008 - Combined Liver-Kidney Transplantation in Pediatric Polycystic Kidney Disease

Publication Number: 8.4431

Amber Kazi, Seattle Children's Hospital/University of Washington, Seattle, WA, United States; Jodi Smith, University of Washington School of Medicine, Seattle, WA, United States; Chloe Douglas, Doernbecher Children's Hospital at Oregon Health & Science University, Portland, OR, United States; Sharon M. Bartosh, University of Wisconsin, Madison, WI, United States; Juhi Kumar, UPMC Childrens Hospital of Pittsburgh, Pittsburgh, PA, United States; Lyndsay A. Harshman, University of Iowa Hospitals and Clinics, IOWA CITY, IA, IA, United States; Namrata G. Jain, Hackensack Meridian School of Medicine, Hackensack, NJ, United States; Sarah Kizilbash, University of Minnesota Masonic Children's Hospital, Minneapolis, MN, United States; Rachel Engen, University of Wisconsin School of Medicine and Public Health, Madison, WI, United States

Background: Polycystic kidney disease (PKD) is a genetic disorder that can affect both the kidney and the liver. PKD is a common indication for combined liver-kidney transplant (CLKT), but currently, there is little consensus for the approach to pediatric multi-organ transplant in PKD.

Objective: Our primary outcomes were: to describe the population of PKD patients in transplant including characteristics of candidates, donors, and recipients, and to compare graft and patient survival between CLKT, kidney-alone transplants (KT), and liver-alone transplants (LT).

Design/Methods: Retrospective cohort study of pediatric simultaneous CLKT, LT, and KT in the United States using the Scientific Registry of Transplant Recipients. Population included patients < 18 years old listed between January 1st, 2001 and December 31st, 2019 with a diagnosis of PKD, Caroli’s disease, congenital hepatic fibrosis, or polycystic liver disease.

Results: There were 684 candidates and 630 transplants that met inclusion criteria (112 listed for CLKT, 89 received such grafts). Compared to liver-alone and kidney-alone candidates, CLKT candidates were more likely to have a history of prior transplant (25% prior KT, 2.7% prior LT), and a higher rate of death on waitlist (8.9% CLKT, 3.1% KT, 2.4% LT) despite a shorter waitlist time. They were also more likely to be listed at multiple centers (44.6% CLKT, 7.8% KT, 12.9% LT). Overall, KT was the most common single organ transplant for PKD (458 KT vs 89 CLKT vs 83 LT). CLKT recipients had a similar MELD/PELD at transplant to LT recipients and a similar incidence of pre-emptive transplantation to KT recipients. CLKT recipients had a longer kidney cold ischemia time , KDPI >35% (34.8% CLKT vs 7% KT), and higher DR mismatch (52.8% with 2 HLA-DR mismatches) compared to KT alone. Thymoglobulin induction was more commonly used for CLKT recipients. CLKT had lower rejection incidence than KT alone (7.9% versus 25.6 %), but similar rejection incidence to LT (18.0 % versus 24.1 %). KT and LT allograft survival were higher among CLKT compared to KT or LT alone at 10 years. There was no difference in patient survival at 10 years.

Conclusion(s): Patients with PKD were more commonly transplanted with a kidney-alone. Patients who were listed for CLKT had prior transplants, higher incidence of death on the waitlist, and received higher KDPI and higher DR mismatched kidneys. Despite this, kidney and liver graft survival were higher in CLKT after 10 years and rejection rates were lower. This data provides evidence and guidance to aid in decision making regarding transplants in pediatric PKD.

Table 1. Characteristics of PKD candidates and recipients who received a CLKT, KT, or LT.


Table 2. Characteristics of donors to PKD recipients for CLKT, KT, or LT.


Table 3. Immunosuppression, patient survival, and graft survival comparing PKD patients who received CLKT, KT, or LT.