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Immune Network ; : 352-364, 2017.
Article in English | WPRIM | ID: wpr-195868


Hyper-IgE syndrome (HIES) is a very rare primary immune deficiency characterized by elevated serum IgE levels, recurrent bacterial infections, chronic dermatitis, and connective tissue abnormalities. Autosomal dominant (AD) HIES involves a mutation in signal transducer and activator of transcription 3 (STAT3) that leads to an impaired T(H)17 response. STAT3 signaling is also involved in the function of RORγt⁺ type 3 innate lymphoid cells (ILC3s) and RORγt⁺T(H)17 cells. The aim of this study was to investigate the role of innate immune cells such as innate lymphoid cells (ILCs), granulocytes, and monocytes in a patient with HIES. Peripheral blood mononuclear cells (PBMCs) from a patient with HIES and three age-matched healthy controls were obtained for the analysis of the innate and adaptive immune cells. The frequencies of ILCs in PBMCs were lower in the patient with HIES than in the controls. Moreover, granulocyte-macrophage colony-stimulating factor (GM-CSF) and IL-17A produced by ILC3s in PBMCs were lower in the patient with HIES than the controls. Compared with the controls, classical monocytes (CD14⁺CD16(low)), which have a high antimicrobial capability, were also lower in the patient with HIES, while non-classical monocytes (CD14(low)CD16⁺) as well as intermediate monocytes (CD14⁺CD16(intermediate)) were higher. Taken together, these results indicate that the impaired immune defense against pathogenic microbes in the patient with HIES might be partially explained by functional defects in ILC3s and inflammatory monocytes.

Humans , Bacterial Infections , Connective Tissue , Cytokines , Dermatitis , Granulocyte-Macrophage Colony-Stimulating Factor , Granulocytes , Immunity, Innate , Immunoglobulin E , Interleukin-17 , Job Syndrome , Lymphocytes , Monocytes , STAT3 Transcription Factor
International Journal of Pediatrics ; (6): 710-713, 2017.
Article in Chinese | WPRIM | ID: wpr-666824


Objective To observe the efficacy of bacterial lysate(bronchovaxom) in the acute phase of respiratory tract infection and to prevent recurrent respiratory tract infections.Methods From April 2014 to A-pril 2015,in pediatric respiratory ward of Shengjing hospital of China Medical University,80 children with recur-rent respiratory tract infection were randomly divided into treatment group and control group,with 40 cases in each group.The control group patients were received routine anti-infection and symptomatic treatment,the treat-ment group patients were received oral bacterial lysis products for 3 months on basis of the conventional anti-in-fection and symptomatic treatment.To observe the duration of clinical symptoms of two groups of children with respiratory tract infection in the acute phase,antibiotic use and hospital stay changes;observed the number of re-spiratory infections occurred again within 1 year;measured the levels of serum immunoglobulin before and 6 months after treatment. Results Compared with the control group,the duration of symptoms such as fever, cough and other symptoms of respiratory tract infection,the duration of pulmonary rales,the use of antibiotics and the length of hospital stay were significantly shortened in the treatment group,and the number of respiratory infections was significantly reduced,the difference significant(P<0.05).After treatment,the levels of IgG and IgA in the treatment group were significantly higher than those before treatment,and the immunoglobulin IgG and IgA in the treatment group were significantly higher than those in the control group(P<0.05).Conclusion Oral bacterial lysis products to prevent respiratory infections,not only can reduce the duration of various clini-cal symptoms of respiratory tract infection,shorten the use of antibiotics and hospital stay,and can improve chil-dren's immunity,reduce the number of respiratory infections again.