1/51. Parathyroid carcinoma secondary to prolonged hyperplasia in chronic renal failure and in coeliac disease.The presentation of parathyroid carcinoma in patients with chronic renal failure is rare, although with improvements in life expectancy associated with this condition there have now been 12 reported cases, including the first case we report here. It has been proposed that in these cases there has been a malignant transformation of benign parathyroid hyperplastic tissue. We also report the first case of parathyroid carcinoma associated with coeliac disease and suggest that the same mechanism may be responsible. We review the presentation, diagnosis, treatment and natural history of the disease.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
2/51. 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 = 1300.725864978keywords = intraepithelial (Clic here for more details about this article) |
3/51. 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 = 0.16666666666667keywords = carcinoma (Clic here for more details about this article) |
4/51. Tissue transglutaminase autoantibodies in patients with non-Hodgkin's lymphoma. case reports.BACKGROUND: Tissue transglutaminase (tTG) has recently been identified as the autoantigen recognized by endomysial antibodies in celiac disease (CD) patients and this has permitted the use of an ELISA test to detect the presence in the serum of autoantibodies specific for the diagnosis of CD. AIM: We report two cases of anti-tTG positivity in patients with non-Hodgkin's lymphoma (NHL) without evidence of CD. case reports: Both patients were males aged 67 and 69 years respectively; both were hospitalized for fever and peripheral adenopathy. Lymph node histology showed an immunoblastic high-grade T-cell NHL at the IVth the stage of disease in both cases. They were included in a multicenter study on the association between CD and NHL. Serological screening for CD showed the presence of serum anti-tTG antibodies, with values within the range of those recorded in untreated CD patients in our laboratory; however, both patients had negative anti-endomysial antibodies and in both cases intestinal histology showed normal mucosa with villi and crypts of normal height and depth (villi/crypts ratio > or = 2.5, within the range of normal subjects for our laboratory), and no increase in intraepithelial lymphocytes. The HLA phenotype was obtained giving the following antigens: Case 1: A 3, A 24(9), B 22, B 35, BW 6, DR 1, DR 11(5), DQ 3, DR 52. Case 2: A 2, A 3, B 51(5), B 8, BW 4, BW 6, DR B1*02, DR B1*03, DR B3*01. Both subjects were also positive for serum anti-smooth muscle antibodies and one for antinuclear antibodies. CONCLUSIONS: (1) serum anti-tTG positivity can be found in subjects with NHL without CD and the real frequency of these 'false positives' must be investigated both in subjects with lymphoproliferative disorders and in patients with autoimmune diseases. (2) In patients with NHL, without CD, anti-tTG positivity may be unassociated with EmA positivity and the biological significance of this finding must be clarified.- - - - - - - - - - ranking = 162.59073312225keywords = intraepithelial (Clic here for more details about this article) |
5/51. role of interferon alpha in promoting T helper cell type 1 responses in the small intestine in coeliac disease.Coeliac disease (CD) is caused by a CD4 T helper cell type 1 (Th1) response in the small intestinal mucosa to dietary gluten. As the major Th1 inducing cytokine, interleukin 12, is undetectable in CD gut mucosa, the mechanism by which Th1 effector cells are generated remains unknown. Interferon (IFN) alpha, a cytokine capable of promoting IFN-gamma synthesis, has been implicated in the development of Th1 mediated immune diseases. Here we report a case of CD-like enteropathy in a patient receiving IFN-alpha for chronic myeloid leukaemia. Morphological assessment of duodenal biopsies taken from the patient showed total villous atrophy, crypt cell hyperplasia, and a high number of CD3 intraepithelial lymphocytes. Both antigliadin antibodies and antiendomysial antibodies were positive. rna analysis revealed pronounced expression of IFN-gamma. Withdrawal of gluten from the diet resulted in a patchy improvement in intestinal morphology, normalisation of laboratory parameters, and resolution of clinical symptoms. By western blot analysis, IFN-alpha protein was seen in the duodenal mucosa from untreated CD patients but not in controls. This was associated with marked expression of IFN-gamma protein in CD mucosa. Collectively, these results suggest a role for IFN-alpha in promoting Th1 responses to gluten.- - - - - - - - - - ranking = 162.59073312225keywords = intraepithelial (Clic here for more details about this article) |
