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C Griscelli

Publications and source records attributed to C Griscelli.

At least 235 records · Page 13Linked to original sources
PubMed1982

Biotin-responsive immunoregulatory dysfunction in multiple carboxylase deficiency.

The immunoregulatory system has recently been shown to require prostaglandins (PG) for its activation in man. We report here an impairment of immunoregulatory function, due to defective PGE monocytic production, in a 12-month-old boy with multiple carboxylase deficiency (MCD). The abnormal immune-response was corrected in vitro by adding PGE to the medium. Moreover, PGE deficiency and immunoregulatory dysfunction responded to biotin administration in vivo. It is suggested that the PGE deficiency in MCD could result from an impaired activity of a biotin enzyme, acetyl CoA carboxylase, since the product of this enzyme reaction, malonyl CoA, is required for prostaglandin synthesis.

Biotin↗
PubMed1982

[Post-splenectomy infections and Pneumococcus vaccination in paediatric surgery (author's transl)].

Morbidity and lethality rates in pneumococal infections are higher among children with underlying diseases associated with restricted or absent splenic function. Vaccination with polyvalent vaccine is indicated in all children who are more than 2 years old and who have been splenectomized or have a congenital asplenia. Since protection by vaccination is 80% only, we combine the vaccination with penicillin prophylaxis for at present at least three to five years after splenectomy and draw the express attention of parents and family physicians to the limited nature of protection afforded by vaccination. An increase in the immunogenicity of polysaccharid antigen vaccine might lead to successful vaccination of children below 2 years of age who are notable for a particularly high risk of infection. First reports have been published in literature on the possibility of re-implantation of splenic tissue after post-traumatic rupture (17, 27, 28) so that it may become possible to employ this method additionally to pneumococcus vaccination. In case of haematological indication for splenectomy this should be postponed as far as possible until the child has completed his fifth year of life.

Antibodies, Bacterial↗
PubMed1982

Respective roles and interactions of T-lymphocyte and PGE2-mediated monocyte suppressive activities in human newborns and mothers at the time of delivery.

Recently the concept of a poorly functional humoral immune response in the newborn was proposed. Data have been presented indicating that the impaired newborn B cell maturation, as shown in vitro in a pokeweed mitogen-induced B cell maturation system, is due both to an immaturity of lymphocyte subsets and to an increased suppressive T activity. In the present work, we present evidence that there exists a predominance of a naturally occurring T lymphocyte suppressive activity in the cord blood in that the removal of the suppressive activity by irradiation allows a normal maturation of newborn B cells. Such normal maturation of newborn B cells can also be obtained using mixed cultures of adult T cells and newborn B cells. Newborn suppressor T cells belong to both EA gamma (+) and EA gamma (-) fractions, and it is not known whether these two groups do or do not belong to different subsets. The PGE2-dependent monocyte suppressive activity does not play any role in the suppression observed in newborns since newborn monocytes are poorly suppressive and since they produce a smaller amount of PGE2 than adult monocytes. Some observations suggest, on the contrary, that the suppressive T lymphocytes can regulate the level of the PGE2-dependent monocyte suppressive activity. It should be noticed that similar observations about T lymphocyte and PGE2-dependent monocyte suppressive activities have been made at the same time using mothers' cells. These observations suggest the possibility that such changes in B cell immune regulation may result from an interaction between maternal and fetal lymphoid cells.

B-Lymphocytes↗
PubMed1982

Defective handling of mannan by monocytes in patients with chronic mucocutaneous candidiasis resulting in a specific cellular unresponsiveness.

Carbohydrate antigens from Candida albicans, essentially mannan, have previously been shown to persist in the serum of some patients with chronic mucocutaneous candidiasis, and to be able to inhibit specifically the candida antigen-induced proliferation of control lymphocytes. Lymphocytes from three out of six patients were shown to be hypersensitive to mannan inhibition. These data were explained by the demonstration of an apparently selective impairment of radiolabelled mannan handling by two patients' monocytes following a normal uptake. This defect was observed both in active and remission phases of the infection suggesting an intrinsic defect of patients' monocytes. In experiments performed with control lymphocytes, it was shown that mannan exerted its suppressive effect by interfering with candida antigen presentation by adherent cells to autologous T lymphocytes. Furthermore, mannan neither was cytotoxic nor induced suppressor T cells. Altogether, these data suggest that the in vivo persistance of mannan, in some patients, is secondary to a primary macrophage dysfunction leading to impairment of specific cellular immune responsiveness.

Antigens, Fungal↗
PubMed1982

Lack of prostaglandin E2-mediated monocyte suppressive activity in newborn and mothers.

