1/12. Cryptococcal osteomyelitis and cellular immunodeficiency associated with interleukin-2 deficiency.We describe an unusual example of cellular immunodeficiency associated with interleukin-2 deficiency in an otherwise healthy 15-year-old boy who had isolated cryptococcal osteomyelitis of the scapula at 10 years of age. His previous medical history was remarkable only for prolonged, severe varicella infection at 6 years of age. He had persistent moderate lymphopenia, anergy, and absent lymphocyte blastogenic responses to mitogens, antigens, or monoclonal T cell antibodies. Subnormal blastogenic responses were seen after exposure to high concentrations of phorbol esters. Immunoglobulin levels and specific antibodies were normal. The patient has been in good health since treatment of his osteomyelitis. However, his lymphocyte blastogenic responses to mitogens have remained absent during 4 years of observation; investigation of the cause revealed a specific interleukin-2 deficiency resulting from defective generation of interleukin-2 messenger ribonucleic acid. Secretion of interleukin-1 by monocytes was normal, suggesting that the abnormal blastogenic response and interleukin-2 production were due to a problem intrinsic to T lymphocytes. The generation of messenger ribonucleic acid for interleukin-4 was not affected. interferon-gamma was produced at subnormal levels. The addition of recombinant interleukin-2 restored lymphocyte blastogenic responses and increased the expression of interleukin-2 receptors. The clinical findings and immunologic abnormalities present in this patient differ from other primary and secondary immunodeficiencies associated with interleukin-2 deficiency. Thus our observations in this patient extend the spectrum of immunodeficiencies associated with abnormalities in the production of this important cytokine.- - - - - - - - - - ranking = 1keywords = varicella (Clic here for more details about this article) |
2/12. Reovirus type 2 isolated from cerebrospinal fluid.An 8-week-old female infant presented with a history of active varicella complicated by escherichia coli sepsis, oral thrush, hypoalbuminemia, intermittent fevers, diarrhea and feeding intolerance. Rhesus monkey kidney cells inoculated with cerebrospinal fluid revealed reovirus-like particles by electron microscopy. Virus neutralization and rna-gel electrophoresis studies identified the isolated pathogen as reovirus serotype 2. This report represents one of only a few to isolate reovirus from the central nervous system in humans.- - - - - - - - - - ranking = 1keywords = varicella (Clic here for more details about this article) |
3/12. vidarabine therapy for severe herpesvirus infections. An unusual syndrome of chronic varicella and transient immunologic deficiency.Six patients with severe herpesvirus infections were successfully treated with vidarabine. One patient had a previously undescribed syndrome of chronic cutaneous varicella infection of eight months' duration, associated with transient but complete duppression of lymphocyte response to conconavalin A. Other diagnoses were severe varicella pneumonia, progressive cytomegalovirus pneumonia associated with acute lymphocytic leukemia, herpes simplex encephalitis, severe zoster associated with stage IV lymphoma, and disseminated herpes simplex in a patient receiving high doses of steroids. All patients showed cessation of new lesions or abrupt clinical improvement between days 2 and 4 after initiation of therapy, and all were cured of their clinical infection. Dramatic improvement in all of our patients and the minimal toxicity observed make vidarabine suitable for use in severe herpesvirus infections.- - - - - - - - - - ranking = 6keywords = varicella (Clic here for more details about this article) |
4/12. A novel X-linked combined immunodeficiency disease.A novel X-linked combined immunodeficiency disease was found in five living males in an extended family in the united states. The age of the affected males ranged from 2.5 to 34 yr. The most prominent clinical abnormalities were a paucity of lymphoid tissue; recurrent sinusitis, otitis media, bronchitis, and pneumonia; severe varicella; and chronic papillomavirus infections. The principal immunologic features of the disorder were normal concentrations of serum immunoglobulins but restricted formation of IgG antibodies to immunogens; normal numbers of B cells and NK cells but decreased numbers of CD4 and CD8 T lymphocytes, particularly the CD45RA subpopulations; diminished proliferative responses of blood T cells to allogeneic cells, mitogens and antigens; and decreased production of IL-2 by mitogen stimulated blood lymphocytes. Thus, affected males in this family carry an abnormal gene on their x chromosome that results in a combined immunodeficiency that is distinct from previously reported disorders.- - - - - - - - - - ranking = 1keywords = varicella (Clic here for more details about this article) |
5/12. Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome).An 8-year-old male underwent excision of an encapsulated thymoma. Four months later he presented with disseminated, fatal varicella. Evaluation of his immune status during the terminal illness revealed hypogammaglobulinemia and lymphopenia consistent with a diagnosis of Good's syndrome (immunodeficiency with thymoma). This is the first case of Good's syndrome reported in a child and the first case of fatal varicella associated with Good's syndrome. The combination of this rare pediatric tumor and immunodeficiency is discussed. Despite specific antiviral therapy, varicella remains a deadly disease in the immunocompromised host.- - - - - - - - - - ranking = 7keywords = varicella (Clic here for more details about this article) |
