tailieunhanh - Báo cáo y học: "Chronic granulomatous disease: a review of the infectious and inflammatory complications"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học Critical Care giúp cho các bạn có thêm kiến thức về ngành y học đề tài: Chronic granulomatous disease: a review of the infectious and inflammatory complications. | Song et al. Clinical and Molecular Allergy 2011 9 10 http content 9 1 10 REVIEW CMA CLINICAL AND MOLECULAR ALLERGY Open Access Chronic granulomatous disease a review of the infectious and inflammatory complications EunKyung Song1 Gayatri Bala Jaishankar1 Hana Saleh3 Warit Jithpratuck3 Ryan Sahni3 and Guha Krishnaswamy1 2 3 Abstract Chronic Granulomatous Disease is the most commonly encountered immunodeficiency involving the phagocyte and is characterized by repeated infections with bacterial and fungal pathogens as well as the formation of granulomas in tissue. The disease is the result of a disorder of the NADPH oxidase system culminating in an inability of the phagocyte to generate superoxide leading to the defective killing of pathogenic organisms. This can lead to infections with Staphylococcus aureus Psedomonas species Nocardia species and fungi such as Aspergillus species and Candida albicans . Involvement of vital or large organs can contribute to morbidity and or mortality in the affected patients. Major advances have occurred in the diagnosis and treatment of this disease with the potential for gene therapy or stem cell transplantation looming on the horizon. Introduction Primary immune deficiencies often present as recurrent infections often with unusual pathogens or infections of unusual severity or frequency. Host defense consists of either nonspecific or specific mechanisms of immunity to invading pathogens Table 1 . Nonspecific mechanisms include barrier functions of skin and mucosa tears saliva mucosal secretions mucociliary responses and clearance by peristalsis as occurs in the bladder or the gastro-intestinal system phagocyte responses neutrophils macrophages or mononuclear cells and their component of pathogen recognition receptors or PRR and cell adhesion molecules or CAMs complement system of proteins C reactive protein CRP cytokines and the PRRs mentioned earlier on the surface of a variety of cell types. .