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Results for “Immunologic Deficiency Syndromes”

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At least 127 records · Page 7Linked to original sources
PubMed1981

[Oropharyngeal lymphoid tissue as a bursa-equivalent structure: embryological and clinical considerations].

If we examine with an ontogenetic viewpoint the tissues giving origin to both thymus and avian bursa of Fabricius, we can't ignore that they are primary lymphoid organs connected to areas of ecto-entodermic junctions. They have a strong development in the embryonic life while tending to regress in the adult. The analysis of the organogenesis clarifies this affirmation. In the human cephalic district we know of various ectoentodermic stratification areas: the branchial pockets and the Remake pharyngeal membrane. The oral-pharyngeal lymphoid tissue is histogenetically linked to the stem-cells of the marrow which migrate to occupy an initially ecto-entodermic milieu. Structural and ontogenetic analogies of embryological nature lead to an identification of the oral-pharyngeal lymphoid tissue in man with the avian bursa of Fabricius. Therefore we deem it very indicative that a humoral immunological deficiency syndrome appears in subjects with the atrophy of the Waldeyer ring; above all considering that an experimental model of the humoral immunological deficit has been reproduced in chicken surgically or hormonally bursectomized.

Animals↗
PubMed1986

Immunologic studies in homosexual and hemophiliac subjects with persistent generalized lymphadenopathy: a comparative analysis.

Hemophiliac patients receiving factor VIII replacement therapy and homosexual subjects are at risk for the acquired immune deficiency syndrome. Immunologic abnormalities, especially depletion of helper T cell populations, have been noted in members of these groups and may parallel their clinical state. We have evaluated the peripheral blood immunologic status of a group of hemophiliac and homosexual subjects with and without persistent generalized lymphadenopathy (PGL). Although individuals with PGL had more abnormal T-lymphocyte parameters, including lower helper/suppressor ratios, than group members without lymphadenopathy, there were no significant differences noted within each respective risk group. However, differences between hemophiliac and homosexual patient groups were noted. T-lymphocyte subpopulations of hemophiliac patients with lymphadenopathy were significantly more abnormal than the corresponding homosexual groups. All groups had decreased lymphocyte proliferative responses to mitogen. Homosexual subjects with lymphadenopathy exhibited the most profoundly depressed lymphocyte mitogenic responses. We conclude that, although abnormalities of T-lymphocyte subpopulation and lymphocyte mitogenic responses exist within each risk group, homosexual subjects (both asymptomatic and with PGL) had lower mitogenic responses than would be expected for their T-lymphocyte helper/suppressor ratios as compared to either control subjects or hemophiliac patients. These findings suggest that additional factors are operative in the mononuclear cell populations of homosexual subjects that depress lymphocyte function. An analysis of these factors may help explain the higher incidence of AIDS in homosexual subjects as compared to hemophiliac patients.

Acquired Immunodeficiency Syndrome↗
PubMed1995

Immunodiagnostic assays.

The immune system is a complex interactive network. Defects in its function can be characterized broadly as being the result of actual deficiencies in the network or misdirection of normal immunologic functions. The assays that are available to detect deficiencies in the immunologic network barely scrape the surface of the possibilities. These assays primarily evaluate humoral immune function, but undetected defects in innate and cellular immunity are sure to exist. Although assays of humoral immunity have allowed the characterization of a number of immunodeficiency syndromes in horses, closer evaluation of the equine immune system using newer assays described in this text, as well as future assays yet to be developed are sure to determine new syndromes. Assays of misdirected immunologic functions have been limited to detection of misdirected antibody responses, but the dependence of antibody production on help from T cells could reflect an underlying defect of cellular immunity. Similar to immunodeficiency syndromes, misdirected responses of the innate and adaptive arms of immunity are sure to occur but will only be detected by more diligent surveillance of diseased horses and application of new immunodiagnostic technologies.

Animals↗
PubMed1987

Immunological changes in beta-thalassemia major patients positive for HTLV-III antibodies.

