a GFD.
Treating celiac disease with a GFD is almost always completely effective. Gastrointestinal complaints and other symptoms are alleviated. Secondary complications, such as anemia and osteoporosis, resolve in almost all patients. People who have experienced lactose intolerance related to their celiac disease usually see those symptoms subside, as well. Although there is no risk and much potential benefit to this treatment, it is clear that avoiding all foods containing gluten can be difficult.
Experts emphasize the need for lifelong adherence to the GFD to avoid the long-term complications of this disorder. They point out that although the disease may have symptom-free periods if the diet is not followed, silent damage continues to occur. Celiac disease cannot be "outgrown" or cured, according to medical authorities.

Key terms

Antibodies — Proteins that provoke the immune system to attack particular substances. In celiac disease, the immune system makes antibodies to a component of gluten.
Gluten — A protein found in wheat, rye, barley, and oats.
Villi — Tiny, finger-like projections that enable the small intestine to absorb nutrients from food.

Prognosis

Patients with celiac disease must adhere to a strict GFD throughout their lifetime. Once the diet has been followed for several years, individuals with celiac disease have similar mortality rates as the general population. However, about 10% of people with celiac disease develop a cancer involving the gastrointestinal tract (both carcinoma and lymphoma).
There are a small number of patients who develop a refractory type of celiac disease, where the GFD no longer seems effective. Once the diet has been thoroughly assessed to ensure no hidden sources of gluten are causing the problem, medications may be prescribed. Steroids or immunosuppressant drugs are often used to try to control the disease. It is unclear whether these efforts meet with much success.

Prevention

There is no way to prevent celiac disease. However, the key to decreasing its impact on overall health is early diagnosis and strict adherence to the prescribed gluten-free diet.

Resources

Periodicals

Gluten-Free Living, (Bimonthly newsletter). PO Box 105, Hastings-on-Hudson, NY 10706.

Organizations

American Celiac Society. 58 Musano Court, West Orange, NJ 07052. (201) 325-8837.
Celiac Disease Foundation. 13251 Ventura Blvd., Suite 1, Studio City, CA 91604-1838. (818) 990-2354. 〈http://www.cdf@celiac.org〉.
Celiac Sprue Association/United States of America (CSA/USA). PO Box 31700, Omaha, NE 68131-0700. (402) 558-0600.
Gluten Intolerance Group. PO Box 23053, Seattle, WA 98102-0353. (206) 325-6980.
National Center for Nutrition and Dietetics. American Dietetic Association, 216 West Jackson Boulevard, Suite 800, Chicago, IL 60606-6995. (800) 366-1655.

Other

National Institute of Diabetes & Digestive & Kidney Diseases. http://www.niddk.nih.gov/health/digest/pubs/celiac/index.htm.

celiac

 [se´le-ak]
pertaining to the abdomen.
celiac disease a malabsorption syndrome characterized by marked atrophy and loss of function of the villi of the jejunum and occasionally the cecum. A distinction was formerly made between infantile, childhood, and adult forms, but they are now all considered to be the same entity. Called also celiac or nontropical sprue and gluten enteropathy.

The condition is related in some way to dietary gluten and is either a hypersensitive reaction to a protein in certain cereal grains or a local toxic inflammatory reaction to gluten. A hereditary factor has been implicated because the disease occurs in familial clusters. Diagnosis is usually made in young to middle-aged adults, but the onset of symptoms often is traced to early childhood.

The symptoms of celiac disease are fairly typical of all malabsorption syndromes. Manifestations include large, foul-smelling, bulky, frothy, and pale-colored stools containing much fat. There are recurrent attacks of diarrhea, with accompanying stomach cramps, alternating with constipation. There is some edema and abdominal distention as in severe malnutrition, extreme weight loss, asthenia, deficiency of vitamins B, D, and K, and electrolyte depletion.

Diagnosis is based on intestinal biopsy and demonstrated pathological changes in the structure of the absorbing cells of the small intestine. In many cases, elimination of gluten from the diet produces a dramatic improvement in symptoms and restoration of normal function of the small intestine. Some patients experience remission within a few days, while others continue to have symptoms for months.

