A. Gurkan et al., Unsuspected bile duct paucity in donors for living-related liver transplantation: Two case reports, TRANSPLANT, 67(3), 1999, pp. 416-418
Alagille's syndrome is a common cause of liver dis ease in children and may
lead to the need for orthotopic liver transplantation Alagille's syndrome
is inherited in an autosomal dominant manner, with variable penetration, an
d may also be present in patients' parents, who may be considered potential
donors for living-related transplantation We report here on two cases in w
hich the living-related donors for children with Alagille's syndrome had no
Liver function abnormalities or characteristic features of Alagille's synd
rome. In both cases, the operation for living-related donation had to be ab
orted because of a paucity of bile ducts discovered intraoperatively. Given
the variable presentation of Alagille's syndrome, we believe that it is ne
cessary preoperatively to evaluate the biliary system of family members who
are potential living-related donors for patients with this condition.