儿童非胆道闭锁性胆汁淤积性肝病的临床及预后分析

摘要/Abstract

摘要： 目的分析儿童非胆道闭锁性胆汁淤积性肝病的临床特征及预后。方法回顾性分析2018年12月至2023年6月就诊于我院消化内科的非胆道闭锁性胆汁淤积性肝病患儿,分析其相关指标的差异性。纳入病患183例,根据预后情况将其分为预后不良组(27例)与预后良好组(156例)。比较两组患儿的病因构成、ALT、TBil、DBil、GGT、TBA、Alb、PT、INR、Hb、肝脏大小、脾脏大小的差异。结果不同病因发病率由高到低依次为:感染性(33.3%)、病因不明(28.4%)、药物性(18.0%)、遗传代谢性(8.2%)、胆管梗阻(7.1%)、其他(4.9%)。其中,男孩110例,女孩73例,男∶女=1.51∶1,不同疾病的男女发病率比较,差异无统计学意义(P>0.05)。在感染性疾病及遗传代谢性疾病中,≤1岁组患儿发病率显著高于>1岁组(P<0.05);在药物性疾病中,≤1岁组发病占比较>1岁组发病占比更低(P<0.05),其余组间比较差异无统计学意义(P>0.05)。预后不良组的ALT、TBil、DBil、PT、INR、肝脏肋下大小、脾脏肋下大小分别为699.00(344.50,1 837.00)U/L、217.00(171.20,276.80)μmol/L、157.20(101.30,197.20)μmol/L、18.10(13.40,27.10)s 、1.58(1.16,2.50)、23.3(0,29.2)mm、0(0,20.5)mm,预后良好组的ALT、TBil、DBIL、PT、INR、肝脏肋下大小、脾脏肋下大小分别为284.10(119.00,1 326.23)U/L、105.66(78.50,158.70)μmol/L、 71.40(49.20,106.68)μmol/L、13.05(11.80,15.23)s、1.12(1.02,1.33)、0(0,19.8)mm、0(0,0)mm,预后不良组较预后良好组更高(P<0.05);预后不良组Alb为35.70(32.00,37.70)g/L,预后良好组则为37.65(34.03,39.70)g/L,预后不良组较预后良好组更低(P<0.05);预后良好和预后不良组的GGT、TBA、Hb方面比较,差异均无统计学意义(P>0.05)。进一步行多因素logistic回归分析发现,DBil、INR、脾脏肋下大小的OR、95%CI分别为(OR=1.026、7.033、1.119,95%CI=1.014～1.038、2.332～21.210、1.050～1.192)。结论儿童非胆道闭锁性胆汁淤积性肝病病因以感染性、药物性为主;遗传代谢性、梗阻性也较为常见;≤1岁患儿病因以感染性、遗传代谢性疾病为主,>1岁则以药物性为主;仍有近1/3病因未明。DBil、INR、脾脏大小是影响患儿预后的独立影响因素。

关键词: 非胆道闭锁, 胆汁淤积, 儿童, 临床特征, 预后

Abstract: Objective To analyze the clinical characteristics and prognosis of non-biliary obstructive cholestatic liver disease in children. Methods A retrospective analysis was conducted on 183 children with non-biliary obstructive cholestatic liver disease who visited the Department of Gastroenterology at our hospital from December 2018 to June 2023. The etiology, clinical characteristics, and prognosis were analyzed. According to the prognosis, patients were divided into a good prognosis group and a poor prognosis group, with 156 patients in the former group and 27 patients in the latter group. We compared the differences in etiology, ALT, TBil, DBil, GGT, TBA, Alb, PT, INR, Hb, liver size, and spleen size between the two groups of children. Results The incidence rate of different causes from high to low was infectious (33.3%), unknown (28.4%), drug (18.0%), genetic/metabolic (8.2%), bile duct obstruction (7.1%), and others (4.9%). There were 110 boys and 73 girls, with a male:female ratio of 1.51∶1. There was no significant difference in the incidence rate of different diseases between boys and girls (P>0.05). In infectious diseases and genetic metabolic diseases, the incidence rate of children ≤ 1-year-old group was significantly higher than that of children>1 year old group (P<0.05); In drug-induced diseases, the incidence rate of ≤ 1-year-old group was lower than that of>1-year-old group (P<0.05). There was no significant difference between the other groups (P>0.05). The ALT, TBil, DBil, PT, INR, liver subcostal size, and spleen subcostal size in the poor prognosis group were 699.00(344.50,1837.00)U/L,217.00(171.20,276.80)μmol/L,157.20(101.30,197.20)μmol/L,18.10(13.40,27.10)s ,1.58(1.16,2.50),23.3(0,29.2)mm,0(0,20.5)mm, while the ALT, TBil, DBil, PT, INR, liver subcostal size, and spleen subcostal size in the good prognosis group were 284.10(119.00,1326.23)U/L,105.66(78.50,158.70)μmol/L,71.40(49.20,106.68)μmol/L,13.05(11.80,15.23)s,1.12(1.02,1.33),0(0,19.8)mm,0(0,0)mm,with higher values in the poor prognosis group compared to the good prognosis group (P<0.05).Alb levels in the poor prognosis group were 35.70 (32.00, 37.70) g/L, while those in the good prognosis group were 37.65(34.03, 39.70) g/L. Alb levels in the poor prognosis group were lower than those in the good prognosis group (P<0.05). In terms of GGT, TBA, and Hb, there was no statistically significant difference between the good and poor prognosis groups(P>0.05). Further multivariate logistic regression analysis was conducted to identify the indicators that differed between the good prognosis and poor prognosis groups. The OR and 95% CI values of DBil, INR, and splenic subcostal size were 1.026 (95% CI: 1.014~1.038), 7.033 (95% CI: 2.332~21.210), and 1.119 (95% CI: 1.050~1.192), respectively. Conclusion Non-biliary obstructive cholestatic liver disease in children is mainly caused by infectious and drug-induced factors,genetic/metabolic and obstructive diseases are also not uncommon. In the children aged <1 year, infectious and genetic metabolic diseases are predominant, while in those aged >1 year, drug-induced diseases are predominant.Nearly one-third of the causes are still unknown. DBil,INR, and spleen size are independent factors affecting the prognosis of the patients.

Key words: Non biliary atresia, Cholestasis, Children, Clinical features, Prognosis

郑丽娟, 薛福敏, 王一琳, 于静, 郭亚琼. 儿童非胆道闭锁性胆汁淤积性肝病的临床及预后分析[J]. 肝脏, 2026, 31(1): 102-106.

ZHENG Li-juan, XUE Fu-min, WANG Yi-lin, YU Jing, GUO Ya-qiong. Clinical and prognostic analysis of children with non-biliary obstructive cholestatic liver disease[J]. Chinese Hepatolgy, 2026, 31(1): 102-106.

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