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慢性肝病儿童肝移植的时机与指征

Timing and Indications for Liver Transplantation for Children with Chronic Liver Disease.

作者信息

Suthantirakumar Risheka Lakshmi, Gupte Girish L

机构信息

Specialised Foundation Programme in Paediatrics, Imperial College London NHS Hospital Trust, London W2 1NY, UK.

Liver Unit (Including Small Bowel Transplantation), Birmingham Children's Hospital, Birmingham B4 6NH, UK.

出版信息

Children (Basel). 2025 Mar 31;12(4):449. doi: 10.3390/children12040449.

DOI:10.3390/children12040449
PMID:40310116
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12025402/
Abstract

Chronic liver disease (CLD) in children poses significant challenges, necessitating timely management to mitigate morbidity and mortality. Liver transplantation (LT) has emerged as a transformative intervention, offering improved long-term survival for paediatric patients with CLD. This review explores the evolving landscape of liver transplantation, focusing on indications and timing considerations. The aetiology of CLD is diverse, encompassing intrahepatic, extrahepatic cholestatic conditions, metabolic diseases, malignancy, and drug-induced liver injury. LT is indicated when children exhibit signs of hepatic decompensation, necessitating a comprehensive evaluation to assess transplant suitability. Indications for LT include biliary atresia, inborn errors of metabolism, hepatocellular carcinoma, and emerging indications such as mitochondrial hepatopathies and acute on chronic liver failure. The timing of transplantation is critical, emphasizing the need for early recognition of decompensation signs to optimise outcomes. Advancements in LT techniques and immunosuppressive therapies have enhanced patient and graft survival rates. Various transplant modalities, including reduced-size LT and living-related LT, offer tailored solutions to address the unique needs of paediatric patients. While LT represents a cornerstone in the management of paediatric CLD, careful patient selection, multidisciplinary collaboration, and ongoing refinements in transplant protocols are imperative for optimizing outcomes and addressing the evolving landscape of paediatric liver disease management.

摘要

儿童慢性肝病(CLD)带来了重大挑战,需要及时管理以降低发病率和死亡率。肝移植(LT)已成为一种变革性干预措施,为患有CLD的儿科患者提供了更高的长期生存率。本综述探讨了肝移植不断变化的格局,重点关注适应证和时机考量。CLD的病因多种多样,包括肝内、肝外胆汁淤积性疾病、代谢性疾病、恶性肿瘤和药物性肝损伤。当儿童出现肝失代偿迹象时,即表明需要进行肝移植,这需要进行全面评估以确定移植的适宜性。肝移植的适应证包括胆道闭锁、先天性代谢缺陷、肝细胞癌,以及线粒体肝病和慢性肝衰竭急性发作等新出现的适应证。移植时机至关重要,强调需要早期识别失代偿迹象以优化治疗结果。肝移植技术和免疫抑制疗法的进步提高了患者和移植物的生存率。各种移植方式,包括减体积肝移植和亲属活体肝移植,为满足儿科患者的独特需求提供了量身定制的解决方案。虽然肝移植是儿科CLD管理的基石,但谨慎的患者选择、多学科协作以及移植方案的持续完善对于优化治疗结果和应对儿科肝病管理不断变化的格局至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/309c/12025402/52ea84ac7b5c/children-12-00449-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/309c/12025402/7b938d3c956d/children-12-00449-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/309c/12025402/52ea84ac7b5c/children-12-00449-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/309c/12025402/7b938d3c956d/children-12-00449-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/309c/12025402/52ea84ac7b5c/children-12-00449-g002.jpg

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