Biomedical subjects
R H Buckley
Publications and source records attributed to R H Buckley.
Developmental immunology and the immunodeficiency diseases.
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Long term use of intravenous immune globulin in patients with primary immunodeficiency diseases: inadequacy of current dosage practices and approaches to the problem.
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Use of intravenous gamma-globulin in antibody immunodeficiency: results of a multicenter controlled trial.
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Symptomatic giardiasis in three patients with X-linked agammaglobulinemia.
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Monoclonal immunoglobulin-secreting lymphoma in a patient with severe combined immunodeficiency disease.
A 3.5 year old boy with X-linked severe combined immunodeficiency disease (SCID), who had been in laminar flow isolation throughout his life, developed a B cell tumour producing up to 3008 mg/dl of an IgM kappa paraprotein 1 month after infusion of both liver and thymus cells from a fetal donor and 6 months after the last of six fetal liver cell infusions given over a 3 year period. Pretransplant studies revealed a high percentage of circulating B lymphocytes. HLA typing suggests that the tumour was of host origin.
Abnormalities in the regulation of human IgE synthesis.
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Immunologic studies before and after splenectomy in a patient with the Wiskott-Aldrich syndrome.
Sequential studies of cellular and humoral immunity were conducted in an infant with the Wiskott-Aldrich syndrome prior to and after a splenectomy for uncontrollable hemorrhage. All measures of cellular immunity showed gradual improvement during the 8-month period after surgery. Serum isohemagglutinins, diphtheria and tetanus antibodies, and the percentage of immunoglobulin-bearing B cells did not change significantly from presplenectomy values. The serum IgE concentration declined from a high of 10,800 IU/ml at 1 month postsplenectomy to a low of 860 IU/ml at 5 months after surgery and the IgG concentration gradually decreased from a high of 1880 mg/dl presplenectomy to a low of 620 mg/dl 8 months later. The platelet count ranged from 64,000 to 206,000/mm3 for the first 6 months after splenectomy. It decreased precipitously 6.5 months after the operation; at the same time there was a marked rise in platelet-bound IgG antibody (PB-IgG). The PB-IgG declined rapidly following vincristine therapy and, after another rise, declined more gradually following steroid therapy.
Fungal infection in chronic granulomatous disease. The importance of the phagocyte in defense against fungi.
Among 245 cases of chronic granulomatous disease which were evaluated, fungal infection occurred in 20.4 percent. Fungi encountered include Aspergillus, Torulopsis and Candida. In 18 percent of the patients with fungal infection, the disease was limited to soft tissue or bone; all did well. Most of the patients had fungal pneumonia and/or widely disseminated disease; diagnosis was usually confirmed by open lung biopsy. Patients with pneumonia or disseminated disease who received no therapy succumbed to infection, whereas more than half the patients who received antifungal therapy were cured. Modalities of treatment included antifungal chemotherapy, surgical removal of infected tissue and granulocyte transfusion. Although several patients showed dramatic improvement during granulocyte transfusions given in combination with antifungal chemotherapy, the improvement achieved was not statistically significant when compared with that achieved with chemotherapy alone. These results emphasized the importance of phagocytic cells in defense against fungi and the need for further evaluation of granulocyte transfusion therapy in compromised hosts in whom fungal infections develop.
Elevated IgA concentration in milk produced by mothers delivered of preterm infants.
Concentrations of immunoglobulins G, M, and A were measured by double-antibody radioimmunoassay in morning milk samples collected during the first month postpartum from 35 mothers delivered of preterm infants and 14 mothers delivered of term infants. Mean concentrations of IgG (1.8, to 2.8 mg/gm protein) and IgM (2.8 to 11.7 mg/gm protein) were similar in milk from both groups of mothers. In contrast, IgA was present in significantly higher concentrations throughout the first month postpartum in milk from mothers delivered of preterm infants than in milk from those giving birth at term (P less than 0.01). To determine the effect of milk flow on IgA concentration, IgA was also measured in complete 24-hour milk collections; milk from mothers with preterm deliveries again contained significantly higher concentrations of IgA than milk from mothers with term deliveries (P less than 0.01). This higher IgA concentration was not secondary to method of milk expression. The concentration of IgA was found, however, to vary inversely with milk volume (P less than 0.01). Although mean values of milk volumes for the groups were not statistically different, the overall lower volumes of milk produced by mothers giving birth preterm resulted in comparable total IgA production per 24 hours. There were no differences in serum IgA concentrations of preterm infants fed their own mother's milk and comparable infants fed a cow milk formula, suggesting that IgA in milk is not absorbed from the intestine in significant amounts.
Colonic nodular lymphoid hyperplasia in a child with antibody deficiency and near-normal immunoglobulins.
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Regulation of IgE synthesis: introduction.
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In vitro studies of IgE synthesis by human blood mononuclear cells.
IgE was detected in increasing quantities in supernatants of cultured human blood mononuclear cells, with peak amounts being found in 7 to 12-day cultures. Inhibitors of protein synthesis or of cell division reduced IgE production. Significantly greater amounts of IgE were made by cells from patients with elevated serum IgE than by those from normal adults. Pokeweed mitogen caused modest (threefold) augmentation of IgE synthesis is normal control cultures but inhibited it in cultures from patients with elevated serum IgE. Co-culturing unfractionated mononuclear cells from some atopic patients with cells from normal people resulted in significant suppression of the expected amount of IgE synthesis but not of IgM or IgG production. This suggests that a suppressor cell population or function is present in normal adults that regulates B cell IgE synthesis and that patients with elevated IgE may be deficient in this function. Results of coculture studies with isolated T and B cell-enriched subpopulations support the possibility that such activity exists in normal T cell populations. The marked augmentation of IgE synthesis by patients' B cells when separated from their own T cells indicates that patients' T cells have some regulatory capacity, but control T cells were more potent in this regard.
Human IgE synthesis in vitro: a reassessment.
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Safety and patient acceptability of intravenous immune globulin in 10% maltose.
The safety and patient acceptance of two preparations of modified (reduced and alkylated) immune globulin for intravenous use were evaluated; one preparation was formulated as a 5% solution in 10% maltose (IGIV-maltose), the other did not contain maltose (IGIV). In this double-blind trial each of 29 immunodeficient patients received three consecutive monthly infusions (100 or 150 mg/kg immune globulin) of one preparation before being crossed over to the other. Only 3 of 29 patients had adverse reactions when on IGIV-maltose, compared with 22 who had side-effects during infusions of IGIV (p < 0.001). Adverse reactions were recorded during 3 of 87 IGIV-maltose infusions and during 51 infusions with the maltose-free IGIV (p < 0.001). 27 patients expressed preference for IGIV-maltose. IGIV-maltose seems safe and will permit rapid infusion of large doses of immune globulin, thus improving the management of patients with antibody deficiency diseases.
IgA in saliva of breast-fed and bottle-fed infants.
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Medusa cells: the morphology and cytochemistry of common amoeboid variants of eosinophils.
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Presidential address. Reflections on the 1970s and a look to the future.
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