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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Antibody-dependent cellular cytotoxicity (ADCC), has been shown to be independent in vitro of thymus-derived lymphocytes, but the precise nature of the effector lymphocyte has not been fully clarified. To further study the identity of the ADCC effector cell type(s), peripheral blood leukocytes were purified by Ficoll-Hypaque density centrifugation and fractionated into surface immunoglobulin-positive [Ig(+)] and surface immunoglobulin-negative [Ig(-)] populations by chromatographic separation on Sephadex G-200 anti-human immunoglobulin columns. After column fractionations, the ADCC effector activity against antibody-coated autologous lymphocytes was predominantly and consistently found in the Ig(-) fraction. This latter population was then further fractionated, by rosetting techniques, into two subpopulations, The first was depleted by lymphocytes with surface receptors for sheep red blood cells [E(+)]and the second was depleted of lymphocytes with receptors for sheep red blood cell-antibody-complement [EAC-(+)]. Analysis of these populations showed that ADCC effector activity was predominantly a property of the Ig(-) lmyphocytes which are E(-) but EAC(+). These lymphocytes have been referred to as "null lymphocytes" and probably represent a subset of bone marrow-derived (B) cells. In addition, variable and low levels of ADCC activity were observed in some Ig(+) populations (B cells). Further purification of the null cell population by filtration over nylon wool columns to reduce the number of contaminating latex ingesting monocytes did not reduce ADCC effector activity. Isolated null cell ADCC effector activity was inhibited by either rabbit anti-human F(ab)2 or normal pooled rabbit gamma globulin, but not by rabbit F(ab)2 anti-human F)ab)2 or media. This supports the contention previously suggested in studies using unfractionated lymphocyte populations that the ADCC effector cell recognizes the Fc portion of the antibody molecule. The variable and low level of activity noted in the Ig(+) populations is unexplained but possibly due to a variable population of null cell-derived Ig(+) lymphocytes within the whole Ig(+) population. In conclusion, these experiments demonstrate that, in vitro, the major ADCC effector activity of circulating human peripheral blood lymphocytes resides in the Ig(-), E(-), EAC-(+) subpopulation termed "null cells." Since it has been noted that in certain disease states, such as immunodeficiency syndromes, autoimmune disorders, and neoplasms, the percentage of this population of lymphocytes in the peripheral blood is elevated, it is speculated that these cells, perhaps through their ADCC function, may play an important pathophysiologic role in these diseases.
J Clin Invest 1975 Dec
PMID:Human antibody-dependent cellular cytotoxicity. Isolation and identification of a subpopulation of peripheral blood lymphocytes which kill antibody-coated autologous target cells. 5 42

Intestinal immune responses are adapted to function at external mucosal surfaces. Specialized forms of antibody, secretory immunoglobulin A (IgA) and immunoglobulin M (IgM), provid humoral immunity but little is known of local cell mediated immune reactions. Antigens in the intestinal lumen gain preferential access via Peyer's patches in which sensitised lymphocytes proliferate before entering the lymphatic system. These lymphoblasts return to the intestinal mucosa via the bloodstream to provide predominantly IgA antibody responses. Secretory IgA antibody can neutralize viruses, bacteria and toxins, and appears to block the entry of some food antigens into the lamina propria. Disturbances of intestinal immunity may result in malabsorption. Immunodeficiency states are often associated with malabsorption due to Giardia lamblia infestation. In alpha chain disease there is a malignant expansion of plasma cells in the intestinal mucosa which secrete an abnormal heavy chain fragment of IgA. Arthus type hypersensitivity reactions to milk proteins and gluten may contribute to the mucosal injury in patients suffering from milk allergy and coeliac disease.
Am J Med 1979 Dec
PMID:An overview of intestinal immunity and malabsorption. 11 6

Atopic dermatitis is a recognizable phenotype that most likely results from several mechanisms. An increasing number of children with atopic dermatitis are recognized as having immune defects, although the exact incidence of such immunodeficiency states among patients with atopic dermatitis is unknown. Children with atopic dermatitis who suffer recurrent or persistent infections should undergo immunologic evaluation. Ideally, this should include evaluation of cell-mediated immunity as well as neutrophil and monocyte chemotaxis. Further investigation into the beta-blockade theory may enhance our understanding of atopic dermatitis. Careful attention to factors exacerbating atopic dermatitis is essential in understanding this problem. Abnormal sweating, dry skin, sensitivity to contactants, and emotional stress should always be considered in evaluation of children with atopic dermatitis.
Pediatr Ann 1976 Dec
PMID:Atopic dermatitis: etiology and pathogenesis. 18 47

An adult with the late onset immunodeficiency syndrome developed intractable diarrhea. Widespread cytomegalovirus (CMV) infection of the gastrointestinal tract was detected antemortem with detailed morphological studies and viral culture. The CMV-type cells were especially numerous in his severely ulcerated colon. Electron microscopy of infected cells in rectal biopsy material revealed the characteristic features of CMV infection. It is likely that the CMV infection contributed to the symptom complex and the mucosal injury. Unusual opportunistic infections as a cause of diarrhea should be considered in patients with late onset immunodefociency, especially if Giardiasis is ruled out.
Gastroenterology 1977 Dec
PMID:Cytomegalovirus infection of the gastrointestinal tract in a patient with late onset immunodeficiency syndrome. 19 23

