原发性免疫缺陷:特异性抗体缺乏,IgG 正常。

Vishaka R Hatcher,Veronica C Alix,Tasha S Hellu,Meredith M Schuldt
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摘要

特异性抗体缺乏症(SAD)是一种常见的原发性免疫缺陷疾病,对于反复发作和/或严重鼻窦肺部感染的年长儿童和成人,应考虑该病。其诊断特点是对多糖疫苗(尤其是肺炎球菌疫苗)的抗体反应不足,而对蛋白抗原的反应正常,血清免疫球蛋白和免疫球蛋白 G (IgG) 亚类的水平正常。SAD 的内在机制尚未完全阐明。据了解,幼儿对多糖的反应性有限,随着年龄的增长,这种反应性会逐渐增强。基于这一现象,目前的共识是,2 岁以后免疫系统就已充分成熟,这是确诊 SAD 的最早年龄。目前对多糖无应答的阈值仍未达成共识,有几家商业实验室可以检测一系列血清型,建议患者在接种疫苗前和接种疫苗后都由同一实验室进行血清型检测诊断评估。一旦确诊,将根据临床严重程度对 SAD 进行治疗。临床医生可考虑使用预防性抗生素和免疫球蛋白替代物。应对这些患者进行密切随访,可能在 12-24 个月后停止更换 IgG。与青少年和成人相比,儿童更有可能缓解 SAD。SAD 患者也可能发展为更严重的免疫缺陷;因此,持续监测仍是护理 SAD 患者的一项重要原则。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Primary Immunodeficiency: Specific antibody deficiency with normal IgG.
Specific antibody deficiency (SAD) is a common primary immunodeficiency disorder that should be considered in older children and adults with recurrent and/or severe sinopulmonary infections. The diagnosis is characterized by inadequate antibody response to polysaccharide vaccine, specifically, pneumococcal, with normal responses to protein antigens and normal levels of serum immunoglobulins as well as immunoglobulin G (IgG) subclasses. The underlying mechanism for SAD is not completely elucidated. It is understood that young children have limited polysaccharide responsiveness, which develops with increased age. Due to this phenomenon, the consensus is that there is adequate immune maturity after age 2 years, which is the earliest for the SAD diagnosis to be established. There remains a lack of consensus on thresholds for polysaccharide nonresponse, and there are several commercial laboratories that measure a range of serotypes, with the recommendation for patients to have their diagnostic evaluation with serotype testing both before vaccination and after vaccination to be conducted by the same laboratory. Once a diagnosis has been made, the management of SAD is based on the clinical severity. Clinicians may consider prophylactic antibiotics as well as immunoglobulin replacement. These patients should be closely followed up, with the possibility of discontinuation of IgG replacement after 12 to 24 months. Children are more likely to demonstrate resolution of SAD than are adolescents and adults. Patients with SAD may also progress to a more severe immunodeficiency; therefore, continued monitoring remains a crucial principle of practice in the care of patients with SAD.
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