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Adult Liver Disease Prognostic Modelling for Long-term Outcomes in Biliary Atresia: An Observational Cohort Study.
Jain, Vandana; Burford, Charlotte; Alexander, Emma C; Dhawan, Anil; Joshi, Deepak; Davenport, Mark; Heaton, Nigel; Hadzic, Nedim; Samyn, Marianne.
Afiliación
  • Jain V; Paediatric Liver, GI and Nutrition Centre and Mowatlabs.
  • Burford C; Paediatric Liver, GI and Nutrition Centre and Mowatlabs.
  • Alexander EC; Paediatric Liver, GI and Nutrition Centre and Mowatlabs.
  • Dhawan A; Paediatric Liver, GI and Nutrition Centre and Mowatlabs.
  • Joshi D; Institute of Liver Studies.
  • Davenport M; Department of Paediatric Surgery.
  • Heaton N; Liver Transplant Surgery, Institute of Liver Studies, Kings College Hospital, London, UK.
  • Hadzic N; Paediatric Liver, GI and Nutrition Centre and Mowatlabs.
  • Samyn M; Paediatric Liver, GI and Nutrition Centre and Mowatlabs.
J Pediatr Gastroenterol Nutr ; 73(1): 93-98, 2021 07 01.
Article en En | MEDLINE | ID: mdl-33720092
OBJECTIVES: 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). METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Atresia Biliar / Enfermedad Hepática en Estado Terminal Tipo de estudio: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Child / Humans Idioma: En Revista: J Pediatr Gastroenterol Nutr Año: 2021 Tipo del documento: Article Pais de publicación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Atresia Biliar / Enfermedad Hepática en Estado Terminal Tipo de estudio: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Child / Humans Idioma: En Revista: J Pediatr Gastroenterol Nutr Año: 2021 Tipo del documento: Article Pais de publicación: Estados Unidos