Cases reported "Celiac Disease"

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1/72. Refractory sprue: recovery after removal of nongluten dietary proteins.

    A 44-year-old woman with diarrhea, weight loss, and a small-bowel biopsy consistent with adult celiac disease failed to improve on a gluten-free diet. Despite in-hospital supervision at two university medical centers and addition of corticosteroid therapy, diarrhea and wegith loss continued, resulting in life-threatening nutritional depletion. She was transferred to the University of chicago and made full nutritional recovery with total parenteral nutrition. Exploratory laparotomy showed no abnormality except the flat intestinal mucosal lesion. diarrhea recurred when a gluten-free diet was resumed. When the patient ate egg, chicken, or tuna alone, severe diarrhea, hypotension, cyanosis, and shock occurred. When these foods--along with gluten--were eliminated from the diet, the patient returned to oral nutrition and made a full clinical recovery. In patients with refractory sprue deletion of other dietary proteins in addition to gluten, as in the present patient, may be lifesaving.
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2/72. Hyposplenism, antiendomysial antibodies and lymphocytic colitis in collagenous sprue.

    A 66-year-old woman was seen repeatedly over a decade to remove recurrent colonic adenomas and investigate episodes of watery diarrhea. Although the diarrhea was believed to be due to lymphocytic colitis, she developed weight loss, hypoproteinemia and hyposplenism that resulted in further studies, specifically to exclude celiac disease. Small intestinal biopsies, however, showed severely 'flattened' villous architecture with trichrome-positive subepithelial collagenous deposits, characteristic of collagenous sprue. Antiendomysial antibodies, known serological markers of celiac disease, were also detected. While collagenous sprue has been considered a distinct small intestinal disorder, the constellation of clinical and pathological findings in this patient suggests a close link with adult celiac disease.
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3/72. azathioprine in refractory sprue.

    We report a patient with life-threatening refractory sprue who was dependent on high doses of corticosteroids to prevent severe diarrhea, malabsorption, and villous atrophy. azathioprine allowed tapering of corticosteroids to lower doses, while maintaining remission in histology and in objective measures of malabsorption. Immunosuppressive therapy, however, is not without risks, particularly in patients with associated hypoglobulinemia.
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4/72. adult celiac disease presented with celiac crisis: severe diarrhea, hypokalemia, and acidosis.

    An acute severe onset of celiac disease is very uncommon in adults. We describe a patient with adult celiac disease who presented with acute diarrhea that lead rapidly to a life threatening hypokalemia and acidosis, the so-called celiac crisis. Celiac crisis, described mainly in children younger than two years of age, has become very rare due to earlier diagnosis and effective therapy of the disease. The case described is an example of the heterogeneous clinical course of celiac disease and emphasizes the need to consider it in the differential diagnosis, even in adults suffering from acute diarrhea and acidosis.
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5/72. celiac disease and Turner's syndrome: patient report.

    Turner's syndrome is a chromosomal disease frequently associated with autoimmune disorders including thyroid disease, inflammatory bowel disease, diabetes mellitus and juvenile rheumatoid arthritis. Recent reports have described an association of celiac disease with Turner's syndrome. We present an additional patient with Turner's syndrome associated with celiac disease. A girl aged 15- 7/12 yr was seen for the complaints of delayed growth and puberty, abdominal pain and chronic diarrhea. She was diagnosed as having celiac disease and a gluten-free diet was initiated. Despite one year of strict diet no signs of puberty were observed. She was then evaluated again for absence of puberty, and 45,XO karyotype Turner's syndrome was diagnosed.
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6/72. Untractable diarrhea due to late onset celiac disease of the adult following pancreatoduodenectomy.

    This case describes a rare cause of severe diarrhea that may occasionally plague the postoperative course of pancreatic resections. Although exceedingly rare this complication has already been described in another two patients undergoing pancreatoduodenectomy with diagnosis of duodenojejunal adenocarcinoma. Surgeons dedicated to pancreatic resections as well as pancreatologists caring for these patients should retain this possibility in their cultural background. diarrhea, due to either exocrine insufficiency or interruption of sympathetic nerves to the small intestine, is a common complaint after pancreatoduodenectomy. A 54-year-old white female after a pancreatoduodenectomy with standard lymphatic clearance developed intractable diarrhea leading to severe cachexia. Recognition of the underlying occult celiac disease and institution of the appropriate dietary regimen allowed quick and expeditious recovery.
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7/72. helicobacter pylori-negative gastric ulcerations associated with celiac disease at first presentation.

