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)

Nine cases of Mediterranean lymphoma with alpha heavy chain disease are described. All patients were young, aged between 11 and 22 years. Neither sex predominated in the series. The clinical presentation of the disease was either gastrointestinal, with chronic diarrhea and malabsorption, or neoplastic, with tumors frequently associated with either obstructive or perforative complications. Finger clubbing and retardation of physical growth (height and weight) and secondary sexual maturation were usually evident. The laboratory manifestations of the malabsorption syndrome were scant and generally not specific. The intestinal lesions predominated in the beginning of the small bowel, and their gross appearance varied from one case to another: diffuse thickening of the intestinal wall, alone or associated with tumors, or isolated segmentary tumors. Massive mesenteric lymph node enlargement was present in all cases; in some cases hepatic, splenic, or extra-abdominal lymph node spread was noted. Histologically, these lesions demonstrated a varied cellular morphology, but seemed to belong constantly to the B-lymphocytic line. The secretion and excretion by these cells of an abnormal IgA (devoid of light chains), which was revealed by immunoelectrophorectic study of the patients' sera, was the main biochemical abnormality of the disease. This disease presents several problems: 1) its relationship with other intestinal lymphomas; 2) the nature and the degree of malignancy of the intestinal cellular infiltrates; and 3) its identification as a clinicopathologic entity, with the possibility that infectious and genetic factors are significant in its etiology and pathogenesis.
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PMID:Mediterranean lymphomas with alpha heavy chain monoclonal gammopathy. 82 21

Twenty-seven intestinal lymphoma patients were studied. Abnormal alpha-chain immunoglobulin was detected in the sera of seven of these patients. The alpha-chain disease patients were from the rural areas of Southern Iran. They were of low socio-economic status and their age ranged from 15-44 years. Predominant clinical features were malabsorption, diarrhoea , abdominal pain, vomiting, and weight loss. Infiltration of mucosa of the small intestine with plasma cells and also distortion and flattening of the villi were common histopathologic characteristics of these patients. Involvement of mesenteric lymph nodes with infiltration of tumour cells was observed in a number of cases. Protein studies revealed no significant differences between the serum immunoglobulin levels of these patients and normal values. Immunoelectrophoresis using monospecific antiserum against H-chain of human IgA demonstrated the abnormal precipitin band of alpha-chain disease protein.
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PMID:Alpha-chain disease and its association with intestinal lymphoma. 82 61

A 30-year-old Turk was admitted with signs of exudative enteropathy together with malabsorption. There was no improvement on a gluten-free diet. Immunological investigations demonstrated atypical IgA-immunoglobulin in the serum which did not precipitate with antisera against L-chains. Peroral ileal biopsy and surgical biopsy material showed a diffuse proliferation of plasma cells in an altered ileal mucosa and in the mesenteric lymph nodes. Skeletal X-rays showed no osteolysis and the bone marrow showed no evidence for multiple myeloma. Treatment with melphalan and steroids resulted in a three year remission. In the terminal stage an intra-abdominal malignant lymphoma developed.
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PMID:[Alpha-chain disease presenting as malabsorption syndrome with exudative enteropathy (author's transl)]. 82 83

To clarify the controversy about the effectiveness of a gluten-free diet in dermatitis herpetiformis, 10 highly motivated patients were investigated. The indices used to assess improvement included deposition of sub-epidermal IgA in unaffected skin, counts of intraepithelial lymphocytes, deposition of IgA in jejunal villi, and electrical tests of glucose absorption. In every patient subepidermal IgA concentrations fell after gluten withdrawal. In all but one patient the dose of dapsone necessary to control symptoms was reduced. Indeed, six patients stopped taking the drug completely within a year. In nine patients biopsy specimens were taken from the jejunum; seven showed abnormalities in jejunal morphology, eight had increased numbers of intraepithelial lymphocytes, and five had increased numbers of IgA-reactive cells in the lamina propria. Two of these three indices improved after gluten withdrawal, which confirmed that all nine patients were adhering to their diet. Routine screening for malabsorption proved to be unsatisfactory for showing the mild jejunal disease found in patients with dermatitis herpetiformis. The electrical test of glucose absorption showed subnormal results in all eight patients tested, however, and in six the results improved after gluten withdrawal.
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PMID:Dermatitis herpetiformis: effect of gluten-free diet on skin IgA and jejunal structure and function. 85 90

