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Biomedical subjects

C Griscelli

Publications and source records attributed to C Griscelli.

At least 217 records · Page 12Linked to original sources
PubMed1983

Specific in vitro antimannan-rich antigen of Candida albicans antibody production by sensitized human blood lymphocytes.

We have developed a new antigenic system for the induction of specific in vitro antibody response in man. The antigen used was purified from the cell wall of Candida albicans strain A and contained greater than 96% polysaccharide mannan. Peripheral blood mononuclear cells from Candida-sensitized donors produced specific antimannan antibodies during a 7-d culture in the presence of mannan absorbed with methylated bovine serum albumin. Two methods were used to detect antimannan antibody responses. Antimannan antibody-producing cells were identified by radioautography with tritiated mannan. Antibody concentration in culture supernatants was measured by an enzyme-linked immunosorbent assay. In both methods, specific IgM and IgG (but not IgA) antibodies were detected. The antibody production to mannan was specific, since an antigenically unrelated polysaccharide (pneumococcal antigen S III) did not bind to methylated bovine serum albumin-mannan-induced blast cells and did not induce antimannan antibody-containing cells. Furthermore, a pulse with an excess of unlabeled mannan abolished [3H]mannan binding, whereas an excess of unlabeled S III did not. Similarly, no antimannan antibody was obtained in influenza virus-stimulated cultures and mannan-stimulated cultures were not inducing antiinfluenza antibodies. The antimannan antibody production was shown to be a T cell-dependent phenomenon. The T helper effect appeared to be radiosensitive. It was under a genetic restriction as it occurred only in autologous or semi-identical but not in allogeneic situations. This system is relatively simple, reproducible, and well suited for the study of specific secondary in vitro antibody responses to polysaccharide antigens in humans.

Antibodies, Fungal↗
PubMed1983

Enhancement by interferon of membrane HLA antigens in patients with combined immunodeficiency with defective HLA expression.

Since interferon is known to enhance HLA A-B expression on lymphocytes from normal donors, we have tested the hypothesis that interferon could reverse the defective membrane expression of HLA antigens observed in some patients with combined immunodeficiency. Leucocytes from four patients with this syndrome, after overnight incubation with preparations of interferon, showed a clear enhancement in the percentage of cells bearing HLA A-B-C and beta 2 microglobulin (but not HLA-DR) antigens as detected by membrane immunofluorescence. Functional HLA-A and B antigens also appeared on patients' T cell blasts treated with interferon, as shown by the ability of these blasts to be destroyed by specific cytotoxic T lymphocytes. Both alpha and beta human interferons were effective. These effects were shown to be mediated by interferon (but not contaminants in our preparations) by the use of specific antiserum to interferon. It is likely that interferon acts on HLA synthesis, since in vitro addition of drugs known to inhibit nucleic acid or protein synthesis completely abolished the enhancing effect of interferon on membrane HLA expression. Interferons can therefore modulate leucocyte HLA expression and synthesis in patients with defective expression of these antigens, a finding which suggests that interferon treatment might be beneficial in this condition.

Antigens, Surface↗
PubMed1983

Evidence that abnormal platelet functions in human Chédiak-Higashi syndrome are the result of a lack of dense bodies.

The structure and functions of platelets from three patients with the Chédiak-Higashi syndrome were examined. Electron-microscopic observations revealed a large reduction in the number of serotonin-storage granules or dense bodies but otherwise normal ultrastructure and normal amounts of alpha-granules and catalase-positive granules. The number of mepacrine-labeled granules was also reduced. Platelets contained normal amounts of beta-thromboglobulin and Platelet Factor 4. The platelet release reaction studied with thrombin as the inducer was impaired. The serotonin uptake by the patients' platelets was low and not inhibited by reserpine, and its metabolism was increased. These findings clearly show that platelets from human Chédiak-Higashi syndrome are deficient in the storage pool of dense granule substances and suggest that this granule defect has an influence on the release mechanism of other granule constituents.

Blood Platelets↗
PubMed1983

[Di George syndrome, exemplary rhomboencephalic neurocristopathy].

Di George's syndrome associates hypocalcemia and hyperphosphoremia (phosphocalcic disorders from absence of the parathyroids), cell immunity disorders related to agenesis or hypoplasia of the thymus, and large vessels malformations (pulmonary artery, aorta) with cardiac lesions. The disorder is the result of deletion of rhomboencephalic neural crests cells, Di George's syndrome being an exemplary rhomboencephalic neurocristopathy. This organic syndrome is associated with early disorders of the same neurological region (the rhomboencephalon), expressed clinically by sucking and swallowing disturbances and cardiorespiratory regulatory disorders. Early rhomboencephalic lesions can lead to velopalatal division and microretrognathism secondary to a lingual motility disturbance. The association of this neurocristopathy with a neural tube lesion constitutes a dysneurulation. Fetal and neonatal retrognathism are evidence of bulbar involvement. Di George's and Pierre Robin's syndromes constitute two fairly similar clinical expressions of the same embryonic deficiency of rhomboencephalic neurulation.

Brain↗
PubMed1982

[Glucagon-induced neutrophil mobilization (author's transl)].

In 19 normal subjects an increase in the number of circulating neutrophils was observed after intramuscular injection of 1 mg glucagon. The response began at the end of the 1st hour following the injection and persisted beyond the 8th hour, with a peak between 2 and 5 hours. No response was obtained in patients with bone marrow aplasia, either primary or associated with acute leukaemia. In 20 patients with chronic primary neutropenia, the degree of response was proportional to the percentage of medullary polymorphonuclears. A comparison between the kinetics of the glucagon-induced granulocyte response and that of the response induced by other neutrophil mobilizing agents suggested that glucagon acts by releasing granulocytes from the bone marrow reserve compartment. The finding that an infusion of dibutyryl cyclic AMP results in granulocyte mobilization suggests that the effects of glucagon are mediated by cAMP at cell level. Since the glucagon response test is harmless and gives rapid and pronounced results, it may be useful in investigation of patients with neutropenia. In addition, the glucagon-induced granulocyte mobilization might improve leucocyte yield in blood donors used for transfusion in agranulocytosis.

Adolescent↗
PubMed1982

Prenatal testing for inherited immune deficiencies by fetal blood sampling.

We attempted to develop a prenatal diagnosis in fetuses at risk for immunodeficiency by fetal blood sampling performed under fetoscopy at 18-22 weeks of gestation. In order to obtain normal values, we first investigated thirty-five control fetuses whose blood punctures were undertaken for the diagnosis of haemoglobinopathies. Surface markers and in vitro mitogen-induced proliferation of the fetal lymphocytes were studied using micromethods. We then examined two fetuses at risk for two different types of severe combined immunodeficiency and established their immunological integrity, hence avoiding an unjustified termination of pregnancy. This immunological integrity was confirmed after birth.

Antigens, Surface↗