The aetiology of several liver diseases in children is age specific and many of these conditions have significant and potentially long-term clinical repercussions if not diagnosed early and managed in a timely fashion. We address 5 clinical scenarios that cover most of the diagnostic and therapeutic emergencies in children: infants with liver disease; acute liver failure; management of bleeding varices; liver-based metabolic disorders; and liver tumours and trauma. A wide spectrum of conditions that cause liver disease in infants may present as conjugated jaundice, which could be the only symptom of time-sensitive disorders – such as biliary atresia, metabolic disorders, infections, and haematological/alloimmune disorders – wherein algorithmic multistage testing is required for accurate diagnosis. In infantile cholestasis, algorithmic multistage tests are necessary for an accurate early diagnosis, while vitamin K, specific milk formulae and disease-specific medications are essential to avoid mortality and long-term morbidity. Management of paediatric acute liver failure requires co-ordination with a liver transplant centre, safe transport and detailed age-specific aetiological work-up – clinical stabilisation with appropriate supportive care is central to survival if transplantation is indicated. Gastrointestinal bleeding may present as the initial manifestation or during follow-up in patients with portal vein thrombosis or chronic liver disease and can be managed pharmacologically, or with endoscopic/radiological interventions. Liver-based inborn errors of metabolism may present as encephalopathy that needs to be recognised and treated early to avoid further neurological sequelae and death. Liver tumours and liver trauma are both rare occurrences in children and are best managed by a multidisciplinary team in a specialist centre.

儿童几种肝病的病因具有年龄特异性，如果不及早诊断和及时治疗，其中许多疾病都会产生重大且潜在的长期临床影响。我们解决了涵盖儿童大多数诊断和治疗紧急情况的 5 种临床情况：患有肝病的婴儿；急性肝功能衰竭；静脉曲张出血的管理；基于肝脏的代谢紊乱; 和肝脏肿瘤和外伤。导致婴儿肝病的多种疾病可能表现为结合性黄疸，这可能是时间敏感疾病的唯一症状 - 例如胆道闭锁、代谢紊乱、感染和血液/同种免疫疾病 - 其中算法多阶段测试是需要准确诊断。在婴儿胆汁淤积症中，算法多阶段测试对于准确的早期诊断是必要的，而维生素 K、特定配方奶粉和针对疾病的药物对于避免死亡率和长期发病率至关重要。小儿急性肝功能衰竭的管理需要与肝移植中心协调、安全运输和详细的年龄特异性病因检查——如果有移植指征，临床稳定和适当的支持性护理是生存的核心。胃肠道出血可能作为门静脉血栓形成或慢性肝病患者的初始表现或在随访期间出现，可以通过药物或内镜/放射干预进行治疗。基于肝脏的先天性代谢错误可能表现为脑病，需要及早发现和治疗，以避免进一步的神经系统后遗症和死亡。肝肿瘤和肝外伤在儿童中都很罕见，最好由专科中心的多学科团队管理。