丰坦相关性肝病:在哥伦比亚的一个参考中心的儿科经验
Andrés David Aranzazu1, Isabel C Sánchez2, Sydney Goldfeder3
1Pediatric Cardiology Fellow, Clínica CardioVID, Universidad Pontificia Bolivariana, Medellín, Colombia.
Cardiology in the young
|July 3, 2025
概括
与Fontan相关的肝病影响了超过11%的患有Fontan循环的儿科患者,这给诊断和治疗带来了挑战. 早期识别和管理对于这些复杂的病例至关重要.
科学领域:
- 儿童心脏病学 儿童心脏病学
- 肝病学 肝病学是一种肝病学.
- 遗传性心脏病是一种先天性心脏病.
背景情况:
- 丰坦相关性肝病 (FALD) 是由于单心室生理学患者的血液动力学变化引起的.
- 它涉及肝脏的结构,功能和临床变化.
- 了解FALD对于患者的长期结果至关重要.
研究的目的:
- 详细介绍了儿科患者与Fontan相关的肝功能障碍的临床经验.
- 为了突出FALD在哥伦比亚队列中的流行率和特征.
主要方法:
- 对12名被诊断患有Fontan相关性肝病的儿科患者进行了回顾性分析.
- 在2001年至2024年期间从单心室计划中收集的数据.
- 基于已建立的FALD诊断标准的纳入标准.
主要成果:
- 在Fontan阶段11.1% (12/108) 的患者出现了FALD.
- 诊断时的平均年龄为14.5岁 (范围未指明),Fontan完成时的平均年龄为3.9岁.
- 33%的患者患有蛋白质损失性肠病/塑性支气管炎; 41%的患者表现出缩性功能障碍; 16%的患者表现出腹缩性功能障碍; 100%的患者表现出某种类型的膜功能不足.
结论:
- 与Fontan相关的肝病仍然是患有Fontan生理学的儿科患者的重大临床问题.
- 对FALD的诊断和治疗方法需要持续关注.
- 医疗保健提供者必须优先考虑早期发现和管理FALD.
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