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

A long-term follow-up of 740 American servicemen splenectomised because of trauma during the 1939-45 war showed a significant excess mortality from pneumonia and ischaemic heart-disease. Mortality from cirrhosis was also increased, but not significantly. The findings confirm that the risk of fatal infections is increased by asplenia; however, the risk of cancer was not increased, as it is in some other immunodeficiency states. Post-splenectomy thrombocytosis and hypercoagulability may account for the increased risk of fatal myocardial ischaemia in this group.
 ...
PMID:Splenectomy and subsequent mortality in veterans of the 1939-45 war. 6 6

Cellular and humoral immune reactivity to primary and secondary challenge with tetanus toxoid, diphtheria toxoid and keyhole limpet hemocyanin was studied in normal dogs and canine bone marrow chimeras prepared for marrow grafting by lethal doses of cyclophosphamide. Short-term chimeras (less than 100 days postgrafting) showed general impairment of immune function as indicated by marked depression of skin test reactivity, lymphocyte blastogenesis in vitro and antibody formation. Long-term chimeras (more than 100 days post-grafting) demonstrated gradual improvement of immunologic capacity related to the stage of postgrafting. Prolonged immunoincompetence was suggested by decreased lymphocyte proliferation in vitro in response to stimulation with tetanus and diphtheria toxoids and by incomplete conversion from 19S to 7S antibody synthesis. Lymphocyte blastogenesis to specific antigens may prove a useful parameter for the in vitro evaluation of defects in cell-mediated immunity following allogeneic bone marrow transplantation. Our findings of prolonged immunodeficiency in the preclinical canine model point out the necessity for vigilance in early detection and treatment of infection and underline the importance of approaches aimed at accelerating immunologic reconstitution.
 ...
PMID:In vitro and in vivo immune response to specific antigens in canine marrow graft recipients. 7 95

We have investigated a new hypothesis for the association between adenosine deaminase (A.D.A.) deficiency and immunodeficiency--namely, that deoxyadenosine rather than adenosine (an equally effective A.D.A. substrate) is toxic to proliferating cells of lymphoid origin. This possibility was explored in mitogen-stimulated lymphocytes cultured with a potent A.D.A. inhibitor, E.H.N.A. (erythro-9[2-hydroxy-3-nonyl] adenine) to simulate A.D.A. deficiency. In this in-vitro system deoxyadenosine was inhibitory at much lower and more physiological concentrations (1 mumol/1), compared with adenosine (100 mumol/1).
 ...
PMID:A role for purine metabolism in the immune response: Adenosine-deaminase activity and deoxyadenosine catabolism. 7 65

Using the indirect ELISA technique, IgM, IgG and IgA antibody titres against the primary test immunogen Helix pomatia haemocyanin (HPH) could be measured. All twenty-seven normal volunteers (age range 26-74 years) developed maximal or high IgM, IgG and IgA antibody titres 2 weeks after primary immunization with 1.0 mg HPH subcutaneously. Lower dose immunization resulted in submaximal responses. Titre kinetics in the three Ig classes correlated in height and time per person. There was no significant difference in antibody response between two adult age groups. Secondary immunization after 6 weeks, again with 1.0 mg HPH, when high titres were still present, resulted in a small titre increase in all three Ig classes. Comparison with the tanned red cell agglutination technique used previously, in combination with the 2-mercaptoethanol treatment of sera, showed the unreliability of 7S titres measured thus early in the primary immune response. Investigation of four patients with humoral immunodeficiency confirmed the class specificity of the test.
 ...
PMID:Class-specific antibody titres (ELISA) against the primary immunogen Helix pomatia haemocyanin (HPH) in man. 8 Feb 96

Three siblings presented in early childhood with central-nervous-system (CNS) dysfunction, candida dermatitis, keratoconjunctivitis, and alopecia. Two were studied immunologically and had absent delayed-hypersensitivity skin-test responses and absent in-vitro lymphocyte responses to candida antigen. One of them had selective IgA deficiency and no antibody response to pneumococcal polysaccharide immunisation, and the other had a subnormal percentage of T lymphocytes in peripheral blood. The first two siblings died with progressive CNS deterioration and overwhelming infection. The third child, who presented with a periorificial candida dermatitis, alopecia, keratoconjunctivitis, and intermittent ataxia at eighteen months of age, had intermittent lactic acidosis and raised excretion of beta-hydroxyproprionate, methylcitrate, beta-methylcrotonylglycine, and beta-hydroxyisovalerate in urine. After four days of oral biotin, 10 mg/per day, the metabolites in her urine were significantly reduced, suggesting a biotin-responsive multiple carboxylase deficiency. These findings, taken with previous reports of immune defects in patients with disorders of branched-chain aminoacid catabolism, suggest a new biochemical basis for primary immunodeficiency disease.
 ...
PMID:Multiple biotin-dependent carboxylase deficiencies associated with defects in T-cell and B-cell immunity. 8 54

