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