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Biomedical subjects

C Griscelli

Publications and source records attributed to C Griscelli.

At least 253 records · Page 14Linked to original sources
PubMed1981

Arthropathy with rash, chronic meningitis, eye lesions, and mental retardation.

Three unrelated children (one girl and two boys) have had since birth a syndrome characterized by a permanent skin rash which becomes more intense during flare-ups associated with fever, lymphadenopathy, splenomegaly, and arthritis symmetrically involving the large joints. In one boy, typical psoriasis was observed at age 3 years. In two patients, roentgenograms of the joints showed early patellar ossification and an abnormal epiphyseal appearance. The three children also had neurologic involvement, with mental retardation, enlarged head circumference, eye lesions, late closure of the anterior fontanel, and a chronic meningitis with infiltration by polymorphonuclear cells. No immunologic abnormalities were found, but polymorphonuclear cells infiltrated the skin, lymph nodes, synovial fluid, and CSF.

Adolescent↗
PubMed1981

Intestinal salivary, and tonsillar IgA and J-chain production in a patient with severe deficiency of serum IgA.

An 18-year-old man with tendency to respiratory infections had a serum IgA level of only 2% of normal whereas his salivary IgA amounted to 50% of the lower normal concentration range. Moreover, both the rectal and jejunal IgA-producing cell populations were of normal size. Nevertheless, a relative increase of salivary IgM and a distinctly raised number of IgM-producing cells in jejunal mucosa indicated an imbalance in his secretory immune system. This possibility was supported by the presence of an excess of J 3 chains in most of his intestinal IgA immunocytes, probably reflecting a reduced synthetic rate of IgA. The number of tonsillar IgA-producing cells was only slightly below the normal range; most of them lacked J chain, as normal, and could thus be a source of his serum IgA, which was mainly monomeric. A marked deficiency of IgA-producing cells in his bone marrow supported the notion that this tissue site normally is the major source of monomeric IgA. This study suggests that a generally defective IgA system may be topically activated owing to the persistent antigenic and mitogenic load on mucosa-associated lymphoid tissues. Our findings are not consistent with a general regulative compartmentalization of monomer- and dimer-producing IgA immunocyte populations.

Adolescent↗
PubMed1981

T-cell subset analysis by monoclonal antibodies in primary immunodeficiencies.

T-cell subsets have been analysed in 23 cases of primary immunodeficiency with monoclonal antibodies. Functional assays investigating T-cell function--proliferative response to mitogens, antigens, and allogeneic cells, cytotoxicity generated against allogeneic target cells and helper function to pokeweek mitogen-induced immunoglobulin synthesis--were performed in parallel in the same patients. The results enabled us to delineate four groups of patients. The first group consisted of patients in whom marker and function studies show concordant data, with either normal or strongly decreased T-cell number and function. The second group consisted of patients in whom various degrees of functional abnormality coexist with subnormal T-cell number and increased suppressor T-cell proportion. In the third group, we collected all patients who showed functional deficiencies without marker abnormalities. Finally, there was a small group composed of patients whose T-cell pattern was strongly suggestive of abnormal differentiation.

Adenosine Deaminase↗
PubMed1981

Dysfunctions of pokeweed mitogen-stimulated T and B lymphocyte responses induced by gammaglobulin therapy.

Lymphocytes obtained from nonimmuno deficient children treated with commercially available preparations of gammaglobulin failed to proliferate and to mature into plasma cells in vitro after stimulation with pokeweed mitogen. The influence of the treatment on lymphocyte functions varied according to the cell population considered. A T helper cell activity was detected in these patients but only in the cell subset bearing receptors for IgG after irradiation. T lymphocytes exerted a suppressive effect that disappeared after irradiation or incubation at 37 degrees C. The suppressive cells were found among E rosette-forming cells depleted of leukocytes bearing receptors for IgG. Their suppressive effect was expressed only in the presence of normal radioresistant T lymphocytes that did not bear Fc receptors for IgG. Similar dysfunctions could be induced in vitro by incubation of normal T and B lymphocytes with gammaglobulin preparations. Because F(ab)'2 fragments or deaggregated preparations of gammaglobulin failed to activate T suppressor lymphocytes, this activation was likely triggered by attachment of Fc portion of denatured IgG to the corresponding membrane receptor. This activation step was prostaglandin E(2)-dependent, suggesting that activated monocytes were involved in the activation process. B lymphocyte responses appeared directly inhibited by attachment of denatured gammaglobulin on membrane Fc receptor. Our observations suggest that immunological effects of gammaglobulin therapy are not limited to antibody transfer, since it also induces subtle modifications of in vitro pokeweed mitogen-stimulated T and B cell responses. These modifications must be considered in interpreting results obtained in immunodeficient patients investigated under gamma-globulin therapy.

