Domino liver transplantation for select metabolic disorders: Expanding the living donor pool
Neslihan Celik,
James E. Squires,
Kyle Soltys,
Jerry Vockley,
Diana A. Shellmer,
Wonbae Chang,
Kevin Strauss,
Patrick McKiernan,
Armando Ganoza,
Rakesh Sindhi,
Geoffrey Bond,
George Mazariegos,
Ajai Khanna
Affiliations
Neslihan Celik
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
James E. Squires
Pediatric Hepatology Children's Hospital of Pittsburgh of UPMC Pittsburgh Pennsylvania
Kyle Soltys
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
Jerry Vockley
Division of Medical Genetics University of Pittsburgh School of Medicine, Center for Rare Disease Therapy, Children's Hospital of Pittsburgh of UPMC Pittsburgh Pennsylvania
Diana A. Shellmer
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
Wonbae Chang
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
Kevin Strauss
Division of Medical Genetics University of Pittsburgh School of Medicine, Center for Rare Disease Therapy, Children's Hospital of Pittsburgh of UPMC Pittsburgh Pennsylvania
Patrick McKiernan
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
Armando Ganoza
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
Rakesh Sindhi
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
Geoffrey Bond
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
George Mazariegos
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
Ajai Khanna
Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine Pittsburgh Pennsylvania
Abstract Domino liver transplantation (DLT) involves transplanting liver from a patient with metabolic disease into a patient with end‐stage liver disease with the expectation that the recipient will not develop the metabolic syndrome or the recurrent syndrome will have minimal affect. The domino donor gets a deceased donor or a segment of live‐donor liver through the deceased donor organ allocation system. Waitlist mortality for the domino recipient exceeds morbidity associated with getting the donor disease. Between 2015 and 2017, four patients with three metabolic disorders at UPMC Children's Hospital of Pittsburgh underwent DLT with domino allografts from maple syrup urine disease (MSUD) patients. These included patients with propionic acidemia (PA) (n = 1), Crigler‐Najjar (CN) syndrome type‐1 (n = 2), and carbamoyl phosphate synthetase deficiency (CPSD) (n = 1). Mean follow‐up was 1.6 years (range 1.1‐2.1 years). Total bilirubin levels normalized postoperatively in both CN patients and they maintain normal allograft function. The PA patient had normal to minimal elevations of isoleucine and leucine, and no other abnormalities on low protein diet supplemented with a low methionine and valine free formula. No metabolic crises have occurred. The patient with CPSD takes normal baby food. No elevation in ammonia levels have been observed in any of the patients. DLT for a select group of metabolic diseases alleviated the recipients of their metabolic defect with minimal evidence of transferrable‐branched chain amino acid elevations or clinical MSUD despite increased protein intake. DLT using allografts with MSUD expands the live donor liver pool and should be considered for select metabolic diseases that may have a different enzymatic deficiency.