Garside J P, Kerrin D P, Brownlee K G, Gooi H C, Taylor J M, Conway S P
Department of Paediatrics and Child Health, Leeds General Infirmary, Leeds, UK.
Pediatr Pulmonol. 2005 Feb;39(2):135-40. doi: 10.1002/ppul.20050.
The aim of this study was to report serum immunoglobulin (Ig) and IgG subclass levels in a large pediatric population with cystic fibrosis, and relate these to measures of disease severity. Total immunoglobulin levels were measured in 154 patients, and IgG subclass levels were measured in 136 patients and compared to age-related normal population data and to levels reported in previously published studies of children with cystic fibrosis. Clinical data were also collected: genotype; height, weight, and BMI standard deviation scores; FEV(1) (as percent predicted); Shwachmann-Kulczycki (S-K) and Northern chest X-ray scores; and Pseudomonas aeruginosa infection status. The clinical well-being of patients with hypo- or hyper-gammaglobulinemia was compared with age- and sex-matched control patients who had normal levels of gammaglobulin. IgG subclass levels were measured, and the results were compared with previous studies. Eleven patients had hypergammaglobulinemia (7.8% compared with 0-69% in the published literature). Patients with hypergammaglobulinemia had lower FEV(1) percent-predicted values, and worse S-K and Northern chest X-ray scores than controls. Three patients had hypogammaglobulinemia (1.9% compared with 0-10.8% in the published literature). There was no difference in any clinical parameter between controls and those with hypogammaglobulinemia. Nineteen patients (14%) had low levels of IgG1, and 40 patients (29%) had low levels of IgG2. The low percentage of patients with abnormally high immunoglobulin levels probably reflects the improved respiratory status of today's children with CF. The low percentage of those with low IgG probably reflects better nutritional status. The finding of worse lung function and clinical scores in patients with hypergammaglobulinemia agrees with the published literature. The high percentage of patients with low IgG2 was unexpected and was not previously reported. The clinical significance of this in patients with CF is unknown.
本研究旨在报告一大群患有囊性纤维化的儿科患者的血清免疫球蛋白（Ig）和IgG亚类水平，并将这些水平与疾病严重程度指标相关联。对154例患者测量了总免疫球蛋白水平，对136例患者测量了IgG亚类水平，并将其与年龄相关的正常人群数据以及先前发表的囊性纤维化儿童研究中报告的水平进行比较。还收集了临床数据：基因型；身高、体重和BMI标准差分数；FEV(1)（预测百分比）；施瓦赫曼 - 库尔奇茨基（S - K）和胸部X线北部分数；以及铜绿假单胞菌感染状态。将低丙种球蛋白血症或高丙种球蛋白血症患者的临床状况与丙种球蛋白水平正常的年龄和性别匹配的对照患者进行比较。测量了IgG亚类水平，并将结果与先前的研究进行比较。11例患者患有高丙种球蛋白血症（7.8%，而文献报道为0 - 69%）。高丙种球蛋白血症患者的FEV(1)预测百分比值较低，S - K和胸部X线北部分数比对照组差。3例患者患有低丙种球蛋白血症（1.9%，而文献报道为0 - 10.8%）。对照组与低丙种球蛋白血症患者在任何临床参数上均无差异。19例患者（14%）的IgG1水平较低，40例患者（29%）的IgG2水平较低。免疫球蛋白水平异常高的患者比例较低可能反映了当今囊性纤维化儿童呼吸状况的改善。IgG水平低的患者比例较低可能反映了更好的营养状况。高丙种球蛋白血症患者肺功能和临床评分较差的发现与已发表的文献一致。IgG2水平低的患者比例较高出乎意料，且此前未被报道。这在囊性纤维化患者中的临床意义尚不清楚。
