| Background and objectivesBiliary atresia (biliary atresia, BA) is one of the most serious digestive tractdiseases of pediatric surgery, and also is the main hepatic disease which cause infantsdeath. The disease has obvious regional and ethnic differences, the incidence ofEuropean white is significantly lower than the children of the east; Incidence ofEuropean and American area is about1/12000~1/5000, incidence of Chinese Taiwanarea is approximately1.46/10000.The incidence of biliary atresia in Chinesemainland haven’t specific statistics at present.The features of biliary atresia areprogressive inflammation and fibrous obstruction of intra and extra hepatic biliarytracts, lead to cholestasis, progressive liver fibrosis and liver cirrhosis. If the childrenaren’t treated timely, they will be died within2years usually. The treatment of biliaryatresia is early has Kasai operation and liver decompensated period will be expectedto undergo liver transplantation. The literature reported that the liver survival rate is36%~63%in4years after Kasai operation. With the progress of medical technology,the survival rate of biliary atresia increased significantly, but there are still a lot ofchildren repeated happened cholangitis post-operation which leaded to liver cirrhosis,portal hypertension and death. This paper collected the clinical data of99childrenwith biliary atresia who underwent operation recent5years in our department andwere followed up completely. Combining with the patient’s medical history,symptoms, laboratory and imaging examinations, operation records, follow-upsituations retrospectively analyzed the prognosis which were effected by operationage, types of biliary atresia, postoperative cholangitis, situation of liver functionrecovery and provided certain reference for the future diagnosis of biliary atresia.MethodsRetrospective analyzed99children in combination with related literature andelectronic medical record system retrieval. These children had been underwent operation and followed-up complete data during August2008to July2013, female54cases,45cases were male, age from1month to6months, an average of (3.33±1.26)months. All children were confirmed for the diagnosis of biliary atresia byintraoperation.These children had different degrees of preoperative biliary obstructionsymptoms, such as: the skin and mucous membrane were yellowing, the color ofdefecate was shallow, even white. The livers of30children were increased, quality ofthem were a bit hard, including15cases’ spleens increased;5cases had symptomssuch as cirrhosis ascites, and subcutaneous varicose vein of abdominal wall whenthey saw a doctor. Patients were followed up by clinic visits, letters or phone calls,average follow-up time were (30.03±9.80) months. The content of follow-up includedknowing about the developmental situation and defecate color of children, whetheroccurred cholangitis, portal hypertension, gastrointestinal hemorrhage, ascites andother symptoms, whether underwent liver transplantation operation. Regularlyreviewed the blood routine, liver function, color doppler ultrasound to know aboutliver function situation, degree of liver cirrhosis and bile excretion condition.According to the types of biliary atresia (typeâ… group, typeâ…¡group, typeâ…¢ group),surgery day age (<60d group,60d~90d,91d~120d group,>120d group), the presenceof cholangitis (with cholangitis group, with not cholangitis group), liver functionsituation (good group, a bit poor group, poor group) grouped the99children withbiliary atresia. SPSS17.0statistical software package was used to analyse the data.Quantitative data was expressed by "mean±standard deviation";2test was used toanalyse qualitative data such as jaundice subsided situation, biliary atresiaclassification, operation day age and with or not cholangitis. Kaplan-Meier survivalanalysis was used to analyse children’s survival condition. Log rank test was used toevaluate the significance of survival differences between3groups of liver function(good group, a bit poor group, poor group). The difference was statistically significantwhen P<0.05.ResultsAfter admission all biliary atresia children had medical examinations: All c hildren’s body skin and mucous membrane were yellow, white clay sample shit,some children had symptoms of hepatosplenomegaly, ascites and even hypertension. To perfect the relevant biochemical examination such as routine blood,liver function with direct bilirubin (DBIL) and indirect bilirubin (IBIL), total bilirubin (TBIL), alanine aminotransferase (ALT), aspertate aminotransferase (AST), gamma GGTP (gamma glutamyl transpeptidase)(gamma GGT) and bile acid (associates) increased significantly. Elevated bilirubin was given