成人体肺静脉高压相关肝病患儿肝纤维化和门脉高压的生物标志物。

Biomarkers of fibrosis and portal hypertension in Fontan-associated liver disease in children and adults.

机构信息

Division of Pediatric Gastroenterology, Hepatology and Nutrition, Riley Hospital for Children, Indiana University, 705 Riley Hospital Drive, ROC 4210, Indianapolis, Indiana 46202, United States.

Division of Pediatric Cardiology, United States.

出版信息

Dig Liver Dis. 2024 Aug;56(8):1335-1342. doi: 10.1016/j.dld.2023.12.017. Epub 2024 Jan 13.

DOI:10.1016/j.dld.2023.12.017

PMID:38220486

Abstract

BACKGROUND

Fontan-associated liver disease (FALD) refers to structural and functional changes of the liver caused by the physiology of the Fontan palliation. Currently, liver biopsy is the gold standard to assess liver fibrosis of FALD.

AIM

Investigate biomarkers correlating with severity of liver biopsy fibrosis in FALD.

METHODS

A retrospective study of post-Fontan patients ≥ 10 years of age who underwent liver biopsy was conducted. Advanced liver disease (ALD) was defined as bridging fibrosis and/or cirrhosis on liver biopsy. AST-to-platelet ratio index (APRI), Fibrosis-4 (FIB-4) and Liver Stiffness Measurement (LSM) from FibroScan were used as non-invasive fibrosis scores.

RESULTS

Sixty-six patients (26/47; 55.3% adults and 13/19 children; 68.4%) had ALD on biopsy. ALD was associated with lower platelet count (151 vs. 198 K/uL, p = 0.003), higher APRI (0.64 vs. 0.32, p = 0.01), higher FIB-4 (0.64 vs. 0.32, p = 0.02). Liver fibrosis score correlated with APRI (0.34, p = 0.02) and FIB-4 (0.47, p = 0.001) in adults. LSM had a high sensitivity at 81.3% with 45.5% specificity at a cut-off 18.5 kPa.

CONCLUSIONS

APRI and FIB-4 had modest discrimination to identify adults with advanced liver disease, but not children, indicating that these values may be followed as a marker of FALD progression in older patients.

摘要

背景

Fontan 相关肝疾病（FALD）是指由于 Fontan 姑息治疗的生理学引起的肝脏结构和功能变化。目前，肝活检是评估 FALD 肝纤维化的金标准。

目的

研究与 FALD 肝活检纤维化严重程度相关的生物标志物。

方法

对接受过肝活检的年龄≥10 岁的 Fontan 后患者进行回顾性研究。肝晚期疾病（ALD）定义为肝活检中桥接纤维化和/或肝硬化。AST 与血小板比值指数（APRI）、纤维化-4（FIB-4）和 FibroScan 的肝硬度测量（LSM）用作非侵入性纤维化评分。

结果

66 例患者（26/47；55.3%为成人，13/19 为儿童；68.4%）肝活检存在 ALD。ALD 与血小板计数较低（151 比 198 K/uL，p = 0.003）、APRI 较高（0.64 比 0.32，p = 0.01）、FIB-4 较高相关（0.64 比 0.32，p = 0.02）。在成人中，肝纤维化评分与 APRI（0.34，p = 0.02）和 FIB-4（0.47，p = 0.001）相关。LSM 在 18.5 kPa 的截断值时具有 81.3%的高灵敏度和 45.5%的特异性。