A 13-month-old boy had a "late-onset" form of combined immunodeficiency and a fulminant Pneumocystis carinii pneumonia of one month's duration. There was no evidence of cutaneous-delayed hypersensitivity responses to diphtheria-tetanus toxoids, Candida albicans, or streptokinase-streptodornase, or of lymphocyte DNA synthesis after in vitro stimulation with the mitogens phytohemagglutinin and concanavalin A, and only 2% to 4% of peripheral blood E-rosetted T lymphocytes. The serum IgM level was normal (62 mg/dL), whereas the other immunoglobulins were markedly reduced. Despite an increased number of Ig-bearing lymphocytes, in vitro Ig secretion after pokeweed mitogen stimulation was substantially reduced. The thymus gland was dysplastic with no Hassalls' corpuscles or thymocytes, and other lymphoid organs showed depletion of T-dependent areas to a greater extent than the B-dependent areas.
 ...
PMID:Diagnostic dilemma of a 13-month-old boy with 'late-onset' combined immunodeficiency. 8 98

Up to 12% of epileptic patients have subnormal IgA serum concentrations. Previous observations suggest that IgA deficiency is correlated with hydantoin treatment and also with the type of seizure. In a followup study it is shown that IgA deficiency in epileptics is a rather constant feature of a given patient. The most pronounced changes in IgA levels were seen in patients in whom the hydantoin medication also changed. However, low IgA levels have also been reported in untreated epileptics. A new classification for the immunodeficiency state in epileptics is introduced.
 ...
PMID:Immunodeficiency in epilepsy: a new view. 9 Jan 39

In a previously healthy 13-year-old girl with disseminated blastomycosis, immunodeficiency was considered because of lymphopenia and the slow response of her lung disease to therapy with amphotericin B. Cellular immunity was found to be profoundly impaired, with absent delayed cutaneous hypersensitivity to several common antigens, a decreased count of thymus-dependent lymphocytes in the peripheral blood and a greatly diminished in-vitro proliferative response of lymphocytes to phytohemagglutinin (PHA). Humoral immunity was intact. Two additional types of therapy were assessed: subcutaneous injection of transfer factor was associated with an unsustained increase in lymphocyte counts and a positive cutaneous response to PHA but no clinical change; parenteral alimentation to ensure an adequate energy intake was associated with rapid clinical improvement, the development of delayed hypersensitivity to four additional antigens, and the return of lymphocyte counts and proliferative response to normal. These findings suggest that increased energy intake rather than transfer factor therapy was responsible for the child's recovery, and they emphasize the importance of adequate nutrition in the maintenance of intact cellular immunity.
 ...
PMID:Cellular immunity and nutrition in refractory disseminated blastomycosis. 9 21

Pyelonephritis followed ureteral inoculation of bacteria in both infant and adult monkeys. Because of the frequency of reflux in infants this was done by bladder inoculation, although ureteral inoculation was necessary in adults. The longer duration of bacteriuria in infants may be attributable to a relative immunodeficiency.
 ...
PMID:Experimental pyelonephritis in the monkey. VI. Infection of infants versus adults. 10 79

The first part of the paper outlines the actual situation of the recent investigations on the interpretative problem of the immunological deficits combined with enzymatic deficits. ADA deficit associated with combined immunodeficiency is an autosomal recessive form. Low enzyme levels, which is produced by a structural gene located on chromosome 20, were detected in chronic lymphatic leukemias where ADA decrease is correlated to low levels of T lymphocytes and the prevalence of an atypical B lymphocyte population. Particularly high levels of ADA were detected in acute lymphoblastic leukemias, in transplant rejection, in blastic crisis of chronic myeloid leukemia... NP deficit is associated with a T branch immunodeficiency, with high levels of inosine, guanosine, hypouricemia, hypochromic microcythemia and hematopoietic tissue megaloblastosis. This enzyme, with trimeric structure, whose structural gene is located on chromosome 14, shows a cytoplasmic location, and its maximum activity is to be found in T lymphocytes separated by rosetting. IGPT deficit is to be held responsible for the neurological Lesch-Nyhan syndrome. This deficit is associated with a depression of B lymphocyte function evaluated as response to the mitogens (PWM, Protein A) or as specific immunoglobulins production. At last the Authors report some personal investigations performed on hemolysates and lymphocytes of subjects with impaired immunity, as well as on some children at birth to establish the correlation between ADA and NP behaviour and immunosurveillance. Lastly, the data on the variations of the enzymatic and immunological parameters of subjects with immunodeficiency associated to enzymopenia after red cell transfusion are reported.
 ...
PMID:[Immunodeficiencies and enzyme deficiencies]. 10 63


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>