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)

Biweekly 200 mg/kg infusions of immune globulin (Gamimune) were given to a 46-year-old woman with severe common variable immunodeficiency, bronchiectasis, and chronic diarrhea with malabsorption. Failure to achieve therapeutically effective serum IgG concentrations in the face of fulminant sepsis was accompanied by a shortened serum IgG half-life of 10.6 days. Currently recommended doses of 200 mg/kg may prove inadequate in very ill patients with sepsis and malabsorption.
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PMID:Intravenous immune globulin therapy. Treatment of a patient with severe immunodeficiency, chronic malabsorption, and fulminant septicemia. 392 22

An 18-year follow-up of a young woman who presented at the age of 10 years with a malabsorption syndrome and recurrent chest infections is reported. The serum immunoglobulins, and in particular the IgA, were increased, cell-mediated immunity was impaired but free alpha heavy chains were not detected. A jejunal biopsy showed plasma cell infiltration of the bowel with villous atrophy. Bronchiectasis, recurrent skin infections, and sinusitis were treated with frequent courses of antibiotics and corticosteroids. An initial presumptive diagnosis of cystic fibrosis was excluded by a normal sweat test. Although difficult to classify her exact type of immune deficiency state, the data would appear to conform to the syndrome of cellular immunodeficiency with normal or near normal immunoglobulins and lymphopenia (Nezelof's syndrome).
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PMID:Plasma cell infiltration of the small intestine, recurrent pulmonary infections, and cellular immunodeficiency (Nezelof's syndrome). 405 Jul 63

The mitotic activity of epithelial lymphocytes (expressed as percentage mitotic figures/3,000 lymphocytes/mucosal biopsy) was determined in a random sample of jejunal biopsies performed on 44 children with malabsorption, diarrhoea, or failure to thrive. The mitotic index (MI) exceeded 0.2% in 19 biopsies obtained from children with untreated celiac sprue (CS); there were no false positives. The remaining 25 biopsies (MI of less than 0.2%) were considered to be "nonceliac" in origin, among which were several with a severe degree of villous flattening. Conditions in this latter category excluded by a low MI included cow's milk protein enteropathy, selective immunoglobulin A deficiency, combined variable immunodeficiency, Crohn's jejunitis, and intractable diarrhoea of infancy. A high MI (greater than 0.2%) prospectively distinguishes mucosal lesions due to untreated CS from other causes of malabsorption, particularly those associated with villous flattening, but in which the MI is less than 0.2%. This index is therefore proposed as a simple, reliable, and prospective histological marker of CS, and one that could: reduce the need to perform multiple biopsies during a gluten-free diet; and avoid the necessity for follow-up "diagnostic" gluten challenges, especially in very young children.
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PMID:Studies of intestinal lymphoid tissue. VIII. Use of epithelial lymphocyte mitotic indices in differentiating untreated celiac sprue mucosa from other childhood enteropathies. 406 81

The mucosal concentrations of seven regulatory peptides and the density properties and integrity of their storage granules have been studied in mucosal biopsies from the human jejunum in eight gastrointestinal disease states and compared with normal controls. In diseases with associated mucosal inflammation (coeliac disease, Crohn's disease with jejunal involvement, postinfective tropical malabsorption, and common variable immunodeficiency) there was a selective increase in fragility of the gastric inhibitory polypeptide (GIP) and somatostatin storage granules. The gastrin, motilin, enteroglucagon, secretin, and vasoactive intestinal polypeptide granules had normal properties in these conditions. In diseases in which diarrhoea occurred in the absence of changes in jejunal mucosal histology (irritable bowel syndrome, pancreatic insufficiency, jejuno-ileal bypass for morbid obesity, and purgative abuse) there were no abnormalities of the storage granules. Increased mucosal concentrations of all peptides except vasoactive intestinal polypeptide (VIP) were found in coeliac disease and selective increases of VIP found in Crohn's disease, motilin in the irritable bowel syndrome and gastrin and GIP in pancreatic insufficiency. It is suggested that the storage granule abnormalities in the diseases with abnormal mucosal histology are secondary to the inflammatory changes.
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PMID:Gastrointestinal regulatory peptide storage granule abnormalities in jejunal mucosal diseases. 614 62

The observation of a 21 year-old man with primary common variable hypogammaglobulinemia and total villous atrophy is reported. Gluten withdrawal induced considerable improvement in malabsorption and mucosal damage. However, protein-losing enteropathy, nodular lymphoid hyperplasia and immunoglobulin deficiency were persistent. To our knowledge, improvement on gluten-free diet has been reported until now in 18 previous cases. This report illustrates the links between immunodeficiency and malabsorption, which are discussed.
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PMID:[Acquired hypogammaglobulinemia and malabsorption with villous atrophy sensitive to a gluten-free diet]. 632 61

