AIM OF THE STUDY: Early treatment by Kasai portoenterostomy (KPE) can delay progression of fibrosis in biliary atresia (BA). Therefore early detection is crucial. Neonatal jaundice is more visible in children with a Caucasian heritage. Therefore we aimed to investigate differences in the referral pattern between Caucasian and non-Caucasian children with suspected BA in the Netherlands
METHODS: Retrospective pilot cohort study, including Dutch children treated for BA whose ethnicity was known. We compared age at primary presentation in an hospital, days between primary presentation and referral to a national referral centre, age at KPE and percentage KPEs < 60 days in Caucasian and non-Caucasian children.
MAIN RESULTS: We included 49 Dutch children treated for BA in the Netherlands between 2008-2017. There were 38 and 11 non-Caucasian patients. There were 14 boys and 35 girls. Age at primary presentation was 38 (28-50) vs. 64 (55-78) days in Caucasian vs non-Caucasian patients, p=0.001. There were 5 (1-7) vs. 5 (2-19) days between first presentation and referral to the national referral centre, p=0.66. Median age at KPE procedure is 56 (49-65) vs 76 (68-85) days in Caucasian vs. non-Caucasian group, p=0.001. 68% of the Caucasian group underwent KPE < 60 days vs. 9% in the non-Caucasian group, p=0.001.
CONCLUSIONS: BA is later recognised in non-Caucasian children in the Netherlands. This leads to delayed treatment and a worse outcome. Efforts should be made to increase awareness in parents as well as caregivers.
Figure 1 Timetable of the Caucasian and non-Caucasian group: primary presentation, referral to a national referral centre and KPE.