The Importance of Early Referral in Pediatric Acute Liver Failure

Bibliographic Details
Main Author: Jerónimo, Mónica
Publication Date: 2015
Other Authors: Moinho, Rita, Pinto, Carla, Carvalho, Leonor, Gonçalves, Isabel, Furtado, Emanuel, Farela Neves, José
Format: Article
Language: por
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/6195
Summary: Introduction: Acute liver failure is a rare disorder associated to high morbidity and mortality despite survival improvement through liver transplantation. The importance of a multidisciplinary approach and early referral to a pediatric liver transplantation center were important conclusions of a national meeting in 2008, from which resulted an actuation consensus.Objectives: To characterize acute liver failure admissions in a Pediatric Intensive Care Unit of the portuguese pediatric livertransplantation center. To compare results before (A) and after (B) 2008.Material and Methods: Observational, retrospective study during a 20 year period (1994-2014). Inclusion criteria: age < 18 years old and acute liver failure (INR ≥ 2 without vitamin K response and hepatocellular necrosis). Children with previous liver disease were excluded.Results: Fifty children were included, with median age of 24.5 months. The most common etiology under 2 years old was metabolic (34.6%) and above that age was infectious (29.2%). Forty six percent were submitted to liver transplantation and 78% of them survived. Overall mortality was 34%. Median referral time was 7 days in period A (n = 35) and 2 days in period B (n = 15; p = 0.006). Pediatric risk of mortality´s median was 14.7 in period A and 6.5 in B (p = 0.019). Mortality was 37% vs 26% in periods A and B, respectively (p = 0.474).Discussion and Conclusions: Overall mortality was similar to the observed in other European centers. Liver transplantation is in fact the most effective therapeutic option. After 2008, there was a reduction in referral time and cases severity on admission; however, mortality has not reduced so far.
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spelling The Importance of Early Referral in Pediatric Acute Liver FailureA Importância da Referenciação Precoce na Falência Hepática Aguda PediátricaChildLiver FailureAcuteLiver TransplantationReferral and Consultation.CriançaFalência Hepática AgudaReferenciaçãoTransplante de Fígado.Introduction: Acute liver failure is a rare disorder associated to high morbidity and mortality despite survival improvement through liver transplantation. The importance of a multidisciplinary approach and early referral to a pediatric liver transplantation center were important conclusions of a national meeting in 2008, from which resulted an actuation consensus.Objectives: To characterize acute liver failure admissions in a Pediatric Intensive Care Unit of the portuguese pediatric livertransplantation center. To compare results before (A) and after (B) 2008.Material and Methods: Observational, retrospective study during a 20 year period (1994-2014). Inclusion criteria: age < 18 years old and acute liver failure (INR ≥ 2 without vitamin K response and hepatocellular necrosis). Children with previous liver disease were excluded.Results: Fifty children were included, with median age of 24.5 months. The most common etiology under 2 years old was metabolic (34.6%) and above that age was infectious (29.2%). Forty six percent were submitted to liver transplantation and 78% of them survived. Overall mortality was 34%. Median referral time was 7 days in period A (n = 35) and 2 days in period B (n = 15; p = 0.006). Pediatric risk of mortality´s median was 14.7 in period A and 6.5 in B (p = 0.019). Mortality was 37% vs 26% in periods A and B, respectively (p = 0.474).Discussion and Conclusions: Overall mortality was similar to the observed in other European centers. Liver transplantation is in fact the most effective therapeutic option. After 2008, there was a reduction in referral time and cases severity on admission; however, mortality has not reduced so far.Introdução: A falência hepática aguda é uma doença rara associada a elevada morbilidade e mortalidade apesar do aumento da sobrevida devido ao transplante hepático. Em 2008, decorreu em Portugal uma reunião sobre esta patologia em pediatria, resultando num consenso de atuação que salientou a importância da abordagem multidisciplinar e referenciação precoce para um centro de transplantação hepática.Objetivos: Caracterizar as admissões por falência hepática aguda no Serviço de Cuidados Intensivos Pediátricos do centro português com transplante hepático pediátrico. Comparar resultados antes (A) e depois de 2008 (B).Material e Métodos: Estudo observacional retrospetivo de 20 anos (1994-2014). Critérios de inclusão: idade < 18 anos e falência hepática aguda (INR ≥ 2 sem resposta à vitamina K e necrose hepatocelular). Excluíram-se as crianças com doença hepática crónica.Resultados: Incluíram-se 50 crianças com idade mediana de 24,5 meses. A causa mais comum de falência hepática aguda abaixo dos 2 anos foi metabólica (34,6%) e acima infeciosa (29,2%). Foram submetidos a transplante hepático 46%, tendo sobrevivido 78%. A mortalidade global foi 34%. A mediana do tempo de referenciação foi 7 dias no período A (n = 35) e 2 no B (n = 15; p = 0,006). A mediana do risco de mortalidade prevista pelo PRISM foi 14,7% no período A e 6,5% no B (p = 0,019). A mortalidade foi 37% vs 26% no período A e B respetivamente (p = 0,474).Discussão e Conclusões: A mortalidade global foi sobreponível à de outros centros europeus, sendo o transplante hepático a opção terapêutica mais eficaz. Após 2008 o tempo de referenciação e a gravidade dos casos na admissão reduziram, ainda sem tradução significativa na mortalidade.Ordem dos Médicos2015-09-11info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfimage/jpegimage/jpegimage/jpegapplication/mswordapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/6195oai:ojs.www.actamedicaportuguesa.com:article/6195Acta Médica Portuguesa; Vol. 28 No. 5 (2015): September-October; 559-566Acta Médica Portuguesa; Vol. 28 N.