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Authors
Advisor(s)
Abstract(s)
Apesar de rara na infância, a Insuficiência Hepática Aguda (IHA) apresenta elevadas taxas
de mortalidade e morbilidade, especialmente se não for identificada e tratada
precocemente. Esta síndrome clínica resulta de lesão hepática aguda e grave num paciente
sem doença hepática prévia e, na infância, quando passíveis de ser determinadas, as suas
principais causas incluem: ingestão de toxinas e os
de fármacos ou drogas, infeções, alterações genéticas e metabólicas, doenças imunomediadas e isquemia. Como ainda não existem opções terapêuticas capazes de reverter a
necrose hepatocelular que está na sua origem, o tratamento baseia-se em cuidados de
suporte e terapêutica dirigida à causa (quando determinada) que são suficientes e permitem
recuperação hepática espontânea em alguns casos; noutros, cerca de 50%, a sobrevivência
exige transplante hepático. Apesar de continuar a apresentar uma elevada taxa de
mortalidade, a introdução de novas técnicas de transplante hepático e as descobertas
recentes na área da imunossupressão permitiram uma melhoria neste parâmetro,
esperando-se que no futuro o maior conhecimento sobre os imunomoduladores envolvidos
e a patofisiologia subjacente possibilitem melhores desfechos clínicos. Os principais
objetivos desta monografia são a atualização das definições, algoritmos de abordagem,
opções terapêuticas disponíveis e avanços prognósticos da IHA Pediátrica.
Although rare, Pediatric Acute Liver Failure (PALF) presents high mortality and morbidity rates, especially if not identified and treated early. This clinical syndrome results from acute and severe liver injury in a patient with no previous liver disease and, in childhood, when determinable, its main causes include ingestion of toxins and drug overdose, infections, genetic and metabolic alterations, immune-mediated diseases and ischemia. As there are still no therapeutic options capable of reversing the hepatocellular necrosis which is at its origin, treatment is based on supportive care and therapy directed at the cause (when determined) which are sufficient and allow spontaneous hepatic recovery in some cases; in others, around 50%, survival requires liver transplantation. Although the mortality rate remains high, the introduction of technical variant grafts and recent discoveries in immunosuppression have allowed an improvement in this parameter, and it is hoped that in the future the greater knowledge of the immunomodulators involved and underlying pathophysiology will enable better clinical outcomes. The main objectives of this monograph are to update definitions, algorithms of approach, available therapeutic options, and prognostic advances in PALF.
Although rare, Pediatric Acute Liver Failure (PALF) presents high mortality and morbidity rates, especially if not identified and treated early. This clinical syndrome results from acute and severe liver injury in a patient with no previous liver disease and, in childhood, when determinable, its main causes include ingestion of toxins and drug overdose, infections, genetic and metabolic alterations, immune-mediated diseases and ischemia. As there are still no therapeutic options capable of reversing the hepatocellular necrosis which is at its origin, treatment is based on supportive care and therapy directed at the cause (when determined) which are sufficient and allow spontaneous hepatic recovery in some cases; in others, around 50%, survival requires liver transplantation. Although the mortality rate remains high, the introduction of technical variant grafts and recent discoveries in immunosuppression have allowed an improvement in this parameter, and it is hoped that in the future the greater knowledge of the immunomodulators involved and underlying pathophysiology will enable better clinical outcomes. The main objectives of this monograph are to update definitions, algorithms of approach, available therapeutic options, and prognostic advances in PALF.
Description
Keywords
Diagnóstico Insuficiência Hepática Aguda Neonatal Insuficiência Hepática Aguda Pediátrica Patofisiologia Prognóstico Terapêutica