Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0007570 (celiac disease)
 13,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cancer of the small intestine is rare compared with other sites in the gastrointestinal tract. Of the four major primary small-bowel tumors (adenocarcinomas, lymphomas, carcinoid, and leiomyosarcomas), adenocarcinomas and lymphomas are associated with diseases that seem to increase the risk of developing these malignancies. In the case of immunoproliferative small intestinal disease and celiac disease, both of which are thought to predispose patients to the development of primary lymphoma, treatment of the predisposing conditions seems to decrease the risk of developing subsequent malignancy. Recognition of the increased risk associated with other conditions, such as immunodeficiency syndromes, nodular lymphoid hyperplasia, Crohn's disease, the gastrointestinal polyposis syndromes, hereditary nonpolyposis colon cancer, neurofibromatosis, long-standing ileostomy, and urinary diversion procedures, may lead to early diagnosis and improved survival.
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PMID:Premalignant conditions of the small intestine. 870 62

Sclerosing cholangitis may be a cause of refractory pain in patients infected with the human immunodeficiency virus. We performed celiac plexus block in three such patients with sever pain from sclerosing cholangitis and a poor response to conventional analgesia. The pain had been centered in the epigastrium and/or upper-right quadrant of the abdomen for 2, 10, and 15 weeks, respectively. Computed tomography-guided celiac plexus block with absolute alcohol and bupivacaine was performed. All three patients reported complete disappearance of the pain immediately after the procedure in two cases and 3 days later in the remaining patient. All patients were discharged free of pain and without analgesics and were followed up for 2, 8, and 11 months, respectively, without recurrence of pain. Celiac plexus block deserves further trial for the treatment of severe pain associated with sclerosing cholangitis in patients with acquired immunodeficiency syndrome. The quality of life of our three patients was considerably improved with this relatively simple procedure.
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PMID:Celiac plexus block as treatment for refractory pain related to sclerosing cholangitis in AIDS patients. 883

Immunoglobulin deficiency, especially deficiency of IgA, has been described in patients with celiac sprue (CS). Our study was performed in an area of high prevalence of CS to determine the prevalence of immunodeficiency states in patients with CS, to examine their clinical characteristics, response to treatment, and HLA phenotypes compared with a group of age- and sex-matched persons with CS but without immunoglobulin deficiency. Fourteen of 604 patients with CS were identified as being selectively deficient in IgA, whereas one had common variable immunodeficiency. At diagnosis, anemia was present in 8 of 14 IgA-deficient patients compared with 10 of 42 controls (p = 0.047), whereas abdominal pain was more common in controls with CS. Autoimmunity and recurrent infection were more prevalent in the IgA-deficient group. Response to gluten-free diet was similar in both groups in terms of histologic structure and recovery of intestinal brush-border enzyme activity. IgA-deficient participants with CS had no increased risk of associated malignancy or lymphoma. HLA phenotypes were similar in both groups. The prevalences of selective IgA deficiency and common variable immunodeficiency in this series of patients with CS are 2.31 in 100 and 0.16 in 100, respectively. Although this group is unique in character, close follow-up coupled with conscientious compliance with a gluten-free diet, remains the mainstay of treatment for these patients.
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PMID:Celiac sprue and immunodeficiency states: a 25-year review. 941 41

On the suspicion that folate deficiency was not being thoroughly investigated we conducted a retrospective study of management in a teaching hospital. Notes from 84 consecutive patients with low red cell folates (mean age 69.5 years, range 21-95, M:F 33:51) were reviewed for haemoglobin, mean cell volume, dietary history, alcohol consumption, drug history, relevant medical history, relevant investigations, treatment, repeat measurement of red cell folate and diagnosis of deficiency. In 52 (61.9%, mean age 72.9 years, range 33-95, M:F 21:51) no diagnosis was reached. In only 32 (38.1%, mean age 63.9 years, range 21-89, M:F 12:20) was a definitive diagnosis established: 5 had coeliac disease, 1 had Crohn's disease, 9 had drug-associated deficiency (4 methotrexate, 3 phenytoin, 1 trimethoprim and 1 valproate), 1 had combined variable immunodeficiency and 16 had dietary deficiency. In most cases of folic acid deficiency no attempt was made to establish aetiology. We recommend that younger patients without an obvious cause are investigated initially by dietary assessment and measurement of anti-endomysial antibody and by duodenal biopsy with small-bowel follow-through if clinically indicated.
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PMID:An audit of the investigation and treatment of folic acid deficiency. 960 41

