Serum Alpha1-antitrypsin in Normal Children and Childhood Liver Disease

中國正常及肝疾患兒童血清中之Alpha1-antitrypsin值

李鍾祥(Chung-Hsiang Lee) ； 李廷堅(Ting-Chien Lee)

《Acta Paediatrica Sinica》 17卷1期 (1976/03) Pp. 13-20

摘要

血清中缺乏alpha1-antitrypsin (α1-AT)與嬰兒早期肝硬化及成人肺氣腫之關係已有許多報告，然而中國小孩之肝疾患與α1-AT缺乏之關係仍未知。作者等取20例正常中國成人，20例臍帶血，51例臺東小孩，55例臺東布農族山地小孩，170例花蓮阿美族山地小孩，40例急性肝炎小孩及5例新生兒肝炎小孩之血清，測定其血中α1-AT值。結果成人正常值爲245±47mg/100ml，沒有發現同型缺乏例，卻發現23.53%之臺東小孩，41.82%之布農族小孩及33.53%阿美族小孩血清α1-AT量較低(40-150mg%)。
作者等同時檢查臺東及山胞小孩血清中α-fetoprotein (α-FP)，hepatitis associated antigen (HAA)，及血清中之glutamic oxalacetic及glutamic pyruvic transaminases (SGOT及SGPT)，結果發現血清α1-AT較低與HAA，α-FP，及SGOT，SGPT之異常無關。
關於臺東及山地小孩有許多血清α1-AT較低例之存在及患肝疾之中國小孩與缺乏α1-AT之關連仍待進一步之研究。

並列摘要

Deficiency of the circulating enzyme inhibitor, alpha1-antitrypsin (α1-AT) has been implicated in the development of cirrhosis in childhood, and of early-onset of panacinar emphysema in adults. Serum α1-AT levels in 20 normal adults, 20 cord blood samples, 51 normal Tai-Tung children, 55 normal aboriginal children of Pu-Long tribe, 170 normal aboriginal children of A-Mei tribe, 40 children with acute viral hepatitis and. 5 infants with neonatal hepatitis were determined. The results showed that the mean serum α1-AT level of 20 normal adults was 245±47mg/100ml and no case of homozygous deficiency was found in normal children or children with liver disease but low (40-150mg/100ml) serum α1-AT levels were found in 23.58%, 41.82% and 33.58% of Tai-Tung, Pu-Long and A-Mei children, respectively. Hepatitis associated antigen (HAA), alpha-fetoprotein (α-FP) and serum glutamic oxalacetic and glutamic pyruvic transaminases (SGOT and SGPT) were also determined. There were no correlation between low serum α1-AT levels and abnormal HAA and SGPT. The high prevalence of low serum α1-AT levels found in Tai-Tung and aboriginal children and the etiological relationship of α1-AT deficiency in Chinese children with liver disease need further studies.