| Celiac Disease | | Counselor, Celiac Disease, otherwise known as celiac sprue is a gluten- sensitive enteropathy. It is a chronic disease of the digestive tract that interferes with the digestion and absorption of food nutrients. People with celiac sprue cannot tolerate gluten, a protein commonly found in wheat, rye, barley, and, sometimes, oats. When people with celiac sprue ingest gluten, the mucosa of their intestines is damaged by an immunologically mediated inflammatory response, resulting in maldigestion and malabsorption. The result is severe diarrhea. We value all your comments, so, if you have a suggestion for a newsletter subject but haven't submitted it yet, or if you have already submitted one but think of another, please take a minute to let us know by clicking on your "Reply" button and dropping us a note. To learn more about AMFS, Inc., the organization run by Physicians and Attorneys that provides medical experts and case review services nationwide, and has produced the following informational newsletter to aid you in understanding complex medical issues, please click here - www.amfs.com. |
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| Pathophysiology: Celiac sprue has a strong hereditary component. The prevalence of the condition in first-degree relatives is approximately 10%. Strong association exists between celiac sprue and 2 human leukocyte antigen (HLA) haplotypes (DR3 and DQw2). Damage to the intestinal mucosa is seen with the presentation of gluten-derived peptides by the HLA molecules to helper T cells. (Helper T cells mediate the inflammatory response.) Absence of intestinal villi and lengthening of intestinal crypts characterize mucosal lesions in untreated celiac sprue. |
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| Frequency/Mortality/Morbidity: The frequency of celiac sprue in the United States is relatively low, about 1 case in 3000 persons. The low frequency of celiac sprue may be because of underdiagnosis of the disease, in part, because of the lack of laboratories with personnel experienced in diagnostic testing of celiac sprue. Although rarely lethal, celiac sprue is a significant and often debilitating maldigestive and malabsorption syndrome affecting multiple organ systems. Patients with celiac sprue are at increased risk for complications such as lymphomas and adenocarcinomas of the intestinal tract. Untreated pregnant women are at risk of miscarriage and at risk of having a baby with a congenital malformation. Short stature often results when celiac sprue prevents nutrient absorption during the childhood years when nutrition is critical to growth and development. |
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| | History: Symptoms of celiac sprue malabsorption can include 1 or more of the following: chronic diarrhea, steatorrhea (malabsorption of ingested fat), abdominal bloating or cramps, flatulence, weight loss, fatigue, anemia, bleeding diathesis, osteopenia, seizure disorders, stunted growth. Diarrhea is the most common symptom in untreated celiac sprue. Diarrhea caused by celiac sprue is due to maldigestion and malabsorption of nutrients. The stools might be watery or semiformed, light tan or gray, and oily or frothy. The stools have a characteristic foul odor. In infants and young children, extensive diarrhea can lead to severe dehydration, electrolyte depletion, and metabolic acidosis. |
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| | Physical: Abdominal examination shows a protuberant and tympanic abdomen due to distention of intestinal loops with fluids and gas. Ascites occasionally can be detected in patients with severe hypoproteinemia. Evidence of weight loss, including muscle wasting or loose skin folds. Other findings include: orthostatic hypotension, peripheral edema, ecchymoses, hyperkeratosis or dermatitis herpetiformis, cheilosis and glossitis, evidence of peripheral neuropathy, chvostek sign or Trousseau sign. |
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| | Causes: Celiac sprue results from a combination of immunological responses to an environmental factor (gluten) and genetic factors. Gliadin (a complex mixture of proline- and glutamine-rich polypeptides obtained by alcohol extraction of wheat gluten) can produce symptoms and histological changes in the small intestine when administered to patients with asymptomatic celiac sprue. Antigliadin antibodies can frequently be identified in untreated patients. Immunoglobulin A (IgA) antibodies to smooth muscle endomysium (a highly specific serologic marker for celiac sprue) can also be found. Recent studies indicate tissue transglutaminase as the endomysial antigen involved, suggesting that autoimmunity plays a role in the pathogenesis. Cell-mediated immune responses are also important for the pathogenesis of celiac sprue, as demonstrated by the presence of large numbers of CD8+ T lymphocytes in the intestinal epithelium. Genetics play an important role in celiac sprue. The incidence of celiac disease in relatives of patients with celiac sprue is significantly higher than in the general population. The prevalence in first-degree relatives of patients with celiac sprue is approximately 10%. Concordance for the disease in HLA identical siblings is about 30% and concordance for the disease in identical twins approaches 70%. |
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| | Lab Studies/Imaging Studies: Include all of the following: Electrolytes and chemistries including protein, cholesterol, and albumin, iron, folate, B-12, PT, stool examination, oral D-xylose and lactose tolerance tests, serology to include IgA and IgG antibodies to gliadin. Radiographic evaluation of the small bowel after barium ingestion is helpful in making a diagnosis of untreated celiac sprue. Abnormal radiographic findings can include dilatation of the small intestine, a coarsening or obliteration of the normally delicate mucosal pattern, and fragmentation or flocculation of the barium in the gut lumen. |
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| | Procedures: Results of the small intestinal biopsy, along with appropriate serum antibodies, usually establish the diagnosis. Histologic findings in celiac sprue primarily involve the mucosa of the small intestine. The submucosa, muscularis, and serosa are usually not involved. The villi are atrophic or absent, and crypts are elongated. The cellularity of the lamina propria is increased with a proliferation of plasma cells and lymphocytes. The number of intraepithelial lymphocytes per unit length of absorptive epithelium is increased. |
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| | Treatment: The primary treatment of celiac sprue is dietary. Removal of gluten from the diet is essential. Complete avoidance of gluten-containing grain products is relatively difficult for patients to achieve and maintain because certain products, such as wheat flour, are virtually ubiquitous in the American diet. A small percentage of patients with celiac sprue fail to respond to a gluten-free diet. In some patients who are refractory, corticosteroids might be helpful. In patients who fail to respond to corticosteroids, other comorbid conditions, such as lymphomas of the small intestine, should be considered. |
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| | Medical/Legal Concerns: Patients with celiac sprue are at increased risk for complications such as lymphomas and adenocarcinomas of the intestinal tract. A small percentage of patients with celiac sprue fail to respond to a gluten-free diet, and, in some patients with refractory disease, corticosteroids might be helpful. In patients who fail to respond to corticosteroids, other comorbid conditions, such as lymphomas of the small intestine, should be considered. Missing these serious cancers is unacceptable. Removal of gluten from the diet is essential to successful treatment of patients with celiac sprue. However, complete avoidance of gluten-containing grain products is relatively difficult to achieve and maintain because certain products, such as wheat flour, are virtually ubiquitous in the American diet. Careful and extensive indoctrination of the patient by the physician and the dietitian is often necessary to achieve full compliance. Compliance is important for pregnant women because, if untreated, pregnant women are at risk of miscarriage and at risk of having a baby with a congenital malformation. Compliance is also important for infants and young children because, with untreated celiac sprue, failure to gain weight and growth retardation are common in this population. |
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