Unit of Paediatric Gastroenterology, Digestive Endoscopy, Hepatology and Care of the Child with Liver Transplantation, University Hospital of Padova, Padova, Italy.
Pediatrics, Department of Medicine, Surgery and Dentistry, University of Salerno, Salerno, Italy.
Transplantation. 2019 Jan;103(1):68-70. doi: 10.1097/TP.0000000000002490.
Nonalcoholic fatty liver disease (NAFLD) has now become a common cause of chronic liver disease in children; however, unlike adults liver transplantation is rarely required as treatment. It is important that in children presenting with NAFLD, secondary causes of fatty liver particularly inherited metabolic defects should be excluded.
A pediatric working group comprised of 3 hepatologists and a liver transplant surgeon was tasked with a set of questions to address the current state of evidence and knowledge about NAFLD in children with particular focus on liver transplantation. A systematic review of the English literature regarding pediatric NAFLD (from birth to 18 years of age) published in the last 2 decades (2000-2018) was carried out. The evidence was evaluated by the subgroup members and further discussed with the wider workshop faculty leading to the recommendations for best practice.
Given the paucity of literature on the subject good quality of evidence was only available on risk factors for NAFLD and medical treatment where the group could make recommendation with high/moderate strength. The evidence on natural history and indications for liver transplantation was poor hence group could not make any recommendations.
Based on the existing literature and subgroups, collective experience NAFLD unlike in adults is a very rare indication for liver transplantation in children. No definitive recommendations could be made about the natural history, indications, and outcome of liver transplantation for NAFLD in children.
非酒精性脂肪性肝病(NAFLD)现已成为儿童慢性肝病的常见病因;然而,与成人不同,儿童很少需要进行肝移植作为治疗手段。重要的是,对于表现为 NAFLD 的儿童,应排除脂肪性肝病的继发性病因,尤其是遗传性代谢缺陷。
一个由 3 名肝病学家和 1 名肝移植外科医生组成的儿科工作组接受了一系列问题的任务,旨在解决目前关于儿童非酒精性脂肪性肝病(从出生到 18 岁)的证据和知识状况,特别关注肝移植。对过去 20 年(2000-2018 年)发表的关于儿科非酒精性脂肪性肝病的英文文献进行了系统回顾。证据由小组成员进行评估,并与更广泛的工作组教员进一步讨论,最终提出最佳实践建议。
鉴于该主题的文献较少,只有关于非酒精性脂肪性肝病的危险因素和药物治疗的高质量证据,因此小组可以提出具有高/中度强度的建议。关于自然史和肝移植适应证的证据较差,因此小组无法提出任何建议。
根据现有文献和小组的经验,与成人不同,非酒精性脂肪性肝病在儿童中是肝移植的一个非常罕见的适应证。对于儿童非酒精性脂肪性肝病的自然史、适应证和肝移植结果,无法做出明确的建议。