Di Giorgio A, Bartolini E, Calvo P L, Cananzi M, Cirillo F, Della Corte C, Dionisi-Vici C, Indolfi G, Iorio R, Maggiore G, Mandato C, Nebbia G, Nicastro E, Pinon M, Ranucci G, Sciveres M, Vajro P, D'Antiga L
Paediatric Liver, GI and Transplantation, ASST-Hospital Papa Giovanni XXIII, Piazza OMS1, Bergamo 24127, Italy.
Department Neurofarba, University of Florence and Liver Unit, Meyer Children's University Hospital, Florence, Italy.
Dig Liver Dis. 2021 May;53(5):545-557. doi: 10.1016/j.dld.2021.03.004. Epub 2021 Mar 26.
Acute liver failure (ALF) is a clinical condition characterized by the abrupt onset of coagulopathy and biochemical evidence of hepatocellular injury, leading to rapid deterioration of liver cell function. In children, ALF has been characterized by raised transaminases, coagulopathy, and no known evidence of pre-existing chronic liver disease; unlike in adults, the presence of hepatic encephalopathy is not required to establish the diagnosis. Although rare, ALF has a high mortality rate without liver transplantation (LT). Etiology of ALF varies with age and geographical location, although it may remain indeterminate in a significant proportion of cases. However, identifying its etiology is crucial to undertake disease-specific management and evaluate indication to LT. In this position statement, the Liver Disease Working Group of the Italian Society of Gastroenterology, Hepatology and Nutrition (SIGENP) reviewed the most relevant studies on pediatric ALF to provide recommendations on etiology, clinical features and diagnostic work-up of neonates, infants and children presenting with ALF. Recommendations on medical management and transplant candidacy will be discussed in a following consensus conference.
急性肝衰竭（ALF）是一种临床病症，其特征为凝血功能障碍的突然发作以及肝细胞损伤的生化证据，导致肝细胞功能迅速恶化。在儿童中，ALF的特征是转氨酶升高、凝血功能障碍，且无已知的先前存在的慢性肝病证据；与成人不同，诊断ALF无需存在肝性脑病。尽管罕见，但未经肝移植（LT）的ALF死亡率很高。ALF的病因因年龄和地理位置而异，尽管在相当一部分病例中病因可能仍不明确。然而，确定其病因对于进行针对疾病的管理和评估LT指征至关重要。在本立场声明中，意大利胃肠病学、肝病学和营养学会（SIGENP）肝病工作组回顾了关于儿童ALF的最相关研究，以就患有ALF的新生儿、婴儿和儿童的病因、临床特征及诊断检查提供建议。关于药物治疗和移植候选资格的建议将在随后的共识会议中讨论。