A young woman presented a mixed congenital and familial immunodeficiency syndrome consisting in an absence of IgA and lowered levels of IgG and IgM, with a defect in cellular immunity. She had a mild malabsorption syndrome with slight alterations of the jejunal mucosa. Non-caseating tuberculoid granulomata were found in skin lesions, in lymph nodes and in the spleen. At age 27 the patient died of a neurological disease of 4 months duration. Autopsy revealed a very widespread demyelinating process involving mainly the right cerebellar hemisphere but also most of the pons and left cerebellum, with the typical morphologic characters of PML. In the hemispheres lesions were limited to microscopical "microglial nodules" with discrete demyelination. A review of 86 published cases of PML revealed 9 other cases in which lesions showed a strong predilection for the subtentorial territories. This sampling allows for tha assumption that some 11% of the cases of PML have this particular lesion distribution. Other pertinent features of this case are briefly discussed.
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PMID:[Progressive multifocal leucoencephalopathy. Observation with predominant pontocerebellar lesions and association with congenital immune deficiency]. 87 53

Two IgA-deficient children with inflammatory myopathy and intestinal malabsorption were evaluated. The myopathy was characterized by weakness of facial and proximal limb muscles, increased serum concentrations of lactic dehydrogenase and creatine phosphokinase, and histologic evidence of inflammation and degeneration of muscle fibers. Features of the intestinal abnormality were blunted villi, interstitial inflammation, and reduction in IgA-containing plasma cells and IgA content of epithelial cells. The myopathy and malabsorption improved with corticosteroid treatment. Circulating antibodies to striated muscle could not be demonstrated in either patient, but one had antibodies to milk and chicken serum proteins. We speculate that IgA deficiency may predispose to the development of inflammatory myopathy.
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PMID:Inflammatory myopathy, IgA deficiency, and intestinal malabsorption. 94 14

D-xylose absorption tests and jejunal morphology have been shown to be unaltered in 12 African patients with pellagra when compared with normal values for Zambian Africa adults; that result is contrary to two previous investigations in India and Egypt respectively. A significant inverse association has been shown, however, between D-xylose absorption and serum immunoglobulin IgG and IgA concentrations; that is consistent with previous studies and probably reflects malabsorption of xylose in the presence of systemic infections. It is concluded that pellagra per se does not alter intestinal structure or monosaccharide absorption.
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PMID:D-xylose absorption and jejunal morphology in African patients with pellagra (niacin tryptophan deficiency). 100 68

A case of extensive primary lymphocytic lymphoma (lymphosarcoma) of the gastrointestinal tract is reported. At the time of presentation the patient had diffuse involvement of the gastrointestinal tract from the stomach to the ano-rectal junction. Malabsorption was present. Pre-treatment immunologic studies showed elevated serum IgA and IgG, but impaired humoral and cellular immunity. Death occurred 13 months after diagnosis.
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PMID:Extensive primary lymphoma of the gastrointestinal tract. 107 44

A male infant with bilateral iris coloboma who had had repeated infections and malabsorption was studied. The levels of total lymphocytes and of T and B cells were normal or high, but IgA became undectable and IgG low, whereas IgM was normal. His lymphocytes did not respond to phytohemagglutinin (PHA), concanavalin A, pokeweed mitogen (PWM) or in mixed lymphocyte reactions (MLR), nor did they respond in vitro when thymosin was included in the test systems. He was skin-test-negative, even to dinitrochlorobenzene. His crudely isolated T lymphocytes and the supernatant of his PHA-stimulated lymphocytes inhibit the response of normal lymphocytes to PHA, PWM, and in MLR. During thymosin treatment skin test and lymphocyte reactivity to mitogen remained negative. He became faintly positive in MLR, and the suppressor activity in the supernatant of his PHA-stimulated lymphocytes no longer inhibited the response of normal lymphocytes to PHA, PWM, or in MLR. In parallel with thymosin treatment he showed quite marked clinical improvement.
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PMID:Suppressor cell activity in a male infant with T-and B-lymphocyte dysfunction treated with thymosin. 108 48

A 46 year old man presented with selective hypogammaglobulinemia, malabsorption and long-standing secondary malnutrition. Although the patient had essentially unmeasurable levels of immunoglobulins G (IgG), M (IgA), he had normal levels of immunoglobulin E (IgE). He was found to be anergic when tested for the delayed cutaneous hypersensitivity reaction. Evaluation of his cell-mediated immunity in vivo and in vitro suggested one discrete lesion, a defective production of the lymphocyte mediator macrophage migration inhibitory factor. With improved nutrition the patient "repaired" this defect in the "efferent" limb of cellular immunity and was no longer anergic.
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PMID:Selective hypogammaglobulinemia with persistence of IgE, malabsorption and a nutritionally dependent, reversible defect in cell-mediated immunity. 109 Jan 48


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