Department of Gastroenterology and Hepatology, Children's Health Ireland Crumlin, Dublin, Ireland.
School of Medicine, University College Dublin, Dublin, Ireland.
Indian J Pediatr. 2024 Mar;91(3):286-293. doi: 10.1007/s12098-023-04694-7. Epub 2023 Jul 13.
The systemic sequelae of chronic liver disease (CLD) may be due to portal hypertension and shunting, malnutrition, and/or a low grade inflammatory state. This article will focus on the consequences of chronic liver disease affecting extrahepatic organs. Portal hypertension underlies many systemic complications of CLD. Aside from varices and ascites, portal hypertension may cause both hepatopulmonary syndrome and portopulmonary hypertension leading to respiratory compromise. Cardiomyopathy may also occur secondary to end stage liver disease. Hepatorenal syndrome is also well recognised and hepatic encephalopathy is a consequence of the effect of liver dysfunction on the brain. Compromise of the immune system is well described in end-stage liver disease leading to sepsis and its consequences. Bony disease including osteoporosis and hepatic arthropathy may both be seen in children with CLD. CLD may be asymptomatic initially but then complications may present as the disease progresses. Furthermore, systemic effects of end stage liver disease may complicate liver transplant. These complications often present insidiously or at the time of acute decompensation. Thus, it is important that healthcare providers are vigilant when caring for children with CLD. This article outlines the secondary complications of CLD with an overview of the definition and diagnosis, pathophysiology, management and prognosis of each.
慢性肝脏疾病(CLD)的全身性后遗症可能是由于门静脉高压和分流、营养不良和/或低度炎症状态所致。本文将重点介绍影响肝外器官的慢性肝脏疾病的后果。门静脉高压是 CLD 许多全身性并发症的基础。除了静脉曲张和腹水外,门静脉高压还可能导致肝肺综合征和门脉性肺动脉高压,导致呼吸功能受损。终末期肝病也可能导致心肌病。肝肾综合征也广为人知,肝性脑病是肝功能障碍对大脑影响的结果。终末期肝病中免疫系统受损的情况也得到了很好的描述,导致败血症及其后果。包括骨质疏松症和肝性关节病在内的骨骼疾病在患有 CLD 的儿童中均可出现。CLD 最初可能无症状,但随着疾病的进展,可能会出现并发症。此外,终末期肝病的全身影响可能会使肝移植复杂化。这些并发症通常在不知不觉中或急性失代偿时出现。因此,医疗保健提供者在照顾患有 CLD 的儿童时保持警惕非常重要。本文概述了 CLD 的继发性并发症,并概述了每种并发症的定义和诊断、病理生理学、治疗和预后。