Antibodies to HTLV-III were found in 6 of 206 Sicilian patients with thalassemia major, aged 1 to 30 years. None of the patients showed clinical signs related to immuno-deficiency syndromes. Immunological investigations carried out in all positive patients showed alterations similar to those found in thalassemic patients without antibodies to HTLV-III. These immunological changes could not depend on the HTLV-III infection, but could be related to the thalassemic condition.

Adolescent↗
PubMed1989

Characterization of a helper T lymphocyte defect in vitamin A-deficient mice.

Vitamin A-deficient mice exhibited sharply decreased IgG1 responses to protein Ag in vivo and in vitro. We traced the cellular basis for these impaired responses to a functional defect in A-deficient Th. Ag-presenting cells and B cells from A-deficient mice showed no functional defects. The A-deficient T cells proliferated normally in response to Ag but failed to provide the B cell stimulus for Ag-specific IgG1 responses. The A-deficient mice had fewer Th by limiting dilution analysis, and retinyl acetate supplementation in vitro restored Th frequency to control levels. Because added retinyl acetate reversed the Th functional block, these vitamin A-deficient T cells must be primarily defective in activation, not in establishment of immunologic memory.

Animals↗
PubMed1993

[Laboratory diagnosis of immune deficiency].

Highly specialized, state-of-the-art diagnostic tests are available for identifying congenital and acquired immune defects. These methods should only be resorted to when less complicated means have created suspicion of an immune defect. The case history, including the family history, represents the core of the diagnostic procedure. Initially, only simple clinical investigations are indicated. These should enable the physician to exclude or delimit a defect in the immune system which then can be defined more closely by specific tests. Screening includes clinical chemistry (erythrocyte sedimentation rate, total serum protein, serum electrophoresis, C-reactive protein, blood count including differential blood count, ferritin, urine analysis, and a quantitative assay of the immunoglobulins A, G and M), bacteriological, serological, and radiological investigations, and finally skin tests with recall antigens. Thereby, it is usually possible to reliably detect primary B cell defects with humoral antibody deficiency syndromes. Lymphocyte subset counts, immunoelectrophoresis, and bone marrow biopsy are necessary for the differential diagnosis, or for the confirmation, of malignant lymphatic proliferation, especially in adults. IgG subclass defects as well as granulocyte dysfunction and complement defects must be excluded in patients who are susceptible to bacterial infection despite normal immunoglobulin concentrations. In suspected cases of primary or secondary (HIV, cytomegalovirus, Epstein-Barr virus) T cell defects, lymphocyte subset counts and, where applicable, T cell function tests are indicated. The majority of secondary immunodeficiency syndromes, in which the primary disease is known, do not currently require specialized diagnosis. Nevertheless, monitoring of the lymphocyte subsets in HIV-positive patients has already become standard practice in health care (for evaluating the prognosis and deciding on the therapy).

Diagnosis, Differential↗
PubMed1979

Congenital ichthyosis: concurrent immunodeficiency and atypical T cells.

A patient with congenital ichthyosis and progressive neurologic anomalies showed disturbances in the specific humoral and cellular defense as well as the presence of atypical lymphoid cells in skin and lymph node. The latter resembled the atypical T cells found in mycosis fungoides and Sézary syndrome. The possibility of the presence of either a cutaneous T cell lymphoma or unregulated T cell stimulation leading to concurrent immunodeficiency in this patient is discussed.

Decerebrate State↗
PubMed1982

Treatment of chronic mucocutaneous candidiasis with 5-fluorocytosine.

A four-year-old girl with chronic mucocutaneous candidiasis (CMC) and an immunologic deficiency syndrome was studied in detail over a period of two years. Her lymphocytes neither produced migratory inhibitory factor nor responded consistently with an increased 3H-thymidine uptake when placed in tissue culture medium with candida antigen. The serum IgE was increased. No autoantibody was demonstrated in the patient's serum and she did not have nay kind of endocrine disorder. Eradication of the candida infection with 5-fluorocytosine, although resulting in marked clinical improvement, did not alter the immunologic abnormalities.

Antibody Formation↗
PubMed1997

The role of Jak3 in lymphoid development, activation, and signaling.

