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    First case of cross-auxiliary double domino donor liver transplantation
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    Abstract:
    We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy (FAP) and liver dysfunction.A two-stage surgical plan was designed, and one domino graft was implanted during each stage.During the first CASE
    Keywords:
    Domino
    Liver function
    Liver disease
    In 1983 we assessed the implications for hepatic transplantation programs by studying mortality from liver disease in a tertiary care children's hospital. The current study reviews the impact of orthotopic liver transplantation (OLT) on survival for the period 1984–1989. Findings showed that deaths from liver disease decreased from 9.2 to 3.8 per year. Twenty-eight infants and children were referred and underwent OLT, with a 64% survival rate. Deaths from biliary atresia, which used to account for 24% of the total, have been reduced to 4.3%. Deaths from liver failure in infancy (which decreased from 49% to 39%) still present formidable challenges for transplantation. The implications of these findings are discussed.
    Liver disease
    Orthotopic liver transplantation
    To assess the utility of prognostic scoring systems for adolescents with biliary atresia (BA) surviving with native liver, for predicting the subsequent requirement for liver transplantation (LT).Single-centre retrospective analysis of 397 BA patients who received Kasai Portoenterostomy (KP) 1980-1996 and survived with the native liver at 16 years. Laboratory and clinical variables at 16 years (timepoint 16 years) were used to calculate (i) LT allocation scores; Model for End-Stage Liver Disease [MELD/MELD-sodium (Na)], and UK End-Stage Liver Disease (UKELD); (ii) Mayo Primary Sclerosing Cholangitis risk score (MayoPSC) and (iii) a modified Paediatric End-Stage Liver Disease (PELD) score. Scores were compared between patients requiring LT after 16 years of age (LT > 16 years), and those who survived with native liver, at the latest follow-up. Additional subgroup analysis for patients with data available at 12 years (timepoint 12 years).MELD (area under the receiver operating characteristic [AUROC] 0.847) and UKELD (AUROC: 0.815) at 16 years of age predict the need for LT > 16 years. No advantage for MELD-Na over MELD was demonstrated. MELD >8.5 and UKELD >47 predicted LT > 16 years with 84% and 79% sensitivity and 73% and 73% specificity. PELD had a similar performance to MELD, but superiority to UKELD. MayoPSC revealed predictive accuracy for LT >16 years (AUROC 0.859), with a score of >0.87 predicting LT > 16 years with 85% sensitivity and 82% specificity. At timepoint 12 years, MELD and MayoPSC predicted LT >16 years. Change in MELD, PELD and MayoPSC between 12 and 16 years of age, was associated with LT >16 years.Adult LT allocation scores may help monitor progress in adolescent BA, but the omission of relevant risk factors limits their utility for listing in this cohort. A BA-specific prognostic score would improve the management of adolescent BA.
    Liver disease
    Primary Sclerosing Cholangitis