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编码α_1-抗胰蛋白酶抑制物(Pi)的等位基因已确定75个以上,Z 等位基因可引起α_1-AT 的分泌缺陷,Pizz 表型和儿童肝病及成入肺气肿有关。但具有此基因表型的新生儿只有17%表现出明显的临床症状,其原因可能与补体系统有关。为研究α_1AT缺乏的肝病和补体活化的关系,作者对34例具Pizz基因而发生肝病的儿童进行研究,其中33例进行了肝活检,并依据肝功能检查将其分成轻型、中型和重型三组。以38例健康儿童作对照。作者以正常人血清加入3mg/ml 菊粉,37℃温育1小时后,1500×g 离心30分钟,上清部分作为C_3d 标准,再加上等体积22%PEG 8000 PBS 混合,4℃温育2小时,1500×g4℃离心20分钟,上清液用PBS 按比例稀释得到标准参考曲线。
More than 75 alleles have been identified for the alpha 1-antitrypsin inhibitor (Pi), the Z allele can cause a defective secretion of alpha 1-AT, and a Pizz phenotype has been associated with childhood liver disease and emphysema. However, only 17% of newborns with this gene phenotype showed significant clinical symptoms, which may be related to the complement system. In order to investigate the relationship between α_1AT-deficient liver disease and complement activation, the authors studied 34 children with Pizz-induced liver disease, 33 of whom underwent liver biopsy and were divided into three groups based on liver function tests: light, medium, and heavy . 38 healthy children as a control. The authors in normal human serum add inulin 3mg / ml, 37 ℃ for 1 hour, 1500 × g centrifuge for 30 minutes, the supernatant as C_3d standard, plus an equal volume of 22% PEG 8000 PBS mixed, 4 ℃ incubation 2 hours, 1500 × g4 ℃ centrifugation for 20 minutes, the supernatant diluted with PBS to obtain a standard reference curve.