临床特征与活检的变化相关 肝纤维化在Fontan循环中的严重程度
Taylor Hartzel Houlihan1, Ari Gartenberg2, Kathryn M Dodds3
1CS Mott Children's Hospital, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, USA.
JACC. Advances
|December 16, 2025
概括
芬坦血流中的肝纤维化随着时间的推移而进展,并且与芬坦手术时的年轻年龄有关. 这表明正在进行的纤维生成和肝脏健康的潜在可修改因素.
科学领域:
- 心脏病学 心脏病学
- 肝病学 肝病学是一种肝病学.
- 儿科医学 儿科医学
背景情况:
- 肝纤维化是Fontan循环 (FC) 患者的常见并发症,严重程度各不相同.
- 了解与纤维化程度相关的因素对于管理这种人群中的肝脏健康至关重要.
研究的目的:
- 为了确定与FC中肝纤维化严重程度相关的临床和血液动力学因素.
- 评估纤维化,患者特征,Fontan血动力学和非侵入性肝脏评估之间的关系.
主要方法:
- 一组173名患有FC的受试者接受了心脏导管治疗,肝脏活检用Sirius红色染色进行纤维化量化,肝脏弹性图以及实验室测试.
- 探索了Sirius红色染色 (纤维化严重程度) 和临床/血液动力学因素之间的关联.
主要成果:
- 西里乌斯红色染色 (中位数为21.6%) 与心室形态学,导管血液动力学或肝脏弹性学无关.
- 纤维化严重程度与阿斯巴酸氨基转移酶/血小板比率相关,并与多年FC暴露呈正相关.
- 芬坦手术时的年龄与纤维化症的严重程度相反相关.
结论:
- 在FC中,肝纤维化随着暴露时间的延长而进展,不论静脉压力如何,都表明持续的纤维化.
- 芬坦手术时的年龄较小与纤维化严重程度的增加有关,突出了改善肝脏结果的潜在可修改因素.
相关概念视频
Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow
200
Chronic liver disease significantly impacts drug metabolism due to alterations in hepatic blood flow and enzyme accessibility. This disruption affects the body's pharmacokinetics—the movement and processing of drugs within the system. Key enzymes crucial for metabolizing medications become less accessible, changing how drugs are processed and utilized. Furthermore, liver disease influences the synthesis of plasma proteins, such as albumin and globulins, which play critical roles in drug...
200
Ultrasound II: Endoscopic Ultrasound and FibroScan
499
Endoscopic Ultrasound (EUS) and FibroScan are valuable diagnostic tools in gastroenterology and hepatology, each with specific applications and techniques.
Endoscopic Ultrasound (EUS):
Endoscopic Ultrasound (EUS):
499
Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test
169
In clinical practice, the direct measurement of hepatic blood flow to evaluate liver function presents significant challenges due to the intricate and specialized nature of the necessary techniques. Consequently, healthcare professionals often rely on empirical estimates derived from thorough patient examinations and liver function tests to gauge liver health. Among the tools at their disposal, the Child–Pugh and MELD scoring systems stand out for their ability to categorize and assess...
169
Heart Failure IV: Classification and Diagnostic Evaluation
293
Heart failure can be classified in various ways, with the most common classifications based on physical activity limitations, disease progression, severity, and treatment strategies.The Functional Classification of Heart Failure divides patients into four categories based on physical activity limitation due to symptom burden.Class I: Patients in this class have cardiac disease but no physical activity limitations. Ordinary activities like walking, climbing stairs, or routine tasks do not cause...
293
Assessment of the Cardiovascular System III: Palpation
968
Palpation involves feeling the body to evaluate texture, size, consistency, and tenderness for assessing cardiovascular health. The following steps are organized in a head-to-toe order:
Jugular Venous Pressure (JVP) Measurement
Position the patient at a thirty- to forty-five-degree angle or in a semi-fowler's position. Look for the highest point of pulsation in the internal jugular vein and measure the vertical distance to the angle of Loius or sternal angle. A normal JVP is 3-4 cm above...
Jugular Venous Pressure (JVP) Measurement
Position the patient at a thirty- to forty-five-degree angle or in a semi-fowler's position. Look for the highest point of pulsation in the internal jugular vein and measure the vertical distance to the angle of Loius or sternal angle. A normal JVP is 3-4 cm above...
968