    Ulcers of the small bowel have repeatedly been described as a late complication of celiac disease and they are considered a signum mali ominis. We report a case of a 53-year-old woman presenting with diarrhea, epigastric pain and abdominal distensions for a period of few weeks. At upper GI endoscopy, biopsies were taken showing complete atrophy of the villi and colonization of the small bowel mucosa. Additionally, uncommon multilocular peptic ulcers were seen in the gastric antrum. These ulcers proved to be helicobacter pylori-negative with no evidence of zollinger-ellison syndrome. Biopsies of gastric ulcers showed signs of a lymphocytic gastritis with an extensive infiltration of the lamina propria by almost exclusively CD3- and CD45R0-positive t-lymphocytes. Intraepithelial t-lymphocytes were found to be increased in the antral as well as the corpus mucosa. Typing the patient for human leukocyte antigens showed a DQA1*0501 and DQB1*0201 phenotype. According to the present report, gastric peptic ulcers seem to be another phenomenon associated with celiac disease. In the case presented here, ulcers were diagnosed together with celiac disease already at first presentation of the patient.
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8/72. abetalipoproteinemia: a case report.

    abetalipoproteinemia is a rare autosomal recessive disorder characterized by steatorrhea, poor weight gain, acanthocytosis and retinitis pigmentosa. Here we peresent a six-month-old patient with abetaliporoteinemia. He had a history of chronic diarrhea from the first month of life. He was cachectic and his motor development was delayed. Microscopic examination of the stool revealed fat. Mild anemia with reticulocytosis, acanthocytosis, low triglyceride, low cholesterol, low-density lipoprotein, high-density lipoprotein, and apolipoprotein A and B were detected. Ophthalmological examination was normal. Peroral jejunal capsule biopsy revealed normal villi and significant lipid deposition in the cytoplasm of affected cells. The patient was given large doses of vitamins E and A.
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9/72. Gastrointestinal dysfunction in immunoglobulin deficiency. Effect of corticosteroids and tetracycline.

    Idiopathic late-onset immunoglobulin deficiency in a young man was associated with achlorhydria and a severe intestinal malabsorption syndrome that did not respond to conventional therapy. Combined therapy with high doses of prednisone and tetracycline hydrochloride resulted in weight gain, cessation of diarrhea, improved absorption of water, fat, and vitamin B12, and production of gastric acid after stimulation with histamine. serum immunoglobulin levels, however, did not increase.
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10/72. Successful infliximab treatment for steroid-refractory celiac disease: a case report.

    celiac disease is a T cell-mediated enteropathy induced by gluten in genetically predisposed individuals. The majority of patients responds to a gluten-free diet but a small number do not. After the exclusion of gluten in the diet, ulcerative jejunititis, and an enteropathy-associated t-cell lymphoma, another treatment modalities, such as systemic steroids and immunosuppressives, may be necessary. This article reports the case of a 47-year-old white woman with immunoglobulin a deficiency. She was diagnosed with celiac disease with subtotal villous atrophy on jejunal biopsy together with positive antiendomysium and antigliadin immunoglobulin g antibodies. Despite close adherence to a gluten-free diet, her weight continued to decrease, she had diarrhea, and her distal duodenal histology showed no improvement. Some improvement in her symptoms was observed with cyclosporine and systemic steroids, but this was not sustained. Recent evidence has suggested that anti-tumor necrosis factor alpha antibodies have a role in the amelioration of an animal model of villous atrophy, and after careful consideration, she was treated with infliximab. There was a dramatic improvement in her weight, symptoms, and distal duodenal histology. The response has been maintained for 18 months while on azathioprine therapy. It is concluded that infliximab is an effective treatment that may be considered in a small number of patients with refractory celiac disease, resistant to other therapy.
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