新生儿引起肝内胆汁淤积是由感染、遗传代谢性疾病、胃肠外营养、基因突变等各种不同病因导致胆汁形成、分泌和排泄障碍。胆汁在体内淤积会影响新生儿的生长发育,严重者出现肝纤维化、肝功能衰竭等不可逆的改变,其发病的机制涉及参与肝脏胆汁酸分泌的各种转运体、肝细胞膜转运蛋白转录和和转录后的调控等。本文归纳总结近年来国内外关于胆汁酸转运体及其转录和转录后的调控,从而为此疾病的防治提供理论依据,为将来降低其发生率和改善预后。

Neonatal intrahepatic cholestasis is caused by infection,genetic metabolic diseases,parenteral nutrition,gene mutation and other different causes leading to bile formation,secretion and excretion disorders．Bile stasis in the body will affect the growth and development of the newborn,and in severe cases,irreversible changes such as liver fibrosis and liver failure will occur．The pathogenesis of bile stasis involves various transporters involved in the secretion of bile acids in the liver,transcription and post transcription regulation of liver cell membrane transporters．In this paper,we summarized the recent studies on bile acid transporters and their transcriptional and post transcriptional regulation at home and abroad,so as to provide a theoretical basis for the prevention and treatment of this disease,and to reduce its incidence and improve its prognosis in the future.

目的 探讨天麻止痫汤分别联合托吡酯和卡马西平治疗对癫痫患者记忆能力及甘丙肽、铁调节转运体1（IREG1）的影响。方法 选择我院2019年6月—2022年6月收治的85例癫痫患者,其中21例予天麻止痫汤联合托吡酯治疗（A组）,23例予天麻止痫汤联合卡马西平治疗（B组）,22例予托吡酯治疗（C组）,19例予卡马西平治疗（D组）。评价4组的治疗效果、记忆能力、甘丙肽和IREG1水平等指标,并进行统计比较。结果 4组治疗总有效率和完全控制率比较差异均无统计学意义（P>0.05）。两两比较,A组总有效率高于C组和D组（P<0.05）,B组总有效率高于C组和D组（P<0.05）,A组和B组总有效率比较差异无统计学意义（P>0.05）;A组患者治疗后中医症候积分、癫痫发作次数及发作持续时间均少于其他3组（P<0.05）;A组患者治疗后视觉再认、图片回忆、视觉再生、联想学习、理解、顺背和倒背数字和记忆商数（MQ）均较治疗前升高,且高于其他3组（P<0.05）;4组治疗后甘丙肽水平升高（P<0.05）,IREG1蛋白表达量降低（P<0.05）,A组治疗后甘丙肽水平高于其他3组,IREG1蛋白表达量低于其他3组（P<0.05）;4组不良反应发生率比较差异无统计学意义（P>0.05）。结论 天麻止痫汤联合托吡酯或卡马西平治疗癫痫的总有效率、安全性均较好,且对患者记忆能力影响较小;而天麻止痫汤在改善癫痫症状、减少癫痫发作次数及发作持续时间、抑制甘丙肽和IREG1异常表达方面更具优势。

Objective To investigate the effects of Tianma Zhixian Decoction combined with topiramate and carbamazepine on memory ability and galanin and iron exporter ferroportin 1（IREG1）in patients with epilepsy．Methods A total of 85 patients with epilepsy were admitted to our hospital from June 2019 to June 2022,including 21 patients treated with Tianma Zhixian Decoction combined with topiramate（Group A）,23 patients treated with Tianma Zhixian Decoction combined with carbamazepine（Group B）,22 patients treated with topiramate（Group C）,and 19 patients treated with carbamazepine（Group D）．The therapeutic effect,memory ability,galanin and IREG1 levels were compared among the four groups．Results There were no significant differences in the total treatment response rate and complete control rate between the four groups（P>0.05）．By pairwise comparison,the total response rate in Group A was significantly higher than those in Group C and Group D（P<0.05）,the rate in Group B was significantly higher than those in Group C and Group D（P<0.05）,there was no significant difference in the total response rate between group A and B（P>0.05）．In group A,the TCM syndrome score,seizure attacks and duration of seizure were significantly less than those of the other three groups（P<0.05）．In Group A,visual re-recognition,picture recall,visual regeneration,associative learning,understanding,numbers recitation forward or backward and MQ values were all significantly improved compared with those before treatment,which was significantly higher than that in the other three groups（P<0.05）．Galanin levels significantly increased in the four groups（P<0.05）after treatment,while IREG 1 protein expression decreased（P<0.05）．Galanin level in group A was significantly higher than the other three groups,IREG 1 protein expression was significantly lower than that in the other three groups（P<0.05）．There was no significant difference in the incidence of adverse reactions among the four groups（P>0.05）．Conclusions Tianma Zhixian Decoction combined with topiramate or carbamazepine is effective and safe in the treatment of epilepsy,with little effect on the memory ability of patients．Tianma Zhixian Decoction has more advantages in improving the complete control rate of epilepsy,improving epilepsy symptoms,reducing the number and duration of seizures,and inhibiting the abnormal expression of galanin and IREG1．