Steroids in biliary atresia: single surgeon, single centre, prospective study
Mark Davenport1, Chris Parsons, Sarah Tizzard
1Department of Paediatric Surgery, King's College Hospital, London SE5 9RS, UK.
Insights
Adjuvant steroid therapy, particularly at higher doses, improved early liver function and jaundice clearance in infants with biliary atresia (BA) after Kasai portoenterostomy (KPE). Long-term survival rates were not significantly affected.
Area of Science:
- Pediatric Surgery
- Hepatology
- Neonatology
Background:
- Biliary atresia (BA) is a serious neonatal liver disease.
- The role of adjuvant steroids after Kasai portoenterostomy (KPE) for BA remains unclear.
- Evidence supporting steroid use in BA management is limited.
Purpose of the Study:
- To evaluate the efficacy of adjuvant steroid therapy in infants with biliary atresia undergoing KPE.
- To compare outcomes between low-dose steroid, high-dose steroid, and no steroid groups.
- To assess the impact of steroids on early liver biochemistry, jaundice clearance, and long-term survival.
Main Methods:
- A retrospective study of 153 infants with isolated BA undergoing KPE before 70 days of age.
- Infants were divided into LOW-dose steroid, HIGH-dose steroid, and NO steroid (including placebo) groups.
- Outcomes measured included early liver biochemistry, jaundice clearance (<20 μmol/L), and actuarial native liver survival.
Main Results:
- High-dose steroids significantly reduced bilirubin, AST, and aspartate-aminotransferase index (APRi) at 1 month post-KPE compared to no steroids.
- Steroid use significantly increased the percentage of infants clearing jaundice at 6 months (67% vs. 52%).
- No significant differences were observed in 4-year patient or native liver survival rates between groups.
Conclusions:
- Adjuvant prednisolone significantly improves early post-operative liver biochemistry in infants with BA.
- Higher doses of prednisolone showed a more pronounced effect on early liver function.
- Steroid therapy increases the proportion of infants achieving jaundice clearance post-KPE, but does not impact long-term survival.
Background & Aims:
The effect of adjuvant steroids in infants with biliary atresia (BA) is not clear and evidence of benefit is lacking.
Methods:
During the period Jan. 2000-Dec. 2011, 153 infants with isolated (CMV IgM-ve) BA underwent Kasai portoenterostomy (KPE) at<70 days. They were divided into three groups: LOW-dose steroid (from a previous randomized trial; starting prednisolone 2mg/kg/day, n=18), HIGH-dose steroid (starting prednisolone 5mg/kg/day, n=44), and NO steroid [n=72+19 placebo (from randomized trial)=91]. Outcome was assessed by early liver biochemistry, clearance of jaundice (<20 μmol/L), and actuarial native liver survival. Data are quoted as median (IQ range) and compared with non-parametric ANOVA, Chi or Log-rank tests as appropriate. p ≤ 0.05 was regarded as significant.
Results:
All three groups were comparable for age (ANOVA, p=0.31) and a surrogate marker of liver fibrosis [aspartate-aminotransferase index (APRi), ANOVA, p=0.67]. At 1 month post KPE, there was a significant reduction in bilirubin [58 (25-91) vs. 91 (52-145)μmol/L, p=0.0015], AST [118 (91-159) vs. 155 (108-193)IU/L, p=0.0015], and APRi [0.49 (0.28-0.89) vs. 0.82 (0.45-1.2), p=0.005] for HIGH vs. NO steroid. There was a significant increase in % clearance of jaundice with the use of steroids [47/91 (52%) vs. 12/18 (67%) vs. 29/44 (66%); steroids vs. no steroids, p=0.037]. There was no statistical difference in 4-year patient survival (96% vs. 94% vs. 95%) or native liver survival (4 year=46% vs. 50 vs. 57%).
Conclusions:
The adjuvant use of prednisolone significantly improved early post-operative liver biochemistry (especially at the higher dose), and increased the proportion of infants who cleared their jaundice at 6 months post-KPE.