This report presents the findings of a study of a 17-year-old male with a selective immunodeficiency to the Epstein-Barr virus, who died of a malignant lymphoma following clinical infectious mononucleosis. Autopsy findings and immunohistochemical techniques demonstrated a malignant lymphoma with B-lymphocyte characteristics which primarily involved the central nervous system (CNS). The relationship of the Epstein-Barr virus to lymphoproliferation is discussed.
Acta Neuropathol 1979 Dec
PMID:Selective immunodeficiency and malignant lymphoma of the central nervous system. Possible relationship to the Epstein-Barr virus. 23 Jun 88

Inherited deficiencies of adenosine deaminase and purine nucleoside phosphorylase have been found to be associated with certain immunodeficiency syndromes which are characterized by deficiencies of mature peripheral lymphocytes. The immunodeficiency states associated with these enzyme deficiencies are thought to arise from blocks in lymphocyte differentiation. Deficiencies of these enzymes have profound and apparently selective effects on lymphocyte differentiation. Their discovery has focused attention on previously unknown relationships between purine nucleotide metabolism and lymphocyte development and function. In this article three aspects of nucleotide-metabolizing enzymes and lymphocyte differentiation will be discussed: 1) the distribution of the enzymes among lymphocyte populations at differing stages of differentiation; 2) the possible biochemical mechanisms which give rise to the immunodeficiencies; 3) the stages of lymphocyte differentiation which are affected by the enzyme deficiencies.
Mol Cell Biochem 1979 Dec 14
PMID:Nucleotide-metabolizing enzymes and lymphocyte differentiation. 23 Nov 99

The eldest brother in a sibship of five children died of acute myelogenous leukemia at 10 years of age. The second and third eldest brothers died of hypoplastic anemia at ages five and nine years, respectively. A surviving 6 year old brother, the proband of the study, has abnormalities that suggest a preleukemic state: mild pancytopenia, platelet dysfunction, immunodeficiency, and bone marrow hypoplasia with approximately 18 per cent blast forms. His 17 year old sister has a mild normochromic normocytic anemia. Cytogenetic studies revealed C-group monosomy in the bone marrows of the proband and the third brother (45, XY, -C); band studies demonstrated that a No. 8 chromosome was missing in the proband (45, XY, -8). At least four of the siblings and their father had cerebellar ataxia, and evidence of a small cerebellum at autopsy examination or by computerized axial tomography. The disorder in this family has major features of two autosomal recessive preleukemic diseases, ataxia-telangiectasia and Fanconi's anemia. However, these and other inherited conditions were excluded by clinical or laboratory criteria, and no environmental causes of the familial disorder were found. The constellation of abnormalities in the family may constitute a new genetic syndrome.
Am J Med 1978 Dec
PMID:A family with acute leukemia, hypoplastic anemia and cerebellar ataxia: association with bone marrow C-monosomy. 28 89

The presence of extra reactions in the HLA typing of a combined immunodeficiency patient may be attributed to B-cell alloantibodies in the HLA typing sera. The presence of these reactions can be used to identify HLA sera containing B-cell antibodies for further B-cell studies. The alloantibodies in this study have some association with the HLA-D determinants and segregate with HLA in normal families.
Transplant Proc 1977 Dec
PMID:Previously unexplained HLA antigens of combined immunodeficiency disease due to Ia alloantigens. 30 19

Pneumocystis carinii pneumonitis is a diffuse bilateral alveolopathy encountered in the immunocompromised host with cancer, a congenital immune deficiency disorder, an organ transplant, severe protein-energy malnutrition or recipients of immunosuppressive therapy for other conditions. The onset is abrupt with fever and tachypnea. No rales are heard and the roentgenogram reveals a diffuse alveolar disease. Once the pneumonitis is evident, the infection is usually fatal if no treatment is given. The diagnosis is best established by the demonstration of the causative organism in specimens obtained by open lung biopsy, or other invasive methods, and stained with Gomori's methenamine silver nitrate, toluidine blue O or polychrome stains. Of the two drugs available for treatment, trimethoprim-sulfamethoxazole is preferred over pentamidine isethionate because of relative difference in adverse effects. With either drug the recovery rate is about 75%. The infection can be prevented in high risk patients by the administration of trimethoprim-sulfamethoxazole prophylactically.
Johns Hopkins Med J 1978 Dec
PMID:Pneumocystis pneumonia: a plague of the immunosuppressed. 30 68

In addition to the x-linked B cell maturation deficit previously reported in CBA/N mice, a functional T cell defect has now been observed. T lymphocyte regulation of the polyclonal PFC response was studied within the context of this x-linked immunodeficiency model. The ability of 1) B cells from (CBA X CBA/CaJ)F1, male mice to respond to nonspecific T cell helper signals and 2) T cells from NCF1 male mice to provide such signals was investigated under in vitro conditions by using bacterial lipopolysaccharide (LPS) as the polyclonal activator. B lymphocytes from both male and female NCF1 mice were receptive to T cell help rendered by NCF1 female T cells. Male T cells. however, were unable to augment polyclonal B cell responses of either NCF1 male or female B cells to LPS. Treatment with ATS + C reduced the polyclonal response of female but not male spleen cells to LPS. This deficit could not be overcome by the use of greater numbers of NCF1 male T cells. The observation that this deficiency in T cell regulation is not due to active suppression suggests that the results may be attributable to an intrinsic T cell defect.
J Immunol 1979 Dec
PMID:T cell regulation of polyclonal B cell responsiveness. II. Evidence for a deficit in T cell function in mice with an X-linked B lymphocyte defect. 31 24


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