Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Serum cold-reactive lymphocytotoxin (LCT) was detected in twenty-two of fifty-six (40%) patients with inflammatory bowel disease (IBD). The frequency of LCT detection was similar in Crohn's disease and ulcerative colitis. Cytotoxicity testing against T or B cell-enriched peripheral blood lymphocytes from normal donors, together with absorption experiments, indicated that LCT in IBD was reactive against determinants on both cell subpopulations. Reactivity against T cells from patients with common variable immunodeficiency was significantly less than with normal donor T cells. LCT in IBD could not be related to prior allogeneic sensitization and its presence appeared to be unrelated to disease activity or drug therapy. No correlation was found between LCT and peripheral blood T- or B-cell numbers. The present findings suggest the need for further investigation of the role of infectious agents in the pathogenesis of IBD.
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PMID:Serum lymphocytotoxins in inflammatory bowel disease. Studies of frequency and specificity for lymphocyte subpopulations. 108 34

The clinical significance of the fastidious organism DF-3 isolated from stool cultures is unclear. We sought to improve our understanding of this organism and to further define its association with human disease. Stool cultures for DF-3 were obtained from three sources: an ongoing study of enteric pathogens in patients infected with the human immunodeficiency virus, a screening procedure in which all stool samples submitted for Clostridium difficile toxin assay were cultured for DF-3, and stool samples submitted specifically for DF-3 culture. Retrospective clinical data were obtained from chart reviews of patients with positive cultures. Antimicrobial susceptibility testing and cell wall fatty acid analysis were performed for each DF-3 isolated. Eight isolates of DF-3 were obtained over a period of 8 months. All patients either had severe underlying disease or were immunocompromised, including three patients coinfected with human immunodeficiency virus and two patients with inflammatory bowel disease. The spectrum of clinical disease ranged from chronic diarrhea with a well-defined response to therapy for DF-3 to an asymptomatic carrier state. Cell wall fatty acid analysis of these isolates demonstrated a consistent pattern with a large peak of 12-methyltetradecanoate. DF-3, a fastidious gram-negative coccobacillus, can be recovered from stool cultures of immunocompromised patients by using selective media. The presence of 12-methyltetradecanoate in cell wall fatty acid analysis assists in identification. The increased use of a selective medium-(cefoperazone-vancomycin-amphotericin B) in the evaluation of diarrhea in immunocompromised hosts, including persons with inflammatory bowel disease, may better define the association of DF-3 with human gastrointestinal disease.
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PMID:Clinical illnesses associated with isolation of dysgonic fermenter 3 from stool samples. 153 8

Gastrointestinal disease is a well recognized feature in patients with common variable immunodeficiency, and is often due to infection with a variety of organisms. Symptoms usually improve with appropriate antibiotic therapy and replacement gammaglobulin. We describe three middle-aged female patients with common variable immunodeficiency who had protracted diarrhoea and weight loss. Despite extensive investigation no infectious cause was found. All patients had granulomas distributed throughout the gastrointestinal tract, but no features of inflammatory bowel disease. There was a poor response to gammaglobulin replacement therapy, antibiotics or symptomatic treatment. We suggest that granulomatous enteropathy is another gastrointestinal manifestation of common variable immunodeficiency.
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PMID:Granulomatous enteropathy in common variable immunodeficiency: a cause of chronic diarrhoea. 185 60

The case is reported of a 39-year-old pregnant woman who presented with fever, abdominal complaints, and diarrhea. Laboratory investigation revealed mononucleosis in the peripheral blood. All microbiological studies were negative, with the exception of finding cytomegalovirus (CMV). Seroconversion was documented; the virus was cultured from urine and subsequently was demonstrated to be present in the inflamed mucosa of the rectum and distal sigmoid, which was found at sigmoidoscopy. This woman was delivered of a neonate with congenital CMV infection but without apparent malformations. The patient experienced recurrences of the bowel disease, in the first of which CMV could still be cultured from a biopsy specimen. In the follow-up period, an otherwise aspecific chronic inflammatory bowel disease remained present. No immunological abnormalities were found, and antibodies to human immunodeficiency virus were negative. This case demonstrates that inflammatory bowel disease can develop as a result of primary infection with CMV.
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PMID:Acute ulcerative proctocolitis associated with primary cytomegalovirus infection. 216 91