º 5 (2015): Setembro-Outubro; 559-5661646-07580870-399Xreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAPporhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/6195https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/6195/4487https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/6195/7495https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/6195/7496https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/6195/7497https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/6195/7592https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/6195/7960Jerónimo, MónicaMoinho, RitaPinto, CarlaCarvalho, LeonorGonçalves, IsabelFurtado, EmanuelFarela Neves, Joséinfo:eu-repo/semantics/openAccess2022-12-20T11:04:46Zoai:ojs.www.actamedicaportuguesa.com:article/6195Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T10:40:14.611191Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv The Importance of Early Referral in Pediatric Acute Liver Failure
A Importância da Referenciação Precoce na Falência Hepática Aguda Pediátrica
title The Importance of Early Referral in Pediatric Acute Liver Failure
spellingShingle The Importance of Early Referral in Pediatric Acute Liver Failure
Jerónimo, Mónica
Child
Liver Failure
Acute
Liver Transplantation
Referral and Consultation.
Criança
Falência Hepática Aguda
Referenciação
Transplante de Fígado.
title_short The Importance of Early Referral in Pediatric Acute Liver Failure
title_full The Importance of Early Referral in Pediatric Acute Liver Failure
title_fullStr The Importance of Early Referral in Pediatric Acute Liver Failure
title_full_unstemmed The Importance of Early Referral in Pediatric Acute Liver Failure
title_sort The Importance of Early Referral in Pediatric Acute Liver Failure
author Jerónimo, Mónica
author_facet Jerónimo, Mónica
Moinho, Rita
Pinto, Carla
Carvalho, Leonor
Gonçalves, Isabel
Furtado, Emanuel
Farela Neves, José
author_role author
author2 Moinho, Rita
Pinto, Carla
Carvalho, Leonor
Gonçalves, Isabel
Furtado, Emanuel
Farela Neves, José
author2_role author
author
author
author
author
author
dc.contributor.author.fl_str_mv Jerónimo, Mónica
Moinho, Rita
Pinto, Carla
Carvalho, Leonor
Gonçalves, Isabel
Furtado, Emanuel
Farela Neves, José
dc.subject.por.fl_str_mv Child
Liver Failure
Acute
Liver Transplantation
Referral and Consultation.
Criança
Falência Hepática Aguda
Referenciação
Transplante de Fígado.
topic Child
Liver Failure
Acute
Liver Transplantation
Referral and Consultation.
Criança
Falência Hepática Aguda
Referenciação
Transplante de Fígado.
description Introduction: Acute liver failure is a rare disorder associated to high morbidity and mortality despite survival improvement through liver transplantation. The importance of a multidisciplinary approach and early referral to a pediatric liver transplantation center were important conclusions of a national meeting in 2008, from which resulted an actuation consensus.Objectives: To characterize acute liver failure admissions in a Pediatric Intensive Care Unit of the portuguese pediatric livertransplantation center. To compare results before (A) and after (B) 2008.Material and Methods: Observational, retrospective study during a 20 year period (1994-2014). Inclusion criteria: age < 18 years old and acute liver failure (INR ≥ 2 without vitamin K response and hepatocellular necrosis). Children with previous liver disease were excluded.Results: Fifty children were included, with median age of 24.5 months. The most common etiology under 2 years old was metabolic (34.6%) and above that age was infectious (29.2%). Forty six percent were submitted to liver transplantation and 78% of them survived. Overall mortality was 34%. Median referral time was 7 days in period A (n = 35) and 2 days in period B (n = 15; p = 0.006). Pediatric risk of mortality´s median was 14.7 in period A and 6.5 in B (p = 0.019). Mortality was 37% vs 26% in periods A and B, respectively (p = 0.474).Discussion and Conclusions: Overall mortality was similar to the observed in other European centers. Liver transplantation is in fact the most effective therapeutic option. After 2008, there was a reduction in referral time and cases severity on admission; however, mortality has not reduced so far.
publishDate 2015
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dc.publisher.none.fl_str_mv Ordem dos Médicos
publisher.none.fl_str_mv Ordem dos Médicos
dc.source.none.fl_str_mv Acta Médica Portuguesa; Vol. 28 No. 5 (2015): September-October; 559-566
Acta Médica Portuguesa; Vol. 28 N.º 5 (2015): Setembro-Outubro; 559-566
1646-0758
0870-399X
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reponame_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
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