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Biomedical subjects

R H Buckley

Publications and source records attributed to R H Buckley.

At least 145 records · Page 8Linked to original sources
PubMed1976

Heterogeneity of lymphocyte subpopulations in severe combined immunodeficiency. Evidence against a stem cell defect.

Surface markers typical of T and B lymphocytes were present on varying proportions of peripheral blood lymphocytes from three infants with severe combined immunodeficiency disease. Despite this, functions mediated by T and B cells were either absent or very minimal in all three, including cell-mediated responses in vivo; the in vitro proliferative response to mitogens, allogeneic cells, or antigens; effector cell function in lymphocyte-antibody lymphocytolytic interaction assays; and in vitro synthesis of IgG, IgA, and IgM. In contrast, mononuclear cells from one of the infants were tested and found capable of lysing both human and chicken antibody-coated erythrocyte targets normally. Co-cultivation experiments with unrelated normal control lymphocytes failed to demonstrate suppressor cell activity for immunoglobulin synthesis in these infants. Augmentations of immunoglobulin production from 310 to 560% over that expected on the basis of individual culture data were noted in co-cultures of one of the infants' cells with two different unrelated normal control cells. These findings suggest that that infant may have had a T helper cell defect or that his T cells were unable to produce soluble factors necessary for B cell differentiation. The finding of cells with differentiation markers characteristic of T and B lymphocytes in each of these patients, though in variable quantities, is further evidence for heterogeneity among patients with the clinical syndrome of severe combined immunodeficiency and argues against the concept that their immunodeficiency was due to a stem cell defect.

Adenosine Deaminase↗
PubMed1975

Enhanced bactericidal activity of phagocytes from patients with chronic granulomatous disease in the presence of sulphisoxazole.

The decrease in the frequency and severity of bacterial infections in four of five children with chronic granulomatous disease (C.G.D.) on long-term sulphonamide therapy was out of proportion to demonstrable direct antibacterial effects of the drug. In an attempt to determine the mechanism for this apparent protective effect, the killing of sulphisoxazole-resistant Escherichia coli and Staphylococcus aureus by leucocytes from five patients with C.G.D. was studied in the presence or absence of 5-50 mug. per ml. sulphisoxazole. With leucocytes from all five patients there was a modest but reproducible enhancement of bactericidal activity in the presence of the sulphonamide. Studies of phagocytosis-associated oxidative metabolism in patients' cell have not revealed a metabolic basis for improved killing.

Blood Bactericidal Activity↗
PubMed1975

Depression of cell-mediated immunity in atopic eczema.

Prompted by recent observations that the thymus exerts an important regulatory influence over IgE antibody production in lower species, we conducted studies of immune function in 21 patients with atopic eczema to seek evidence for a similar relation in man. Skin tests for delayed hypersensitivity to Candida albicans and streptokinase-streptodornase (SK-SD) revealed a striking degree of anergy that correlated with the severity of the eczema. A correlation was also noted between the extent of the dermatitis and the magnitude of the serum IgE concentration. Other immunologic abnormalities did not appear related to the severity of eczema put pertained to the group as a whole. These included significantly (p = less than 0.0001) lower mean percentages of spontaneous sheep erythrocyte (E) or T cell rosettes and of rosettes with neuraminidase-treated sheep erythrocyte (En) rosettes, and significantly lower in vitro lymphocyte responsiveness of the mitogens concanavalin A (p = 0.0013) and poleweek mitogen (p = 0.0002) and to Candida antigen (p = 0.0017) than in normal subjects. Responses to phytohemagglutinin and tetanus toxoid were also depressed but differences were not statistically significant. An increased percentage (p = 0.0324) of peripheral blood B lymphocytes bearing the complement receptor was noted, but, except for a slight increase in lymphocytes bearing IgD, percentages of lymphocytes bearing other immunoglobulins (including IgE) were not elevated.

Adolescent↗
PubMed1975

Serum IgD and IgE concentrations in immunodeficiency diseases.

Concentrations of IgD and IgE were measured in sera from 165 patients with well-defined immunodeficiency in an effort to find information possibly relevant to the roles of antibodies of these classes in host defense. Values for both immunoglobulins were generally quite low in patients who had marked deficiencies of all three major immunoglobulins, although occasional normal or high normal values for IgD were seen in hypogammaglobulinemic patients. Group mean IgD concentrations were also depressed in patients with Wiskott-Aldrich syndrome and in those with selective IgA deficiency; IgE concentrations were depressed in patients with X-linked immunodeficiency with hyper-IgM and in those with ataxia telangiectasia. IgD and IgE were both significantly elevated in patients with extreme hyperimmunoglobulinemia E and undue susceptibility to infection and in a patient with the Nezelof syndrome; none of these patients had histories suggestive of atopy. In addition, the mean IgE concentration was significantly elevated in patients with selective IgA deficiency, many of whom were atopic, and in those with the Wiskott-Aldrich syndrome. The highest IgD concentration (163 mg/100 ml) was found in serum from a boy with variable immunodeficiency who had a lifelong history of severe recurrent pharyngeal infections, primarily streptococcal in etiology. Recurrent staphylococcal infection was a feature common to many but not all patients with elevated serum IgE concentration. These data may prove useful in the future delineation of biologic roles for antibodies in these two immunoglobulin classes.

Adolescent↗
PubMed1975

Bone marrow and thymus transplantation in ataxia-telangiectasia.

Serum IgA concentrations were found to be normal in a boy with ataxia-telangiectasia and selective IgA deficiency several months after an infusion of bone marrow cells from an HL-A and MLR compatible normal sib. In addition, transient improvements were noted in both his in vivo and in vitro cellular immune responsiveness through the third month posttransplant. Despite this, no evidence of chimerism could be detected in karyotypic studies of his peripheral blood lymphocytes and bone marrow cells. No clinical or significant immunologic improvement was noted in his affected female sib following implantation of a fetal thymus or injection of transfer factor.

Ataxia Telangiectasia↗
PubMed1975

Clinical and immunologic features of selective IgA deficiency.

Selective absence of serum and secretory IgA is probably the most common form of human immunodeficiency. High frequencies of recurrent sinusitis, otitis media, pneumonia, and atopy were noted among a group of 75 such patients, all but 4 of whom were Caucasian. Seven instances of familial absence of IgA were detected among 106 relatives of 34 of the group; in 1 family 1 member from each of 3 successive generations was affected. Two IgA-deficient children were later found to have normal amounts of serum IgA. Despite their humoral deficit, B lymphocytes bearing surface IgA were detected in 9/9 IgA-deficient patients in immunofluorescence studies of their peripheral blood lymphocytes. Although in vitro lymphocyte responses to 2 putative T-cell mitogens and to allogenic cells were normal, results of spontaneous rosette formation studies with sheep erythrocytes raise the possibility of a lymphocyte subpopulation deficit in this condition.

Absorption↗