A toddler with common variable hypoimmunoglobulinemia (CVH), inflammatory bowel disease, and recurrent Pneumocystis carinii pneumonia (PCP) on intravenous gammaglobulin (IVIG) replacement was evaluated for a combined cellular immunodeficiency. He had a normal number of circulating T-cells, natural killer (NK) cells, T-cell subset percentages, and his peripheral blood mononuclear (PBM)-derived B-cell number was low. PBM mitogen blastogenesis and mixed lymphocyte reaction (MLR) were normal. MLR activated T-cells expressed class I and II MHC antigens, interleukin 2 (IL-2), and B-cell growth factor (IL-5)-related receptors. The patient's T-cells induced control B-cell maturation with pokeweed mitogen (PWM-PC), and did not suppress PWM-PC production by allogeneic PBM. Bone marrow (BM) CD19+ B-cell number varied between 10 and 44% of all PBM, and the BM B-cell-enriched fraction failed to differentiate to PWM-PC with autologous or allogeneic T-cell help. The NK activity assayed using K562 target cells was deficient, 9.2 x 7.7% (6.9-9.2%) pt, control 35.9 x 35.8% (16.3-67.2% +/- 12.8). In the presence of interferon-alpha, 800 U/ml, the patient's NK activity increased to 17.2 x 14.9% (12.6-17.2%), control 35.9 x 51.0% (36.5-72.3% +/- 12.0). The patient's cell-mediated lympholysis of HLA nonidentical, allogeneic stimulators was normal. Maintaining trough serum IgG levels above 500 mg/dl was required to suppress recurrent PCP. This functional NK deficiency may be relevant to the development of recurrent PCP in IVIG-treated CVH patients.
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PMID:Common variable hypogammaglobulinemia, recurrent Pneumocystis carinii pneumonia on intravenous gamma-globulin therapy, and natural killer deficiency. 278 54

Salmonellae have demonstrated an extraordinary capacity to adapt to a wide range of ecologic niches and to the peculiarities of modern society, such as the mass production of food products. The vast majority of infections in the United States are caused by serotypes not specifically adapted to human or animal hosts, whereas the most frequent isolate in developing countries is S. typhi, which is highly adapted to human hosts. The number of isolates reported in the United States has been increasing steadily since 1975, largely a result of outbreaks associated with the mass production of food products, particularly poultry, which is frequently contaminated. Salmonella infection occurs when ingested organisms bypass gastric defenses, multiply within the intestinal lumen, penetrate the intestinal mucosa, and multiply within macrophages of the reticuloendothelial system. They may then disseminate via the systemic circulation. Several virulence factors have been identified. The wide range of pathologic and clinical manifestations are subdivided into four syndromes, each requiring a distinct diagnostic and therapeutic approach: (1) gastroenteritis, (2) enteric fever, (3) bacteremia with or without metastatic disease, and (4) asymptomatic carriage. Although any serotype can cause any of these syndromes, certain serotypes are associated with specific presentations. Serious complications of bacteremic infection include infections of the aorta, endocardium, bone, and meninges. Salmonella infection is particularly severe in patients who have AIDS, leukemia, lymphoma, immunodeficiency of other causes, inflammatory bowel disease, schistosomiasis, and macrophage dysfunction. Diagnosis is based on culture of the organism from appropriate sites. Several serologic tests have been developed that warrant further evaluation. Chloramphenicol, ampicillin, amoxicillin, and trimethoprimsulfamethoxazole have clearly established efficacy. Experience with third generation cephalosporins and quinolones is preliminary and fragmentary, but results suggest that they may prove to be efficacious in certain clinical circumstances. Antibiotic resistance has become a major problem in certain geographic areas. The three vaccines for S. typhi that are currently in use internationally provide only moderate protection for short periods of time.
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PMID:The spectrum of Salmonella infection. 307 16

