Peri-Operative High-Dose v Post-Operative Low Dose Steroid Therapy in the Management of Biliary Atresia: a Preliminary Report

background: the use of high-dose steroid therapy peri portoenterostomymay have a positive impact on the frequency ofcholangitis and survival rate.methods: a prospective study was conducted on two groupsof patients (less than three months of age) suffering from biliaryatresia from 1999 to 2005. the patients in group i (g i)were managed peri-operatively by high-dose methylprednisolonewhile the other group (g ii) received low dose methylprednisoloneonly post-operatively (2mg/k/day for 1 month).infants in gi (n=30) received methylprednisolone for 3 successivedays before operation (10-8-6mg/kg/day), and 10 mg/kat the day of operation respectively. thereafter the dose wastapered in the next successive 6 days by 8, 6, 5, 4, 3, and 2mg/kg/day and continued for one month.results: seventy two infants with biliary atresia were operated(39 girls and 33 boys). twenty-six of the 30 patients(86%) in g i became jaundice-free within 90 days after portoenterostomywhile only seven (15%) of the 42 patients in gii had normal bilirubin (p<0.0001). episodes of postoperativecholangitis in g i were 20% (6 of 30), and 53% (24 of 42) ing ii (p<0.005). the difference in 3-year survival rate betweenthe two groups is also remarkable: eighty seven percent (26 of30) in gi versus 29% (13 of 45) in g ii (p<0.005). death relatedto biliary atresia occurred in 1 (3.3%) patient in gi comparedwith 12 (29%) patients in g ii (p<0.005).conclusion: these results provide strong evidence that perioperativehigh dose steroid therapy is not only safe in this patientspopulation, but because of its anti-inflammatory andcholerrhetic effects has a positive impact on preventing recurrentcholangitis, and ultimately survival.iran j med sci 2008; 33(2): 79-83.