Early predictors of unfavourable outcome in progressive cholestasis of northwestern Quebec.

ABSTRACT:

Background

Progressive cholestasis of northwestern Quebec (PCNQ) is a rare and severe form of cirrhosis affecting children from Quebec's First Nations. First described by our group in 1981 and historically named North American Indian childhood cirrhosis, such a condition often requires liver transplantation during the pediatric age. This study aimed at suggesting a more culturally sensitive name for the disease and identifying early prognostic factors for an unfavourable outcome.

Methods

We retrospectively collected data of all 14 consecutive patients diagnosed with PCNQ over the last 20 years and compared children listed for liver transplant before 18 years of age (LT, n = 7) to those with milder disease progression (no-LT, n = 7).

Results

Compared with

SUBMITTER: Khendek L 

PROVIDER: S-EPMC9473563 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature