Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia.

UNLABELLED: The objective of this study was to evaluate adjuvant corticosteroids after Kasai portoenterostomy for biliary atresia. The study consisted of a prospective, 2-center, double-blind, randomized, placebo-controlled trial of post-Kasai portoenterostomy corticosteroids (oral prednisolone: 2 mg/kg/day from day 7 to day 21 and 1 mg/kg/day from day 22 to day 28). The data were compared with chi2 or Mann-Whitney tests, as appropriate. Seventy-one postoperative infants with type 3 biliary atresia were randomized to receive either oral prednisolone (n = 36) or a placebo (n = 37). At 1 month, the median bilirubin level was lower in the steroid group (66 versus 92 micromol/L, P = 0.06), but no difference was evident at 6 (P = 0.56) or 12 (P = 0.3) months. The proportion of infants with a normal bilirubin level (<20 micromol/L) at 6 (47% versus 49%, P = 0.89) and 12 months (50% versus 40%, P = 0.35) was not significantly different. The need for transplantation by 6 (12% versus 13%, P = 0.99) and 12 months (26% versus 35%, P = 0.47) was not significantly different. The steroid effect was more pronounced in younger infants (less than 70 days at Kasai portoenterostomy, n = 51), with a reduced bilirubin level at 1 month (64 versus 117 micromol/L, P = 0.01) and with a greater proportion with a normal bilirubin level at 12 months (54% versus 37%, P = 0.22). CONCLUSION: There was a beneficial effect on the rate of reduction of bilirubin in the early postoperative period (specifically in infants less than 70 days old at surgery), but this steroid regimen did not reduce the need for liver transplantation.

The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study.

We carried out a retrospective review of infants with biliary atresia splenic malformation (BASM). We found that 56 infants (10.2%) met the criteria for inclusion from a series of 548 infants (from January 1977 to December 2004). Syndromic infants were more likely to be female (P = .04) and to have a higher incidence of antenatal pathology (specifically maternal diabetes; 12.5% vs 1.2%; P < .0001). Situs inversus was noted in 21 (37%) and cardiac abnormalities in 25 (45%) infants. There were no differences in liver histology (eg, degree of liver fibrosis) or in the HLA genotype between BASM and nonsyndromic infants. Five-year and 10-year estimated native liver survival were 46% and 32%, respectively. There were 7 long-term survivors with their native liver and a follow-up of more than 10 years; all were anicteric. BASM is a distinct subgroup, with an implied onset during the embryological phase of organ development.