35例新生儿肝炎综合征血浆α_1-ACT变化及其临床意义初探

Alteration of plasma α_1-antichymotrypsin in neonatal hepatitis syndrome and its clinical significance

目的 探讨新生儿肝炎综合征的遗传发病机制 ,为防治新生儿肝炎综合征提供理论基础。方法 应用火箭免疫电泳法测定 3 5例肝炎综合征新生儿患儿、3 1例健康新生儿和 46例成人 (成人对照组 3 0例 ,患儿父母 16例 )血浆α1 抗糜蛋白酶 (α1 ACT)水平 ,对新生儿肝炎综合征患儿急性发病期、临床痊愈期以及健康新生儿血浆α1 ACT水平的变化进行比较 ,经家系调查 ,筛查出先天性α1 ACT缺陷。采用全自动生化分析仪检测了先天性α1 ACT缺陷患儿急性发病期血清的总胆红素 (TB)、直接胆红素 (DB)、丙氨酸氨基转移酶 (ALT) ,用相关分析法在α1 ACT缺陷患儿中评估α1 ACT与血清TB、DB、ALT的关系。结果 新生儿肝炎综合征患儿急性发病期血浆α1 ACT水平 (0 3 85 5± 0 12 2g/L)显著高于临床痊愈期 (0 2 5 2 7± 0 0 72g/L) (t =5 5 5 ,P <0 0 0 1) ,也显著高于儿童对照组 (0 2 5 0 0± 0 0 44 2g/L) (t =5 85 4,P <0 0 0 1) ,新生儿肝炎综合征患儿临床痊愈期血浆α1 ACT水平 (0 2 5 2 7± 0 0 72g/L)与正常对照组则无显著性差异 (0 2 5 0 0± 0 0 44 2g/L) (t =0 181,P >0 0 5 )。 3 5例患儿中 8例血浆α1 ACT缺陷 ,经家系调查 ,5 (5 /8)例确定为先天性α1 ACT缺陷。 5例先天性α1 ACT缺陷的患儿在?

Objective To investigate the hereditary pathogenesis of neonatal hepatitis syndrome in order to provide a theoretical basis for prevention and treatment of neonatal hepatitis syndrome Methods Plasma α 1 ACT levels were tested by rocket immunoelectrophoresis in 35 patients with hepatitis syndrome, 31 healthy neonates and 46 healthy adults (including 30 control adults and 16 parents of patients) Plasma α 1 ACT levels were compared between illed and health children Based on family studies, congenital α 1 ACT deficiency was determined Total bilirubin(TB), direct bilirubin(DB) and alanine aminotransterase (ALT) were tested with automatic biochemical instrument among patients with congenital α 1 ACT deficiency A relative analysis was carried out between α 1 ACT and TB, DB, and ALT Results Plasma α 1 ACT levels in acute period of disease group were higher than that in clinically curative period of disease group [(0 385 5±0 122 0) vs (0 252 7±0 072 0), t =5 55, P <0 001] and in childhood control group [(0 385 5±0 250 0) vs (0 250 0±0 044 2), t =5 854, P <0 001] No difference was found in plasma α 1 ACT levels between clinically curative period of disease group and childhood control group [(0 252 7±0 072 0) vs (0 250 0±0 044 2), t =0 181, P >0 05] Eight of 35 had α 1 ACT deficiency,and on the basis of family studies, 5 of 8 were classified as congenital α 1 ACT deficiency Among these patients with congenital α 1 ACT deficiency, there was an obvious negative correlation between TB, DB, ALT in serum and plasma α 1 ACT in acute period( r =-0 933,-0 884,-0 822 respectively, P <0 05) Conclusion Congenital α 1 ACT deficiency may be one of the hereditary pathogenesis in neonatal hepatitis syndrome Measurement of plasma α 1 ACT level may be of some help to determine the state of the illness and evaluate its severity in neonatal hepatitis syndrome.