Neonatal Dubin-Johnson Syndrome and its Differentiation from Biliary Atresia.

ABSTRACT:

Background and aims

The aim was to determine if liver biochemistry indices can be used as biomarkers to help differentiate patients with neonatal Dubin-Johnson syndrome (nDJS) from those with biliary atresia (BA).

Methods

Patients with genetically-confirmed nDJS or cholangiographically confirmed BA were retrospectively enrolled and randomly assigned to discovery or verification cohorts. Their liver chemistries, measured during the neonatal period, were compared. Predictive values were calculated by receiver operating characteristic curve analysis.

Results

A cohort of 53 nDJS patients was recruited, of whom 13 presented with acholic stools, and 14 underwent diagnostic cholangiography or needle liver biopsy to differentiate from BA. Thirty-five patients in the cohort, w

SUBMITTER: Liu T 

PROVIDER: S-EPMC9647112 | biostudies-literature | 2023 Feb

REPOSITORIES: biostudies-literature