Department of Paediatrics, Jeroen Bosch Hospital, 's-Hertogenbosch, The Netherlands.
Eur J Pediatr. 2011 Feb;170(2):169-77. doi: 10.1007/s00431-010-1358-5. Epub 2010 Dec 18.
Primary immunodeficiencies (PIDs) are characterized by an increased susceptibility to infections due to defects in one ore more components of the immune system. Although most PIDs are relatively rare, they are more frequent than generally acknowledged. Early diagnosis and treatment of PIDs save lives, prevent morbidity, and improve quality of life. This early diagnosis is the task of the pediatrician who encounters the child for the first time: he/she should suspect potential PID in time and perform the appropriate diagnostic tests. In this educational paper, the first in a series of five, we will describe the most common clinical presentations of PIDs and offer guidelines for the diagnostic process, as well as a brief overview of therapeutic possibilities and prognosis.
原发性免疫缺陷病（PIDs）的特征是由于免疫系统的一个或多个成分的缺陷而导致易感性增加。尽管大多数 PID 相对罕见，但它们比通常承认的更为频繁。PID 的早期诊断和治疗可以挽救生命，预防发病，并提高生活质量。这种早期诊断是首次遇到孩子的儿科医生的任务：他/她应该及时怀疑潜在的 PID，并进行适当的诊断测试。在这篇教育论文中，是五个系列中的第一篇，我们将描述 PID 的最常见临床表现，并提供诊断过程的指南，以及治疗可能性和预后的简要概述。
