Celiac disease (also known as idiopathic steatorrhea, nontropical sprue, gluten enteropathy, and celiac sprue) is characterized by poor food absorption and intolerance of gluten, a protein in wheat and wheat products. Malabsorption in the small bowel results from atrophy of the villi and a decrease in the activity and amount of enzymes in the surface epithelium. The prognosis is good with treatment (eliminating gluten from the patient’s diet), but residual bowel changes may persist in adults.
In celiac disease, an intramucosal enzyme defect produces an inability to digest gluten. Resulting tissue toxicity produces rapid cell turnover, increases epithelial lymphocytes, and damages surface epithelium of the small bowel.
Celiac disease affects 1 of every 133 people in the United States and results from environmental factors and a genetic predisposition, but the exact mechanism is unknown. A strong association exists between the disease and two human leukocyte antigen haplotypes, DR3 and DQw2. It may also be autoimmune in nature. It affects twice as many females as males and occurs more commonly among relatives, especially siblings. This disease primarily affects whites and those of European ancestry.
Many diseases and conditions are associated with celiac disease, including:
❑ anemia
❑ lactose intolerance
❑ skin disorders such as dermatitis herpetiformis (a burning, itching, blistering rash)
❑ type 1 diabetes mellitus
❑ thyroid disease
❑ Down syndrome
❑ unexplained infertility or miscarriage
❑ osteoporosis or osteopenia
❑ autoimmune disorders, such as rheumatoid arthritis and systemic lupus erythematosus.
Celiac disease produces clinical effects on many body systems:
❑ GI symptoms include recurrent attacks of diarrhea, steatorrhea, abdominal distention due to flatulence, stomach cramps, weakness, anorexia and, occasionally, increased appetite without weight gain. Atrophy of intestinal villi leads to malabsorption of fat, carbohydrates, and protein as well as loss of calories, fat-soluble vitamins (A, D, and K), calcium, and essential minerals and electrolytes. In adults, celiac disease produces multiple nonspecific ulcers in the small bowel, which may perforate or bleed.
❑ Hematologic effects include normochromic, hypochromic, or macrocytic anemia due to poor absorption of folate, iron, and vitamin B12 and to hypoprothrombinemia from jejunal loss of vitamin K.
❑ Osteomalacia, osteoporosis, tetany, and bone pain (especially in the lower back, rib cage, and pelvis) are some of the musculoskeletal symptoms of celiac disease. These signs and symptoms are due to calcium loss and vitamin D deficiency, which weakens the skeleton, causing rickets in children and compression fractures in adults.
❑ Neurologic effects may include peripheral neuropathy, seizures, or paresthesia.
❑ Dry skin, eczema, psoriasis, dermatitis herpetiformis, and acne rosacea are some of the dermatologic effects of celiac disease. Deficiency of sulfur-containing amino acids may cause generalized fine, sparse, prematurely gray hair; brittle nails; and localized hyperpigmentation on the face, lips, or mucosa.
❑ Endocrine symptoms include amenorrhea, hypometabolism and, possibly, with severe malabsorption, adrenocortical insufficiency.
❑ Psychosocial effects include mood changes and irritability.
Symptoms may develop during the first year of life, when gluten is introduced into the child’s diet as cereal. Clinical effects may disappear during adolescence and reappear in adulthood. One theory proposes that the age at which symptoms first appear depends on the strength of the genetic factor: A strong factor produces symptoms during the child’s first 4 years; a weak factor, in late childhood or adulthood.
An elevated alkaline phosphatase level may indicate bone loss, which is commonly experienced before diagnosis. Low cholesterol and albumin levels may reflect malabsorption and malnutrition. Mildly elevated liver enzymes and abnormal blood clotting may also be noted as well as anemia.
Antibody blood tests useful in screening for celiac disease include antiendomysial antibody (IgA), antitransglutaminase (IgA), antigliadin (IgA and IgG), and total serum IgA. Combined, these antibodies provide a sensitive and specific indicator for the presence of celiac disease.
Treatment requires elimination of gluten from the patient’s diet for life. Even with this exclusion, a full return to normal absorption and bowel histology may not occur for months or may never occur.
Supportive treatment may include supplemental iron, vitamin B12, and folic acid; reversal of electrolyte imbalance (by I.V. infusion, if necessary); I.V. fluid replacement for dehydration; corticosteroids to treat accompanying adrenal insufficiency; and vitamin K for hypoprothrombinemia.
Explain the necessity of a gluten-free diet to the patient (and to his parents, if the patient is a child). Advise eliminating wheat, barley, rye, and oats as well as foods made from these grains, such as breads and baked goods; suggest substituting corn or rice. Consult a dietitian for nutritional instruction on a gluten-free diet. Depending on individual tolerance, the diet initially consists of proteins and gradually expands to include other foods. Assess the patient’s acceptance and understanding of the disease, and encourage regular reevaluation.
❑ Observe the patient’s nutritional status and progress by daily calorie counts and weight checks. Also, evaluate his tolerance to new foods. In the early stages, offer small, frequent meals to counteract anorexia.
❑ Assess the patient’s fluid status: record intake, urine output, and number of stools (may exceed 10 per day). Watch for signs of dehydration, such as dry skin and mucous membranes, and poor skin turgor.
❑ Check serum electrolyte levels. Watch for signs of hypokalemia (weakness, lethargy, rapid pulse, nausea, and diarrhea) and low calcium levels (impaired blood clotting, muscle twitching, and tetany).
❑ Monitor prothrombin time, hemoglobin level, and hematocrit. Protect the patient from bleeding and bruising. Use the Z-track method to give iron I.M. If the patient can tolerate oral iron, give it between meals, when absorption is best. Dilute oral iron preparations, and give them through a straw to prevent staining teeth.
❑ Protect the patient with osteomalacia from injury by keeping the bed side rails up and assisting with ambulation, as necessary.
❑ Advise the patient to contact the Gluten Intolerance Group or the Associated Celiac Disease Foundation for information and support.
Review other book chapters online related to Celiac Disease:
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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Title: Professional Guide to Diseases (Eighth Edition)
Authors: Springhouse
Publisher: Lippincott Williams & Wilkins
Copyright: 2005
ISBN: 1-58255-370-X
» Next page: Celiac Disease (The 5-Minute Pediatric Consult)
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