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.

Data from early in vivo experiments demonstrated that the child-size Jarvik heart was capable of providing partial to nearly complete circulatory support with acceptable adverse effects on blood. However, bearing thrombosis was responsible for device malfunction in most cases. To overcome this problem, original pin bearings were replaced with novel conical bearings. This study evaluated chronic in vivo performance of the modified child-size Jarvik heart in the pediatric setting. Six juvenile sheep were implanted with the modified child-size Jarvik heart. Cardiac and pump output were measured daily. Serial blood samples were drawn to evaluate hematology, biocompatibility, and end-organ function. End-organ damage and device thrombosis were examined at necropsy. No device malfunction occurred during animal experiments up to 70 days. Mean cardiac output of the animals was 3.4 L/min. The child-size Jarvik heart was able to deliver a blood flow ranging from 1.4 to 2.5 L/min at speed from 10,000 rpm to 14,000 rpm. Mean plasma-free hemoglobin was 9.8 +/- 5.6 mg/dl, indicating no hemolysis. Acute elevation occurred in some organ function tests after the implant surgery but returned to normal range thereafter. These indices and necropsy showed no end-organ damage. No device thrombosis was observed. The current in vivo experience shows that the modified child Jarvik 2000 heart retained its hemodynamic function and excellent biocompatibility, and the conical bearings permitted it to remain free of thrombus.

To overcome the shortage of kidney donors, diverse methods have been utilized, including living donor kidney transplantation (LDKT) and extended criteria for deceased donor grafts. Currently, LDKT is a major treatment option. However, in Jeju, deceased donor kidney transplantation (DDKT) has been restricted due to the prolonged cold ischemia time (CIT) caused by the island's geographic disadvantages and transportation limitations. We report the first LDKT in Jeju, which demonstrates a means of overcoming prolonged CIT. A 67-year-old male patient with diabetic nephropathy underwent a preemptive ABO compatible LDKT (ABO type O+) from his 62-year-old wife. The operation was uneventful, using anti-thymocyte globulin based on the recipient's medical and immunologic risks. The patient recovered without significant complications and was discharged on postoperative day 15. Follow-up Doppler ultrasonography showed good blood flow to the kidney, and his serum creatinine levels steadily decreased and remained stable. The first successful LDKT in Jeju was significant in that kidney transplantation was implemented by compensating for its geographic limitations. In addition, we review machine perfusion as another method of avoiding prolonged CIT.

Short donor renal vessels during donor nephrectomy represent a technical challenge. The allograft of vessels from deceased donors can be an option for reconstruction; however, cryopreserved vessels are not routinely prepared for living donor kidney transplantation (LDKT). We report a reconstruction of the damaged short renal artery (RA) in LDKT using a polytetrafluoroethylene (PTFE) graft. A 45-year-old male patient underwent ABO-incompatible LDKT from his wife. After donor nephrectomy, we detected a hematoma surrounding the proximal RA of the allograft. The injured segment of the RA was transected, and the short RA was connected to the right external artery of the recipient; however, the blood flow was interrupted by the graft location. Once the arterial anastomosis was removed, the graft was flushed with cold saline, and a PTFE graft was used for the reconstruction of the short RA. Immediate blood flow to the renal graft was excellent without sign of parenchymal infarction until fascial closure. Renal graft Doppler on postoperative day 7 and 3 months showed good blood flow. In this patient, the use of PTFE graft presented no additional morbidity to the kidney transplantation, and no postoperative complications related to its use were noted.