Diana García de Olarte

Primary Immunodeficiency Diseases in Latin America: First Report from Eight Countries Participating in the LAGID

The Latin American Group for Primary Immunodeficiencies, formed in 1993, presently includes 12 countries. One goal was to study the frequency of primary immunodeficiencies in various regions of the American continent and to enhance knowledge about these diseases among primary-care physicians, as well as allergist–immunologists. Important for this purpose was the development of a registry of primary immunodeficiencies using a uniform questionnaire and computerized database. To date, eight countries have collected information on a total of 1428 patients. Predominantly antibody deficiencies were reported in 58% of patients, followed by cellular and antibody immunodeficiencies associated with other abnormalities in 18%, immunodeficiency syndromes associated with granulocyte dysfunction in 8%, phagocytic disorders in 9%, combined cellular and antibody immunodeficiencies in 5%, and complement deficiencies in 2% of patients. The information gathered from this initial analysis of data will serve to expand the patient database to more areas within participating countries and to new countries and to increase collaboration toward better diagnosis and treatment of these diseases.

Lung Puncture-Aspiration as a Bacteriologic Diagnostic Procedure in Acute Pneumonias of Infants and Children

Lung puncture-aspiration is simple and generally innocuous. For the study of acute pneumonia it is superior to examination of naso pharyngeal, throat or laryngeal cultures and gives more positive re sults than does blood culture. It can be extremely useful in investi gational studies and with selected patients in whom identification of the precise pathogen is necessary for optimal antibiotic therapy.Lung puncture-aspiration is simple and generally innocuous. For the study of acute pneumonia it is superior to examination of naso pharyngeal, throat or laryngeal cultures and gives more positive re sults than does blood culture. It can be extremely useful in investi gational studies and with selected patients in whom identification of the precise pathogen is necessary for optimal antibiotic therapy.

Interleukin 4 and Interferon-Gamma Secretion by Antigen and Mitogen-Stimulated Mononuclear Cells in the Hyper-IgE Syndrome: No TH-2 Cytokine Pattern

BACKGROUND Enhanced production of TH-2 cytokines plays a key role in increased IgE production in allergic diseases. Reports about the cytokine profile secreted by peripheral blood mononuclear cells of patients with hyper-IgE syndrome, however, are controversial, suggesting alternative causes for increased IgE production in this syndrome. OBJECTIVE We wished to determine whether mononuclear cells from patients with hyper-IgE syndrome have a pattern of interleukin-4 (IL-4) and interferon-gamma (IFN-gamma) production characteristic of a predominance of TH-2 cells and whether the cytokine production pattern is constant over time. METHODS IL-4 and IFN-gamma secretion by peripheral blood mononuclear cells stimulated with phytohemagglutinin and D. pteronyssinus was measured by ELISA in culture supernatants. Patients with the hyper-IgE syndrome were evaluated 3 times at 4-week intervals and compared with asthmatic patients and normal subjects. RESULTS In PHA-stimulated cultures, patients with hyper-IgE syndrome had an IL-4 and IFN-gamma secretion similar to that of controls, while asthmatic patients had increased IL-4 and decreased IFN-gamma production. Cultures stimulated with D. pteronyssinus showed a variable pattern of secretion for both cytokines. CONCLUSIONS In allergic diseases, increased serum IgE level is the result of a TH-2 pattern of cytokine production, with high IL-4 and decreased IFN-gamma protein secretion. The increased serum IgE concentration typical of the hyper-IgE syndrome is likely the result of a different immunoregulatory process.

A Study of Granulocyte Respiratory Burst in Patients with Allergic Bronchial Asthma

The respiratory burst of phagocytes plays an important role in the tissue damage that accompanies the inflammatory response. One of these conditions is allergic bronchial asthma, therefore, to evaluate the activation state of peripheral granulocytes the generation of reactive oxygen metabolites was evaluated using Luminol-enhanced chemiluminescence (LCL) and reduction of cytochrome C by superoxide. The resting granulocytes of the asthmatic patients under crisis showed a higher LCL compared to the noncrisis patients and control subjects. The granulocytes stimulated with PMA presented a significant increase in the respiratory burst in both groups of asthmatics. The granulocytes of noncrisis asthmatics challenged with Ops-Zym and with fMLP+Ops-Zym showed a higher metabolic activity, whereas the asthmatics under crisis presented no difference between reactive oxygen generation and that of the control group. The quantitative analysis of superoxide generation by granulocytes of the same patients did not show differences among the groups. Our findings suggest that the granulocytes of crisis and noncrisis asthmatics seem to be in a hyperreactive state and with a higher metabolic response when compared to the control group. However, the patients present a different behavior depending on stimulus used to activate cells. This could indicate that in peripheral blood exist different granulocyte populations depending on the inflammatory response taking place in the respiratory tract.

Increase in granulocyte-macrophage-colony-stimulating factor secretion and the respiratory burst with decreased l-selectin expression in hyper-IgE syndrome patients

BACKGROUND The hyper-IgE syndrome is a primary immunodeficiency characterized by severe recurrent abscesses, pneumonia with pneumatocele formation, and elevated serum IgE. Eosinophilia, neutrophil chemotactic defects, and marked tissue damage are frequently present in this syndrome. OBJECTIVE To study whether functional changes in cytokines, adhesion molecules, and neutrophils might help explain these clinical observations. METHODS The following functions were analyzed in patients with the hyper-IgE syndrome and in controls: (1) production of granulocyte-macrophage-colony-stimulating factor by peripheral blood mononuclear cells by ELISA; (2) respiratory burst and reactive oxygen intermediates production by peripheral neutrophils using the luminol-enhanced chemiluminescense technique; and (3) expression of L-selectin on granulocytes and lymphocytes by flow cytometry. RESULTS Patients with hyper-IgE syndrome had significantly increased production of granulocyte-macrophage-colony-stimulating factor by resting or stimulated mononuclear cells, increased generation of reactive oxygen intermediates by neutrophils treated with opsonized zymosan, and reduced L-selectin expression on quiescent and activated granulocytes and lymphocytes. CONCLUSIONS Our results suggest that an important feature of the hyper-IgE syndrome is the increased production of granulocyte-macrophage-colony-stimulating factor, which may explain the reduced L-selectin expression, decreased chemotaxis, and increased oxygen radical production and tissue damage in this disease.