Mutations in a number of lymphoid signaling molecules lead to immunodeficiencies in mice and humans. Among these, one very pleiotropic syndrome results from deficiencies in an array of cytokine signaling pathways utilizing a cytokine receptor common gamma chain, gammac, and the tyrosine kinase Jak3. Recent advances in our understanding of the role of gammac and Jak3 in lymphocyte development and function highlight the importance of cytokine receptor signaling pathways in regulating lymphoid homeostasis and responsiveness.

Animals↗
PubMed1975

The recognition and classification of immunodeficiency diseases with bacteriophage phiChi 174.

A standard antigen to assess humoral responses is needed for the immunodeficiency syndromes. Bacteriophage phiChi 174 is the best available antigen. Large, standardized batches can be prepared, stored and distributed; samples for assay can be transported by mail. We have systematically studied the immune response to this antigen in over 200 persons, including 49 immunodeficiency patients. Phage provides consistent delineation of humoral responses in primary immunodeficiency diseases.

Antibodies, Viral↗
PubMed2004

An imbalance of naive and memory/effector subsets and altered expression of CD38 on T lymphocytes in two girls with hyper-IgM syndrome.

In this report we evaluated CD4(+) T, CD8(+) T and natural killer (NK) cell counts, the levels of naive/memory subsets within the CD4(+) T lymphocyte population, expression of CD38 on T lymphocytes, and CD4(+) and CD8(+) T cell cytokine production in two girls with hyper-IgM (HIM) syndrome. Both girls developed recurrent infections early in infancy, presenting a wide spectrum of clinical manifestations, with a strikingly different disease severity between them. CD4(+) T cell counts were low in both children (patient 1: 214 cells/mm(3) and patient 2: 392 cells/mm(3)), and the CD4/CD8 T cell ratio was 0.4 for patient 1, the patient with the more severe disease, and 1.4 for patient 2. NK cell numbers were low in patient 1 (60 cells/mm(3)) and borderline (286 cells/mm(3)) with regard to normal levels in patient 2. An imbalance of naive and memory/effector cell subsets was found in both girls, with the percentage of CD45RA(+) 27(+) (naive) CD4(+) T lymphocytes being 5.8 and 12.4 for patients 1 and 2, respectively. Expression of CD38 on the surface of T lymphocytes was low in patient 1. Detection of intracellular interferon (IFN)-gamma and tumour necrosis factor (TNF)-alpha in CD4(+) and CD8(+) T lymphocytes upon PMA-Io stimulus was preserved in both children. In conclusion, we found low numbers of CD4(+) T lymphocytes and a dramatic redistribution of naive and memory/effector CD4(+) T lymphocytes in two girls with non-X-linked HIM syndrome. Furthermore, we found low expression of CD38 on T lymphocytes and low numbers of NK cells in the patient with the more severe disease, indicating a possible role for these cells in the pathogenesis of this immunodeficiency.

ADP-ribosyl Cyclase↗
PubMed1992

[Alterations in lymphocyte subsets in variable immunodeficiency syndrome].

Besides functional defects, different phenotypical alterations of lymphocytes have been described in patients with CVID. A decrease in LAM-1- (= Leu8) and CD21 expression was observed on B cells. Some patients with CVID show alterations in their T-cell subpopulations: The most striking features of this subgroup are low CD4/CD8-ratio, an increase in CD57/CD8 double-positive lymphocytes, and a strong enhancement of HLA-DR and "memory" markers such as CD29 and LFA-3 on T cells.

B-Lymphocytes↗
PubMed1975

The pituitary dwarf mouse: a model for study of endocrine immunodeficiency disease.

Pituitary dwarf mutants of the Snell-Bagg and Ames mouse strains develop severe immunodeficiency of the thymus-dependent system which frequently leads to a fatal wasting syndrome. This immunodeficiency is a consequence of defective pituitary influences which will cause 1) an inadequate production of immunocompetent cells due to a central developmental defect primarily affecting the thymus and 2) the inability of immunocompetent cells to undergo a rapid and efficient antigen-induced proliferation and differentiation into antibody-forming cells. The relevance of the dwarf mouse model to a possible association between human immunodeficiency and endocrine disease is briefly discussed.

Adrenocorticotropic Hormone↗