RESUMO
Hyperammonaemia in children can lead to grave consequences in the form of cerebral oedema, severe neurological impairment and even death. In infants and children, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias. Few studies have assessed the role of extracorporeal therapies in the management of hyperammonaemia in neonates and children. Moreover, consensus guidelines are lacking for the use of non-kidney replacement therapy (NKRT) and kidney replacement therapies (KRTs, including peritoneal dialysis, continuous KRT, haemodialysis and hybrid therapy) to manage hyperammonaemia in neonates and children. Prompt treatment with KRT and/or NKRT, the choice of which depends on the ammonia concentrations and presenting symptoms of the patient, is crucial. This expert Consensus Statement presents recommendations for the management of hyperammonaemia requiring KRT in paediatric populations. Additional studies are required to strengthen these recommendations.
Assuntos
Terapia de Substituição Renal Contínua/métodos , Hiperamonemia/terapia , Diálise Peritoneal/métodos , Distúrbios Congênitos do Ciclo da Ureia/terapia , Arginina/uso terapêutico , Carnitina/uso terapêutico , Criança , Pré-Escolar , Técnica Delphi , Dieta com Restrição de Proteínas , Humanos , Terapia de Substituição Renal Híbrida , Hiperamonemia/metabolismo , Lactente , Recém-Nascido , Nutrição Parenteral/métodos , Fenilacetatos/uso terapêutico , Fenilbutiratos/uso terapêutico , Guias de Prática Clínica como Assunto , Diálise Renal/métodos , Benzoato de Sódio/uso terapêutico , Distúrbios Congênitos do Ciclo da Ureia/metabolismo , Complexo Vitamínico B/uso terapêuticoRESUMO
Background: A common practice in the management of critically ill patients is fluid resuscitation. An excessive administration of fluids can lead to an imbalance in fluid homeostasis and cause fluid overload (FO). In pediatric critical care patients, FO can lead to a multitude of adverse effects and increased risk of morbidity. Objectives: To review the literature highlighting impact of FO on a multitude of outcomes in critically-ill children, causative vs. associative relationship of FO with critical illness and current pediatric fluid management guidelines. Data Sources: A literature search was conducted using PubMed/Medline and Embase databases from the earliest available date until June 2017. Data Extraction: Two authors independently reviewed the titles and abstracts of all articles which were assessed for inclusion. The manuscripts of studies deemed relevant to the objectives of this review were then retrieved and associated reference lists hand-searched. Data Synthesis: Articles were segregated into various categories namely pathophysiology and sequelae of fluid overload, assessment techniques, epidemiology and fluid management. Each author reviewed the selected articles in categories assigned to them. All authors participated in the final review process. Conclusions: Recent evidence has purported a relationship between mortality and FO, which can be validated by prospective RCTs (randomized controlled trials). The current literature demonstrates that "clinically significant" degree of FO could be below 10%. The lack of a standardized method to assess FB (fluid balance) and a universal definition of FO are issues that need to be addressed. To date, the impact of early goal directed therapy and utility of hemodynamic parameters in predicting fluid responsiveness remains underexplored in pediatric resuscitation.