priority to the direct bilirubin. Radionuclide dynamic imaging liver magnetic resonance, magnetic resonance cholangio pancreatography and abdomen colour supported the diagnosis of biliary atresia. By intraoperative exploration, the99patients were diagnosed as biliary atresia, among them,8cases were belonging to the typeâ… of biliary atreia (8.1%),3cases were belonging to the typeâ…¡of biliary atreia(3.0%),88cases were belonging to the type â…¢ of biliary atreia (88.9%).typeâ… , type â…¡and type â…¢ of biliary atresia children’s recent rate of jaundice subsided were87.5%,66.6%and39.7%separately. The difference was statisticallysignificant (P<0.05). Typeâ… , typeâ…¡and typeâ…¢ patients’ rate of liver functionrecovered well were75.0%,66.6%and36.3%separately. The difference was statistically significant (P<0.05). According to the surgery age, divided the children with biliary atresia into4groups (<60d group,60d~90d group,91d~120dgroup,>120d group). The60d group contained18cases(18.1%),60d~90d group contained40cases(44.4%),91d~120d group contained22cases(22.2%),>120d group contained19cases(19.3). Four groups of biliary atresia children’s recent rate of jaundice subsided were61.1%,55.0%,31.8%and21.0%separately.The difference was statistically significant (P<0.05).Compared the four groupsof different surgery age separately pairwise. The difference of surgery age <60d group and surgery age>120d group was statistically significant (P<0.05). The difference between surgery age60d~90d group and surgery age>120d group was statistically significant (P<0.05). More differences had no statistical significance (P>0.05).The biliary atresia children can be divided into≤90d group and>90d group according to the surgery age. Two groups of biliary atresia children’s recent rate of jaundice subsided were56.8%and26.8%separately. The diffe rence was statistically significant (P<0.05). The rate of liver function recoveredwell of Four groups (<60d group,60d~90d group,91d~120d group,>120d group)were61.1%,50.0%,27.2%and15.7%separately. The difference was statistically significant (P<0.05).58cases appeared cholangitis in two years after Kasai surgery. The incidence of cholangitis was58.5%(58ï¼99).20cases (34.4%) first attacked within a month after surgery.30cases (51.7%) first attackedwithin3~6months after surgery,among them,13cases recurrent attacked in6months.7cases first attacked within6~12months after surgery.1cases first attacked within1year after surgery. Children’s recent rate of jaundice subsidedwere31.0%who had cholangitis.63.4%of the patients who had not cholangitis turned jaundice subsided.The difference was statistically significant (P<0.05).The rate of liver function recovered well of two groups were35.0%and69.0%separately. The difference was statistically significant (P<0.05). According tothe situation of children’s liver function, children’s hepatic function recoverywas divided into good group, a bit poor group and poor group.The number of3groups were25cases(25.2%),60cases(60.6%)and14cases(14.2%)separately. Kaplan Meier-survival curve analysis was used to analyze these groups of biliary atresia children. The survival differences among the good group of liver function, a bit poor group of liver function and poor group of liver function were tested by Log-rank method. The difference was statistically significant. It revealed that liver function was closely related to the survival, the worse the liver function, the lower the survival rate, the prognosis would be worse.This group contained99cases biliary atresia children, the follow-up time was2-60months, mean follow-up time was (30.03±9.80) months.57cases survived(2cases underwent liver transplantation surgery),42cases died and the survival rates of2years and4years were82.0%and46.0%respectively.The2children who underwent liver transplantation surgery developed normally and Color ultrasound, liver function, relevant test results were normal. ConclusionKasai operation is the primary therapy for biliary atresia children. The Kasaioperation can make adequate drainage of bile, recover the liver function gradually,win operation time for liver transplantation. The condition of liver function recovery,operation age, type of biliary atresia and have or not cholangitis are related to theoutcome of Kasai operation.The better recovery of liver function, the better thepostoperative curative effect, the higher the survival rate.The prognosis oftypeâ… biliary atresia children are better than the type â…¢ biliary atresia children. Theprognosis of Surgery age within90d group are better than the Surgery age exceed90dgroup. The prognosis of chidren who haven’t cholangitis after kasai operation arebetter than who have cholangitis. |