An excess of adult blood adherent cells (monocytes) inhibits mitogen, antigen and allogeneic cell-induced lymphocyte proliferations. This inhibition is dependent on the number of the adherent monocytes in the cultures and is substantially reduced (by 60%) by indomethacin or anti-PGE2 antiserum. Newborn monocytes exert only a weak inhibitory effect and produce about eight times less PGE2 than adult monocytes. The production of PGE2 and the suppression can be induced by incubating newborn monocytes with mixed leucocyte culture supernatants. Both monocytes from mothers at the time of delivery and from newborn infants, usually exert a poor suppressive activity related to a low production of PGE2. We strongly suggest that the expression of the PGE2-mediated suppression by monocytes is under the control of activated short-lived suppressor lymphocytes.

Adult↗
PubMed1982

Inability of newborns' or pregnant women's monocytes to suppress pokeweed mitogen-induced responses.

Although an excess of human adult blood adherent cells inhibits the pokeweed mitogen- (PWM) induced normal adult lymphocyte proliferation and B cell maturation into immunoglobulin-containing cells (ICC), adherent cells collected from newborn infants or pregnant women at time of delivery were unable to exert a similar suppressor activity. After activation by Concanavalin A (Con A), newborns' and pregnant women's adherent cells acquired a suppressor activity comparable to that of control adult adherent cells. The adherent suppressor cell was shown to be radioresistant (3000 rad), indicating its probable monocytic origin. Both monocyte-suppressor activities (MSA) observed in adulthood (spontaneously) and in the neonatal period (after activation) were dependent on prostaglandin E2 (PGE2) secretion, because they were abolished by indomethacin or a specific anti-PGE2 antiserum. Expression of MSA appeared to be under a negative regulation exerted by naturally occurring T suppressor lymphocytes present in the blood of newborns or pregnant women, because incubation of adult monocytes or Con A-activated newborn monocytes with newborns' or pregnant women's T lymphocytes resulted in a dramatic decrease of their MSA. These results strongly suggest that the lack of MSA in the neonatal period and in late pregnancy is a consequence of activation of T suppressor lymphocytes.

B-Lymphocytes↗
PubMed1982

[Acute lymphoblastic leukemia in childhood: importance of sex as a prognostic factor (author's transl)].

In order to appreciate their prognostic value in acute lymphoblastic leukemia (ALL) of childhood, the main initial clinical and biological features have been analysed in 63 patients. In all cases, follow-up was longer than 3 years; in 43 it was longer than 5 years. The survival rate in first remission was 52% after 3 years and 30% after 5 years. No relapse was observed after 5 years. In this series, the presence of very high leukocyte count (less than 30 x 10 9/l or 30,000/mm3) and sex were the only significant prognostic factors. A poorer prognosis of ALL was indeed present in boys. The sex-related difference is independent from other prognostic factors and may require special attention.

Adolescent↗
PubMed1982

[Immunologic study of familial lymphohistiocytosis. Eight new case reports (author's transl)].

We report 8 cases of familial lymphohistiocytosis collected in 6 families. Several data argue for an hyperactivation of the reticuloendothelial system (RES). An abnormal visualization of all organs was observed in a scintigraphic study after 99technetium labelled red blood cells injection. Blood monocytes contained very low peroxidase activity as detected by cytoenzymology and secreted large quantities of prostaglandin E2 (PGE2). Adherent cells isolated from blood exercised a strong suppressor effect on the proliferation of normal lymphocytes induced by phytohemaglutinin. This effect was reduced by indomethacin and therefore appears PGE2-dependent. One patient's serum exerced an inhibitory activity on antigen-induced proliferation of normal lymphocytes and on mixed leucocyte reaction. In contrast, cellular and humoral functions were not deeply impaired. The hyperactivation of the RES remains unexplained and not related to a graft versus host reaction, which could be excluded in 3 of our patients. Therapeutic attempts were not efficient, all patients dying despite steroid, vincaleucoblastin and indomethacin therapy.

Female↗
PubMed1981

Monocyte alpha-naphtyl esterase deficiency in chronic granulomatous disease.

Monocytes from five unrelated children (four boys and a girl) with chronic granulomatous disease were studied for their ability to reduce nitroblue tetrazolium dye after stimulation with zymosan, and for their alpha-naphtyl butyrate esterase activity. As expected, monocytes ingested zymosan particles but failed to reduce nitroblue tetrazolium dye. However, monocytes from two boys out of the five patients were alpha-naphtyl butyrate esterase-negative, whereas both their neutrophils and monocytes were positive for granular naphtol AS-D esterase activity.

Carboxylic Ester Hydrolases↗