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 prospective study of 32 patients with primary upper small intestinal lymphoma in our region revealed 10 cases of alpha heavy-chain disease. Patients were mostly in the second and third decades of life and males predominated. Weight loss, diarrhea, and abdominal pain were the most common complaints and clubbing the most frequent physical findings. Laboratory tests revealed a malabsorption pattern on intestinal x-rays, and malabsorption of xylose, fat, and vitamin B12 was frequently noted. Dense plasmacytic infiltrate of the lamina propria of small bowel was the most frequent pathologic finding while true neoplasm of the lymphoid system (ie, immunoblastic sarcoma) was encountered in 20% of the cases.
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PMID:Alpha heavy-chain disease in southern Iran. 41 71

A case of alpha-chain disease (alpha-CD) is described presenting as a malabsorption syndrome complicated by severe kalipenic nephropathy. Antibiotics and cyclophosphamide were effective in controlling the malabsorption and thr renal dysfunction but did not affect the basic pathological lesion. Eventually the disease became generalized, affected lymph nodes, liver and bone marrow and caused multiple intestinal perforations. Detailed histological studies failed to demonstrate the development of reticulum-cell sarcoma as a terminal complication of the disease and are suggestive of a single malignant plasmacytic cell process.
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PMID:alpha-Chain disease causing kaliopenic nephropathy and fatal intestinal perforations. 82 42

The clinical and pathological features of 17 "Western" type primary abdominal lymphomas (WTL) are compared with 14 of "Mediterranean" type (MTL). The MTL involved only young adult Mulatto and African patients in whom malabsorption and abdominal pain were the major clinical features. The WTL also predominantly affected Mulatto patients but four cases occurred in Caucasians, and the mean age at presentation was two decades later. An obstructive presentation was the most common; only one patient in this group had evidence of malabsorption. The WTLs were located mainly in the distal small bowel and were nearly all of monomorphic lymphocytic or histiocytic type. The MTLs were sited mainly in the duodenum and jejunum and were of an unusual pleomorphic histologic type. A spectrum of cells from those resembling atypical lymphocytes to large histiocytic types were seen, some of the latter resembling Reed-Sternberg cells. The pattern of mesenteric node infiltration in the MTLs was also unusual in that preservation of the medullary sinuses was a common finding. A notable feature of the MTLs was the presence of a heavy infiltration of mature-looking plasma cells associated with a fairly severe villous atrophy in the lamina propria of the small bowel. In the WTLs the adjacent small bowel did not show this feature. In addition to the above cases three patients with a similar heavy plasma cell infiltrate and villous atrophy but without evidence of a lymphoma are described. These cases may represent examples of MTL in a pre-malignant phase. Also included in this study are three patients with alpha-chain disease (alpha-CD), all with a heavy plasma cell infiltration and villous atrophy of the lamina propria and a pleomorphic type lymphoma involving the mesenteric nodes in all, and the small bowel in two. The lymphomas in alpha-CD have been interpreted as immunoblastic sarcoma by Lukes and Collins. Both genetic and environmental factors may be operative in the MTLs including the cases of alpha-CD.
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PMID:Primary intestinal lymphoma of "Western" and "Mediterranean" type, alpha chain disease and massive plasma cell infiltration: a comparative study of 37 cases. 82 14

Literature and original data are reported on a case of alpha-chain disease associated with small intestine lymphoma in a male patient aged 20. The disease manifested clinically with chronic diarrhea, malabsorption, hypoproteinemia, hypocalcemia, cachexia, discretely detected alpha-chain monomer. Morphologically, there was lymphoplasmacyte infiltration of the intestinal mucosal layer; capsule collagenization, clustering immunoblasts among microlymphocytes, a pronounced macrophagal reaction, intercellular crystalloid and lymph masses, follicular pattern disappearance, sites of cellular polymorphism revealed in an axillary lymph node. The latter finding evidenced for developing immunoblastic sarcoma.
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PMID:[Morphologic changes of the small intestine and lymph nodes in alpha-chain disease]. 314 43

Immunoproliferative small intestinal disease (IPSID) is prevalent in the Mediterranean region and in many Third World countries but is rare in Southeast Asia. Between 1980-1990, 4 cases of IPSID were admitted to Ramathibodi Hospital, Bangkok. Three were males and the mean age was 32 +/- 20.2 years. All patients presented with chronic diarrhea of 7 months to 6 years duration, and weight loss of 15 to 31 kg. All were malnourished, three cachectic, and one patient showed growth retardation. Intestinal parasites were found in all cases: two had multiple infections and three had uncommon protozoal infections (coccidium, cryptosporidium). Barium radiographs revealed intestinal mucosal fold thickening with malabsorption pattern in all cases. Alpha chain IgA was detected in one patient. The remainder underwent exploratory laparotomy and the histological finding was of plasma lymphocytic infiltration of the small intestinal mucosa. All patients responded to oral tetracycline with complete remission occurring in one case. During the follow-up period, 3 cases had progressive retractable clinical courses but all died 2 to 5 years after the diagnosis. The causes of death in these patients were secondary bacterial infection (1 case), intestinal tuberculosis (1 case), fungal infection (1 case) and immunoblastic sarcoma in another case. The results of this study confirm the occurrence of IPSID in Thailand. IPSID responds to oral antibiotic therapy and complete remission may be achieved during the early reversible benign phase, thus an awareness of its occurrence is of clinical importance.
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PMID:Immunoproliferative small intestinal disease (IPSID) in Thailand. 836 83