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Results for “Immunologic Deficiency Syndromes”

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At least 19 recordsLinked to original sources

[Primary immunologic deficiency syndromes in man].

Current concepts on the immune system, the genesis of human T- and B-lymphocytes, and their functional properties are presented as well as current data on the pathogenesis and classification of hereditary defects of the human immune system. The main forms of immunity deficiency conditions are described. The methods for diagnosis and treatment of different forms of immunologic deficiency are substantiated. The relationship between immunity deficiency conditions and malignancies, allergic and autoimmune processes, and morphological changes in various organs and tissues is described.

B-Lymphocytes

[Natural antibodies (isohemagglutinins, heterophilic, antierythrocytic and antibacterial) in children with congenital immunologic deficiency syndromes].

Examination of 30 patients with immunodeficient diseases showed that the system of natural antibodies was considerably changed depending on the form and the extent of deficiency of the immune system: in agammaglobuinemia the antibodies under study were almost completely absent, and in immunological insufficiency with ataxia-teleangiectasis the production of antibacterial antibodies proved to be sharply decreased. The data obtained can be used both for the diagnosis of immunodeficiencies and for control of the restoration of the immunological competence after a number of therapeutic measures, particularly after the transplantation of the thymus-sternum complex.

Adolescent

Immunodeficiency in familial erythrophagocytic lymphohistiocytosis.

4 children with familial erythrophagocytic lymphohistiocytosis and hyperlipidaemia were found to have a previously unrecognised immunological deficiency syndrome which included defects in both humoral and cellular immunity and a plasma inhibitor of in-vitro lymphocyte blastogenesis. The inhibitory activity was proportional to the increase in the triglyceride concentration in the patients' plasma. Immunological deficiency, to which hyperlipidaemia may be a contributing factor, appears to be a significant feature of familial erythrophagocytic lymphohistiocytosis.

Antibody-Dependent Cell Cytotoxicity

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

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