Possible transmission of a human lymphotropic retrovirus (LAV) from mother to infant with AIDS.
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Acquired Immunodeficiency Syndrome↗
Biomedical subjects
C Griscelli
Publications and source records attributed to C Griscelli.
Selective tropism of lymphadenopathy associated virus (LAV) for helper-inducer T lymphocytes.
Lymphadenopathy associated virus ( LAV ) has been isolated from patients with the acquired immunodeficiency syndrome (AIDS) or lymphadenopathy syndrome. Since the immune deficiency in AIDS seems to be primarily related to the defect of the helper-inducer T lymphocyte subset, the possibility that LAV is selectively tropic for this subset was investigated. Fractionation of T lymphocytes was achieved by cellular affinity chromatography with monoclonal antibodies. In a hemophilic patient who was a healthy carrier of LAV , reverse transcriptase activity and virus particles detected by electron microscopy were found only in cultures of helper-inducer lymphocytes. When infected with LAV in vitro, lymphocyte subsets from normal individuals yielded similar results. Virus production was associated with impaired proliferation, modulation of T3-T4 cell markers, and the appearance of cytopathic effects. The results provide evidence for the involvement of LAV in AIDS.
Acquired Immunodeficiency Syndrome↗
Isolation of new lymphotropic retrovirus from two siblings with haemophilia B, one with AIDS.
A human T-lymphotropic retrovirus was isolated from cultured T lymphocytes from two siblings with haemophilia B. Patient 2 was healthy, but patient 1 had acquired immunodeficiency syndrome. The retrovirus differed from human T-cell leukaemia virus (HTLV) but it was similar to the lymphadenopathy-associated retrovirus (LAV) in its morphology and its major core protein (P25). Both patients had antibodies against LAV and patient 1's retrovirus, detected by an enzyme-linked immunosorbent assay or a radioimmunoprecipitation assay. Seroepidemiological data indicated the transmission of this retrovirus by plasma products.
Acquired Immunodeficiency Syndrome↗
[Nosologic aspects of systemic forms of very-early-onset juvenile arthritis. Apropos of 17 cases].
The case-reports of seventeen patients who experienced onset of systemic juvenile arthritis between the neonatal period and the age of ten months are studied. Group I consists of six cases in which onset took place after the age of five months. The condition progressed towards recovery with minimal residual joint disease, towards polyarticular involvement or towards perennial systemic manifestations. Group II individualizes three patients with neonatal manifestations, and chronic polymorphonuclear cell meningitis and major radiologic changes as typical features. The eight patients in Group III, some of whom had neonatal onset, exhibited febrile exacerbations with involvement of skin, joints and lymph nodes, often produced by various antigenic stimulations (infections, immunizations, gammaglobulin injections and delayed hypersensitivity reactions). In group III exacerbations were often shorter but more frequent than in group I. After several years follow-up, clinical and radiological evaluation showed no signs of residual joint disease in this group. Biological features in each of the three groups were manifestations of major non-specific inflammation. Histologic examination of lymph nodes and skin showed no specific abnormality. IgA and IgD concentrations were especially high in group III. The reality of the syndrome described many years ago by Wissler and Fanconi appears to be open to debate in the light of manifestations recorded in group III.
Adrenal Cortex Hormones↗
Impaired natural killer activity in lymphohistiocytosis syndrome.
In six patients with well-documented lymphohistiocytosis syndrome, natural killer activity was found to be profoundly impaired and could not be increased by incubation in vitro with interferon. This abnormality was not found in parents of the affected children. A clear correlation with the activity of the disease was observed, although a delay of a few weeks (possibly reflecting the life span of NK cells in blood) was seen in the disappearance of NK activity after the onset of the disease and its reappearance after remission. No absolute correlation was observed between NK activity and percentage of leukocytes detected by the Leu-7 monoclonal antibody. Our findings indicate that testing NK activity is useful for the diagnosis of lymphohistiocytosis syndrome and can be used as an index of activity of the disease, among other major clinical and biologic signs of this syndrome. Reversal of the NK activity defect (rather than detection of Leu-7 positive cells) appears to be a good criterion of complete remission.
Antibodies, Monoclonal↗
[AIDS in the infant].
Recently, we and others have encountered a group of infants who developed AIDS. They were born from parents who belonged to identified risk factors for AIDS. The clinical and immunologic features were described. The existence of a pediatric form of AIDS transmitted other than by transfusion suggests the possibility of transplacental perinatal or postnatal transmission.
Acquired Immunodeficiency Syndrome↗
[A case of AIDS in a hemophilia B patient in France].
AIDS is reported in a 14 year old hemophiliac B. This patient was treated on demand and used 800 F IX IU per kg B.W. and per year. He presented with severe opportunistic infection (Toxoplasmosis) and profound impairment of cellular immunity. He suffered of chronic active hepatitis and he was chronic HBs Ag carrier. A retrovirus (LAV) was isolated from his cultured T lymphocytes. He had no other identified risk factor than severe hemophilia B. The occurrence of AIDS in hemophiliacs suggests a relationship between the transfusion of blood products and the disease. The cause of AIDS is unknown. A possible relationship between LAV and AIDS is discussed.
