Dressler F, Peter H H, Müller W, Rieger C H
Department of Pediatrics, Hannover Medical School, FRG.
Acta Paediatr Scand. 1989 Sep;78(5):767-74. doi: 10.1111/j.1651-2227.1989.tb11141.x.
The clinical and immunological data of 5 patients with transient h hypogammaglobulinemia of infancy (THI) are presented and compared to published data. In THI, there is distinction between patients who can mount a specific immune response, by producing specific antibodies to bovine serum albumin, diphtheria toxin, and isohemagglutinins, and patients with more severe forms of immunodeficiency. With only 5 reported cases of THI in 11 years and analysis of more than 8000 sera-, our data support the notion that THI is a relatively rare disorder. THI can only be diagnosed with certainty in retrospect, although the ability to mount an active, specific immune response is a strong indicator for the diagnosis. We propose a more accurate definition of THI to help avoid clinical misjudgement.
本文呈现了5例婴儿暂时性低丙种球蛋白血症（THI）患者的临床和免疫学数据，并与已发表的数据进行了比较。在THI中，能够通过产生针对牛血清白蛋白、白喉毒素和同种血凝素的特异性抗体来引发特异性免疫反应的患者，与免疫缺陷更严重形式的患者有所不同。在11年里仅报告了5例THI病例，且对8000多份血清进行了分析，我们的数据支持THI是一种相对罕见疾病的观点。尽管能够引发活跃的特异性免疫反应是诊断的有力指标，但THI只能在回顾时才能确切诊断。我们提出了一个更准确的THI定义，以帮助避免临床误诊。
