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

A malignant lymphoma of the small bowel developed in a 55-yr-old woman who had intestinal malabsorption for 20 yr, late-onset hypo-gamma-globulinemia, and nodular lymphoid hyperplasia. The tumor arose in the first jejunal loop and appeared to be of B-immunoblastic type (diffuse large noncleaved cells). This case indicates that nodular lymphoid hyperplasia in the context of immunodeficiency may be a condition leading to lymphoid malignancy.
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PMID:Jejunal malignant lymphoma in a patient with adult-onset hypo-gamma-globulinemia and nodular lymphoid hyperplasia of the small bowel. 357 29

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.
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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.
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PMID:Alpha-chain disease. Report of a case from Taiwan. 379 Nov 62

In addition to abnormalities in systemic immune function, patients with the acquired immunodeficiency syndrome (AIDS) and the pre-AIDS syndromes have significant abnormalities in the distribution of T-cell subsets in the intestinal tract. Such immune deficits predispose such patients to opportunistic infections and tumors, many of which involve the gastrointestinal tract. For example, Candida albicans often causes stomatitis and esophagitis. Intestinal infections with parasites (Cryptosporidium, Isospora belli, Microsporidia) or bacteria (Mycobacterium avium-intracellulare) are associated with severe diarrhea and malabsorption, whereas viruses like cytomegalovirus and herpes simplex virus cause mucosal ulcerations. Clinically debilitating chronic diarrhea develops in many AIDS patients for which no clear cause can be identified. Enteric pathogens like Salmonella and Campylobacter can be associated with bacteremias. Kaposi's sarcoma and lymphoma involving the intestinal tract are now well-recognized complications of AIDS. Although AIDS is not associated with a pathognomonic liver lesion, opportunistic infections and Kaposi's sarcoma or lymphoma may involve the liver.
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PMID:Gastrointestinal manifestations of the acquired immunodeficiency syndrome. 382 11

A high incidence of intestinal lymphoma has been noted in the Middle East since the early 1960's. Initially thought to be a single entity, Middle Eastern intestinal lymphoma is now perceived as at least 3 distinct types. Mediterranean lymphoma (MTL), also known as alpha chain disease and immunoproliferative small intestinal disease (IPSID), occurs in the proximal small intestine of young adults who present with malabsorption. Histologically a phase of plasma cell infiltration is followed by invasive lymphoma. In approximately 50% of cases an alpha 1 heavy chain paraproteinaemia is present. Intestinal lymphoma of so called Western type (WTL) occurs in older patients without malabsorption or a paraproteinaemia. The lymphoma is of follicular center cell type and there is no preceding plasma cell infiltrate. Recent work suggests that both MTL and WTL belong to a distinctive group of lymphomas that specifically arise from mucosa associated lymphoid tissue. The third type of Middle Eastern intestinal lymphoma occurs predominantly in the ileo-caecal region of children and is histologically indistinguishable from Burkitts lymphoma.
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PMID:Middle Eastern intestinal lymphoma. 384 95

The sprue syndromes, tropical and nontropical sprue, were both described as disease entities in the 1880s and share similar morphological features with varying degrees of villus atrophy of the small intestinal mucosa, and both present clinically with malabsorption. Recent cell kinetic studies of the turnover of the intestinal epithelium in sprue have convincingly demonstrated that the flat mucosa is caused by increased efflux (cell death) with compensatory crypt hyperplasia. The pathogenetic insult in tropical sprue appears to be a persistent overgrowth of the small intestine by enteric pathogens after a bout of turista. The pathogenesis of nontropical sprue is determined by both genetic factors, demonstrated with a strong association with certain HLA haplotypes (B8, DR3, DR7 and DC3) and presumably also environmental events (virus infection?), which render the mucosa susceptible to gluten. The cause of the malabsorption syndrome is multifactorial and results from both intraluminal and cellular events. The digestion of proteins, carbohydrates, and lipids is compromised due to decreased pancreatic and biliary secretion. The absorption of the digestive products is also severely affected due to decreased activity of microvillus enzymes (dipeptidases and disaccharidases) and a presumed reduction in the number of transport carriers. The clinical presentation is identical and the distinction between tropical and nontropical sprue is based on the history (ie, exposure to a tropical environment) and the response to treatment. Tropical sprue is cured by treatment with tetracycline and folic acid, whereas nontropical sprue responds to a gluten-free diet. Nontropical sprue is associated with dermatitis herpetiformis by common genetic and morphological features, and the skin lesions in dermatitis herpetiformis are also responsive to a gluten-free diet. Finally, there appears to be an increased incidence of intestinal malignancies (lymphoma, adenocarcinoma) in nontropical sprue.
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PMID:The sprue syndromes. 390 13

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.
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PMID:Immunoproliferative small intestinal disease and primary small intestinal lymphoma. Relation to alpha chain protein. 392 29

Primary small intestinal lymphoma (PSIL) represents a heterogenous group of disorders with variable clinical and pathologic features and a characteristic age, socioeconomic, and geographic distribution. In developed countries, PSIL usually occurs as a localized ileal tumor, shows a bimodal age distribution, and most frequently presents with abdominal pain and obstructive symptoms. Histologically, most of these tumors are diffuse histiocytic, lymphocytic, or undifferentiated lymphomas. Other variants of PSIL, collectively referred to as immunoproliferative small intestinal disease, occur most often among young patients of poor socioeconomic status in Third World countries, mostly in the Middle East and Mediterranean area. They are characterized by involvement of long loops of the upper small intestine and commonly present with abdominal pain, diarrhea, malabsorption, and clubbing of the fingers. A subgroup of these patients shows a serological abnormality with the appearance of part of the alpha heavy chain of IgA in the serum. Histologically, the lesion appears as a dense diffuse lymphoplasmacytic infiltrate of the mucosa of the upper jejenum or duodenum. A form of malignant lymphoma of true histiocytic origin complicates long-standing celiac disease. The contrasting clinical, epidemiological, histopathological, and immunological features of these variants of PSIL raise interesting questions about the pathogenesis of small bowel lymphoma.
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PMID:Primary lymphomas of the small intestine: east-west contrast. 395 68

Four young women presented with diarrhea, malabsorption, and intestinal pseudoobstruction. Intestinal biopsy specimens (both peroral and full-thickness) showed flat small intestinal mucosa, sparsity of crypts, and a widespread lymphoid infiltrate in the lamina propria, muscularis propria, and myenteric plexus. There was no neuron or nerve fiber loss or damage in the plexus; muscle cell absence in the vicinity of lymphoid cell infiltration in the muscularis propria probably accounted for the pathogenesis of pseudoobstruction. Immunochemical stains showed that the infiltrate was polyclonal, and none of the patients has developed lymphoma on clinical follow-up of 4-16 yr. Transient improvement in symptoms occurred after antibiotic therapy in 3 patients, and 1 patient had improvement after treatment with cyclophosphamide and prednisone; however, symptoms of pseudoobstruction persist in all. These cases illustrate yet another cause of intestinal pseudoobstruction which is histologically distinct from visceral myopathies and neuropathies. The pathogenesis of this illness may be related to that of diffuse immunoproliferative diseases seen in Third World countries.
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PMID:Intestinal pseudoobstruction caused by diffuse lymphoid infiltration of the small intestine. 402 67

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.
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PMID:Immunochemical studies in four cases of alpha chain disease. 498 31


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