Postoperative high-dose steroids do not improve mid-term survival with native liver in biliary atresia

Postoperative adjuvant steroid treatment is reported to improve jaundice-free survival in biliary atresia (BA) patients and to reduce the need for early liver transplantation. However, evidence of all retrospective studies is very limited, although high-dose corticosteroids were favored. The aim of this dosage finding study was to test the most promising corticosteroid protocol in a smaller but representative series, in order to optimize the settings of upcoming prospective and long-term multicenter studies.

Our prospective single-center and open-labeled pilot study on high-dose steroids included 49 consecutive BA patients. Basic data of the study group were not different from 29 controls. In the study group, 20 consecutive patients were treated after the Kasai with methylprednisolone (10 mg/kg day 1 to 5 and 1 mg/kg day 6 to 28).

Overall survival with native liver was 63% after 6 months and 31% after 2 yr, with no statistical difference between the study and control groups. After 2 yr, 27% of all patients were still jaundice-free. With regard to predictive parameters, we found, 6 months after the Kasai, bilirubin < 20 micromol/L as highly sensitive (97%) and specific (93%) for jaundice-free survival with native liver.

In contrast to previous reports, this pilot study shows that high-dose steroid pulses after Kasai procedure are not effective in postoperative adjuvant therapy protocols and should be avoided in upcoming multicenter steroid studies. Therefore, we recommend extended and randomized multicenter studies to pre-evaluate the supposed effectiveness of alternative steroid protocols, by comparing, 6 months after the Kasai procedure, the number of patients with normal bilirubin.