P0362 SERUM SELENIUM CONCENTRATIONS OF CIRRHOTIC CHILDREN

Results: 88 infants with neonatal cholestasis were reviewed.The 3 commonest diagnoses were BA (n = 24), CMV hepatitis (n = 11) and parenteral nutrition-related cholestasis (n = 4).12 infants had various known causes of neonatal cholestasis, while the cause was unknown for 37 infants.No alpha 1-antitrypsin deficiency was noted.Of the 24 cases of BA, HPE was not performed in 5 (21%) who were all referred late (ranged 132-192 days, median 134 days).One infant's parents refused surgery.All these 6 infants had since died (median age of death: 8 months).Of the 18 infants who had HPE, 1 died 6 weeks after HPE due to post-operative complications.Nine infants (50%) had persistent jaundice 6 months after HPE.One infant underwent LT at a neighboring country at 1 year of age and is alive and well 6 years after the LT.The remaining 8 had all died.Eight infants (44%) were jaundice-free 6 months after HPE.Of these, 6 (75%) were alive with minimal morbidity (median follow-up 5 years), while 2 had major complications (esophageal varices and hypersplenism).The overall success rate for HPE was 44%.Of the 7 infants who had HPE <60 days of age, 5 (71%) were jaundice-free by 6 months, while only 3 of the 11 infants (27%) who had HPE >60 days were jaundice-free by 6 months (p = 0.18).The overall survival with native liver 1 year after HPE was 33%.Conclusion: BA is the most important cause of neonatal cholestasis in Malaysian infants, but a ´1-antitrypsin deficiency is not seen.Late referral is common in infants with BA, and there was a trend for better outcome if HPE was performed <60 days of age.For infants with BA in country where access to LT is limited, early referral for HPE offered the best prospect for long term survival.