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)

The cause of death was obtained for 77 members of the Coeliac Society resident in England and Wales. Proportional mortality analysis revealed a significant deficiency in deaths from ischaemic heart-disease in males and a non-significant deficiency in females. It is suggested that this apparent protective effect of coeliac disease might result from malabsorption of dietary lipids. The study also confirmed findings of an excess of deaths from lymphoma and upper gastrointestinal neoplasms.
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PMID:Death from ischaemic heart-disease and malignancy in adult patients with coeliac disease. 5 82

Of 18 patients with small-intestinal lymphoma associated with villous atrophy and crypt hyperplasia of uninvolved mucosa, most presented with acute obstruction or perforation preceded by abdominal pain and weight-loss. Malabsorption, thought to be adult coeliac disease, preceded the diagnosis of lymphoma in 5 cases. The lymphomas were composed of malignant histiocytes and were classified on morphological grounds as malignant histiocytosis. Of particular interest was the finding by immunohistochemical techniques of all major immunoglobulin classes within malignant cells of single tumors. Characterisation of this group of lymphomas as a specific entity should help in the further understanding of the relationship of malabsorption and lymphoma and in the rationalisation of treatment.
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PMID:Intestinal lymphoma associated with malabsorption. 7 67

All adult cases of primary intestinal lymphoma seen during the years 1953--77 at Groote Schuur Hospital, South Africa, were reviewed. Seventy percent of patients with solitary lymphoma and 80% with immunoproliferative small intestinal disease were mulatto. Patients in the latter group presented with malabsorption and those with a solitary lymphoma presented with intestinal obstruction. Four of 15 patients tested for the presence of alpha-heavy-chains were found to have alpha-heavy-chain disease. Family studies of potential genetic and immunologic factors showed some significant differences in IgA and IgG levels in the families of two patients with alpha-heavy-chain disease. Elevated alkaline phosphatase of intestinal origin was found in four of six patients with immunoproliferative small intestinal disease and in a high proportion of relatives. Fifty percent of the six patients were of blood group B. Minor blood groups, ABH secretor state and Pi phenotype distribution were similar to those of the control subjects. HLA gene frequency was particularly increased in the HLA-A9 antigen. These studies suggest that genetic factors may be relevant to the pathogenesis of immunoproliferative small intestinal disease.
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PMID:Primary intestinal lymphoma in South Africa. 10 20

Five out of 74 patients with adult coeliac disease (ACD) diagnosed from 1965 through 1977 developed intestinal lymphoma. The age at diagnosis of ACD was 54-63 years (mean 58) and in all patients an initial improvement was noted on a gluten-free diet. The interval between the diagnoses of ACD and lymphoma was 0.5-6 years (mean 3). In 4 patients the diagnosis of lymphoma was preceded by relapse of diarrhoea and loss of weight despite an adequate diet; 2 patients developed perforation of the bowel. In all patients who subsequently developed lymphoma a pronounced lymphocytopenia, 0.4-1.3 X 10(9)/l, was found at the diagnosis of ACD. It is suggested that ACD patients over 40 who have relapse of malabsorption symptoms despite an adequate diet should be investigated for the presence of an intestinal lymphoma. The suspicion of a malignant transformation may be especially strong if a previous, pronounced lymphocytopenia has been recorded. Three patients died 1-12 months after the diagnosis of lymphoma. However, 2 patients are in good condition 14 and 20 months, respectively, after laparotomy and removal of the tumour, indicating that an early diagnosis and treatment may have a reasonably palliative effect in some cases.
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PMID:Lymphoma of the small intestine in adult coeliac disease. 36 82

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

Alpha Heavy Chain Disease has generally been described in the Mediterranean area in young people with a diffuse gastrointestinal lymphoma and malabsorption. We describe a patient from the United States with polypoid lymphoma of the gastrointestinal tract and a prolonged clinical course, who had the characteristic alpha heavy chain disease protein. He did not have malabsorption, and the gastrointestinal tract, though containing multiple discrete lymphomatous polyps had little plasmacytic reaction. Lymphosarcoma cell leukemia developed late in the course and the malignant cells had the characteristics of B lymphocytes but did not contain alpha heavy chains. The data support the contention that the lymphoma in this form of alpha heavy chain disease may involve B lymphocytes but that the abnormal protein may be produced by other cells. Polypoid varieties of gastrointestinal lymphoma in North America must now be considered part of the spectrum of alpha heavy chain disease and the diagnostic protein searched for diligently.
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PMID:New presentation of alpha heavy chain disease: North American polypoid gastrointestinal lymphoma. Clinical and cellular studies. 41

