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)

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

A 59 year old woman with insulin-dependent diabetes mellitus and chronic diarrhea was found to have mild steatorrhea, selective plasma IgA deficiency and adrenal insufficiency. Significant adrenal secretion of corticosteroids resulted only after prolonged stimulation with large doses of exogenous ACTH. Plasma ACTH levels were not elevated during clinical adrenal insufficiency or after metyrapone administration but did respond normally to vasopressin and insulin-induced hypoglycemia. These studies were interpreted as showing both primary adrenal insufficiency and impaired pituitary reserve for ACTH secretion in response to the feedback stimulus. No deficiency was found in secretion of other pituitary tropic hormones. Jejunal biopsy showed a lack of IgA-containing plasma cells. With cortisone replacement, diarrhea subsided and a malabsorption pattern on a film of the small bowel was no longer seen. IgA deficiency has been noted frequently with steatorrhea but rarely with diabetes and only once previously with adrenal insufficiency.
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PMID:Atypical adrenal insufficiency with failure of the pituitary feedback receptor. A case with associated diabetes mellitus and selective IgA deficiency with steatorrhea. 17 48

Transmissible gastroenteritis or TGE is a virus diarrhoea which occurs in pigs of all ages and is associated with high mortality rates in the young piglets. Growth of virus in the columnar epithelium of the small intestine causes atrophy of the intestinal villi, malabsorption, watery diarrhoea and dehydration. Faecal excretion of virus usually continues up to fourteen days after infection but chronic carriers have been found to occur. TGE is self-limiting on the majority of pig-breeding farms but the virus may persist in particular conditions and an enzootic form of the disease will appear in this case. In typical outbreaks, the diagnosis can usually be based on clinical symptoms. When the disease runs an enzootic course, a clinical diagnosis will be out of the question. TGE should be differentiated from colibacillosis and from another virus diarrhoea, the aetiology of which is not precisely known. A rapid and correct diagnosis may be established by direct fluorescent antibody studies of frozen sections of the small intestine in infected piglets. When sows have been spontaneously infected, their offspring will be protected by lactogenic immunity. The presence of TGE antibodies of IgA class in the milk is required to ensure complete immunity of the piglets lasting for weeks on end. Intramuscular inoculation of a commercially available vaccine in sows will only stimulate the production of antibodies of the IgG class in the milk. These antibodies will merely afford short-lived immunity. The vaccine cannot prevent symptoms of disease from appearing in piglets following infection with virulent TGE virus but it does reduce mortality
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PMID:[Transmissible Gastroenteritis in Swine (author's transl)]. 17 23

An 8-year-old boy presented with idiopathic late-onset immunoglobulin deficiency manifested principally by recurrent suppurative chest infections and chronic diarrhoea with malabsorption. Nodular lymphoid hyperplasia and giardiasis were shown on small bowel biopsy. Investigation of the immune system showed low serum levels of IgG, IgM, and IgA, negative skin tests to four recall antigens, absent mixed lymphocyte reactivity, and impared lymphocyte responses to mitogens in vitro. Serum complement and granulocyte function studies were normal. Maintenance therapy with gammaglobulin and antibiotics gave a good response.
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PMID:Idiopathic late-onset immunoglobulin deficiency with associated defect in cell-mediated immunity. 30 36

Immunoglobulin (Ig)-containing cells were quantitated immunohistochemically in biopsy specimens from the proximal jejunal mucosa. The numbers of IgA, IgM, and IgG immunocytes in a defined "mucosal tissue unit" were, on the average, raised 2.4, 4.6, and 6.5 times, respectively, when 13 adult patients with untreated coeliac disease (CD) were compared with 15 patients who had a histologically normal mucosa. The IgA-:IgM-:IgG-cell ratios averaged 66:28:6 in untreated CD and 79:18:2.6 in the controls. Similar quantitative data in 10 patients with treated CD were intermediate. IgD- and IgE-containing cells were rare in all patient groups. Most patients in a heterogeneous malabsorption group showed a jejunal Ig-containing cell population similar to that seen in CD, indicating that the local immunocyte pattern may not be specific for the latter disease. The only significant alteration in serum Ig levels related to CD was a raised concentration of IgA compared with normal. This was consistent with the increased amounts of extracellular IgA revealed in the mucosa. However, there was no indication of a defect in the transport of dimeric IgA and IgM through SC-producing cells, which in the CD mucosa were present in both crypt and surface epithelium.
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PMID:Immunoglobulins in jejunal mucosa and serum from patients with adult coeliac disease. 32 52

