A Nomogram Predicting the Prognosis of Children With Biliary Atresia After Hepatoportoenterostomy

Abstract

Background: Although Kasai portoenterostomy (KPE) is performed timely for most children with biliary atresia (BA), the native liver survival (NLS) is still poor due to the progressive liver fibrosis. Many children have to receive liver transplantation (LT) within 2 years after KPE. Early prediction of the prognosis permits the implementation of prophylactic treatments for BA children. However, studies about the prediction are limited.Objective: The purpose of this study is to establish a nomogram to predict the prognosis of BA children within 2 years after KPE.Methods: The follow-up data of 151 BA children were retrospectively reviewed, and were randomly divided into a training cohort for constructing a nomogram (n = 103) and a validation cohort (n = 48). In the training cohort, patients were divided into Group A and Group B according to whether death or LT were observed within 2 years post-KPE. Multivariate Cox regression based on the baseline characteristics, liver function indicators and LSM (liver stiffness measurement) values at KPE and 3 months after KPE was utilized for the establishment of the nomogram in predicting the prognosis of BA within 2 years after KPE. The discrimination and calibration of the nomogram were internally and externally validated.Results: Fifty-six BA children were included in Group A and 47 were included in group B. Age at KPE, METAVIR score F4, LSM at 3 months, first onset of cholangitis within 3 months, and jaundice clearance time were the independent predictors for the prognosis of BA children within 2 years after KPE (all P < 0.05). The developed nomogram based on these independent predictors showed good discrimination and calibration by the internal and external validation. Its performance was better than each predictor in predicting the prognosis (all P < 0.05).Conclusions: The established nomogram based on the indicators from the first 3 months after KPE may be useful for predicting the prognosis of BA children within 2 years post-KPE and helpful for the consideration of LT.

A Novel Model for Predicting the Clearance of Jaundice in Patients With Biliary Atresia After Kasai Procedure

“…Wu et al found that an LSM value > 7.7 kPa was a predictive factor for discriminating BA patients from other cholestatic patients, and patients with an LSM value > 16 kPa often require early liver transplantation ( 29 ). Liu et al also showed that the LSM value at 3 months was associated with 2-years NLS post- KP ( 31 ). In our study, the preoperative LSM values in the uncleared jaundice group were significantly higher.…”

“…Additionally, the LSM value mainly reflects the degree of liver fibrosis, which is one of the main pathological features of BA (28). Previous studies have applied the LSM assessment for the diagnostic and prognostic prediction of BA (29)(30)(31). Wu et al found that an LSM value > 7.7 kPa was a predictive factor for discriminating BA patients from other cholestatic patients, and patients with an LSM value > 16 kPa often require early liver transplantation (29).…”

“…Although many studies have indicated several risk factors associated with the short- and long-term outcomes of BA patients post-KP ( 17 , 35 , 36 ), there are few established visual predictive models to determine its prognosis. Liu et al constructed a nomogram to predict the 2-year NLS in BA patients post-KP and showed good performance in a validation cohort ( 31 ). However, there was a difference compared with the results of our study.…”

Predicting the outcomes of Kasai portoenterostomy for biliary atresia: a cohort study

“…DB and ALB are factors reflecting the excretory and synthetic functions of liver; the results indicate that only patients with excellent comprehensive liver functions can survive with their native liver. Some studies have been reported about predicting NLS for infants after KPE, most focused on factors before jaundice resolution, and some are not routine parameters, [9][10][11][12][13][14][15] the predictive values are not certain.…”

A nomogram model based on preoperative grey-scale US features and routine serum biomarkers to predict the outcome of infants with biliary atresia after Kasai portoenterostomy

“…Many studies have tried to identify the outcome predictors after KPE. Several studies (26)(27)(28)(29)(30) reported that pre-KPE liver biopsy and elastography can predict KPE outcome by evaluating liver degree. However, although liver biopsy is the gold standard for fibrosis, it is invasive and unsafe for BA infants, and the obtained histological material is insufficient for an appropriate analysis (27).…”