6/12. Reinfection with varicella-zoster virus in immunocompromised patients.A small epidemic of varicella/atypical generalized zoster among 6 immunocompromised patients and one previously healthy person is described. The 6 immunocompromised patients suffered from lymphoproliferative diseases in terminal stages treated with chemotherapy and reported varicella in their childhood. They developed a generalized maculopapular rash with hemorrhagic bullae and necroses. The infection passed from one patient to another during a 3-month period in the department. They were placed in adjacent rooms and nursed by the same staff. The most specific diagnostic tool was the detection of varicella-zoster virus antigen from vesicles by ELISA technique. The epidemic was supposed to be caused by exogenous reinfection with varicella-zoster virus, and illustrated that generalized zoster may be even so infectious as varicella and that immunocompromised patients should be protected against reinfection.- - - - - - - - - - ranking = 9keywords = varicella (Clic here for more details about this article) |
7/12. Varicella pneumonia in a bone marrow-transplanted, immune-reconstituted adenosine deaminase-deficient patient with severe combined immunodeficiency disease.bone marrow transplantation provides an important modality for "enzyme replacement" and the immune reconstitution of patients with adenosine deaminase (ADA) deficiency and severe combined immunodeficiency disease. We report a patient with ADA deficiency who develops severe varicella pneumonia 6 years after successful bone marrow transplantation and immune reconstitution. Marked abnormalities in T-cell mitogen responsiveness and pokeweed mitogen-induced polyclonal immunoglobulin synthesis occurred. Coculture experiments suggested the presence of increased suppressor activity. T-cell phenotyping showed decreased T3 and T4 subsets. These abnormalities slowly resolved over several months as the patient recovered from the varicella infection. ADA enzyme levels and metabolite concentrations in urine and erythrocytes remained unchanged. These findings, together with the chromosome and immune studies, suggested that the bone marrow graft remained intact. These studies indicate that immunologically reconstituted ADA-deficient patients may be at higher risk for complications related to varicella infection and suggest that the institution of preventive measures is important.- - - - - - - - - - ranking = 3keywords = varicella (Clic here for more details about this article) |
8/12. An inverted ratio for T-helper/T-suppressor cells, and selective deficiency of cell-mediated immunity, in a girl with cartilage hair hypoplasia.Clinical and immunological data are presented in a thirteen-year-old girl with cartilage-hair hypoplasia. From early infancy, the girl suffered from recurrent respiratory infections, and at ten years of age she had a severe and protracted attack of varicella. Immunologic investigations revealed very low mitogenic responses to PHA and ConA and an inverted ratio for T-helper/T-suppressor cells. However, antibody-mediated immunity was normal. The results emphasize that selective cell-mediated immune dysfunction, which is most often found in short-limbed dwarfism with cartilage-hair hypoplasia, may be due to increased T-suppressor- as well as decreased T-helper cell activity.- - - - - - - - - - ranking = 1keywords = varicella (Clic here for more details about this article) |
9/12. Generalized varicella in severe combined immunodeficiency with B lymphocytes.A six-month-old male infant, whose elder brother had died of progressive vaccinia due to combined immunodeficiency, contracted varicella-zoster infection, and died of disseminated varicella ten days after onset of the disease. As with his elder brother, the blood levels of immunoglobulins were normal, and specific varicella antibodies appeared in his serum, although in low concentrations. His t-lymphocytes, although their number was subnormal, could be stimulated with phytohaemagglutinin, and production of migration inhibitory factor could also be demonstrated. The necropsy confirmed thymic dysplasia. In addition to histological changes, severe combined immunodeficiency and foci of malignant lymphoma were present. On this basis the disease was classified, according to the WHO recommendations, as severe combined immunodeficiency with B lymphocytes, complicated by malignant lymphoma.- - - - - - - - - - ranking = 7keywords = varicella (Clic here for more details about this article) |
10/12. A family of selective immunodeficiency with normal immunoglobulins: possible autosomal dominant inheritance.We report here our findings in two Japanese siblings who experienced recurrent bacterial and viral infections since early infancy. Recent symptoms included diarrhoea, conjunctivitis, rashes, headache, sore throat, joint pain, vomiting and vertigo, all similar to those seen in toxic shock syndrome, except for shock. These symptoms improved following gammaglobulin treatment. staphylococcus aureus with coagulase type IV was continuously isolated from nasal smears producing toxic shock syndrome toxin-1 (TSST-1). serum antibodies did not or only poorly responded to TSST-1, diphtheria toxoid, varicella virus and rubella virus, whereas total and subclass levels of serum immunoglobulin and in vitro dna synthesis of lymphocytes stimulated by TSST-1, Staph. aureus, varicella vaccine and mitogens were normal. In the family, ten other members in three generations (five males: five females) including the mother had similar clinical symptoms. Thus, the disease may be inherited in an autosomal dominant fashion.- - - - - - - - - - ranking = 2keywords = varicella (Clic here for more details about this article) |
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