Lieberman J, Tachiki K H
Department of Medicine, Veterans Administration Medical Center, Sepulveda, CA 91343.
Chest. 1989 Jan;95(1):111-6. doi: 10.1378/chest.95.1.111.
Twelve CF heterozygous and two CF homozygous women who were tested serially for the CF-lectin activities during one to two months of their menstrual cycles, were found consistently to have negative tests during menses. The specific hormonal alteration during menses that affects the assay is unknown. However, estrogenic medication administered without progesterone to postmenopausal heterozygous women also caused false-negative tests, suggesting that a balance between progesterone and estrogen is critical for the CF-lectin activity; mere depletion of these hormones alone does not interfere. The addition of estrogen in vitro also inhibited the CF-lectin activity at a physiologic concentration of 10(-6) M, and addition of an equivalent concentration of progesterone blocked this inhibitory effect. Future studies of the CF-lectin or its assay as a potential CF-carrier test must be limited to those women not menstruating at the time of blood drawing and not receiving estrogens. Hemolysis or contamination of the serum with RBC should invalidate that sample for testing, although the effect of RBC contamination can be circumvented with the addition of mannose. A more objective, simplified means of performing the CF-lectin assay is reported. A preliminary blind study taking these issues into consideration was 100 percent correct in detecting 16 CF patients and 17 obligate heterozygotes, and revealed 4 percent of 64 control subjects to have positive tests. The presence of this lectin-like factor may reflect the underlying biochemical defect responsible for this disease.
12名囊性纤维化(CF)杂合子女性和2名CF纯合子女性在月经周期的1至2个月内连续接受CF凝集素活性检测,结果发现她们在月经期间检测结果始终为阴性。月经期间影响该检测的具体激素变化尚不清楚。然而,对绝经后杂合子女性单独使用雌激素药物也会导致假阴性检测结果,这表明孕酮和雌激素之间的平衡对CF凝集素活性至关重要;仅这些激素的减少本身并不会产生干扰。在体外添加生理浓度为10(-6) M的雌激素也会抑制CF凝集素活性,而添加同等浓度的孕酮可阻断这种抑制作用。未来将CF凝集素或其检测作为潜在CF携带者检测的研究必须仅限于在抽血时未处于月经期且未接受雌激素治疗的女性。溶血或血清被红细胞污染应使该样本检测无效,不过添加甘露糖可避免红细胞污染的影响。据报道,有一种更客观、简化的CF凝集素检测方法。一项考虑到这些问题的初步盲法研究在检测16名CF患者和17名必然杂合子方面的准确率为100%,并显示64名对照受试者中有4%检测结果为阳性。这种凝集素样因子的存在可能反映了导致该疾病的潜在生化缺陷。