Ten cases of primary upper small-intestinal lymphoma associated with alpha-chain protein in serum were discovered in a prospective study of the sera of patients with immunoproliferative small-intestinal disease (IPSID). Patients were mostly young males presenting with abdominal pain, weight loss, and diarrhea and showing laboratory evidence of carbohydrate, fat, and vitamin B12 malabsorption and hypoalbuminemia. The more frequently encountered pathologic abnormality was a diffusely nodular jejunal mucosa produced by a plasmacytic infiltrate of variable cell maturity involving a varible depth of small bowel wall with or without involvement of the mesenteric or para-aortic-lymph node complex and, in one instance, the liver. A less frequent picture included circumferential ulcerative and constrictive transmural tumors of the upper small intestine produced by a malignant lymphoma with involvement of abdominal lymph nodes. Small-intestinal surface epithelial abnormalities, a dense mantle of mature plasma cells overlying the lymphoma, a pronounced follicular lymphoid hyperplasia adjacent to and at distances from the lymphoma were other features of note in our IPSID cases associated with alpha-chain protein.
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PMID:Primary upper small-intestinal lymphoma and alpha-chain disease. Report of 10 cases emphasizing pathological aspects. 41 96

The authors discuss the main features of the complex pathophysiology of patients subjected to duodenocephalopancreatectomy, and particularly the difficulties inherent in clinical assessment of the digestive and metabolic impairment consequent upon the duodeno-gastro-pancreatic mutilation. Out of a total of 57 cases of this description, they singled out for recheck 23 patients who had undergone duodenocephalopancreatectomy not less than six months and not more than seven years before (chronic pancreatitis, 11 cases; various malignancies of the periampullar area, 10 cases; Zollinger-Ellison syndrome, 1 case; retroperitoneal lymphoma, 1 case). Seventy-six per cent of patients who had been gainfully employed were able to resume their jobs after surgery. Steatorrhea, assessed in terms of fecal fats, was present in all cases; notwithstanding, 70% of the patients gained weight (average increase 7 kg). All patients were on enzyme replacement therapy. Only 4% developed diabetes, and none developed postoperative peptic ulcers. Conversely there was a high incidence (65%) of bone structure reshuffling, signally osteoporosis, probably imputable to steatorrhea and vitamin D malabsorption, plus the often associated increase of serum alkaline phosphatase activity.
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PMID:[The biological results of duodenocephalopancreatectomy. Clinical evaluations based on a long-term follow-up]. 53 2

A new Peruvian case of malabsorption associated with primary diffuse lymphoma of the small bowel and mesenteric lymph glands is reported, having found no alpha chains either in blood or in urine. Like in most of the 8 previous cases, medium oral doses of oxytetracycline supressed diarrhea, markedly atenuated steatorrhea, restored the sense of well-being to the patient, and significantly increased his body weight. Critic is made on the denominations "mediterranean lymphoma" and "primary upper small intestinal lymphoma" ("PUSIL") that have been inappropriately assigned to the condition.
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PMID:[Mediterranean lymphoma in Peru. Its treatment with oral oxytetracycline]. 55 Jul 91

In addition to lymphoma, there is an increased incidence of gastrointestinal carcinoma in patients with malabsorption due to celiac disease. This is frequently manifested by a loss of response to gluten withdrawal. Four such cases are described: one patient had lymphoma and the other three had cancer of the esophagus, jejunum, and pancreas, respectively. The literature indicates that carcinoma of the esophagus and small bowel is particularly common in patients with celiac disease. These findings suggest that celiac disease should be considered a premalignant condition and that such patients should undergo a regular radiographic survey to detect early cancer.
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PMID:Small-bowel malabsorption and gastrointestinal malignancy. 62 26


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