Pediatric liver-kidney transplantation for hepatorenal fibrocystic disease from a living donor.

The indications for and the timing of LT and/or KT for the patients with HRFCD are based on the severity of liver and kidney involvement. Most organs come from living donors, because the number of deceased donors is extremely low in Japan. Therefore, patients with HRFCD may need two organs from living donors. Four patients with HRFCD underwent living donor LT and KT from a single donor. The type of transplantation included combined LKT in one case, sequential LKT in two cases, and sequential KLT in one case. Although the case of combined LKT died because of sepsis, the other cases were doing well. Sequential LKT was successfully performed at the proper timing for each transplant; however, both of the donors suffered from a gastroduodenal ulcer after liver donation because of the psychological burden related to the relatively short period between two donations. In conclusion, living donation for LKT with cautious surgical procedures is not harmful for donors and recipients. However, changes in the allocation system established for deceased donors for HRFCD should be considered to avoid the need for two organ donations from the same living donor.