The patients studied were diagnosed as suffering from alpha-chain disease by their clinicopathological features, malabsorption findings, X-ray, and presence of abnormal alpha-chain protein in their serum. The objective of the study was to determine any possible defect of the immune system in such patients. The rosette technique and surface immunofluorescence were used to enumerate the circulating T and B lymphocytes in these patients. They were also skin-tested with tuberculin and given sensitizing doses of dinitrochlorobenzene. Their serum immunoglobulins were also quantitated. It was found that the proportion of circulating B lymphocytes was much higher than normal, whereas that of T lymphocytes was lower than normal. Furthermore, they could not be sensitized to DNCB and their skin test to tuberculin was negative. It was concluded that the disease was a B-cell disease of IgA type, associated with low level of cellular immunity.
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PMID:T and B lymphocytes in alpha-chain disease. 41 62

Detailed investigations of 9 patients with dermatitis herpetiformis are presented. In all cases cutaneous lesions were controlled by dapsone alone or by dapsone and gluten free diet. Granular IgA deposits were found in 7 patients, linear IgA deposits in one, and C3 component of complement in one. 3 patients out of 8 tested, carried the specific HLA-B8 antigen. Despite an extensive investigation, no malabsorption was detected. Jejunal biopsies were performed in 8 cases. Jejunal villous flattening was observed in one patient. It improved after a 2 months gluten free diet on subsequent jejunal biopsies. D. H. seems peculiar in France as compared with case reports from other countries: low prevalence of gluten sensitive enteropathy; rare occurrence of the specific HLA-B8 antigen; incidence of D. H. seems to be low in France. It is noticeable that french incidence of coeliac disease is low as well. This suggests a genetic difference in the investigated population (low prevalence of HLA-B8 antigen) and/or different alimentary habits, particularly a low dietary gluten amount.
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PMID:[Dermatitis herpetiformis (author's transl)]. 48 15

A six-year old boy who had suffered from the age of two with chronic diarrhea has been found to be severely retarded in statural growth. Examinations discovered marked lymphopenia with a T lymphocyte defect accompanied by absent IgA and IgE. In contrast with other cases described in literature, in this case the immunodeficiency was not accompanied by bone or cartilage alterations. The only factor apparently responsible for his lack of growth was the malabsorption.
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PMID:Harmonic dwarfism, lymphopenia, deficit of IgA and IgE in a 6-year old boy. 61 Apr 13

The clinical, biochemical, immunological and histopathological features in a patient with alpha-chain disease are described. The patient, a 20-year-old Coloured man, presented with severe steatorrhoea, malabsorption, abdominal pain and progressive general deterioration. An heterogeneous abnormal band with IgA immunochemical specificity was detected on electrophoresis of the patient's serum and urine. This protein was identified as free alpha-chain and was present in serum, urine, saliva and jejunal juice. A jejunal mucosal biopsy specimen showed distinctive appearances associated with alpha-chain disease. Bone marrow involvement was found and abnormal lymphoid cells were seen in the circulation together with an increased B lymphocyte population derived from bone marrow. This is the third South African patient with alpha-chain disease to be diagnosed. The patient has shown a partial remission after 12 months' chemotherapy. There was rapid symptomatic response and normalisation of protein parameters which were not paralleled by an objectively discernible response as assessed by haematological examination, intestinal absorption studies and histology of the jejunal mucosa.
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PMID:Alpha-chain disease in a non-Mediterranean climate. A case report. 81 7

alpha CD is an immunoproliferative disorder which is most frequently, but not always, associated with the disease entity referred to as Mediterranean lymphoma with malabsorption. In most cases, the evolution of the disease and its morbid anatomy suggests an immune deficiency state in which an abnormal clone of intestinal plasma cells incapable of producing the complete IgA molecule proliferates, perhaps in response to microbial antigenic stimulation. In the majority of cases, this proliferation is composed of mature appearing plasma cells which lack morphologic features of malignancy. Although possibly reversible, this proliferation appears to be highly susceptible to the evolution of secondary neoplastic clones, which appear in most instances after a clinical course of unpredictable duration and manifest themselves in the form of single or multiple immunoblastic sarcomas.
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PMID:The pathologic anatomy of alpha chain disease. 82 78


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