Clinical Characteristics Of The Cholestatic Liver Disease In Children

Objective:To analyze the clinical characteristics of the cholestatic liver disease in children Methods:From January 1,2017 to December 1,2019,155 children with cholestatic liver disease were retrospectively collected from the first hospital of Jilin University,including 103 males and 52 females,with the age of 4 days-14 years.All of the above patients met the following diagnostic criteria: total bilirubin(TBIL)< 85.5 μ mol / L with direct bilirubin(DBIL)> 17.1 μ mol / L,or TBIL > 85.5 μ mol / L with DBIL in the proportion of TBIL > 20%;at the same time,presenting with liver disease,that is,hepatomegaly or change of liver texture and abnormal increase of alanine transferase.Results:1)Etiology compositionThe different etiology composition ratio was from high to low: infectivity(56 cases,36.1%)> unknown cause(42 cases,27.1%)> anatomical or structural abnormalities(39 cases,25.2%)> toxicity(11 cases,7.1%)> other etiology(4 cases,2.6%)> genetic metabolism(3 cases,1.9%).Among them,cytomegalovirus infection(CMV)(48 cases)was the most common in infectivity,biliary atresia(37 cases)was the most common in anatomical or structural abnormalities,and total parenteral nutrition(9 cases)was the most common in toxicity.2)Distribution of patients in different age groupsThe proportion of cholestatic liver disease in children of different age ranges from high to low in the following order:(≤1 year,123 cases)>(> 6 years,14 cases)>(3-6 years,12 cases)>(1-3 years,6 cases);the proportion of cholestatic liver disease in children of different age ranges within 1 year old:(1-2 months,44 cases)>(< 1 month,29 cases)>(2-3 months,24 cases)>(3-4 months,13 cases)>(5-6 months,6 cases)>(>6 months 4 cases)>(4-5 months,3 cases).3)Etiology composition of different gendersThe most common causes of cholestatic liver disease were: cytomegalovirus infection,biliary atresia,total parenteral nutrition and unknown cause;there was no significant difference between the sexes in the proportion of each cause(P > 0.05).4)Comparison of serum AST,ALT,γ-GT,ALP,TBIL,DBIL and TBA between cytomegalovirus infection group and biliary atresia groupThere were significant differences in serum AST,ALT,γ-GT,ALP,TBIL,DBIL and TBA between the two groups(P < 0.05).The biochemical index of biliary atresia group was significantly higher than cytomegalovirus infection group.5)The color doppler ultrasound manifestations of biliary atresiaThe color Doppler ultrasound manifestations of biliary atresia can be divided into porta hepatis region abnormalities(22 cases,59.5%)and gallbladder region abnormalities(33 cases,89.2%).Among them,most of the abnormalities in the porta hepatis region had "Triangular cord"(20 cases);the common manifestations of the abnormalities in the gallbladder region were abnormal contraction rate of the gallbladder(18 cases,accounting for 48.6%)and rigidity of the gallbladder wall(17 cases,accounting for 45.9%).Conclusion:1)In our study,CMV infection,biliary atresia and unknown etiology are the most common causes of cholestatic liver disease in children;2)In this study,nearly 79.3% of the children were within 1 year old,while within 1 year old,they were within 0-3 months old.It is suggested that the age of this month is the high incidence period of cholestatic liver disease in children,which should be paid attention to clinically;3)There was no gender difference in the etiological distribution of CMV infection,biliary atresia,total parenteral nutrition and unknown causes;4)The degree of liver injury and cholestasis of biliary atresia was significantly higher than that of CMV infection.5)Among the various ultrasonographic manifestations of biliary atresia,the combination of “triangular cord”,rigid gallbladder wall and contraction rate of gallbladder may be helpful for the diagnosis of biliary atresia.