Cases reported "Celiac Disease"

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1/12. An uncommon association: celiac disease and dermatomyositis in adults.

    We describe the case of a patient with a two-year history of adult dermatomyositis (DM) who developed malabsorption revealing celiac disease. Our observation raises the question of an association between DM and celiac disease as part of a continuum, suggesting that celiac disease may be included within the spectrum of the gastrointestinal manifestations of DM and polymyositis (PM). From a practical point of view, our data indicate that the diagnosis of celiac disease should be suspected in PM/DM patients exhibiting malabsorption syndrome. Based on our findings, we further emphasize that an evaluation for celiac disease, including anti-gliadin antibodies, anti-endomysium antibody and tissue trans-glutaminase antibodies should be considered in PM/DM patients presenting with unusual and unexplained gastrointestinal features. This could lead to the early management of such patients, resulting in decreased morbidity (i.e., malnutrition and malignancy) related to misdiagnosed celiac disease.
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2/12. Nodular regenerative hyperplasia of the liver in a patient with celiac disease.

    We present the case of dual adult celiac disease and liver disease with portal hypertension (esophageal varices); a percutaneous liver biopsy was compatible with nonspecific reactive hepatitis. Clinically, celiac disease was characterised by poor response to a gluten-free diet, with the development of a biochemical cholestasis and marked malnutrition. Our patient died of cerebral hemorrhage, at the age of 50 years, without associated risk factors. The necropsy demonstrated the existence of a nodular regenerative hyperplasia of the liver, splenic atrophy, gelatinous transformation of the bone marrow, and lymphocytic colitis. We discuss the different types of liver disorders associated with celiac disease and the possible relation between nodular regenerative hyperplasia and celiac disease, based on immunologic mechanisms.
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3/12. Very severe iron-deficiency anemia in a patient with celiac disease and bulimia nervosa: a case report.

    A 29-year-old woman presented to the emergency department with exhaustion, fatigue, and abdominal pain. She reported having received a diagnosis of bulimia nervosa 10 years before. On examination, she had a marked pallor and was severely malnourished. Laboratory analysis revealed a dramatically low hemoglobin level of 1.7 g/dL (ref: 11.5-15.8 g/dL). serum iron was quantified as 1.4 micromol/L (ref: 7-26 micromol/L), ferritin as 5 ng/mL (ref: 10-120 ng/mL), and the level of serum transferrin as 212 mg/dL (ref: 200-360 mg/dL). A duodenal biopsy revealed villous atrophy in the mucosal layer indicative for celiac disease. This diagnosis was confirmed by serum levels of endomysial antibodies, tissue transglutaminase antibody, and antigliadin antibodies. The newly diagnosed gluten-sensitive enteropathy is likely to be in part responsible for the severe symptoms reported. The extent of hemoglobin decline in combination with an astonishing lack of critical symptoms seen in this patient is a rarity. We conclude that anorectic patients with severe anemia and malnutrition should be evaluated for the presence of additional somatic conditions.
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4/12. Congenital intractable diarrhea with possible defective crypt regeneration: report of a case.

    A male infant, born uneventfully from a consanguinous marriage, presented with intractable watery diarrhea from his third day of life, with subsequent malnutrition and failure to thrive. He received central parenteral nutrition beginning at three months of age after a poor response to a semielemental diet and peripheral parenteral nutrition. He was totally dependent on central parenteral nutrition thereafter. Although diarrhea disappeared with strict bowel rest, intolerance to minimal enteral feedings persisted throughout his 2 years 4 months of life. Investigations including stool examinations and repeated cultures, immune function studies, radiologic studies of the small bowel and screening for galactosemia and cystic fibrosis could not demonstrate a specific cause for the diarrhea. Repeated small intestinal biopsies at 1 month, 4 months and 1 year 5 months of age showed persistent villous atrophy with crypt hypoplasia and a low crypt mitotic index. Electron microscopic examination revealed normal-appearing microvilli. This child may have had a congenital enteropathy due to an inborn crypt regeneration defect causing lifelong intolerance to enteral feedings.
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5/12. celiac disease presenting with chilblains in an adolescent girl.

    chilblains, or pernio, are cutaneous lesions that may accompany systemic illnesses including states of malnutrition and autoimmune diseases. We report an adolescent girl in whom chilblains were the chief presenting sign of celiac disease. A gluten-free diet led to weight gain and resolution of the chilblains. We speculate that in this patient, weight loss due to celiac disease contributed to the development of chilblains.
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keywords = malnutrition
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6/12. Susceptibility to tuberculosis in patients with coeliac disease.