6/51. Diagnostic duodenal bulb biopsy in celiac disease.BACKGROUND AND STUDY AIMS: Duodenal or jejunal biopsies are needed to establish the diagnosis of celiac disease. It is widely advocated that these biopsies be taken from the distal duodenum. patients AND methods: After finding two index cases with diagnostic biopsies taken from the duodenal bulb, we retrospectively analyzed biopsies from the descending duodenum and the duodenal bulb of 51 patients with suspected or diagnosed celiac disease. The diagnosis of celiac disease and classification of the histological changes were performed by one pathologist. RESULTS: In the two index cases the diagnosis of celiac disease could only be established by taking the biopsies from the duodenal bulb, and not from the descending duodenum. In the retrospective analysis the number of intraepithelial lymphocytes was on average higher, but not significantly, in the descending part of the duodenum. CONCLUSIONS: Most patients with celiac disease show similar mucosal changes in biopsies taken from the descending part of the duodenum and from the duodenal bulb. But in patients who have already been on a gluten-free diet in childhood and later abandoned their diet, an additional duodenal bulb biopsy should be done.- - - - - - - - - - ranking = 162.59073312225keywords = intraepithelial (Clic here for more details about this article) |
7/51. 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.- - - - - - - - - - ranking = 0.16666666666667keywords = carcinoma (Clic here for more details about this article) |
8/51. Inflammatory cytokines in small intestinal mucosa of patients with potential coeliac disease.T helper cell type 1 (Th1) response to gluten has been implicated in the pathogenesis of coeliac disease (CD). To characterize immunological activation and mild inflammations leading to overt CD in potential coeliac patients, jejunal biopsies were obtained from family members of patients with CD or dermatitis herpetiformis (DH). Nine family members and one latent CD, eight CD patients and eight normal controls furnished jejunal biopsy specimens. Immunohistochemical staining of sections for interleukin-1alpha (IL-1alpha), IL-2, IL-4, interferon-gamma (IFN-gamma), tumour necrosis factor alpha (TNF-alpha), CD3, gammadelta-T cell receptor (gammadelta-TCR), and alphabeta-TCR was carried out with monoclonal antibodies. Further, expression of IL-4 and IFN-gamma messenger rna was detected by radioactive in situ hybridization in these same samples. In lamina propria, CD patients and potential CD patients had higher densities of IL-2 (P = 0.028, P = 0.043), IL-4 (P = 0.021, P = 0.034) and IFN-gamma positive cells (P = 0.000, P = 0.009) than did controls. Moreover, CD patients showed a higher density of TNF-alpha positive cells (P = 0.012, P = 0.001) than the other two groups, and expression of IFN-gamma mRNA (P = 0.035) was higher in them than in the other two study groups. Additionally, higher densities of TNF-alpha and IFN-gamma positive cells occurred in potential CD patients with high gammadelta-TCR intraepithelial lymphocytes (IELs). Our findings support the hypothesis that lamina propria T cells and macrophages, through their secretion of cytokines, play a central role in the pathogenesis of coeliac disease. The inflammatory cytokines found in potential CD specimens strongly suggest that these inflammatory markers can be identified long before visible villous changes have occurred.- - - - - - - - - - ranking = 162.59073312225keywords = intraepithelial (Clic here for more details about this article) |
9/51. association of primary sclerosing cholangitis, ulcerative colitis and coeliac disease in female siblings.Primary sclerosing cholangitis is a rare, cholestatic liver disease, most commonly affecting young men. The association of primary sclerosing cholangitis with other autoimmune disorders, although rare, indicates a genetic predisposition for this disease. We describe, for the first time, the association of primary sclerosing cholangitis, ulcerative colitis and coeliac disease in two sisters. Ulcerative colitis was mild and preceded liver disease in both patients. There were no symptoms of coeliac disease, and its silent form was diagnosed on the basis of serological tests. Both patients carried HLA molecules DR3 and DQ2. Although HLA DR4 was not found, there was a rapid progression of liver disease to cirrhosis and cholangiocarcinoma in one patient. The familial occurrence of primary sclerosing cholangitis, ulcerative colitis and coeliac disease supports the hypothesis of genetic predisposition for these diseases.- - - - - - - - - - ranking = 0.16666666666667keywords = carcinoma (Clic here for more details about this article) |
10/51. 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 = 812.95366561124keywords = intraepithelial (Clic here for more details about this article) |
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