qin_130104免疫缺陷病.pptVIP

治疗： 目前用于治疗AIDS的药物有叠氮脱氧胸苷（AZT）、苏拦明（Suramin)、双脱氧胞苷 (ddc)、双脱氧面苷(ddl )等。主要通过干扰病毒DNA合成，从而抑制HIV在体内增殖，缓解症状，延长病人生存期。 此外，发现许多抑制蛋白酶、阻止HIV与靶细胞结合或融合的药物，能分别作用于细胞感染的不同阶段，以达到抗HIV的效果，均尚处于研究阶段。 中草药中发现括贝母苷、甘草甜素、及地丁、空心苋、紫草等抽提物有抑HIV的作用。能缓解症状，还在研究和总结中。 免疫缺陷病的诊断和治疗 家族史对于原发性免疫缺陷病的诊断上十分重要，也为确定合适的治疗方案提供重要的依据。 治疗上主要采取的方法 一．抗感染 抗生素治疗 二．输入免疫球蛋白或免疫细胞 替代疗法，而无法重建免疫功能 三．骨髓移植 干细胞移植，使受损的免疫系统达到免疫重建，用于治疗致死性免疫缺陷病 四．基因治疗 将外源基因导入受者的淋巴细胞再回输患者体内，可产生正常的基因产物替代缺失或不正常的基因产物 掌握IDD, PIDD, SIDD, CID, SCID, AIDS的概念 IDD的基本特征 IDD的分类 HIV引起AIDS的机制 IDD的治疗原则 免疫：识别和排除抗原性异物的功能 * * XLA: Bruton 酪氨酸激酶缺陷，B细胞发育停滞在前B * * Under normal circumstances, the complex is latent in neutrophils and is activated to assemble in the membranes during respiratory burst. NADPH oxidase generates superoxide by transferring electrons from NADPH inside the cell across the membrane and coupling these to molecular oxygen to produce superoxide anion, a reactive free-radical. Superoxide can be produced in phagosomes, which contain ingested bacteria and fungi, or it can be produced outside of the cell. In a phagosome, superoxide can spontaneously form hydrogen peroxide that will undergo further reactions to generate reactive oxygen species (ROS). Superoxide kills bacteria and fungi by mechanisms that are not yet fully understood (see article on superoxide), but may inactivate critical metabolic enzymes, initiate lipid peroxidation, and liberate redox-active iron, which allows the generation of indiscriminate oxidants such as the hydroxyl radical. It is presumed that superoxide kills bacteria directly, as the virulence of many pathogens is dramatically attenuated when their superoxide dismutase (SOD) genes are deleted. However, downstream products of superoxide also include hydrogen peroxide and hypochlorous acid, the reactive agent in bleach. * HIV infection is associated with immunosuppression caused by a dramatic reduction in the helper T cell population. The loss of helper T cells may be caused