Dallas Allergy Immunology, 7777 Forest Lane, Dallas, TX 75230, USA.
Am J Otolaryngol. 2011 Jul-Aug;32(4):329-37. doi: 10.1016/j.amjoto.2010.05.001. Epub 2010 Aug 17.
The objective of the study was to review the diagnosis and treatment of primary immunodeficiency disease (PID) and the role of otolaryngologists in the management of PID.
A search was conducted of PubMed and the Web sites of organizations for PID patients for literature pertaining to the diagnosis and treatment of PID, with an emphasis on the role of otolaryngologists. The reference lists of selected articles were reviewed for additional articles.
Patients with PID commonly present with respiratory tract infections (eg, recurrent ear, nose, or throat infections) and chest disease. Diagnostic delays or inadequate treatment of PID may lead to significant morbidity and premature mortality. Immunoglobulin (Ig) replacement is the cornerstone of therapy for most patients with PID. Although intravenous Ig is the most popular route of administration in the United States, subcutaneous Ig administration may be appropriate for patients with poor venous access, those who are unable to tolerate intravenous Ig, or those who prefer the independence and flexibility of self-administration.
Recognition and diagnosis of PID by otolaryngologists are critical to optimizing patient outcomes. Several therapeutic regimens for Ig replacement are now available that offer patients increased flexibility and independence.
本研究旨在回顾原发性免疫缺陷病（PID）的诊断和治疗，以及耳鼻喉科医生在 PID 管理中的作用。
通过 PubMed 和 PID 患者组织网站搜索与 PID 的诊断和治疗相关的文献，重点关注耳鼻喉科医生的作用。同时，还对选定文章的参考文献进行了综述，以获取更多相关文章。
PID 患者常因呼吸道感染（如反复耳部、鼻部或喉部感染）和胸部疾病而就诊。PID 的诊断延迟或治疗不当可能导致严重的发病率和过早死亡。免疫球蛋白（Ig）替代疗法是大多数 PID 患者的治疗基石。尽管静脉注射免疫球蛋白（IVIG）在美国是最常用的给药途径，但对于静脉通路不良、无法耐受 IVIG 或希望具有自我管理独立性和灵活性的患者，皮下免疫球蛋白（SCIG）给药可能更为合适。
耳鼻喉科医生对 PID 的识别和诊断对于优化患者的治疗结果至关重要。目前有多种 Ig 替代治疗方案可供选择，为患者提供了更大的灵活性和独立性。
