Oral

BACKGROUND AND AIM
Biliary atresia (BA) is a progressive, destructive inflammatory obliterative cholangiopathy of neonates affecting varying lengths of both intrahepatic and extrahepatic bile ducts. We aimed to determine the population incidence and outcome of BA in central states of peninsular Malaysia.
 
METHODS
We conducted a retrospective cohort study including all surgically confirmed BA bo in 3 states of peninsular Malaysia (Kuala Lumpur, Selangor and Putrajaya) between 2014 and 2018. Data were collected from medical records of all hospitals in the region. Population statistics were obtained from the Statistics Department, Malaysia. Successful Kasai portoenterostomy (KP) was defined as serum bilirubin <34 mmol/L at 3 months post-surgery. Final outcome, including survival with native liver, after LT and without LT at 3 years, was determined
 
RESULTS
During the study period, 668,850 live births were recorded in the 3 states while 71 (male 36, female 35) cases of BA were confirmed, giving an overall population incidence of 1.06/10,000 (range 0.58 – 1.69) population. There was no gender predilection. Malay children composed 81% of the entire cohort and have the highest incidence of 1.26/10,000 among all ethnic groups. The mean age at surgery was 68 (range 33 – 133) days. Jaundice clearance at 3 months was 67%. At 3 years, 2 patients were lost to follow-up. Of the remaining 69 patients, 43 (62.3%) survived with native liver, 11 (15.9%) survived after LT, and 15 (21.7%) succumbed without LT. Survival with native liver and overall 3-year survival rates were 62.3% and 78.2%, respectively.

CONCLUSIONS
The current study, the first in Malaysia to determine population incidence of BA, showed that the overall outcome of Malaysian infants with BA was adversely affected by a lack of timely LT. It provides important information in the planning early screening, planning and allocating of appropriate resources for LT in Malaysian children with BA.

REFERENCES
Lee WS, Chai PF, Lim KS, Lim LH, Looi LM, Ramanujam TM. The outcome of biliary atresia in Malaysia: a single‐centre study. J Paediatr Child Health 2009,45:279-285.