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Biomedical subjects

C Griscelli

Publications and source records attributed to C Griscelli.

At least 289 records · Page 16Linked to original sources
PubMed1978

Impaired defense against vaccinia in a child with T-lymphocyte deficiency associated with inosine phosphorylase defect.

A case of progressive vaccinia associated with a profound deficiency of cellular immunity and a defect in inosine phosphorylase is described. A striking contrast was observed between humoral immunity, which showed little if any impairment, and a severe cellular defect affecting both markers and functions of T lymphocytes. The child died despite treatment with methosazone, levamisole, transfer factor, irradiated blood transfusions, and a thymus graft. An adequate serum level of antibody to vaccinia virus was obtained by transfer of specific immunoglobulins, but this failed to stop the progression of the disease. This observation suggests that host defense against vaccinia infection is mainly mediated by cellular immunity.

Animals↗
PubMed1978

Selective defect of precursor T cells associated with apparently normal B lymphocytes in severe combined immunodeficiency disease.

Two patients, one with an autosomal and the other a sex-linked form of severe combined immunodeficiency, had more than 95% B cells in their peripheral blood. Despite an increased absolute number of B lymphocytes, the patients were unable to produce serum antibodies. In each patient, geno- or pheno-identical bone marrow transplantation was followed by the visualization of a thymus shadow and the appearance of both cellular and humoral functions. Chromosome of allotype studies showed that the T cell originated from the donor whereas serum immunoglobulins were synthesized by host B cells. In these patients the pathogenesis appears to be a selective defect of bone marrow precursor T cells without concomitant intrinsic B cell defect. The successful outcome of the graft in these two patients, who are now, respectively, 5 years and 11 months of age and free of infections, indicates that the preferred form of therapy in such patients is transplantation of bone marrow stem cells, which populate the thymus and mature slowly into T cells that cooperate fully with host B cells in synthesis of antibody.

B-Lymphocytes↗
PubMed1978

Specific inhibition of in vitro Candida-induced lymphocyte proliferation by polysaccharidic antigens present in the serum of patients with chronic mucocutaneous candidiasis.

A specific inhibitory activity of in vitro proliferative responses of normal human lymphocytes to Candida metabolic antigen was found in the serum of 6 out of 23 children with chronic mucocutaneous candidiasis. In each of the six patients, the presence of an inhibitory activity was associated with Candida-specific cellular defects, characterized by a negative-skin test and a lack of in vitro lymphocyte proliferation. The presence of a circulating inhibitor was detected during relapses of the disease and disappeared under antifungal therapy. This inhibitory effect was not associated with any toxicity on tested lymphocytes. The factor was shown to be nondialysable, thermostable, nonprecipitable with ammonium sulfate and absorbable on anti-Candida antibodies or concanavalin A-coupled agarose columns. Altogether, these results suggest that the inhibitory factor is not an immunoglobulin, but rather a polysaccharidic antigen of Candida albicans. An inhibition of Candida-induced proliferative response of normal human lymphocytes was also obtained by addition of polysacharide antigens or purified mannans from C. albicans to cultures. Candida polysaccharidic antigens appeared, therefore, to be involved in specific depression of cellular functions observed in chronic candidiasis.

Adolescent↗
PubMed1978

[Adenomegalic cytophagic sinusal histiocytosis. Morphological and immunologic study of a case].

The case of a 14-year-old boy of North-African origin, presenting a phagocytic sinus histiocytosis is reported. The main features of this now classical disease are illustrated: its localization to the neck, its chronicity, the fact that it is well tolerated, the pseudotumoral appearance of the adenomegaly as well as the intensive phagocytic (essentially lymphocytophagic) activity of the sinus macrophages. The bringing to light in a picture of hyperimmunity of an elevated percentage of antibodies against the measles and EB viruses, associated with a temporary depression of the cellular immunity, suggest that this lymphophagocytosis could be controlled and facilitated by preferential opsonization of lymphoid cells carrying on their membrane the antigen of the virus or viruses initially responsible.

Adolescent↗
PubMed1978

[Chédiak-Higashi disease (report on a new case) (author's transl)].

A typical case of Chédiak-Higashi disease is reported in a French four years old boy (no parents consanguinity). The clinical aspect of oculo-cutaneous and pilar hypopigmentation, associated with recurrent infections led to the diagnosis of C. H. disease which was confirmed by the presence of giant leukocytes granulations in the blood and giant melanosomes in epidermal melanocytes; these giant pigmented granules, are made of a limiting unique membrane with granular matrix and periodic filamentous structures, which correspond to stade I, II and III melanosomes. In keratinocytes, melanosomes are rare, never isolated and scattered in cytoplasm, but grouped in giant melanosomes complexes. No specific immunity deficiency is found in this patient but the chemotaxis of neutrophil polymorphonuclear leukocytes was decreased but restored by levamisole treatment. Concanavaline A Cap formation by polymorphonuclear leukocytes was abnormally increased, but returned at a normal level after cyclic GMP incubation and levamisole treatment. Unfortuntely, the patient died soon after accelerated phase had begun, in an anatomo-clinical picture of pseudo-lymphoma. A pathogenic discussion of CH disease is presented with the help of a study of 102 cases of literature.

Chediak-Higashi Syndrome↗
PubMed1978

Beta thalassemia with reticulocytopenia: clinical, biochemical, and ultrastructural studies.

An unusual form of beta thalassemia is described in two children of unrelated families. Its main features are a severe anaemia with a low reticulocyte count associated with an erythroblastic hyperplasia; these characteristics indicate a completely ineffective erythropoiesis. The results of the study of haemoglobin synthesis performed on the bone marrow in vitro showed a greater imbalance in chain synthesis than that typically found in Cooley's anaemia. Ultrastructural studies revealed, in erythroblasts, all the features observed in Cooley's anaemia, although these features were more widely encountered. In addition, two peculiar findings were noted: (1) many inclusion bodies, which were partially or totally surrounded by smooth membranes, and which may indicate an autophagic phenomenon; and (2) a spongy appearance of the chromatin in rare erythroblasts of one of the two patients which resembled that found in congenital dyserythropoietic anaemia type I. These findings suggest that the great imbalance in the synthesis of the haemoglobin chain is responsible for the presence of an increased number of inclusion bodies and results in the death of nearly all the late erythroblasts. Furthermore, the present results point out the need for further study of the synthesis of the globin chains in atypical congenital dyserythropoietic anaemias.

Blood Cell Count↗