Genetic investigation into an increased susceptibility to biliary atresia in an extended New Zealand Maori family.

ABSTRACT:

Background

Biliary atresia (BA), a fibrosing disorder of the developing biliary tract leading to liver failure in infancy, has an elevated incidence in indigenous New Zealand (NZ) Māori. We investigated a high rate of BA in a group of children (n = 12) belonging to a single Māori iwi (or 'tribe', related through a remote ancestor).

Methods

Population and geographical data was used to estimate the rate of BA in Māori sub-groups, and a pedigree linking most of the affected children was constructed from oral and documented history. Array genotyping was used to examine hypotheses about the inheritance of a possible genetic risk factor, and the history of the affected population, and Exome Sequencing to search for candidate genes.

Results

Most of these affected children (n

SUBMITTER: Cameron-Christie SR 

PROVIDER: S-EPMC6299523 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature