Division of Immunology, Children's Hospital Boston and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA.
J Allergy Clin Immunol. 2009 Dec;124(6):1161-78. doi: 10.1016/j.jaci.2009.10.013.
More than 50 years after Ogdeon Bruton's discovery of congenital agammaglobulinemia, human primary immunodeficiencies (PIDs) continue to unravel novel molecular and cellular mechanisms that govern development and function of the human immune system. This report provides the updated classification of PIDs that has been compiled by the International Union of Immunological Societies Expert Committee on Primary Immunodeficiencies after its biannual meeting in Dublin, Ireland, in June 2009. Since the appearance of the last classification in 2007, novel forms of PID have been discovered, and additional pathophysiology mechanisms that account for PID in human beings have been unraveled. Careful analysis and prompt recognition of these disorders is essential to prompt effective forms of treatment and thus to improve survival and quality of life in patients affected with PIDs.
奥格登·布鲁顿发现先天性无丙种球蛋白血症 50 多年后，人类原发性免疫缺陷病（PIDs）仍在不断揭示新的分子和细胞机制，这些机制控制着人类免疫系统的发育和功能。本报告提供了国际免疫学协会专家委员会在 2009 年 6 月于爱尔兰都柏林举行的两年一次会议后，对 PIDs 进行的最新分类。自 2007 年出现上一次分类以来，已经发现了新形式的 PID，并且已经揭示了导致人类 PID 的其他病理生理学机制。对这些疾病进行仔细分析和及时识别对于及时采用有效的治疗方法至关重要，从而提高患有 PIDs 的患者的生存率和生活质量。
