Domino liver transplantation for maple syrup urine disease in children: A single-center case series.

BACKGROUND: Domino liver transplant (DLT) represents another type of liver donor to expand the donor pool. Recent reports of successful DLT in children with maple syrup urine disease (MSUD) show promising long-term outcomes. METHODS: It was a retrospective study. All children with MSUD were paired with either recipients with end-stage liver disease (ESLD) or non-MSUD metabolic disease. Each pair underwent simultaneous liver transplant (LT), where the MSUD recipient received the graft from a living-related donor and the liver explanted from the MSUD donor was transplanted to the respective paired domino recipient. We report our experience regarding the techniques and outcomes of DLT at our center. RESULTS: Eleven children with MSUD and 12 respective DLT recipients were enrolled, one of which was domino split-liver transplantation. DLT recipients included seven ESLD, two propionic acidemia (PA), one glycogen storage disease(GSD) type-1, one GSD type-3, and one Citrullinemia. Post-LT ICU and hospital stays were comparable (p > .05). Patient and graft survival was 100% and 66.6% in the MSUD group and DLT recipients at a mean follow-up of 13.5 and 15 months. There was no death in the MSUD group as compared to four in the DLT group. The amino acid levels rapidly normalized after the LT in the children with MSUD and they tolerated the normal unrestricted diet. No vascular, biliary, or graft-related complications were seen in the post-transplant period. No occurrence of MSUD was noted in DLT recipients. CONCLUSION: DLTs have excellent post-surgical outcomes. DLT should be strongly considered and adopted by transplant programs worldwide to circumvent organ shortage.

Living Donor Liver Transplant in patients with Hepatorenal Syndrome without the use of Intraoperative Renal Replacement Therapy, a single-center experience.

BACKGROUND: Continuous Renal Replacement Therapy (CRRT) is often used to support the intraoperative course during liver transplantation (LT) for patients with HRS. However, the use of intraoperative CRRT (IOCRRT) is not without its problems. Living donor liver transplantation (LDLT) is a planned operation and is possible without IOCRRT as the recipient can be optimized. AIM: To study the peritransplant outcomes of patients with CLD and HRS undergoing LT without IOCRRT. METHODS: Analysis of LT program database for perioperative outcomes in patients with HRS from Feb 2017 to Dec 2018. RESULTS: 87/363 (23.9%) adult LDLT patients had HRS, of whom 31 (35.6%) did not respond (NR) to standard medical therapy (SMT) prior to LT. Modified perioperative protocol enabled the NR patients (who were sicker and in persistent renal failure) to undergo LT without IOCRRT. Postoperative renal dysfunction was similar (2 in NR and 2 in R) at 1 year. Post-LT survival was also not different at one month (83.87% in NR and 87.5% in R [p = .640]) and at 1 year (77% in NR vs 80.4% in non-responders [p = .709]). CONCLUSION: IOCRRT can be avoided in HRS patients undergoing LDLT without compromising their outcomes (post-LT survival and RD), even in patients who have not responded to SMT, preoperatively.

Correlation between thromboelastography and rotational thromboelastometry values in adult liver transplant recipients.

BACKGROUND AND AIMS: Viscoelastic haemostatic assays (VHA) namely Thromboelastogram (TEG) and Rotational thromboelastometry (ROTEM) are used for global assessment of coagulopathy and guiding transfusion during living donor liver transplant (LDLT).We conducted a study to compare the interchangeability of the values obtained from these devices in patients with End stage liver disease (ESLD) undergoing LDLT. METHODS: In 76 patients undergoing LDLT, ROTEM and TEG were performed and assessed for interchangeability using Spearman Correlation. The direction and strength of correlation between equivalent parameters was calculated using Inter Class Correlation (ICC) and Bland Altman analysis. RESULTS: The correlation ρ between CT (clotting time) of ROTEM and R of TEG was 0.16 (P = 0.19).The ICC was 0.15, with 95% confidence interval (CI) of -0.38-0.48 (P = 0.25).The ρ of CFT (ROTEM) with K (TEG) was 0.425 (P=<0.001). The ICC was0.49 with 95% CI of 0.17-0.69, P = 0.003.Alpha of ROTEM correlated with Angle of TEG with ρ of 0.475 (P=<0.001). The ICC was 0.61, with 95% CI of 0.36-0.76, P=<0.001.Maximum Clot firmness (MCF) correlated with maximum amplitude (MA) with ρ=0.76 (P=<0.001).The ICC was 0.86, with 95% CI of 0.77-0.92, P=<0.001. Lysis index (L30) of ROTEM correlated clot lysis (CL30) of TEG with ρ of 0.16 (P = 0.18).However, the ICC was 0.45, with 95% CI of 0.11-0.66, P = 0.08. The correlation between CT of ROTEM and R of TEG as well as L30 of ROTEM and CL30 of TEG was not significant.The strongest correlation was found between MCF and MA (P < 0.001). However the MCF/MA showed an agreement of only 86% (ICC = 0.86). CONCLUSION: Values from ROTEM and TEG were not found to be interchangeable.