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Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia
Scott Nightingale1, Michael O Stormon, Edward V O'Loughlin
1*Department of Gastroenterology, John Hunter Children's Hospital †Discipline of Paediatrics and Child Health, University of Newcastle, Newcastle, Australia ‡Transplant and Regenerative Medicine Centre §Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children ||Department of Paediatrics, University of Toronto, Toronto, Canada ¶Department of Gastroenterology, The Sydney Children's Hospital Network-Westmead #Discipline of Paediatrics and Child Health, Faculty of Medicine, University of Sydney **Department of Surgery, The Sydney Children's Hospital Network-Westmead, Sydney, Australia ††Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario ‡‡Department of Pediatrics and School of Epidemiology, Public Health and Preventative Medicine, University of Ottawa, Ottawa, Canada §§Department of Gastroenterology, The Sydney Children's Hospital Network-Randwick, Sydney, Australia ||||Department of Paediatrics, University of Otago, Christchurch, New Zealand ¶¶Department of Surgery, The Sydney Children's Hospital Network-Randwick ##School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, Australia ***Division of General Surgery, Hospital for Sick Children †††Department of Surgery, University of Toronto, Toronto, Canada.
Insights
Biliary atresia (BA) infants needing liver transplantation (LT) can be identified by specific lab values post-hepatoportoenterostomy (HPE). Total bilirubin and albumin levels at 3 months post-HPE predict native liver survival at 2 years.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Neonatal Surgery
Background:
- Biliary atresia (BA) is a severe neonatal liver disease.
- Most infants with BA require liver transplantation (LT) after hepatoportoenterostomy (HPE).
- Predicting native liver survival is crucial for managing BA patients.
Purpose of the Study:
- To identify clinical and laboratory factors predicting 2-year native liver survival in BA infants post-HPE.
- To establish prognostic indicators for BA patients undergoing HPE.
Main Methods:
- Retrospective cohort study of 217 BA patients in Australia and Canada (1986-2009).
- Analysis of clinical and laboratory data at 3 months post-HPE.
- Univariate and multivariate logistic regression to identify predictors of native liver survival.
Main Results:
- Serum total bilirubin (TB) and albumin levels at 3 months post-HPE were independent predictors of 2-year native liver survival.
- Optimal cut-off values: TB <74 μmol/L and albumin >35 g/L.
- Three distinct groups based on TB and albumin levels showed significantly different survival rates.
Conclusions:
- Serum TB and albumin levels at 3 months post-HPE are valuable prognostic markers for BA patients.
- Low albumin (<35 g/L) in non-jaundiced infants post-HPE indicates poor prognosis.
- Poorer linear growth suggests a need for early nutritional support in select BA patients.
Objectives:
Most infants with biliary atresia (BA) require liver transplantation (LT) after hepatoportoenterostomy (HPE), including those who initially clear jaundice. The aim of the present study was to identify clinical and routine laboratory factors in infants with BA post-HPE that predict native liver survival at 2 years.
Methods:
A retrospective cohort study was conducted in 217 patients with BA undergoing HPE in Sydney, Australia and Toronto, Canada between January 1986 and July 2009. Univariate and multivariate logistic regression using backwards-stepwise elimination identified variables at 3 months after HPE most associated with 2-year native liver survival.
Results:
Significant variables (P < 0.05) on univariate analysis included serum total bilirubin (TB) and albumin at 3 months post-HPE, bridging fibrosis or cirrhosis on initial liver biopsy, ascites of <3 months post-HPE, type 3 BA anatomy, age at HPE of >45 days, change in length z scores within 3 months of HPE, and center. On multivariate analysis, TB (P < 0.0001) and albumin (P = 0.02) at 3 months post-HPE, and center (P = 0.0003) were independently associated with native liver survival. Receiver operating characteristic analysis revealed an optimal cut-off value of TB <74 μmol/L (4.3 mg/dL; area under the receiver operating characteristic curve 0.8990) and serum albumin level >35 g/L (3.5 mg/dL; area under the receiver operating characteristic curve 0.7633) to predict 2-year native liver survival. TB and albumin levels 3 months post-HPE defined 3 groups (1: TB ≤74 μmol/L, albumin >35 g/L; 2: TB ≤74 μmol/L, albumin ≤35 g/L; 3: TB >74 μmol/L) with distinct short- and long-term native liver survival rates (log-rank P < 0.001). Length z scores 3 months post-HPE were poorer for group 2 than group 1 (-0.91 vs -0.30, P = 0.0217) with similar rates of coagulopathy.
Conclusions:
Serum TB and albumin levels 3 months post-HPE independently predicted native liver survival in BA when controlling for center. Serum albumin level <35 g/L in infants with BA who were no longer jaundiced at 3 months post-HPE was a poor prognostic indicator. Poorer linear growth and absence of significant coagulopathy suggest a role for early aggressive nutritional therapy in this group.
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