Treatment consists of placing the patient on a gluten-free diet that excludes all cereal grains except corn and rice. Since many prepared foods contain wheat, barley, rye, or oats to provide bulk, the patient must be cautioned to read all labels on packaged foods, even ice cream, salad dressings, condiments, and foods one would not expect to contain cereal products. (See also gluten.) Administration of corticosteroids may be necessary for some adults who do not respond to a gluten-free diet. There is evidence that celiac disease is associated with lymphoma and carcinoma of the small bowel; this is especially true of patients who have not been treated with a gluten-free diet.

ce·li·ac dis·ease

[MIM*212750]
a disease occurring in children and adults characterized by sensitivity to gluten, with chronic inflammation and atrophy of the mucosa of the upper small intestine; manifestations include diarrhea, malabsorption, steatorrhea, nutritional and vitamin deficiencies, and failure to thrive, or short stature.

celiac disease

n.
A chronic gastrointestinal disease characterized by sensitivity to gluten and malabsorption from the small intestine, resulting in diarrhea, stools with high fat content, and nutritional and vitamin deficiencies. Also called celiac sprue, gluten-sensitive enteropathy, nontropical sprue.

celiac disease

Etymology: Gk, koilia + L, dis, opposite of; Fr, aise, ease
an inborn error of metabolism characterized by the inability to hydrolyze peptides contained in gluten. Gluten is found in wheat, oats, and barley. The disease affects adults and young children, who suffer from abdominal distension, vomiting, diarrhea, muscle wasting, and extreme lethargy. A characteristic sign is a pale, foul-smelling stool that floats on water because of its high fat content. There may be a secondary lactose intolerance, and it may become necessary to eliminate all milk products from the diet. Most patients respond well to a high-protein, high-calorie, gluten-free diet. Rice and corn are good substitutes for wheat, and any vitamin or mineral deficiencies can be corrected with oral preparations. Prognosis for full recovery is excellent. Failure to respond generally indicates misdiagnosis. Also called celiac sprue, gluten-induced enteropathy, nontropical sprue. Compare malabsorption syndrome.

coeliac disease

A malabsorptive syndrome caused by hypersensitivity of intestinal mucosa to alpha-gliadin, a gluten extract composed of glutamine and proline-rich proteins that is found in wheat, barley, rye, and oats.
 
Clinical findings
Diarrhoea, copious fatty stools, abdominal distension, weight loss, haemorrhage, osteopenia, muscle atrophy, peripheral neuropathy, CNS and spinal cord demyelination (sensory loss, ataxia), amenorrhoea, infertility, oedema, petechiae, dermatitis herpetiformis (especially if HLA B27), alopecia areata. Occult bleeding occurs in half of patients.
 
Lab
Transaminases (ALT, AST) are increased in ± 40% of patients with CD, which usually normalises with a gluten-free diet; iron deficiency, anaemia due to occult bleeding.

Diagnosis
Duodenal biopsy; anti-gliadin and anti-endomysial antibodies.
 
Management
Eliminate gliadin from diet.

Prognosis
Without treatment, 10–15% develop lymphoma (e.g., immunoblastic lymphoma; less commonly, T cell lymphoma), a risk that increases with disease duration. CD predisposes to gastrointestinal lymphoma and carcinoma of the oral cavity and oesophagus; the otherwise rare small intestinal adenocarcinoma is 80-fold more common in CD.

celiac disease

Celiac sprue, celiac syndrome, gluten-sensitive enteropathy GI disease A malabsorptive syndrome caused by hypersensitivity of intestinal mucosa to α-gliadin, a gluten extract composed of glutamine and proline-rich proteins, present in wheat, barley, rye, and oats Clinical Diarrhea, copious fatty stools, abdominal distension, weight loss, anemia, hemorrhage, osteopenia, muscle atrophy, peripheral neuropathy, CNS and spinal cord demyelination–sensory loss, ataxia, amenorrhea, infertility, edema, petechiae, dermatitis herpetiformis, especially if an HLA B27 haplotype Lab Transaminases–ALT, AST are ↑ in ± 40% of Pts with CD, which usually normalizes with gluten-free diet; occult bleeding occurs in ±12 of Pts with CD and may be severe enough to cause iron deficiency anemia; Pts may also have alopecia areata, antigliadin and antiendomysial antibodies Diagnosis Small intestine Bx Management Eliminate gliadin from diet; without treatment 10-15% develop lymphoma–eg, immunoblastic lymphoma, less commonly, T cell lymphoma; CD predisposes to GI lymphoma, and carcinoma of the oral cavity, esophagus; the otherwise rare small intestinal adenoCA is 80-fold more common in CD. Cf Tropical sprue.

ce·li·ac dis·ease

(sē'lē-ak di-zēz')
A disease occurring in children and adults characterized by sensitivity to gluten, with chronic inflammation and atrophy of the mucosa of the upper small intestine; manifestations include diarrhea, malabsorption, steatorrhea, and nutritional and vitamin deficiencies.
Synonym(s): gluten enteropathy, coeliac disease.