High doses of intravenous immune globulin produced meaningful clinical responses in three patients with altered immune function. One patient had a history of repeated infection with gram-positive pneumococci, another patient had second-degree burns and previously undiagnosed chronic leukemia of unknown etiology, and the third patient had frequent infections of the intestinal tract with symptoms of malabsorption and gluten intolerance. All three of the cases are examples of antibody immunodeficiency. After receiving intravenous immune globulin, all three patients experienced complete remission of infection.
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PMID:Intravenous immune globulin therapy for antibody immunodeficiency. 651 65

A patient had common variable immunodeficiency, chronic malabsorption, and Campylobacter jejuni infection. Infection was diagnosed by jejunal aspiration. A follow-up jejunal aspirate was culture positive at the same time that a stool culture was negative. Infection resulted in worsening of chronic diarrhea, but it was not associated with clinical features of colitis or proctitis. The duration of infection was prolonged and initial antimicrobial therapy was ineffective. Single drug therapy with erythromycin ethylsuccinate and then chloramphenicol led to the emergence of resistant organisms. After five months of bacterial excretion, combination therapy with metronidazole and neomycin sulfate eliminated the organism. This case emphasizes that the clinical manifestations and response to therapy of C jejuni infection can be altered in immunodeficient patients.
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PMID:Jejunal infection with Campylobacter. 671 98

Protracted diarrhoea in early infancy gives rise to many diagnostic and therapeutic problems. Jejunal biopsy often reveals villous atrophy of varying degrees. Severe reduction of small intestinal absorptive area causes secondary monosaccharide malabsorption, as well as secondary disaccharide deficiency, consequences which are relevant in any attempts at oral feeding. Morphologic, metabolic, endocrinological and microbiological studies have to be undertaken in order to establish a definitive diagnosis in protracted diarrhoea, but these studies often fail to reveal the aetiology of the disease. Immunologic abnormalities like phagocyte dysfunction, thymic atrophy and hypoplasia of B-cell regions in lymph nodes might be secondary events, but some types of immunodeficiency are of primary importance in the development of protracted diarrhoea. Total parenteral nutrition in many cases has to be instituted, with all its implications and hazards: septicaemia is the most dangerous of these. Zinc deficiency and acrodermatitis enteropathica may occur during total parenteral nutrition, and zinc deficiency secondarily contributes to the symptoms of diarrhoea. Parenteral administration of zinc is able to overcome these effects.
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PMID:Protracted diarrhoea: secondary monosaccharide malabsorption and zinc deficiency with cutaneous manifestations during total parenteral nutrition. 677 99

The authors report a case of a forty-six year old woman suffering from generalized prurigo nodularis. This dermatosis was associated with a cellular immunodeficiency; therefore a clofazimine therapy 300 mg/a day was instituted for six months. Ten months after the cessation of the clofazimine therapy, there appeared a malabsorption syndrome that was temporarily improved by gluten--free diet the real etiology was only ascertained during laparotomy, when masses of crystals in the small intestine mucosa as well as in mesenteric lymph nodes were observed. Therefore when it is necessary to prescribe clofazimine to take advantage of become its immunoregulating properties, it must always become in mind that an intestinal complication may ensue: this is well recognized in articles appearing in journals devoted to leprosy.
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PMID:[Exudative enteropathy caused by clofazimine: apropos of a case]. 681 19

Several diseases of the small intestine, including gluten-sensitivity, present with malabsorption and a "flat" mucosa. Determination of the mitotic index of epithelial lymphocytes provides a simple, objective method of assessing, and thus of predicting, whether a flat mucosa is due to gluten-sensitivity (index greater than 0.2%), or not (index less than 0.2%). The use of this index in circumstances especially likely to cause diagnostic confusion--for example, intestinal lymphoma; Crohn's jejunitis of immunodeficiency--is illustrated in this paper. Of seven cases, five (two primary lymphoma, three immunodeficiency) had been treated with a gluten-free diet without benefit; a mitotic index performed on the initial biopsy in each of these patients could have predicted from the outset that none was gluten-sensitive. Of the remaining two cases, determination of the mitotic index on the biopsy initially obtained from a man with severe hypogammaglobulinaemia would have indicated that he was also gluten-sensitive. Empirical use of a gluten-free diet was avoided in the other patient (with flat small intestinal mucosa and low mitotic index) in whom the diagnosis was ultimately shown to be due to Crohn's disease of jejunum.
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PMID:Studies of intestinal lymphoid tissue. VI--Proliferative response of small intestinal epithelial lymphocytes distinguishes gluten- from non-gluten-induced enteropathy. 682 70


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