Human immunodeficiency virus (HIV) is often combined with unexplainable diarrhoea and weight loss. This study was designed to see if changes in the intestinal mucosal structure could explain the malabsorption found in HIV-infected patients with diarrhoea. Twenty acquired immunodeficiency system (AIDS) patients, 19 men and 1 woman, CD4 < 0.01, with severe weight loss and with non-infectious chronic diarrhoea, were evaluated using a new intestinal function test (D-xylose breath test). Fifteen of the subjects were examined with an upper intestinal endoscopy with biopsy specimens taken from the duodenal mucosa. The function test showed that the D-xylose uptake was markedly decreased to the same extent as for patients with coeliac disease (breath index AIDS patients 9.4 (4.3-14.4), coeliac patients 15.6 (7.6-23.6), reference level 2.5 (2.4-2.9), urine excretion AIDS patients 20% (13-26), coeliac patients 22% (14-24), reference level 37% (32-42)). The severe malabsorption could not be explained by the slight mucosal changes occasionally seen by light microscopy with small mucosal inflammation and almost normal villi. However, electron microscopy showed enterocytes with signs of hypofunction and degeneration correlating better to the intestinal malabsorption found in patients with advanced HIV infection and chronic diarrhoea.
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PMID:Changes in small intestinal structure and function in HIV-infected patients with chronic diarrhoea. 1006 44

Gastrointestinal lymphomas comprise a group of distinctive clinicopathological entities of B- or T-cell type, with primary gastrointestinal Hodgkin's disease being extremely uncommon. Most low-grade B-cell gastrointestinal lymphomas are of mucosa-associated lymphoid tissue (MALT) type, so called because they recapitulate the features of MALT rather than those of lymph nodes. Paradoxically, however, most MALT lymphomas arise in the stomach, which normally contains no organized lymphoid tissue. Gastric MALT lymphomas appear to arise in MALT acquired as a reaction to infection of the stomach by Helicobacter pylori and their growth can be inhibited by eradication of this organism from the stomach. Low-grade MALT lymphomas, which usually have a very favorable clinical course, may undergo high-grade transformation but high-grade diffuse large B-cell lymphomas may also arise de novo. Immunoproliferative small intestinal disease (IPSID) is a special form of MALT lymphoma characterized by synthesis of alpha heavy-chain immunoglobulin and a restricted geographic distribution. Other B-cell lymphomas that tend to arise in the gastrointestinal tract include mantle cell lymphoma, which presents as lymphomatous polyposis, Burkitt's lymphoma, and B-cell lymphomas associated with immunodeficiency states. Enteropathy (celiac disease)-associated T-cell lymphoma (EATL) is the most common primary gastrointestinal T-cell lymphoma This is a clinically aggressive tumor that arises from the intraepithelial T-cell population.
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PMID:Gastrointestinal lymphomas of T- and B-cell types. 1007 40

Non-Hodgkin's lymphoma (NHL) of the gastrointestinal (GI) tract accounts for 4% to 20% of all NHLs and is the most common extranodal site of presentation. The stomach is the major organ involved by GI lymphoma. Helicobacter pylori infection, immunosuppression after solid-organ transplantation, celiac disease, inflammatory bowel disease, and human immunodeficiency virus (HIV) infection may be risk factors for GI lymphoma. A significant proportion of gastric lymphomas are of low-grade histology and arise from mucosal-associated lymphoid tissue (MALT). Such MALT lymphomas may be associated with H. pylori infection and may undergo complete regression following eradication of H. pylori. Lymphoma of the small bowel, colon, and rectum may also occur, but are less common than gastric lymphoma. Distinct clinicopathologic entities, such as primary intestinal T-cell lymphoma, immunoproliferative small intestinal disease, and multiple lymphomatous polyposis have been described. Surgery, radiation therapy, and chemotherapy have been used in the treatment of GI lymphomas. However, the optimal management of these lymphomas has never been determined by prospective randomized clinical trials. Such trials by cooperative groups are needed to answer many of the vital unanswered questions concerning extranodal lymphomas of the GI tract.
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PMID:Lymphoma of the gastrointestinal tract. 1037 89

Although it is well known that patients with type 1 diabetes mellitus are susceptible to other autoimmune diseases, the simultaneous occurrence of clustered distinct autoimmune diseases is uncommon. We report a 16-year-old girl, previously diagnosed as having coeliac disease and IgA deficiency, who at 13 years of age developed a clustering of distinct autoimmune diseases, including type 1 diabetes mellitus, rheumatoid arthritis (RA) and euthyroid autoimmune thyroiditis, eventually resulting in a simultaneous long-term remission. The clinical picture was associated with a functional immunodeficiency characterized by a defect in proliferative responses to T cell predominant mitogens and a normal response to the B cell predominant mitogen. In addition, the T cell activation markers HLA-DR, IL-2 receptor and transferrin receptor) were not upregulated. The clinical course of this immunodeficiency paralleled the outcome of the autoimmune diseases. After the abrupt onset, spontaneous clinical remission of both diabetes mellitus and RA was observed. Insulin was first reduced in dose and then discontinued completely at 15 months, in the presence of normal C peptide secretion and normal metabolic control (HbA1c 5.8%). Anti-glutamate decarboxylase (GAD65) and anti-IA-2 antibodies remained persistently high. During the remission phase a normalization of the functional immune defect was observed. The gradual resolution of the multisystemic diseases as well as the normalization of immune function in our patient is unusual. This case may be of considerable value in furthering our knowledge of the immunological mechanisms implicated in these rare multireactive syndromes.
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PMID:Simultaneous peripubertal onset of multireactive autoimmune diseases with an unusual long-lasting remission of type 1 diabetes mellitus. 1110 28

Common variable immunodeficiency is a disorder characterised by hypogammaglobulinemia with B-lymphocytes in peripheral blood and repeated infections. We report a child with a diagnosis of diabetes mellitus and celiac disease during lactation, and in whom common variable immunodeficiency was diagnosed at the age of 5. During evolution of the disease he presented multiple respiratory infections in spite of substitution therapy with gamma globulins. He presented pulmonary fibrosis with a pulmonary volume reduced, and a spirometric restrictive patron. Immunologically, he presents reduction in CD4 lymphoid population. He expresses the alleles DQ2 A1 0501 and B1 which are strongly associated with susceptibility to insulin-dependent diabetes mellitus and celiac disease, but don't express antigens HLA class II DR3 and DR4 that are more frequent in these entities. The main disease and all the complications had affected his curve pondostatural.
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PMID:Common variable immunodeficiency, insulin-dependent diabetes mellitus and celiac disease. 1126

We describe a case report of a patient who developed common variable immunodeficiency (CVID) after autologous haematopoietic stem cell transplantation (SCT) for recurrent Evans syndrome. The disease manifested as attacks of haemolytic anaemia, thrombocytopenia and neutropenia from the age of 12 years. Presence of autoantibodies to blood elements was confirmed together with C4 deficiency. The patient also suffered from dermatitis herpetiformis Duhring without signs of coeliac disease. Autologous T cell-depleted peripheral blood stem cell (PBSC) transplant following conditioning regimen was performed at the age of 20 years. Immunological reconstitution was incomplete and 2 years after SCT he fulfilled laboratory criteria for common variable immunodeficiency (CVID). The patient was found to be a carrier of a risk haplotype for development of CVID DRB1*03/DQB1*0201. We conclude that T cell-depleted SCT here performed for autoimmune manifestations can hasten development of CVID in genetically predisposed patients.
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PMID:Development of common variable immunodeficiency in a patient with Evans syndrome treated by autologous stem cell transplantation. 1291 16


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