1/117. 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 = mucosa (Clic here for more details about this article) |
2/117. Unusual association of thyroiditis, Addison's disease, ovarian failure and celiac disease in a young woman.The coexistence of autoimmune endocrine diseases, particularly autoimmune thyroid disease and celiac disease (CD), has recently been reported. We here present a 23-year-old woman with a diagnosis of hypothyroidism due to Hashimoto's thyroiditis, autoimmune Addison's disease, and kariotypically normal spontaneous premature ovarian failure. Considering the close association between autoimmune diseases and CD, we decided to search for IgA anti-endomysium antibodies (EmA) in the serum. The positivity of EmA and the presence of total villous atrophy at jejunal biopsy allowed the diagnosis of CD. On a gluten-free diet the patient showed a marked clinical improvement accompanied, over a 3-month period, by a progressive decrease in the need for thyroid and adrenal replacement therapies. After 6 months, serum EmA became negative and after 12 months a new jejunal biopsy showed complete mucosal recovery. After 18 months on gluten-free diet, the anti-thyroid antibodies titre decreased significantly, and we could discontinue thyroid substitutive therapy. This case emphasizes the association between autoimmune polyglandular disease and CD; the precocious identification of these cases is clinically relevant not only for the high risk of complications (e.g. lymphoma) inherent to untreated CD, but also because CD is one of the causes for the failure of substitute hormonal therapy in patients with autoimmune thyroid disease.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
3/117. The clinicopathological features of extensive small intestinal CD4 T cell infiltration.methods: Four patients with clinicopathological features suggesting a new distinct entity defining extensive small intestinal CD4 T cell infiltration were observed. RESULTS: All four patients presented with chronic diarrhoea, malabsorption, and weight loss. biopsy specimens of the small intestine disclosed extensive and diffuse infiltration of the lamina propria by pleomorphic small T lymphocytes, which were positive for CD3, CD4, CD5, and the beta chain of T cell receptor in all three cases studied and negative for CD103 in all three cases studied. It is notable that, in all invaded areas, the infiltrating cells showed no histological change throughout the whole evolution. In three patients, lymphocyte proliferation was monoclonal and there was extraintestinal involvement. In one patient, lymphoproliferation was oligoclonal and confined to the small intestine. In all four patients, there was no evidence of coeliac disease. Although none of the four patients responded to single or multiple drug chemotherapy, median survival was five years. CONCLUSION: Extensive small intestinal CD4 T cell infiltration is a rare entity, distinct from coeliac disease and associated with prolonged survival.- - - - - - - - - - ranking = 7.3910631029983keywords = lamina propria, propria, lamina (Clic here for more details about this article) |
4/117. A new cause of 'non-responsiveness' in coeliac disease?A 42 year old man presented with gluten-responsive coeliac disease and secondary pancreatic insufficiency. Subsequently his symptoms relapsed and repeat small intestinal biopsy showed villous atrophy and infiltration by leukaemic cells, despite continuation of a gluten-free diet. Serious causes of relapse and non-responsiveness in coeliac disease include enteropathy-associated t-cell lymphoma, ulcerative jejunitis and an end-stage hypoplastic mucosa. This is the first report of non-responsiveness due to infiltration by leukaemia.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
5/117. Ichthyosis revealing coeliac disease.We describe the case of a twenty-nine year-old woman presenting a lamellar desquamation on the abdomen, submammary folds and on the limbs. During recent years the patient suffered neither from bowel disorders, nor from dryness of the skin. Laboratory tests revealed high values of parathormone and a high level of antiendomysial antibodies. The total bone-mineral density was about 78% of normal levels. A duodenal mucous membrane specimen showed total atrophy of the villi. skin biopsy revealed an acquired ichthyosis mimicking an ichthyosis vulgaris. The patient underwent a gluten-free diet, supported by folic acid and vitamin d and six months later, after the regression of secondary hyperparathyroidism, a remarkable improvement of the cutaneous symptoms was obtained.- - - - - - - - - - ranking = 0.0016303744603877keywords = membrane (Clic here for more details about this article) |
6/117. Gluten-free diet improves iron-deficiency anaemia in patients with coeliac disease.Two cases of newly-diagnosed asymptomatic coeliac disease with 3 years of unexplained severe iron-deficiency anaemia are presented. Oral iron supplementation had no effect on their serum iron levels and, therefore, had no influence on their anaemia. Upper gastrointestinal endoscopy confirmed normal macroscopic findings. Duodenal biopsies revealed subtotal villous atrophy of the mucosa of the small intestine. A strict gluten-free diet led to an increase in serum iron, resolution of anaemia, and restitution of normal mucosal morphology. Thus, severe iron-deficiency anaemia associated with asymptomatic coeliac disease is responsible to gluten-free diet.- - - - - - - - - - ranking = 2keywords = mucosa (Clic here for more details about this article) |
7/117. 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 = 1keywords = mucosa (Clic here for more details about this article) |
8/117. 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 = 4keywords = mucosa (Clic here for more details about this article) |
9/117. 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 = 1keywords = mucosa (Clic here for more details about this article) |
10/117. Latent coeliac disease in a child with epilepsy, cerebral calcifications, drug-induced systemic lupus erythematosus and intestinal folic acid malabsorption associated with impairment of folic acid transport across the blood-brain barrier.A 15-year-old boy with epilepsy and cerebral calcifications, treated with valproic acid, ethyl phenylbarbiturate and ethosuximide, was referred for drug induced systemic lupus erythematosus. Anti-gliadin (AGA) and anti-endomysium (EMA) antibody tests were both positive (EMA titre 1:50). Endoscopic duodenal biopsy showed intense chronic inflammation without villous atrophy or crypt hyperplasia. The child was discharged with a gluten-containing diet. The follow-up showed an increase in EMA titre (1:200) and the persistence of AGA. After 15 months, a second endoscopic intestinal biopsy showed flat mucosa and villous atrophy. Three serum folic acid determinations showed 1.8, 2.4, 2.0 ng/ml (reference range 2.5-16.9 ng/ml) prior to the two intestinal biopsies, but returned to normal levels (11.8 ng/ml) after a gluten-free diet and oral supplementation together. Two years later, the frequency of epileptic seizures was unchanged despite ongoing anti-epileptic treatment and a gluten-free diet. As cerebral calcification and epilepsy are reminiscent of the findings in congenital folate malabsorption, oral loading tests with 5 mg folic acid were carried out and showed impaired intestinal absorption and a defect in the transport across the blood-brain barrier. Low CSF folate levels (13.9 and 12.6 ng/ml, reference range 15-40 ng/ml) and an alteration in the CSF/serum folate ratio (1.43 and 1.16, normal ratio 3:1) were also found as well as increased levels of cystathionine both in CSF (40 micromol/l, reference range 18-28 micromol/l) and in serum (32 micromol/l, reference value <0.10 micromol/l). CONCLUSION: Impairment of intestinal folic acid absorption with a defect in folic acid transport across the blood-brain barrier has been demonstrated in a case of epilepsy and cerebral calcifications associated with coeliac disease.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
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