Acquired Immunodeficiency Syndrome↗
Epstein-Barr serology in immunodeficiencies: an attempt to correlate with immune abnormalities in Wiskott-Aldrich and Chediak-Higashi syndromes and ataxia telangiectasia.
Epstein-Barr (EB) virus serology was correlated with the results of immunological investigations of three inherited immunodeficiency diseases, in an attempt to understand the immune mechanisms controlling EB virus infection. In nine patients with Wiskott-Aldrich syndrome (WAS), the constant lack of anti-EB virus associated nuclear antigen (EBNA) was accompanied by a consistent impairment of allogeneic cytotoxicity. We confirmed a frequent absence of anti-EBNA antibody in ataxia telangiectasia (AT), and we showed a correlation between the level of anti-EBNA response and the mixed leucocyte response (MLR), i.e., an absence of anti-EBNA antibody correlated with a decreased MLR. In two of three untreated patients with Chediak-Higashi syndrome (CHS), high persistent titres of anti-EA antibodies were observed, which were possibly related to a defective natural killer (NK) cell activity. In spite of previous infection with EB virus, none of the 41 patients exhibited clinical signs attributable to the virus, suggesting that residual or compensatory mechanisms must have limited activation of the virus. In patients with AT and WAS these mechanisms may include NK cell activity, which is not depressed in these syndromes, whereas in patients with CHS, they may involve T cell cytotoxicity.
Adolescent↗
[Treatment of the Wiskott-Aldrich syndrome by a graft of allogeneic bone marrow].
We herein describe the first French case of successful bone marrow transplantation (BMT) in a patient with the Wiskott-Aldrich syndrome. Although the patient required hospitalization for a total of one year during his first 4 years of life for bleeding, eczema, protracted diarrhea and multiple infections, the bone marrow transplantation has permitted a complete and stable correction of the thrombocytopenia, the eczema and the immunodeficiency. The patient was prepared by a total body irradiation (850 rads) with a partial lung shielding and anti-lymphocyte globulins. The BMT was immediately followed by a severe but transient herpetic infection and acute graft versus host reaction (grade II) which resolved after steroid therapy. The thrombocytopenia disappeared 3 months after the BMT. The infections and the eczema did not reappear. Immune functions are entirely normal and all blood cells have been shown to be of donor origin (the sister of the recipient). The boy is growing normally and is doing well 3 1/2 years thereafter. He only suffered from bilateral cataracts secondary to the irradiation requiring lens extraction. One can now expect a success rate of 75% in bone marrow transplantation in patients with Wiskott-Aldrich syndrome as evaluated from a world review. In contrast, symptomatic treatment of the disease leads to a mean survival of 7 years, survival rarely exceeding 18 years.
Bone Marrow Transplantation↗
[Autoimmune hemolytic anemia in children. Apropos of 14 cases].
Autoimmune hemolytic anemia is a rare disorder in childhood. The therapeutical difficulties encountered are described in a series of 14 patients aged 6 weeks to 10 years, 8 of them being under the age of 1 year at time of diagnosis. A remission was observed spontaneously in 1 patient and was obtained with steroids in 8 others. One patient died from acute irreversible hemolysis. Four patients were splenectomized because of an immediate or delayed resistance to steroids. After splenectomy, two patients died from infection, one was cured and the last patient is still in remission after one year. Steroid therapy is the primary treatment of autoimmune hemolytic anemia. The initial dose of 2 mg/kg/day has to be maintained until the remission (normal hemoglobin level and reticulocyte count) is achieved, then progressively decreased leading to alternate day therapy for several months. Resistance to steroid requires splenectomy, although it is an hazardous treatment in young children. The indication for immunosuppressive agents in childhood is difficult to define. It can be proposed in patients with steroid dependency in order to reduce the dose of steroid and/or to avoid long term steroid therapy.
Acute Disease↗
[Prognosis of the systemic forms of juvenile chronic arthritis. Apropos of 100 cases].
The records of 100 children presenting with systemic juvenile rheumatoid arthritis were studied retrospectively. The precocity of onset and intensity of initial extra-articular signs did not seem to be correlated with a more severe outcome. On the other hand, the number of arthritides present during the first 6 months seemed to be associated with a different prognosis: the oligo- or abarticular forms generally had a better prognosis. In girls, onset was earlier and remissions were twice more frequent than in boys. Contrary to all other joints, the hip was susceptible of a radiological improvement in 25% of cases. Joint disabilities, especially of the wrist, were initially related to inflammation and pain; secondarily, they were chiefly related to the anatomic evolution, resulting in fusion of the joint spaces. At the last clinical examination after a 10 year's evolution, half of patients were in clinical remission, either without articular sequellae, with sequellae, the most severe of which being the hip involvement; 25% of patients still had systemic symptoms; 25% presented with polyarthritis with persisting biological and articular inflammatory signs.
