Five-year native liver survival analysis in biliary atresia from a single large Chinese center: The death/liver transplantation hazard change and the importance of rapid early clearance of jaundice

Abstract Background：To explore the relevant factors for early liver transplantation (LT) after Kasai portoenterostomy (KP).Methods：Retrospective analysis was performed for 200 children with biliary atresia, who underwent LT with hepatic failure after KP. According to the interval between KP and LT, they were divided into three groups: G1 (≤6-month), G2 (6-month~2-year) and G3 (>2-year). Gender, age of Kasai portoenterostomy, jaundice-clearance, cholangitis after KP and liver function indexes before LT were compared among the three groups.Results：The proportion of patients with age of KP (≤90-day) in G1 was lower than that in G3 (P=0.003). Jaundice-clearance occurred in 6 (7.6%), 26(28.6%) and 26 (86.7%) patients after KP in G1, G2 and G3 respectively (P<0.001). There were statistical differences in the incidence of early cholangitis, late cholangitis and repeated cholangitis among the three groups (P=0.035, <0.001 and 0.022). The native liver survival (NLS) rate of children at operation age >90-day was lower than that of children at operation age ≤90-day (P=0.002). The NLS rate of the children with jaundice-clearance after KP was significantly better than that of the children without jaundice-clearance (P<0.001). The NLS rate of the children with early cholangitis after operation was lower than that in children without early cholangitis (P=0.026). The NLS rate of patients of G2 and G3 with cholangitis after KP was lower than that in children without cholangitis (P=0.017). Multiple logistic regression analysis showed uncleared jaundice after KP was a risk factor for the NLS survival time in patients.Conclusion：The age of KP (>90-day), jaundice-unclear and early cholangitis could reduce the NLS time after KP, which were related to early liver transplantation. Jaundice-unclear was a risk factor for early liver transplantation.

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The prognosis of type III biliary atresia with a hilar cyst on ultrasonography might not be better than that without

10.21203/rs.2.10787/v1 ◽

2019 ◽

Author(s):

Quan-yuan Shan ◽

Bao-xian Liu ◽

Zhi-hai Zhong ◽

Hua-dong Chen ◽

Yu Guo ◽

...

Keyword(s):

Survival Rate ◽

Biliary Atresia ◽

Survival Curves ◽

Type Iii ◽

Kaplan Meier ◽

Significant Difference ◽

Native Liver ◽

Eventual Outcome ◽

Native Liver Survival ◽

Better Than

Abstract Background Biliary atresia (BA) with a hilar cyst is an uncommon variant of BA that may be misunderstood to have a relatively favorable prognosis. Methods A single-center retrospective review of patients that matched BA with a hilar cyst (n=27) with BA without a cyst (n=27) over a 5-years period was done. All patients were diagnosed as type III BA by histologic examination and cholangiograms. Results There were no significant intergroup differences between baseline characteristics and outcomes after Kasai portoenterostomy surgery in two groups. The Kaplan–Meier survival curves showed no significant difference in cumulative native liver survival rate of the two groups (p = 0.584). Of the 27 BA patients with a hilar cyst, 8 were showed the cyst communicated with the gallbladder on cholangiograms, and Kaplan-Meier survival curves indicated that the cumulative native liver survival rate was significantly shorter (P=0.045) in them than those who the cyst was not communicated with the gallbladder. Conclusions Type III BA with a hilar cyst had no better prognosis compared with Type III BA without a cyst. A cyst communicated with the gallbladder may be associated with a poor eventual outcome for Type III BA with a hilar cyst.

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