成人巨天冬氨酸氨基转移酶血症4例临床特征及诊断方法分析

Clinical characteristics and laboratory detection in adult patients with macro-aspartate aminotransferasemia

目的探讨成人巨天冬氨酸氨基转移酶（macro-AST）血症的临床特点和诊断方法。方法回顾性分析4例成人macro-AST血症患者的临床资料。结果 4例macro-AST血症患者中男性1例,女性3例,发病至确诊时间为1-7年。所有患者均表现为孤立性血清AST升高,且已排除各种急、慢性肝病、心脏疾病、肌肉疾病及风湿免疫病。应用聚乙二醇沉淀聚合物法测定4例患者的聚乙醇沉淀活性为89.7%-97.8%;应用冷藏法测定所有患者血清AST活性经冷藏后均明显下降（降幅为55.5%-93.1%）。经过10-36个月的规律随访,所有患者健康状况均良好。结论macro-AST血症是孤立性AST升高的少见原因,可见于健康人群,提高对该病临床特征及诊断方法的认识,可以避免不必要的反复检查及治疗,减少误诊率。

Objective To explore the clinical characteristics and diagnostic methods for adult patients with macro - asparate aminotransferansemia.Methods The clinical data including clinical manifestations and biochemical indexes of 4 adult patients with macro - AST were retrospectivelyanalyzed. Polyethylene glycol precipitation assay and refrigerated storage method were applied for making the diagnosis. Results Amongthese four patients, 3 of them were females and 1 male. All these four patients had persistently isolated elevations of AST and none of themhad other evidence of acute or chronic hepatic diseases, skeletal muscle, myocardial or hemolytic disorders. The polyethylene glycol precipitationassay has been applied for screening macro - enzymes in patients, and it had been found that precipitable activities of PEG （ % PPA） in these fourpatients were 89 .7 %-97 .8 % . The AST activities in these four patients with macro - AST were reduced by more than 50 % （55 .5 % -93 . 1 % ）after refrigerated storage. After follow - up for 10 ？36 months, all of these patients remained healthy. Conclusion Macro - asparate aminotransferasemiais a rare, but benign cause of isolated elevation of AST level. Early diagnosis of macro - AST can avoid unnecessary cost and misdiagnosis.