Antigens, Surface↗
PubMed1981

Nonspecific alpha-naphthyl acetate esterase activity of T-lymphocytes: study in healthy newborns and children, in immune deficiencies and juvenile rheumatoid arthritis.

The aim of this study was to compare the E rosette-forming cells and nonspecific alpha naphthyl esterase (ANAE)-positive lymphocyte values in normal and pathologic situations. In newborns, the ANAE-positive lymphocytes represented less than 60% of the E rosette population. During the first year of life, E rosette-forming cells (E-RFC) reached normal values as soon as one month whereas only three-fourths of the T cells exhibited an ANAE-positive staining. In adult T cell populations, nearly 90% were ANAE-positive. Our observations of immune deficiencies suggested that the relative proportions of E-RFC and ANAE-positive lymphocytes were generally comparable to normal values. However, in the majority of the patients with very low or absent E-RFC (severe combined immune deficiencies, Di George syndrome, and congenital rubella), some ANAE-positive lymphocytes could be detected. Our immunologic survey shows that the ANAE-positive lymphocytes were in a normal range 2 years after a bone marrow transplantation in severe combined immune deficiencies patients. One child who exhibited a normal amount of E-RFC and whose lymphocytes failed to respond in vitro to mitogens had practically no ANAE-positive lymphocytes. An elevated amount of ANAE-positive cells in juvenile rheumatoid arthritis may reflect an augmentation of the T helper functions which permanently stimulated in vivo immunoglobulin production.

Adolescent↗
PubMed1981

[Selective defect on interferon secretion associated with impaired natural killing activity (author's transl)].

A 4-year-old boy suffering from repeated, severe bacterial (staphylococcus, pneumococcus, klebsiella, moraxella) and viral (adenovirus) infections did not show any deficiency of either humoral, cellular or non-specific immunity when investigated by classical immunological tests. In contrast, a profound defect of interferon secretion was found in leucocyte cultures induced by mitomycin-treated Raji lymphoblastoid cells or soluble antigens. This was associated with a profound impairment of non-specific "natural" killer (NK) cytotoxic activity of peripheral leucocytes tested in a 4 hour-chromium release assay against K 562 target cells. Addition of leucocyte interferon in culture increased the cytotoxic potential of the patient's leucocytes. Intramuscular administration of interferon completely (but transiently) reversed the NK defect in vivo. The expression of HLA-A and B on the membrane of platelets was diminished, whereas the expression of HLA-A, B and la antigens on the membrane of lymphocytes was normal. This observation indicates that an apparently primitive defect of interferon secretion may result in a special type of immune deficiency with complex biological consequences, some of which can be reversed by interferon therapy.

Antibody-Dependent Cell Cytotoxicity↗
PubMed1981

Role of prostaglandin E2 in the induction of nonspecific T lymphocyte suppressor activity.

Activated human monocytes and concanavalin A (Con A)-activated T lymphocytes are known to suppress T and B lymphocyte proliferation and B cell maturation into immunoglobulin-producing cells. We have now shown that monocyte suppressive activity is predominantly mediated through release of prostaglandin E2 (PGE2), which is active only in the presence of a "short-lived," radiosensitive T lymphocyte subset. PGE2, at high concentration, can activate T suppressor lymphocytes (TS), which display the same characteristics as Con A-activated TS lymphocytes. Moreover, Con A activation of TS lymphocytes was obtained only in the presence of PGE2, as specific anti-PGE2 antiserum or indomethacin prevented TS activation; this suggested a double signal as a prerequisite for activation of the nonspecific TS cell subset. We propose that TS lymphocytes modified by Con A become sensitive to small amounts of PGE2 produced by monocytes that must be present during the Con A-stimulated activation phase of suppressive cells.

B-Lymphocytes↗
PubMed1981

[Bubble babies].

Explore the source record for details and available documents.

Child Development↗
PubMed1980

Natural killer and killer cell activities in patients with primary immunodeficiencies or defects in immune interferon production.

