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)

Peripheral blood T and non-T lymphoid cells were examined in 31 patients with a variety of primary immmunodeficiencies for their locomotor activity toward casein and endotoxin-activated serum. T cells from 1 and non-T cells from 2 of 7 patients with Bruton type agammaglobulinemia had poor locomotor activity in this system. Both T and non-T lymphoid cells from all patients with common variable immunodeficiency disease had normal locomotor response to casein and EAS. Heterogeneity in locomotor abnormaltiy of T and non-T cells toward casein and endotoxin-activated serum was observed in other immunodeficient patients. No direct correlation was observed between the proportion of cells in the several T cell subsets and the abnormalities of locomotion of T cells.
Thymus 1979 Sep
PMID:Subpopulations of human T lymphocytes. VIII. Locomotion of lymphocyte subpopulations in patients with primary immunodeficiency disorders. 31 30

Nude mice have poorly developed Peyer's patches with very small or no germinal centers and little lymphoid cell proliferation, and a marked decrease in the number of gut-IgA plasma cells. Thymus grafts, which restore the T lymphocyte population of their lymphoid organs to nearly normal levels, lead to a considerable development of the Peyer's patches and of their germinal centers, assocaited with a considerable increase in gut IgA plasma cells, and in the serum IgA level. These findings are consistent with the postulated relationship between the Peyer's patches germinal center cells and the gut IgA plasma cells, and might help to explain the association of thymic defects, low serum IgA, and lack of intestinal IgA plasma cells observed in some immunodeficiency syndromes of man. Nude mice also have marked decrease in the number of lymphocytes present within the intestinal epithelium. These intraepithelial lymphocytes lymphocytes, which have been shown to be of T nature, are restored to normal numbers after thymus grafting.
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PMID:Peyer's patches, gut IgA plasma cells and thymic function: study in nude mice bearing thymic grafts. 80 33

In vitro infectivity of the MT4 lymphoid cell line with human immunodeficiency virus (HIV) has been studied in correlation with the degree of expression of the CD4 molecule at the cell surface. To modulate this CD4 expression in vitro, pre-incubation with phorbol myristate acetate (PMA) was used. The lowest CD4 expression was obtained after 1 to 5 hours. Thereafter, a partial re-expression of OKT4 was observed, e.g., when the incubation time with PMA was extended to 20 hours. Reverse transcriptase (RT) activity decreased and was delayed proportionally to the length of incubation of cells with PMA. This observation was confirmed by the comparable variation of cytopathic effects and of p24 antigen release in culture supernatants. The decrease in HIV infectivity hence correlated with that of OKT4 expression when PMA treatment did not exceed a few hours. By contrast, after extended treatment, infectivity remained decreased although OKT4 expression reappeared.
Thymus 1992 Dec
PMID:Phorbol ester induces down-regulation of CD4 molecule expression and resistance to in vitro infection by HIV1. 128 70

Thymus hyperplasia may be congenital (thymomegaly) and acquired (acquired thymomegaly, "tumour-like" hyperplasia, lymphofollicular hyperplasia). Congenital thymomegaly is represented by variants with hypo- and hyperfunction of an adequately formed thymus and results from the congenital neuro-endocrine disturbances. Thymomegaly with a thymus hypofunction is a marker of the immunodeficiency. Acquired thymomegaly is also represented by two variants with hypo- and hyper-function of the thymus and is in fact a "phenocopy" of the congenital one. "Tumor-like" thymus hyperplasia is a rare form of its pathology with a thymic weight reaching 800 g thus resulting in a syndrome of the mediastinum organs compression. Lymphofollicular thymus hyperplasia can not be always considered to be a thymus pathology but it is characteristic of autoimmune and infectious-allergic diseases with lymphoid follicles localizing in the hyperplastic intralobular perivascular spaces. Thymus parenchyma changes depend on the main disease and play a main role in the development of thymus dysfunction, for example, in the autoimmune diseases. Formation of lymphoid follicles in the dilated perivascular spaces is a non-specific process.
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PMID:[Thymus hyperplasia: classification, problems of patho- and morphogenesis, importance in human pathology]. 179 75

Chicken anaemia agent (CAA) causes severe anaemia, loss of body weight, and hypoplasia of thymus at day 14 after inoculation of one-day-old chickens. Several reports have described an enhancement of concurrent infections with f.e. Marek's disease virus, Infectious Bursal Disease virus, and Reovirus. Immunohistochemical methods were used to describe the immunopathological lesions of the thymus that probably form the basis of the immunodeficiency caused by CAA. Monoclonal antibodies and antisera against leucocytes, T lymphocytes, CD4, B lymphocytes, mononuclear phagocytes, MHC class II, and keratin were used. At day 14 after inoculation, the thymic cortex was completely depleted of thymocytes, whereas the medulla was not. T-cell areas in the spleen also lacked T lymphocytes. In contrast the cortex still contained stromal cells with MHC class II molecules and keratin. At day 21, the cortex had completely regenerated and all clinical signs of CAA infection had disappeared. Labelling experiments with BrdU in 4-week-old control chickens demonstrated that 25% of the divided cells was detected in the medulla and 75% in the cortex. The tissue tropism of CAA may, apart from the preference for rapidly dividing cells, be directed by specific cell determinants.
Thymus 1989
PMID:Transient depletion of cortical thymocytes induced by chicken anaemia agent. 256 Feb 70

