Strober W, Wochner R D, Barlow M H, McFarlin D E, Waldmann T A
J Clin Invest. 1968 Aug;47(8):1905-15. doi: 10.1172/JCI105881.
Immunoglobulin metabolism has been studied in five patients with ataxia telangiectasia and in control subjects. Serum IgG levels were normal, increased, or decreased, reflecting normal, increased, or decreased synthetic rates, respectively. Serum IgM concentration was normal in three cases and slightly elevated in two cases. IgM turnover studies in the three cases with normal serum IgM levels showed normal IgM synthetic and catabolic rates. None of the five patients with ataxia telangiectasia had detectable serum IgA, and the maximum IgA synthetic rates possible for these patients were 0.3-10% of the normal mean synthetic rate (24 +/- 15 mg/kg per day) of 12 control individuals. Three of the patients had normal IgA fractional catabolic rates: 22% of the intravascular pool per day vs. 25 +/- 4% in controls. In two patients, fractional catabolic rates 4 and 20 times normal were found. In these cases, metabolic turnover, in vitro precipitation, radioimmunoelectrophoresis, and (or) the C'la fixation and transfer test provided evidence for the presence of a circulating antibody directed against IgA causing immune elimination of the molecule. These studies suggest that therapy with exogenous IgA may not be possible in some patients with ataxia telangiectasia or in other subjects with dysgammaglobulinemia.
对5例共济失调毛细血管扩张症患者和对照受试者的免疫球蛋白代谢情况进行了研究。血清IgG水平正常、升高或降低，分别反映合成率正常、升高或降低。血清IgM浓度在3例患者中正常，2例患者略有升高。对3例血清IgM水平正常的患者进行的IgM周转率研究显示，IgM的合成和分解代谢率正常。5例共济失调毛细血管扩张症患者中无一例检测到血清IgA，这些患者可能的最大IgA合成率为12名对照个体正常平均合成率（24±15mg/kg/天）的0.3 - 10%。其中3例患者的IgA分数分解代谢率正常：每天血管内池的22%，而对照为25±4%。在2例患者中，发现分数分解代谢率分别为正常的4倍和20倍。在这些病例中，代谢周转率、体外沉淀、放射免疫电泳和（或）C'la固定及转移试验为存在针对IgA的循环抗体导致该分子的免疫清除提供了证据。这些研究表明，在一些共济失调毛细血管扩张症患者或其他伴有球蛋白异常血症的受试者中，可能无法进行外源性IgA治疗。
