Premachandra B N, Yu S Y
Metabolism. 1979 Sep;28(9):890-4. doi: 10.1016/0026-0495(79)90087-8.
Prealbumin (PA) measurements were made by electrophoretic and radial immunodiffusion techniques in three alpha-1-antitrypsin deficient Pi-D) serum samples. The deficiency was characterized as phenotype ZZ (homozygous). In two out of three serums PA was undetectable, as revealed by the absence of radiothyroxine distribution in the PA area, whereas quantitative estimates of PA by radial immunodiffusion showed very low levels (2--7 mg/100 ml) thus corroborating electrophoretic observations; low PA binding of T4 tracer was noted in another Pi-D serum. The total protein and laboratory thyroid function (thyroxine, triiodothyronine, and free thyroxine index concentration) measurements were normal, and the decrease in PA could not be explained on the basis of surgery, protein malnutrition, or cirrhosis. These and other observations described in this preliminary communication have served to raise the possibility of severe prealbumin deficiency being associated with alpha-1-antitrypsin deficiency, while the presence of low-but-not-absent PA in another Pi-D case might also suggest subgroup classification of the phenotype ZZ based on degrees of PA deficiency.
采用电泳和放射免疫扩散技术对3份α1-抗胰蛋白酶缺乏(Pi-D)血清样本进行了前白蛋白(PA)测定。该缺乏症表现为ZZ表型(纯合子)。在3份血清中的2份中,PA无法检测到,这通过PA区域中放射性甲状腺素分布的缺失得以揭示,而通过放射免疫扩散对PA进行的定量估计显示水平极低(2 - 7毫克/100毫升),从而证实了电泳观察结果;在另一份Pi-D血清中,观察到T4示踪剂的PA结合较低。总蛋白以及实验室甲状腺功能(甲状腺素、三碘甲状腺原氨酸和游离甲状腺素指数浓度)测量结果均正常,并且PA的降低无法用手术、蛋白质营养不良或肝硬化来解释。本初步交流中描述的这些以及其他观察结果,引发了严重前白蛋白缺乏与α1-抗胰蛋白酶缺乏相关联的可能性,而在另一个Pi-D病例中存在低但并非缺失的PA,这也可能提示基于PA缺乏程度对ZZ表型进行亚组分类。