波尔托鼻状血管疾病和下坡静脉:单独的临床实体?
Inês Pestana1, Carlos Noronha Ferreira2, Rita Luís3,4
1Department of Gastroenterology, Hospital Amato Lusitano ULSCB, Castelo Branco, Portugal.
GE Portuguese journal of gastroenterology
|February 5, 2025
概括
这一案例凸显了波尔图鼻状血管疾病 (PSVD) 与肺动脉高血压 (PAH) 之间的罕见联系. 诊断需要先进的成像和肝活检来确认PSVD和相关的PAH.
科学领域:
- 肝病学 肝病学是一种肝病学.
- 心脏病学 心脏病学
- 血管医学 血管医学
背景情况:
- 波多鼻状血管疾病 (PSVD) 的特点是没有肝硬化的特定组织学发现,其致病性尚不清楚.
- 肺动脉高血压 (PAH) 可以增加中心静脉压力,可能导致食道变形.
- 很少报告PSVD和PAH之间的关联.
研究的目的:
- 描述一个复杂的病例,患者呈现出暗示上部胃肠道出血的症状.
- 为了说明识别PSVD和PAH同时发生的诊断挑战.
- 强调特定诊断工具在确认这些病情方面的重要性.
主要方法:
- 一名53岁的妇女出现了黑色斑块,腹上疼痛和血液凝结.
- 诊断工作包括内镜,超声波,心声回声,血管造影,心肺血液动力学和横肝活检.
- 治疗包括利尿剂和停止服用β-阻断剂.
主要成果:
- 患者表现出严重的贫血,食道静脉,以及中度至严重的PAH.
- 肝脏活检显示结节性再生性增生,表明PSVD.
- 血液动力学评估证实了非临床显著的鼻状门高血压 (PHT).
结论:
- 这一案例凸显了PSVD与肺高血压之间不常见的关联.
- 输肝活检和血液动力学测量对于诊断PSVD和PAH至关重要.
- 对于患有这种复杂的并发症的患者来说,早期识别和治疗至关重要.
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