Living related liver transplantation in 2 families of Byler's disease

Recently, we have experienced 4 cases of Byler's disease (BD) in 2 families in which both parents needed to be a donor of living related liver transplantation (LRLT) for their each children because of sibling onset. In this paper, we report those clinical course, and discuss the problems on timing of LRLT and donor selection. Cases: Family 1 had 3 patient with BD in 4 siblings. Although one died from severe pneumonia on 3 months after birth, Two siblings needed to have LRLT. Elder daughter have received identical graft 6 years ago from her mother and now in healthy status, but younger son of blood group A, had an incompatible graft, from father of blood group B and died 28 days after surgery from repeated rejection and sepsis. Family 2 has also 2 sibling patient with BD. In contrast to family 1, the blood group analysis of this family showed all A. Therefore, both patients could receive identical graft each from their parents. However, elder daughter who had a liver graft from her father on 1 year ago, is in a condition of chronic rejection, and may need retransplantation. Whereas, younger son is in healthy without any episodes of rejection or infection.