The successful development of fetal tissue transplantation has resulted in therapeutical solutions for patients with a variety of diseases. Fetal liver transplants as well as bone marrow transplants, can completely cure patients with severe combined immunodeficiency disease. These transplants can also be applied to treat other types of immunodeficiency, hemopathies, and inborn errors of metabolism, in association with immunosuppressive therapy. Despite complete HLA incompatibility between transplanted stem cells and host cells, functional activities of donor-derived T-lymphocytes are not restricted. In severe forms of Di George syndrome, immunological reconstitution can be obtained by fetal thymus transplantation. It is expected that, in the near future, pure stem cell transplants and gene transplants will develop and will provide remarkable solutions for the therapy of a large number of diseases.
Thymus 1987
PMID:Fetal tissue transplantation, bone marrow transplantation and prospective gene therapy in severe immunodeficiencies and enzyme deficiencies. 332 5

An infant with partial trisomy 22 syndrome and recurrent infections showed severe cellular immunodeficiency and serum IgG reduction. A marked increase of E-RFCs was observed after in vitro incubation of peripheral blood lymphocytes with thymostimulin (TS), a calf thymic extract. The treatment with TS resulted in reduction of infections, correction of cellular immunodeficiency and increase of IgG levels. Such a primary thymic hormone dependent immunodeficiency may be due to an altered development of branchial arches, commonly present in trisomy 22 syndrome. The same pathogenetic mechanism may account for thymus dysplasia observed in other chromosomal syndromes.
Thymus 1986
PMID:Thymic hormone dependent immunodeficiency in an infant with partial trisomy of chromosome 22. 379 28

Human T lymphocytes bear characteristic membrane antigens which allow for identification on these cells and their subpopulations with monoclonal antibody reagents directed against the specific cell-surface antigens. During a study of T lymphocyte subpopulations in a group of 41 infants from a high risk nursery, three of the seven black infants studied were found to be missing cells reactive with the monoclonal antibody OKT4 which identifies the helper-inducer subset of T cells. Immunological evaluation of these infants and study of their family members revealed that the OKT4 non-reactive lymphocytes reacted normally with another antibody, OKT4A, which also identifies the helper-inducer subset of T cells. The deficiency of the antigen recognized by the OKT4 antibody appeared not to reflect T cell immaturity and was not associated with obvious immunodeficiency. The OKT4 negative phenotype appeared to be transmitted in an autosomal recessive mode. Our studies suggest that heterozygosity for this phenotype is relatively common among the black population and that heterozygotes are not easily distinguishable from the random population on the basis of lymphocyte reactivity with the OKT4 monoclonal antibody.
Thymus 1985
PMID:Ethnic heterogeneity in the distribution of the OKT4 antigen on lymphocytes: studies in three black families. 393 38

Using a partially purified preparation, thymosin fraction 5, we have documented that thymosin can correct many of the immunological deficiencies resulting from the lack of thymosin function in animal models and in humans. Ongoing studies indicate that there is a family of biologically active peptides within fraction 5 that act on T-cell subpopulations to maintain normal immunological reactivity. Several of these peptides have been purified to homogeneity. Two peptides, thymosin alpha 1 and beta 4, have been sequenced and chemically synthesized. Thymosin fraction 5 has been used in most clinical trials reported to date, including children with immunodeficiency disease and patients with autoimmune diseases and cancer. Most recently, the National Cancer Institute has initiated a number of Phase I and Phase II clinical trials with thymosin fraction 5 and synthetic alpha 1 as part of a new Biological Response Modifier Program. Preliminary results from two of these studies look encouraging.
Thymus 1984
PMID:Thymosins: structure, function and therapeutic applications. 608 3

Transplantation of allogeneic thymus into thymic deficient individuals will restore T-cell function including ability to demonstrate alloreactivity. In allogeneically reconstituted athymic (nude) mice, alloreactivity as manifested by positive mixed leukocyte reactivity, cell mediated lympholysis and skin graft rejection is present for all alloantigens, save those of the thymus donor. Such reconstituted animals possess double tolerance, for self and for donor. Cultured thymic fragments have been used to correct the immunodeficiency of thymic deficient humans. In a patient with severe combined immunodeficiency who acquired T-killer activity after allogeneic cultured thymic fragment transplant, mixed leukocyte response to the thymus donor was nearly absent. Patients with cancer of the lung, although not profoundly T deficient, received cultured thymic fragments in a project designed to enhance their immunity. Diminished alloreactivity for donor cells was seen in 2 of 3 such patients.
Thymus 1982 May
PMID:Diminished reactivity to alloantigen following transplantation of cultured thymic fragments. 621 56


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