Matthews W J, Williams M, Oliphint B, Geha R, Colten H R
N Engl J Med. 1980 Jan 31;302(5):245-9. doi: 10.1056/NEJM198001313020501.
To investigate some aspects of immune function in cystic fibrosis, we measured serum immunoglobulins in 419 patients. Twenty-two per cent of the 154 patients less than 10 years old had hypogammaglobulinemia-G, whereas the older patients had normal or elevated serum immunoglobulins. A single mechanism accounting for the extraordinary prevalence of hypogammaglobulinemia in young patients with cystic fibrosis was not defined in studies of T and B-lymphocyte function in vitro or in studies of IgG metabolism in vivo. Analysis of objective clinical data, including arterial blood gases, chest roentgenograms, and bacteriologic cultures, indicated that the patients with hypogammaglobulinemia had significantly less severe lung disease than did age-matched patients with cystic fibrosis and normal or elevated IgG levels. We conclude that progression of lung disease may be due in part to a hyper-immune response.
为了研究囊性纤维化患者免疫功能的某些方面，我们检测了419例患者的血清免疫球蛋白。154例年龄小于10岁的患者中，22%患有低丙种球蛋白血症-G，而年龄较大的患者血清免疫球蛋白正常或升高。在体外T和B淋巴细胞功能研究或体内IgG代谢研究中，未发现单一机制可解释年轻囊性纤维化患者低丙种球蛋白血症的异常高患病率。对包括动脉血气、胸部X线片和细菌培养在内的客观临床数据进行分析表明，与年龄匹配的囊性纤维化患者且IgG水平正常或升高的患者相比，低丙种球蛋白血症患者的肺部疾病严重程度明显较低。我们得出结论，肺部疾病的进展可能部分归因于免疫反应过度。