Natural cell-mediated cytotoxicity (NCMC) against K-562 target cells was explored in patients with various primary immunodeficiencies. (a) NCMC was present in 2 patients lacking detectable B cells. Conversely, NCMC was depressed in 2 patients with severe combined immunodeficiency presenting a normal number of circulating B cells. These data exclude a major role for B cells in NCMC. (b) NCMC was depressed mainly in patients with severe combined immunodeficiency or with defects affecting cellular immunity, which suggests a major role in NCMC for cells of T lineage. (c) Four patients with various clinical features associated with a lack of immune interferon production exhibited a strikingly depressed NCMC. Interferon thus appears as a major in vivo activator of natural killer (NK) cell activity. (d) Eight patients exhibiting a depressed NK cell activity had a normal killer (K) cell activity against L1210 cells in the presence of rabbit anti-L1210 antiserum. These data are consistent with the assumption that NK and K cell activity are mediated through two distinct cellular mechanisms.

Adolescent↗
PubMed1980

Transplantation of lymphoid cells in patients with severe combined immunodeficiency (SCID).

The effects of bone marrow or fetal lymphoid organ transplants in 15 patients with severe combined immunodeficiency disease are reported. The benefits and dangers inherent to the various transplantation strategies are discussed. Our studies indicate that, even in the group of patients with B cells, bone marrow transplantation may be the best procedure to obtain the reconstitution of cellular and humoral functions.

B-Lymphocytes↗
PubMed1980

[The Buckley syndrome: recurring, severe staphylococcal infections, eczema and hyperimmunoglobulinemia E. (author's transl)].

Fifteen patients aged between three and 27 years were examined clinically and immunologically. Common to all patients were severe recurring cutaneous and pulmonary staphyloccal infections, chronic eczema, eosinophilia and an extremely elevated serum IgE level. Eight of the patients had in addition facial dysplasia characterised by coarse features, prognathism and poorly formed external ears. Marked osteoporosis, particularly of the vertebral bodies, was observed in eight patients. A constant defect of granulocyte chemotaxis was found in only three patients; fluctuating or constantly normal chemotaxis occurred in six patients. Polycloncal hypergammaglobulinemia was detected in 14 patients, elevated IgD in two patients, a partial T-cell defect in two patients and a history of lack of antibody response in one patient. Therapeutic trails anti-H2 receptor-antihistamines did not produce lasting or satisfactory clinical or immunological results in the pathogenetically unidentified disease.

Adolescent↗
PubMed1980

Chronic progressive encephalitis in children with x-linked hypogammaglobulinemia.

This report is on six cases of a chronic relentlessly progressive encephalitis occurring in boys with congenital hypogammaglobulinemia presumably of the x-linked type, which are thought to represent a separate neurological entity. Intellectual deterioration, dysarthria, spasticity, ataxia, optic atrophy and an increase of lymphocytes in the cerebrospinal fluid, were the main clinical signs. The pathological picture was that of a viral encephalitis, but all virological investigations on brain biopsies and CSF were negative. The significance of intra-cisternal tubuloreticular inclusions in brain endothelial cells, similarities with chronic rubella encephalitis, and the role of the immunological deficiency are discussed. Sofar, the cause of this new type of encephalitis remains obscure.

Adolescent↗
PubMed1980

Fulminant meningococcemia in a child with hereditary deficiency of the seventh component of complement and proteinuria.

A previously healthy 14-year-old boy presented with fulminant meningococcemia. He was found to have a total deficiency of C7. His serum totally lacked bactericidal activity against Neisseria meningitidis. Addition of purified C7 restored the serum hemolytic and bactericidal activity. Susceptibility to disseminated Neisseria infections has previously been reported in 3 patients with C7 deficiency, as well as in a few patients with deficiency of C5, C6 and C8. These findings emphasize the importance of intact complement mediated bactericidal activity in host defense against disseminated Neisseria infections. Evaluation of the complement system in individuals with Neisseria infections appears mandatory.

Adolescent↗
PubMed1980

Neutrophil chemotaxis in juvenile chronic arthritis.

The leucocyte infiltration observed in histological lesions from patients with juvenile chronic arthritis (JCA) suggests the possibility of an abnormal leucochemotaxis. A group of 21 patients with JCA which fulfilled the Eular criteria (Oslo Symposium 77) were investigated with a paired group of 21 children. The chemotactic assay used was a microscopical direct observation technique. The chemotaxis of the patient's leucocytes was in the normal range, as was the chemotactic activity of their serum. However, their serum had an enhancing effect on the chemotaxis of normal leucocytes. An attempt to characterise this chemotactic enhancing factor was undertaken. It was not dialysable, heat stable, destroyed at 80 degrees C, nor precipitated by ammonium sulphate at 45%; it could be migrated on PAGE with albumin, and, by precipitation with a goat antihuman albumin antiserum, seemed to be bound to the albumin fraction. The function of this factor in the regulation of the inflammatory process is discussed.

Adolescent↗