Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome.

@article{Brinkert2013CombinedLT,
  title={Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome.},
  author={Florian Brinkert and Anja M Lehnhardt and Carmen Montoya and Knut Helmke and Hansjoerg Schaefer and Lutz Fischer and Bjorn A P Nashan and Carsten Bergmann and Rainer Ganschow and Markus Josef Kemper},
  journal={Transplant international : official journal of the European Society for Organ Transplantation},
  year={2013},
  volume={26 6},
  pages={640-50}
}
In ARPKD, mutations in the PKHD1 gene lead to remodeling of the kidneys and liver. These may result in progressive liver fibrosis with portal hypertension requiring combined liver and kidney transplantation (CLKT). There is currently no consensus on the indication for CLKT and data on long-term outcomes are scarce. We analyzed in detail the pretransplant liver symptomatology, laboratory and ultrasound data, histological studies, and genotypes in eight patients undergoing CLKT. The median age… CONTINUE READING