Acute liver failure with hemolytic anemia in children with Wilson's disease: Genotype-phenotype correlations?

Background: Wilson's disease (WD) is a rare autosomal recessive inherited disorder of copper metabolism. Acute liver failure (ALF) and hemolytic anemia represent the most severe presentation of WD in children. No clear genotype-phenotype correlations exist in WD.

Biological and Histological Assessment of the Hepatoportoenterostomy Role in Biliary Atresia as a Stand-Alone Procedure or as a Bridge toward Liver Transplantation.

: In patients with biliary atresia (BA), hepatoportoenterostomy (HPE) is still a valuable therapeutic tool for prolonged survival or a safer transition to liver transplantation. The main focus today is towards efficient screening programs, a faster diagnostic, and prompt treatment. However, the limited information on BA pathophysiology makes valuable any experience in disease management.

Transient elastography of the liver in children with Wilson's disease: Preliminary results.

Purpose: In chronic liver diseases, liver stiffness (LS) is increased, primarily because of liver fibrosis, but other factors, such as intrahepatic deposits, may also be involved. We hypothesized that intrahepatic copper accumulation occurring in Wilson's disease (WD) also leads to an increase in LS. The aim of this study was thus to investigate the changes in LS during treatment in pediatric patients with WD.