Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of nonmalignant immunoproliferative small intestinal disease (IPSID) presenting with severe malabsorption are described. The first patient had a lymphocytic infiltrate of the lamina propria and lymphoid hyperplasia of the mesenteric lymph nodes and responded to oral tetracycline. The second patient had a polyclonal plasmacytic infiltration of the lamina propria and of the mesenteric nodes and responded only to cytotoxic treatment with cyclophosphamide. These cases represent examples of non-alpha-chain disease benign IPSID.
 ...
PMID:Malabsorption associated with nonmalignant immunoproliferative small intestinal disease. 359 77

Experience with 30 patients with immunoproliferative small intestinal disease followed prospectively between 1971 and 1986 is described. All presented with malabsorption or growth retardation and had similar clinical, biochemical, and radiological features, irrespective of the presence of lymphoma or immunological abnormality. Alpha-chain disease protein was detected in 4 of the 11 patients who had a non-lymphomatous, predominantly plasmacytic infiltration of the small bowel; and in 5 of the 19 cases with diffuse intestinal lymphoma. The importance of exploratory laparotomy to include full-thickness intestinal biopsy in patients who have a benign infiltrate on peroral biopsy is demonstrated by the finding of lymphoma in operative specimens in 9 of 15 patients with mature, lymphoplasmacytic cells, and 5 of 8 patients with atypical, lymphoplasmacytic cells. The majority of patients with fully established benign disease, even those elaborating alpha-chain disease protein, appeared to have a good prognosis. No patient with immunoproliferative small intestinal disease developed immunologically demonstrated alpha-chain disease or frank lymphoma, when this was not found initially at explorative laparotomy.
 ...
PMID:Immunoproliferative small-intestinal disease: clinical features and outcome in 30 cases. 368 73

The occurrence of alpha-chain disease (alpha-CD) in a Chinese patient, a resident of Taiwan, is reported. The patient's clinical features were characterized by severe malabsorption and ran a rapidly deteriorated course. Duodenal biopsy showed total villous atrophy with a heavy infiltration of the lamina propria by lymphoplasmacytic cells. Colonic biopsy revealed immunoblastic lymphoma. alpha-CD protein was demonstrated in the patient's serum and concentrated urine by the immunoselection method, and also detected in the cellular infiltrate by immunoperoxidase technique. Postmortem examination disclosed that the lymphoma involved not only the entire gastrointestinal tract and mesenteric nodes, but also the retroperitoneal and peripheral lymph nodes. Immunoperoxidase studies support the concept of a common clonal origin of the lymphoplasmacytic proliferation and immunoblastic lymphoma in alpha-CD.
 ...
PMID:Alpha-chain disease. Report of a case from Taiwan. 379 Nov 62

Intestinal flora was explored in twelve patients affected with alpha-chain disease at different stages (stage A: 2 cases; stage B: 6 cases; stage C: 4 cases). Bacterial overgrowth in the jejunum was observed in 11 cases, but intestinal flora was diverse and no one species was always present; although a 3-month oral antibiotic treatment induced complete remission in one patient (stage A) it was not possible to demonstrate any pathogenic bacterial species. Intestinal lambliasis was present in 40 p. 100 of cases. Virologic studies were negative. At stages A and B of the disease, antibiotic treatment was able to improve malabsorption and/or plasma protein digestive losses in 62 p. 100 of cases; this effect seemed related to the reduction of the bacterial flora and to giardiasis eradication.
 ...
PMID:[Bacteriological, parasitological and virological study of the digestive flora in alpha-chain disease]. 392 86

Forty-three patients with immunoproliferative small intestinal disease and primary small intestinal lymphoma were studied prospectively. Eighteen patients in whom alpha-chain protein was detected in the serum had significantly more features of malabsorption, and disease was localized more commonly in the jejunum. In all of these patients, a diffuse lymphoplasmacytic infiltrate was found in the intestine; in three patients lymphoma was found only in mesenteric lymph nodes. Twenty-five patients with lymphoma in whom alpha-chain protein failed to be detected had significantly more features of intestinal obstruction, and disease was found more commonly in the ileum. Five of these patients had lymphoma associated with a diffuse mucosal infiltrate that was indistinguishable from the first group. In patients available for follow-up, no difference was found in cumulative survival over 30 months in the two groups, with approximately 40% mortality at 6 months.
 ...
PMID:Immunoproliferative small intestinal disease and primary small intestinal lymphoma. Relation to alpha chain protein. 392 29

Studies of a number of properties of the pathological gammaA-proteins in the first four cases of the recently recognized alpha-chain disease demonstrate that, as in gamma-heavy-chain disease, the abnormal protein is devoid of light chains and represents a portion of the alpha-heavy chain related to the Fc-fragment. In two patients, serum electrophoresis showed a broad abnormal band, whereas in the two others the pathological protein was not noticeable on the electrophoretic pattern. The diagnosis of alpha-chain disease can be established without purification of the protein by immuno-electrophoresis and gel diffusion experiments using selected antisera to gammaA and a reference alpha-chain disease protein. All four proteins belonged to the alpha1-subclass, displayed electrophoretic heterogeneity, and showed a strong tendency to polymerize. The polymers occurred in vivo and were held together both by disulfide bonds and by strong noncovalent forces. Two of the three purified proteins had a very high carbohydrate content. The abnormal protein was always found in concentrated urines in variable but generally low amounts. It was not detected in parotid saliva but was present in significant amounts in jejunal fluid of all four patients. The alpha-chain disease protein was shown to be associated with the secretory piece in external secretions of two patients. The clinicopathological features were strikingly similar in the four patients. All patients were affected with a neoplastic and mostly plasmacytic proliferation involving primarily the whole length of the small intestine and the mesenteric nodes and all exhibited a severe malabsorption syndrome. While Israeli authors have emphasized the frequency of this type of abdominal lymphoma in young Arabs and non-Ashkenazi Jews, two of our patients were Kabyles, one a Syrian Arab, and one an Eurasian. Cellular studies showed that the pathological protein was synthesized by the proliferating cells in the lymphoid tissue of the digestive tract and in the mesenteric nodes, and that there was no detectable light-chain synthesis at the intracellular level.
 ...
PMID:Immunochemical studies in four cases of alpha chain disease. 498 31

