1/82. 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.- - - - - - - - - - ranking = 1keywords = sprue (Clic here for more details about this article) |
2/82. 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.- - - - - - - - - - ranking = 1.2keywords = sprue (Clic here for more details about this article) |
3/82. 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.- - - - - - - - - - ranking = 1keywords = sprue (Clic here for more details about this article) |
4/82. Postvagotomy unmasking of nontropical sprue.adult celiac disease which became clinically evident after exophagectomy and vagotomy is described. The diagnosis was suggested by small bowel roentgenography and confirmed by intestinal biopsy and response to a gluten-free diet. The possibility that vagotomy unmasks asymptomatic adult celiac disease is discussed.- - - - - - - - - - ranking = 0.8keywords = sprue (Clic here for more details about this article) |
5/82. Approaching the patient with chronic malabsorption syndrome.The causes of chronic malabsorption may be categorized as decreased intestinal absorption, most commonly caused by celiac sprue; or maldigestion caused by pancreatic insufficiency. The initial step in the evaluation of these patients should include stool studies to confirm fat malabsorption. If fat malabsorption is confirmed, endoscopy with small-bowel biopsies and aspirates for bacterial culture usually follows. A normal endoscopic examination should lead to assessment of pancreatic function. In the setting of normal pancreatic function and the absence of bile acid deficiency, a barium radiograph of the small bowel should be made, looking for anatomical abnormalities. Celiac sprue is an intolerance to gluten caused by a combination of genetic, environmental, and immunologic factors. It classically causes malabsorption. However, it is likely that many patients who exhibit only minor manifestations of the disease go unrecognized and untreated. A presumed diagnosis of celiac sprue is confirmed after a clinical and endoscopic response to a gluten-free diet. Serological markers are available with high degrees of sensitivity and specificity, but duodenal biopsy remains the gold standard for diagnosis. A minority of patients are unresponsive to a gluten-free diet, and intestinal lymphoma should be suspected in these cases.- - - - - - - - - - ranking = 0.6keywords = sprue (Clic here for more details about this article) |
6/82. Refractory sprue syndrome with clonal intraepithelial lymphocytes evolving into overt enteropathy-type intestinal T-cell lymphoma.INTRODUCTION: Recently, patients with refractory sprue have been shown to contain a clonal proliferation of phenotypically abnormal intraepithelial lymphocytes in their intestine. Whether this signifies early enteropathy-type intestinal T-cell lymphoma (EITCL) or a reactive condition is not clear. We report on a patient presenting with the findings of refractory sprue who subsequently developed overt EITCL. MATERIAL AND methods: Duodenal biopsies from 1997 (refractory sprue) and duodenal and jejunal biopsies from 1998 (intestinal T-cell lymphoma) were compared by immunohistochemistry and PCR for the detection of T-cell receptor (TCR)-gamma gene rearrangements. Clonal PCR products were sequenced. RESULTS: The duodenal biopsies from both 1997 and 1998 and the jejunal tumor biopsy showed villus atrophy and an increase of intraepithelial lymphocytes with an abnormal immunophenotype (CD3 , CD4-, CD8- and TCR-beta-). In all duodenal specimens including the one from 1997, and the jenunal tumor biopsy, an identical clonal amplificate was detected by enzymatic amplification of the TCR-gamma gene. CONCLUSION: These data suggest that refractory sprue containing a clonal proliferation of phenotypically abnormal intraepithelial lymphocytes may represent an early manifestation of EITCL. The detection of immunohistochemical negativity for several antigens normally found on intraepithelial lymphocytes such as CD8 or the TCR-beta chain in combination with clonal T-cell populations by PCR may be helpful in identifying refractory sprue with a malignant transformation.- - - - - - - - - - ranking = 1.8keywords = sprue (Clic here for more details about this article) |
7/82. somatostatinoma of the ampulla of vater in celiac sprue.The increased incidence of gastrointestinal lymphoma and adenocarcinoma in patients with celiac sprue is well recognized, with 10% to 15% developing a gastrointestinal malignancy. Somatostatinomas are rare neuroendocrine tumors that occur most commonly within the pancreatic head or duodenum. Although fewer than 100 cases have been reported, somatostatinomas are often associated with multiple endocrine neoplasia-1 syndrome and von Recklinghausen's disease. The unusual case of a 43-year-old woman with celiac sprue in which a somatostatinoma involving the ampulla of vater was identified and resected is presented. To our knowledge, somatostatinomas have not been previously reported in patients with celiac sprue.- - - - - - - - - - ranking = 1.4keywords = sprue (Clic here for more details about this article) |
