摘要：
目的 探讨月龄＜3个月的胆汁淤积症与胆道闭锁(BA)患儿进行胆道造影术的指征.方法 选取2016年4月至2017年4月,在首都儿科研究所附属儿童医院新生儿科接受胆道造影术的110例患儿为研究对象.根据胆道造影术诊断结果,将其分为BA组(n=36)和胆汁淤积症组(n=74).回顾性分析BA组和胆汁淤积症组患儿的一般临床资料、生化指标及超声检查结果.采用成组t检验,对2组患儿年龄、黄疸持续时间及天冬氨酸氨基转移酶(AST)、总胆红素(TBIL)、直接胆红素(DBIL)、白蛋白、球蛋白、血清Ca2+、胆汁酸、血氨、乳酸和碱性磷酸酶(ALP)水平等进行统计学比较.采用Mann-Whitney U秩和检验,对2组患儿γ-谷氨酰转肽酶(GGT)、丙氨酸转氨酶(ALT)水平等进行统计学比较.采用x2检验,对2组患儿性别构成比、白陶土样便发生率及肝大、脾大、GGT水平≥300 U/L、TBIL浓度≥150.0 μmol/L和DBIL浓度≥100.0 μmol/L所占比例进行统计学比较.本研究遵循的程序符合2013年修订的《世界医学协会赫尔辛基宣言》的要求.结果 ①2组患儿年龄、黄疸开始时间、黄疸持续时间、肝大和脾大所占比例等一般临床资料比较,差异均无统计学意义(P＞0.05).②2组患儿性别构成比比较,差异有统计学意义(x2=13.580,P＜0.001).BA组患儿白陶土样便发生率及GGT水平≥300 U/L、TBIL浓度≥150.0 μmol/L、DBIL浓度≥100.0 μmol/L与超声检查结果提示为BA患儿所占比例,均显著高于胆汁淤积症组,并且差异均有统计学意义(x2=18.396、37.914、25.728、27.957、53.606,P＜0.001).BA组患儿GGT水平和DBIL浓度分别为526.0 U/L (409.9～1 187.4 U/L)和(116.3±32.2)μmol/L,显著高于胆汁淤积症组的101.9 U/L(71.8～440.7 U/L)和(88.8±38.8) μmol/L,2组分别比较,差异均有统计学意义(Z=2.955,P=0.003;t =3.214,P=0.006).③白陶土样便,GGT水平,超声检查结果提示胆囊收缩＜50％或者胆囊皱缩、僵硬或者无胆囊,以及超声检查结果提示肝门三角区纤维化,对于诊断BA患儿的敏感度分别为55.6％、76.4％、75.0％及17.6％,特异度分别为81.1％、86.5％、91.9％及95.9％,准确度分别为72.7％、83.3％、86.4％及71.3％,阳性预测值分别为58.8％、72.2％、81.8％及66.7％,阴性预测值分别为78.9％、88.9％、88.3％及71.7％.结论 女性患儿、白陶土样便、GGT水平≥300 U/L、TBIL浓度≥150.0 μmoL/L、DBIL浓度≥100.0 μmoL/L,以及超声检查结果提示胆囊收缩不良或者胆囊僵硬、皱缩或者无胆囊,肝门三角区纤维化,均为月龄＜3个月BA患儿的高危因素,但是不能作为确诊依据.对于月龄＜3个月的合并上述高危因素的患儿,需进行胆道造影术进一步明确诊断.%Objective To explore the indications of cholangiography for children with cholestasis and biliary atresia (BA) younger than 3 months old.Methods A total of 110 children who underwent cholangiography from April 2016 to April 2017 in Department of Neonatology,Children's Hospital,Capital Institute of Pediatrics were selected into this study.According to different results of cholangiography,they were divided into BA group (n =36) and cholestasis group (n =74).The general clinical data,biochemical indexes and ultrasonic examination results of 2 groups were analyzed retrospectively.Independent-samples t test was used to statistically analyze the age,duration of jaundice,and aspartate aminotransferase (AST),total bilirubin (TBIL),direct bilirubin (DBIL),albumin,globulin,serum Ca2+,bile acid,blood ammonia,lactic acid and alkaline phosphatase (ALP) levels between 2 groups.Mann-Whitney U rank sum test was used to analyze the levels of gamma-glutamyl transferase (GGT) and alanine transaminase (ALT) statistically.The gender composition ratio,incidence of kaoline stool,and the proportions of liver and spleen enlargement,GGT level ≥300 U/L,TBIL concentration ≥150.0 μmol/L and DBIL concentration ≥100.0 μmol/L were statistically analyzed by chi-square test.The study was in accordance with World Medical Association Declaration of Helsinki revised in 2013.Results ①There were no statistical differences between 2 groups in the general clinical data,such as age,the onset time of jaundice,duration of jaundice,and proportions of liver and spleen enlargement (P ＞ 0.05).②There was statistically significant difference between two groups in gender composition ratio (x2=13.580,P＜0.001).The incidence of kaoline stool,and proportions of GGT level ≥ 300 U/L,TBIL concentration ≥ 150.0 μmol/L,DBIL concentration ≥ 100.0 μmol/L and the proportion of BA revealed by ultrasound examination in BA groups all were statistically higher than those in cholestasis group (x2=18.396,37.914,25.728,27.957,53.606;all P＜0.001).GGT level and DBIL concentration in BA group were 526.0 U/L (409.9-1 187.4 U/L) and (116.3± 32.2) μmol/L,respectively,which were significantly higher than 101.9 U/L (71.8-440.7 U/L) and (88.8±38.8) μmol/L in cholestasis group.The differences between 2 groups were statistically significant (Z =2.955,P =0.003;t =3.214,P =0.006).③The sensitivities of kaoline stool,GGT level,ultrasonic examination results indicating that gallbladder contraction was less than 50％,and gallbladder with shrinkage and stiffness or without gallbladder,and ultrasound examination results indicating hepatic portal triangular fibrosis in diagnosis of BA were 55.6％,76.4％,75.0％ and 17.6％,respectively,and the specificities were 81.1％,86.5％,91.9％ and 95.9％,respectively,and the accuracy were 72.7％,83.3％,86.4％ and 71.3％,respectively,and the positive predictive values were 58.8％,72.2％,81.8％ and 66.7％,respectively,and the negative predictive values were 78.9％,88.9％,88.3％ and 71.7％,respectively.Conclusions Female children,kaoline stool,GGT level ≥ 300 U/L,TBIL concentration ≥ 150.0 μmoL/L,DBIL concentration ≥100.0 μmoL/L,ultrasonic examination results showed that poor gallbladder contraction or stiffness,shrinkage or absence of gallbladder,hepatic portal triangle fibrosis are all high risk factors for BA patients younger than 3 months old,but those items still cannot be used as the basis for diagnosis of BA.Cholangiography is required for patients younger than 3 months combined with the above-mentioned high-risk factors of BA to make final diagnosis.