Antineutrophil cytoplasmic autoantibodies (ANCA) have been used as markers of systemic vasculitides, including microscopic polyarteritis (MPA) and Wegener's granulomatosis. The diagnostic potential of ANCA assays together with antibodies against the neutrophil enzymes myeloperoxidase (MPO) and proteinase 3 for detecting a systemic vasculitis was tested in a Chinese patient population. 672 sera were received for ANCA assay, and ANCA detected by indirect immunofluorescence was positive in 73 sera from 42 patients. Of the 42 patients, 3 had cytoplasmic ANCA, while 39 had a perinuclear pattern. There was no patient with Wegener's granulomatosis. Two cytoplasmic ANCA positive patients suffered from ulcerative colitis. Another cytoplasmic ANCA positive patient was a carrier of human immunodeficiency virus. Of the 39 perinuclear ANCA positive patients, 10 had MPA. Eight of them were tested for anti-MPO antibody, and all were positive. Other immune disorders that were perinuclear ANCA positive included: 13 patients with systemic lupus erythematosus, 3 with mixed connective tissue disease, 1 with Goodpasture's syndrome, 2 with inflammatory bowel disease, and 2 patients with IgA nephropathy. Anti-MPO antibody was not specific for MPA, and 7 out of the 13 patients with systemic lupus erythematosus were anti-MPO antibody positive. Our study suggests that ANCA and anti-MPO antibody are not specific for MPA in a Chinese population. They would alert the clinician of the possibility of vasculitis, but a clinicopathological correlation is essential in making the diagnosis.
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PMID:Use of antineutrophil cytoplasmic autoantibodies in diagnosing vasculitis in a Chinese patient population. 791 85

We report on 56 children with sclerosing cholangitis (SC) seen between 1972 and 1992. The first symptoms occurred at a mean age of 3.7 years; 15 infants had neonatal cholestatic jaundice. At diagnosis, cholestatic jaundice was present in 25 children, hepatomegaly in 54, splenomegaly in 41, and ascites in 12. Serum alkaline phosphatase activity was increased in 49 patients and gamma-glutamyltransferase activity in all patients tested. Most often the histopathologic findings were extensive portal fibrosis and neoductular proliferation. Cholangiography showed abnormal intrahepatic bile ducts in all children and abnormal extrahepatic bile ducts in 35 (63%). The children were separated into three groups: (1) those with SC of neonatal onset (27%); (2) those with SC of postneonatal onset associated with another disease (55%)--histiocytosis X in 14 children, immunodeficiency syndromes in 8, chronic inflammatory bowel disease or autoimmune hepatitis in 8, and congenital psoriasis in 1; and (3) those with SC of postneonatal onset without an associated disease (18%). Biliary cirrhosis was present in all but three children after 6 months to 19.3 years of follow-up. Eleven children died of portal hypertension or liver failure, and six died of a complication related to the associated disease. Fifteen children had liver transplantation; 11 of these are alive 6 months to 6 1/2 years later without recurrence of SC. The overall estimated median survival time of children with SC was 10 years from clinical onset. These results indicate that SC should be suspected in all children with a chronic cholestatic disease and increased serum gamma-glutamyl transferase activity, especially when diseases known to be associated with SC are present. The prognosis is poor, but liver transplantation should be considered except in those with severe immunodeficiency syndromes.
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PMID:Sclerosing cholangitis in children. 828 75

Today, Pneumocystis carinii pneumonia (PCP) is typically associated with AIDS. However, in the pre-AIDS era, PCP was known to be associated with various immunodeficiency states, malignancies, and immunosuppressive therapy, particularly the use of corticosteroids. PCP has been reported to occur during immuno-suppressive therapy of some chronic inflammatory states, but it has never been reported in patients with inflammatory bowel disease. We report two patients with ulcerative colitis who developed PCP during high-dose corticosteroid therapy, and review the literature regarding non-AIDS PCP. PCP should thus be added to the list of bronchopulmonary complications in inflammatory bowel disease. This report should give gastroenterologists further impetus to limit immunosuppressive therapy to its minimal effective dose.
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PMID:Pneumocystis carinii pneumonia in patients with ulcerative colitis treated with corticosteroids. 847 Jun 40

The occurrence of chronic diarrhea in infants younger than three months suggests disaccharidase deficiency, cow's milk or soy protein intolerance, cystic fibrosis or an immunodeficiency state, while chronic diarrhea in children three to 18 years of age suggests celiac disease, late-onset primary lactose deficiency and inflammatory bowel disease. Gastrointestinal infection is the most common cause of chronic diarrhea in children of all ages. Diarrhea that develops after the introduction of cow's milk, cereals and fruits suggests an enzyme deficiency or protein intolerance. Watery, explosive stools suggest sugar intolerance, and foul-smelling, greasy, bulky stools suggest fat malabsorption. Marked weight loss suggests malabsorption, inflammatory bowel disease, hyperthyroidism or malignancy. The presence of neutrophils or red blood cells in the stool indicates bacterial gastroenteritis or inflammatory bowel disease, while the presence of eosinophils suggests protein intolerance or parasitic infestation. A toddler who is thriving and cheerful despite having diarrhea may have chronic nonspecific diarrhea of childhood.
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PMID:Evaluating the child with chronic diarrhea. 862 43


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