Extrahepatic biliary atresia: a disease or a phenotype?
DH Perlmutter, RW Shepherd - Hepatology, 2002 - journals.lww.com
In infants with extrahepatic biliary atresia (BA) there is obliteration or discontinuity of the
hepatic or common bile ducts at any point from the porta hepatis to the duodenum. When
untreated, the condition results in severe liver injury and death in all cases. The
development of the hepatoportoenterostomy procedure in 1959 by Kasai has permitted long-
term survival in some of the affected infants (10%-20%). The advent of liver replacement
therapy has permitted long-term survival in many of the remaining infants. The estimated …
hepatic or common bile ducts at any point from the porta hepatis to the duodenum. When
untreated, the condition results in severe liver injury and death in all cases. The
development of the hepatoportoenterostomy procedure in 1959 by Kasai has permitted long-
term survival in some of the affected infants (10%-20%). The advent of liver replacement
therapy has permitted long-term survival in many of the remaining infants. The estimated …