    An increased prevalence of past tuberculosis is reported in an adult coeliac population. Of 76 adult coeliac disease patients, 6 had had a history of tuberculosis. This compared with the finding of no cases in a population of 81 patients with non-inflammatory bowel diseases, (p = 0.023), which was matched for age, sex, smoking, ethnic origin and social class. The 'expected' number of cases of tuberculosis amongst ACD patients has also been calculated based on local annual notification rates; this was 2.9. Radiological evidence of past tuberculosis was found in 13 (17%) ACD patients, compared with 4 (5%) control patients (p less than 0.05). It is postulated that the increased prevalence of past tuberculosis in ACD patients is the result of depressed cell mediated immunity and/or malnutrition.
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keywords = malnutrition
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7/12. Malabsorption: common causes and their practical diagnosis.

    Malabsorption may present as non-specific ill health and malnutrition in the elderly. Outside major medical centers, however, investigation is often thought to be difficult and unrewarding, and screening tests unreliable. Therefore, a direct approach is suggested to identify treatable small bowel disease in those elderly patients with a clinical possibility of malabsorption. It is based on the use of plain abdominal x-ray, endoscopy with duodenal biopsy, and small bowel meal.
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8/12. Abnormal sweat electrolytes in a case of celiac disease and a case of psychosocial failure to thrive. review of other reported causes.

    sweat electrolytes were initially elevated in a child who was diagnosed as having celiac disease and also in one with psychosocial failure to thrive. Subsequent sweat tests were normal after nutritional status of the patients had improved with therapy. The reports of elevated sweat electrolytes in conditions other than cystic fibrosis are discussed. It is emphasized that sweat test methods other than the quantitative pilocarpine iontophoresis method are not reliable. The occurrence of false-positive sweat tests demonstrates the need for care in the interpretation of elevated sweat electrolytes and the necessity of performing repeat quantitative pilocarpine iontophoresis for the establishment of the diagnosis of CF. celiac disease and malnutrition from other causes may cause sweat electrolytes to be elevated.
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keywords = malnutrition
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9/12. Copper deficiency in infants with active celiac disease.

    celiac disease was diagnosed in two unrelated infants aged 7 and 7.5 months with severe malnutrition. They showed typical clinical, biological, and histological signs of the disease. Moreover, accompanying copper deficiency was suggested by severe hypocupremia and persistent neutropenia; bone radiographs were also compatible with this diagnosis. Rapid and complete correction of these anomalies could only be obtained after addition of oral copper sulfate to the gluten-free diet. Mechanisms possibly involved in the development of copper deficiency in young infants with celiac disease are: chronic malabsorption; high copper needs in rapidly growing infants; and possibly increased biliary and digestive losses. It is therefore suggested that young children with severe celiac disease should be monitored for their copper status.
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keywords = malnutrition
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10/12. Ox bile treatment of severe steatorrhea in an ileectomy-ileostomy patient.

    Bile salt therapy is not used in patients with steatorrhea due to bile salt deficiency because of fear that severe diarrhea would be caused or exacerbated. We report a patient who previously had had colectomy, partial ileectomy, and ileostomy for Crohn's disease. She had severe steatorrhea due to bile salt deficiency and severe diarrhea (the latter apparently due to fatty acid inhibition of electrolyte and water absorption). The diarrhea was improved by loperamide, but severe steatorrhea and malnutrition persisted. The steatorrhea and malnutrition were corrected by ox bile, without an increase in diarrhea. Presumably, the deleterious effect of bile salts per se on small bowel absorption of water and electrolytes was mitigated by correction of fat malabsorption. At least in this patient, bile salt therapy was highly beneficial.
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keywords = malnutrition
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