Alpha-chain disease with involvement of small intestine-resulting in characteristic villus atrophy and malabsorption has not been reported in this country. We studied a 57-yr-old male who presented with a polypoid tumor of the hepatic flexure of the colon. There was no evidence of malabsorption as manifested by a normal fat balance, serum carotene, and D-xylose absorption studies and the small bowel biopsy did not show villus atrophy. The tumor in the colon was surgically removed and diagnosed as a malignant lymphoma of lymphocytic type. Tumor tissue infiltrated in the mesentery could not be excised. Alpha-chain disease protein was demonstrated in serum and urine, and also in tumor tissue by immunoperoxidase techniques. The alpha-chain disease protein was further purified and classified as subclass 1. The patient had a good clinical response to cyclophosphamide and prednisone, but still has intraabdominal lymphoma with gastric involvement, and his serum alpha-chain protein persists. This case report may represent a distinct variant of alpha-chain disease.
 ...
PMID:Colonic lymphoma producing alpha-chain disease protein. 680 99

Three cases of follicular lymphoid hyperplasia extending to the whole length of small intestine are reported in three young adult patients of low economic status. The disease was revealed by chronic diarrhea with malabsorption and/or protein-losing enteropathy. In one patient, all transitional patterns were found between the hyperplastic follicles and a small intestinal multicentric centrocytic-centroblastic lymphoma. No abnormalities in humoral or cellular immunity were found in the three patients. In particular, serum immunoglobulins (except IgG in one case) and plasma cell populations of small intestinal lamina propria were normal. Diffuse follicular lymphoid hyperplasia of the small intestine in the absence of primary immunoglobulin deficiency appears to be a rare condition associated with (or leading to) intestinal malignant lymphoma in most cases. Patients usually belong to the same populations as those suffering from alpha-chain disease.
 ...
PMID:Diffuse follicular lymphoid hyperplasia of the small intestine without primary immunoglobulin deficiency. 709 Nov 67

Human intestinal lamina propria T cells have a low expression of the CD45RA antigen and a high expression of the CD45RO antigen. This phenotype is characteristic for memory T cells. In addition, T cells in the effector compartment of the mucosa bear surface antigens that are very rarely found in other sites of the immune system. Intestinal T cells also express functional IL-2 receptors, and IL-2 receptor alpha-chain mRNA, and are able to synthesize high amounts of IL-2. However, other markers of memory T cells, as CD29, are not expressed in high density in the lamina propria, indicating that lamina propria T cells differ from "classical" memory T cells. This is supported by functional studies in nonhuman primates infected rectally with Chlamydia trachomatis that show that lamina propria T cells do not proliferate after stimulation with antigen but rather provide helper function for immunoglobulin synthesis. These findings indicate a specific state of differentiation of lamina propria T cells that is adapted to the specific requirements in the gut. In inflammatory bowel disease (IBD) and in celiac disease, an increase in the number of CD25-positive activated T cells is found in involved mucosa. It has been shown that mucosal T-cell activation induces epithelial cell damage and mucosal transformation. Thus, a T cell-mediated damage may contribute to the pathogenesis of IBD. HIV-infected patients have a decreased number of CD4-positive T cells in the intestinal lamina propria. The number of CD25-positive activated T cells is also significantly decreased in the intestine compared to controls. Correlating with the presence of HIV-infected mononuclear cells in the mucosa, mucosal atrophy with hyporegeneration and enterocyte dysmaturation is observed. HIV might thus cause impairment and depletion of activated regulatory T cells in the intestinal lamina propria, which could lead not only to a breakdown of the mucosal immune barrier, resulting in a variety of opportunistic infections, but also to malabsorption, due to mucosal atrophy or enterocyte dysfunction. These findings indicate a close relationship between mucosal T cells and enterocyte proliferation and maturation.
 ...
PMID:Cell differentiation and proliferation in the gastrointestinal tract with respect to the local immune system. 797 5

The major obstacle for oral delivery of administered therapeutic proteins is malabsorption in the intestine. This malabsorption could be overcome by induction of neonatal FcRn [Fc (CH2 and CH3 domains of human IgG1 antibody) receptor]-mediated transcytosis in the intestine using recombinant fusion of CH2 and CH3 moieties of human IgG to a therapeutic protein. To this end we developed recombinant hGH (human growth hormone) fused to the N-terminus of Fc moieties [CH2-CH3 or h (hinge)-CH2-CH3] from human IgG1. These recombinant proteins secreted by the methylotrophic yeast Pichia pastoris functionally induced secretion of insulin-like growth factor 1 by HepG2 cells in the response to hGH moiety in the fusion proteins. In a transport study using polarized T84 cells, 3.7% of added dimeric hGH-h-Fc was transported in the apical-to-basolateral direction within 1 h by FcRn-mediated transcytosis of 1 cm(2) monolayers. However, transport of monomeric hGH-Fc (only 0.43%) was much less effective, yet its transport was 2.3 times higher than that of hGH. Finally, we concluded that, upon recombinant fusion, maintenance of dimeric structure of Fc moieties is crucial for the induction of FcRn-mediated transcytosis.
 ...
PMID:Expression and characterization of human growth hormone-Fc fusion proteins for transcytosis induction. 1706 88


<< Previous 1 2 3 Next >>