Adolescent↗
[Activation by injectable gamma globulins of the suppressive functions of the humoral response tested in vitro].
In subjects with normal immune system injectable gammaglobulins produce unexpected effects: B cells become unable to mature into plasmocytes in vitro when cultured in the presence of pokeweed mitogen. This inhibitory effect seems to be associated with binding of the denatured gammaglobulins to B cell membrane receptors for the IgG Fc fragment (Fc gamma). In addition, injectable gammaglobulins activate a radiosensitive suppressor T cell population without Fc receptors for IgG. Activation of the suppressive function inhibiting of B cells into plasmocytes results from excessive secretion of prostaglandin E2 (PGE 2) by monocytes. We have previously demonstrated that PGE 2 in excess activates certain suppressor T cells. The secretion of PGE 2 by monocytes is thought to be associated with the binding of denatured IgG's to membrane receptors for the corresponding Fc. Activation of suppressor T cells was reproduced in vitro by incubating total T cells or T cells without Fc gamma receptors in the presence of either complete gammaglobulins or of the aggregates they contain. In contrast, preparations deprived of aggregates and F (ab)'2 fragments isolated from gammaglobulins do not produce any detectable activation of suppressor T cells. The results obtained in vitro may not necessarily correspond to the effects of injectable gammaglobulins on humoral response in vivo. We should point out, however, that a fall in plasma IgE levels may be observed in some subjects and that in exceptional cases of IgG and IgA hypergammaglobulinaemia a fall in IgG and IgA has been recorded after gammaglobulin injections.
Antibody Formation↗
Bone-marrow transplantation for inborn error of phagocytic cells associated with defective adherence, chemotaxis, and oxidative response during opsonised particle phagocytosis.
Two girls had delayed umbilical cord detachment, recurrent bacterial infection, inability to form pus, and marked leucocytosis. Their phagocytes were defective in tests of adherence, random migration, chemotaxis, and oxidative burst. NK activity was virtually absent. This rare disorder, due to an inherited absence of a 180 kilodalton membrane glycoprotein on polymorphonuclear cells, is usually lethal within 2 years. Allogeneic HLA-matched bone-marrow transplantation done at ages 4 months in one patient and 2 years in the other after intensive conditioning was successful and resulted in nearly complete correction of phagocytic cell function and NK activity within two months. One patient died 9 months after transplantation from severe chronic graft-versus-host disease with obstructive bronchopneumopathy. The other is doing well 1 year after transplantation and showing stable chimerism and normal phagocytic function.
Bone Marrow Transplantation↗
Cellular immune deficiency in two siblings with hereditary orotic aciduria.
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Child↗
Common variable hypogammaglobulinemia in children. Clinical and immunologic observations in 30 patients.
We made clinical and immunologic observations of 30 children with common variable hypogammaglobulinemia. The mean age at diagnosis was 10.5 years, five years after clinical onset. Diagnosis was initially made based on a history of recurrent otobronchopulmonary infections, diarrhea, or both. The most common complications included short stature, bronchiectasis, and malabsorption, often associated with giardiasis or sprue. Nine patients had associated autoimmune diseases (eg, atrophic gastritis, arthritis, and hemolytic anemia). Three patients died, one of chronic respiratory insufficiency, one of chronic persisting hepatitis, and one of osteogenic sarcoma. Humoral and cellular immune functions of all patients were examined.
Adolescent↗
Tandem translocation t(14;14) in isolated and clonal cells in ataxia telangiectasia are different.
In a patient affected by ataxia telangiectasia (AT), an invading clone with a t(14;14) was found in PHA-, but not in pokeweed-stimulated, lymphocytes. With high resolution R-banding, the proximal breakage was visualized at the junction of bands q11.1-q11.2. This breakpoint differs from that (q12) of noninvading rearrangements of chromosome 14 in AT and non-AT patients. These differences are discussed.
Ataxia Telangiectasia↗
Severe combined immunodeficiency disease: a pathological analysis of 26 cases.
Autopsy material and clinical information were analyzed in 25 cases of untreated or unsuccessfully treated severe combined immunodeficiency disease and one case successfully treated by bone marrow grafting. Two cases were adenosine deaminase deficient and one was nucleoside phosphorylase deficient. The histological appearance of the thymus fell into four clearly recognizable patterns: simple dysplasia, dysplasia with corticomedullary differentiation, dysplasia with pseudoglandular appearance, and atrophic pattern. Three cases lacked lymph nodes and belonged to the category of thymic dysplasia with pseudoglandular appearance. From the data, the following conclusions can be made: (i) The thymic atrophic pattern is a phase in a dynamic process of which the end result is simple dysplasia or dysplasia with corticomedullary differentiation. (ii) The pseudoglandular pattern represents a disease process of early intrauterine onset. (iii) At least a proportion of the cases represent a T-cell defect rather than a lymphoid stem-cell defect. (iv) The lymphoid germinal centers are not the source of plasma cells. (v) The graft-versus-host reaction probably causes lymphoid cells depletion in lymph nodes and spleen.
Autopsy↗