8/82. A new autoantibody in celiac disease.A 74-year-old female patient with therapy-resistant sprue (celiac disease) and a new IgA autoantibody is presented. This autoantibody was demonstrated by immunofluorescence and differs from the conventional antibody against type 3 connective tissue (so-called endomysium antibody). It reacts predominantly with the muscularis and less strongly with the muscularis mucosae of the monkey esophagus. The reaction is not retiform but punctiform; the typical reaction sites of the antibody against type 3 connective tissue are negative. Especially on esophageal sections the antibody can be mistaken for the characteristic antibody directed against tissue transglutaminase, in particular at low magnification. The antigen of the new antibody is as yet unknown.- - - - - - - - - - ranking = 0.2keywords = sprue (Clic here for more details about this article) |
9/82. Heterogeneity of intraepithelial lymphocytes in refractory sprue: potential implications of CD30 expression.BACKGROUND: Refractory sprue is defined as primary or secondary failure to respond to a gluten free diet in patients with coeliac disease-like enteropathy and may signify cryptic or overt enteropathy associated T cell lymphoma. AIMS: To study in detail jejunal morphology and immunophenotypes in patients with refractory sprue in the search for features that might be useful to predict prognosis. patients: Seven patients are described, representing all such cases identified in our hospital over a 13 year period. methods: biopsy and/or surgical resection specimens were examined by morphology, immunohistochemistry, including enzymatic and immunofluorescent detection, and molecular biology. RESULTS: All patients had phenotypically abnormal intraepithelial lymphocytes (IELs) that lacked CD8, T cell receptor alpha beta (or gamma delta), and/or expressed CD30 in addition to variable expression of the natural killer cell receptor CD94. A monoclonal T cell population was present in six cases, data from the seventh being inconclusive. Three patients had overt lymphoma with CD30 tumour tissue intervening between intact mucosa that contained neoplastic IELs. Intriguingly, CD30 IELs were observed both a long way away from, and in direct continuity with, the tumours in these patients. Such CD30 cells were hardly detected in patients without tumours, two of which are in good health several years after the initial diagnosis. CONCLUSIONS: Our data suggest that abnormal IELs in patients with refractory sprue are phenotypically heterogeneous. CD30 expression by these cells may indicate a worse prognosis, including the occurrence of overt lymphoma.- - - - - - - - - - ranking = 1.4keywords = sprue (Clic here for more details about this article) |
10/82. Enteric drainage of a pancreas allograft is safe for patients with celiac sprue.Enteric drainage of exocrine secretions in whole organ pancreas transplantation is generally avoided in patients with pre-existing small bowel disease; however, bladder drainage is associated with a 20% rate of urinary tract-related complications. This is a case report of a type 1 diabetic patient with celiac sprue and renal failure. We performed a simultaneous cadaveric kidney pancreas transplant enterically draining the exocrine pancreas. There were no complications. The patient is now more than 6 months post-transplant with excellent function of both renal and pancreas allografts. We conclude that enteric drainage of pancreas allografts in patients with celiac sprue may be performed safely. Whole organ pancreas transplantation is being performed with greater success than ever before, mostly as a result of lessons learned from past experience (1). Enteric drainage of allograft exocrine secretions is preferred for simultaneous pancreas/kidney (SPK) recipients to avoid urinary tract complications associated with bladder drainage. However, most agree that diabetics with pre-existing bowel disease should have bladder drainage of allograft exocrine secretions, so as to prevent the devastating complication of a bowel leak. We describe here a successful case of enteric drainage of an SPK transplant in a patient with celiac sprue. We believe that, when carefully performed, enteric drainage of pancreas allografts is a safe approach for diabetic patients with celiac sprue, and may avert the serious complications associated with bladder drainage.- - - - - - - - - - ranking = 1.6keywords = sprue (Clic here for more details about this article) |
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