腹部出血和血管脆弱性与神经纤维素炎1型相关
Haruka Sato1, Fumito Okada2, Yoshiki Asayama3
1Department of Radiology, Faculty of Medicine, Oita University, 1-1 Idaigaoka, Hasama-machi, Yufu, Oita, 879-5593, Japan. sato-h@oita-u.ac.jp.
Japanese journal of radiology
|January 23, 2025
概括
神经纤维素瘤类型1 (NF-1) 可能导致严重的腹部出血,原因是血管脆弱. 这一案例凸显了在受影响患者中识别和管理NF-1血管病变的关键需求.
科学领域:
- 血管医学 血管医学
- 遗传学 是一个遗传学.
- 胃肠病学 胃肠病学
背景情况:
- 神经纤维素瘤类型1 (NF-1) 是一种遗传性疾病,大约在3000-5000个人中影响1人.
- NF-1与各种并发症有关,包括恶性瘤和血管疾病,影响预期寿命.
- NF-1血管病变是一种罕见但严重的血管并发症,其特点是结构异常和极度血管脆弱.
研究的目的:
- 在NF-1患者中报告一次性腹部出血病例.
- 强调NF-1血管病变所带来的诊断和管理挑战.
- 强调在接受侵入性手术的NF-1患者中识别血管脆弱性的重要性.
主要方法:
- 一个67岁的女性患者的病例报告,患有反复出现的腹部出血.
- 诊断成像包括计算机断层扫描 (CT) 扫描以确定出血的来源.
- 手术探索 (紧急腹腔切除术) 以评估血管完整性和管理出血.
主要成果:
- CT扫描显示出出血性和西格结肠动脉的伪动脉瘤.
- 腹腔切除术证实了极端的血管脆弱性,需要进行多次手术,因为持续出血.
- 这位患者被诊断为NF-1血管病变.
结论:
- 在出现不明原因腹部出血的患者中,NF-1血管病需要仔细考虑.
- 在NF-1中极端的血管脆弱性需要精心的手术和干预策略.
- 早期识别和量身定制的治疗对于改善NF-1血管病变的结果至关重要.
相关概念视频
Esophageal Varices-I: Introduction
71
Esophageal varices are dilated, tortuous veins which are found mainly in the submucosa of the lower esophagus but which may also appear higher up or extend into the stomach. They develop due to increased pressure in the portal venous system, often as a result of liver cirrhosis. This condition scars and damages the liver, impeding normal blood flow through the portal vein. To compensate, blood seeks alternative pathways, forming fragile new vessels (varices) in the esophagus and stomach. These...
71
Esophageal Varices-II: Clinical Features and Management
45
Esophageal varices often manifest as gastrointestinal bleeding episodes, presenting symptoms like hematemesis (vomiting of blood), hematochezia (passing fresh blood via the rectum), and melena (black, tarry stools). Other signs can include weight loss, anorexia, abdominal discomfort, jaundice, pruritus, altered mental status, and muscle cramps.
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol...
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol...
45
Disorders of Hemostasis
693
Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
693
Vascular Spasm
1.2K
The vascular phase, also known as vasospasm, is the initial stage of hemostasis, crucial for preventing excessive bleeding when a blood vessel is injured. After a vessel is cut, nerves in the damaged area trigger pain and other sensory impulses. Simultaneously, the smooth muscles in the vessel wall contract, resulting in a vascular spasm. This contraction reduces the vessel's diameter at the injury site, slowing or stopping blood loss through the vessel wall. Vascular spasms typically last...
1.2K
Disorders of the Autonomic Nervous System
532
The autonomic nervous system (ANS) is an intricate network of nerves that controls functions such as the regulation of heart rate, digestion, and blood pressure regulation. When this system malfunctions, it can lead to various disorders that affect multiple bodily functions. One common feature of many autonomic disorders is the involvement of smooth blood vessels, which play a crucial role in regulating blood flow throughout the body.
Raynaud's disease, also known as Raynaud's...
Raynaud's disease, also known as Raynaud's...
532
Inflammatory Bowel Disease I: Ulcerative Colitis
134
Introduction
Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
134


