From the Department of Pediatrics I, Center for Pediatric and Adolescent Medicine, University Hospital Heidelberg, Heidelberg, Germany.
European Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany.
J Pediatr Gastroenterol Nutr. 2023 Jul 1;77(1):115-120. doi: 10.1097/MPG.0000000000003777. Epub 2023 Mar 17.
Acute liver failure (ALF) is rare but life-threatening. Common causes include intoxications, infections, and metabolic disorders. Indeterminate etiology is still frequent. No systematic data on incidence, causes, and outcome of ALF across Europe are available. Via an online survey we reached out to European Reference Network Centers on rare liver diseases. Numbers and etiology of ALF cases during 2020 were retrieved and diagnostic and treatment availabilities assessed. In total, 455 cases (306 adult, 149 pediatric) were reported from 36 centers from 20 countries. Intoxication was the most common cause in adult and pediatric care. The number of cases with indeterminate etiology is low. Diagnostic tools and specific treatment options are broadly available within this network. This is the first approach to report on etiology and outcome of ALF in the pediatric and adult population in Europe. High diagnostic yield and standard of care reflects the expert status of involved centers.
急性肝衰竭(ALF)较为罕见,但危及生命。常见病因包括中毒、感染和代谢紊乱。病因仍不明确的情况仍较为常见。目前尚无关于欧洲 ALF 的发病率、病因和结局的系统数据。我们通过在线调查联系了欧洲罕见肝病参考网络中心。检索了 2020 年 ALF 病例的数量和病因,并评估了诊断和治疗的可及性。来自 20 个国家的 36 个中心共报告了 455 例病例(306 例成人,149 例儿童)。在成人和儿童护理中,中毒是最常见的病因。病因不明的病例数量较少。该网络内广泛提供诊断工具和特定治疗选择。这是首次报告欧洲儿科和成人 ALF 的病因和结局。高诊断率和标准的治疗反映了参与中心的专业地位。