Herter,

Christian A., U.S. physician, 1865-1910.
Gee-Herter disease - Synonym(s): Gee disease
Herter disease - gluten sensitivity, manifested by diarrhea, malabsorption, steatorrhea, nutritional and vitamin deficiencies. Synonym(s): celiac disease
Heubner-Herter disease - Synonym(s): Gee disease

ce·li·ac dis·ease

(sē'lē-ak di-zēz') [MIM*212750]
A disease occurring in children and adults characterized by sensitivity to gluten, with chronic inflammation and atrophy of the mucosa of the upper small intestine.
Synonym(s): gluten enteropathy, coeliac disease.

celiac

pertaining to the abdomen.

celiac disease
a malabsorption syndrome in humans characterized by marked atrophy and loss of function of the villi of the jejunum (and rarely, the cecum). Called also celiac sprue, gluten-induced enteropathy, nontropical sprue, and adult, childhood or infantile celiac disease. Occasionally suspected as the cause of villous atrophy in dogs.

Patient discussion about celiac disease

Q. Is celiac genetic? I have one son with celiac disease from my first marriage and me second wife is now pregnant,I was wondering what are the chances for this soon to be born daughter of mine to have celiac as well- if I maybe carry the genetic flaw and is there a way to find out?

A. Celiac disease is a very common illness (about 1 in a 100 people suffer from it in different levels), and it is known to have a strong genetic connection. However, there is not one specific mutation that you can get genetic testing to see if you are carrying it. Your soon to be born daughter will have a higher chance than the regular population to suffer from the disease, but it does not necessarily mean she will.

Q. How do you diagnose celiac? My daughter is 3 years old and is constantly vomiting, has diarrhea and stomach aches. Could this be celiac?

A. This could in fact be celiac. The initial step in screening should include: IgA endomysial antibodies (EMA), IgA tissue transglutaminase (tTG), IgG tissue transglutaminase and Total IgA antibodies. The patients with positive antibody tests, and those with an IgA deficiency, should have a small bowel biopsy to confirm the diagnosis and assess the degree of damage, which is performed endoscopically (looking inside the body by inserting a tube into it).

Q. Is FTT a symptom of celiac? My 1.5 year old son has FTT (failure to thrive) and stomach aches. What could be causing it?

A. Failure to thrive lacks a precise definition, in part because it describes a condition rather than a specific disease. Children who fail to thrive don't receive or are unable to take in, retain, or utilize the calories needed to gain weight and grow as expected. FTT can be caused from many different things: social factors, conditions involving the gastrointestinal system like gastroesophageal reflux, chronic diarrhea, cystic fibrosis, chronic liver disease, and celiac disease. From a chronic illness or medical disorder, an intolerance of milk protein, infections or metabolic disorders.

More discussions about celiac disease
References in periodicals archive ?
In addition, he said, "We did not find evidence of increased dementia risk prior to the diagnosis of celiac disease either.
Celiac disease is characterized by damage to the lining of the small intestine, causing gastrointestinal dysfunction and debilitating symptoms.
Table 1: Risk Factors for Celiac Disease Table 2: Studies Reporting Temporal Trends for Celiac Disease and Calculated Rate of Change Table 3: 6MM, Sources of Prevalence and Diagnosis Rates of Celiac Disease Table 4: 6MM, Sources Not Used in Epidemiological Forecast for Celiac Disease Table 5: 6MM, Total Prevalent Cases of Celiac Disease, Both Sexes, All Ages, N, Select Years 2013-2023 Table 6: 6MM, Age-Specific Total Prevalent Cases of Celiac Disease, Both Sexes, N (Row %), 2013 Table 7: 6MM, Sex-Specific Total Prevalent Cases of Celiac Disease, All Ages, N (Row %), 2013 Table 8: 6MM, Diagnosed Prevalent Cases of Celiac Disease, Both Sexes, All Ages, Select Years 2013-2023
Sarah Sleet, chief executive of Coeliac UK said: "This latest research shows that nearly a quarter of people with celiac disease have now been diagnosed and gives an up to date picture of the diagnosis levels across the U.
Children with celiac disease experience a multitude of problems during their time in school.
Twenty five treatment-naive patients with celiac disease were included in this study.
In fact, according to a recent study by NPD Group, three million Americans have celiac disease, which is four times more common than it was 50 years ago.
Generally, everyone experiences a few symptoms at times that are also the classic presentation of celiac disease, like diarrhoea, weight loss and fatigue, (may also include flatulence, constipation, bloating, indigestion, abdominal pain) and attribute it to allergy or sensitivity to gluten.
I will first use celiac disease-associated protein-coding genes to delineate their regulatory pathways and then study the transcriptional programs of lincRNAs present in celiac disease loci.
Treatment for celiac disease consists of supplementation for nutrient deficiencies or conditions (such as anemia) resulting from malabsorption and adherence to a gluten-free diet.
The primary celiac disease trigger in wheat is a kind of gluten protein called gliadin, but celiac patients can be sensitive to other types as well.