在遗传性出血性长管切开症中出现胃肠道出血,伴随着非常严重的血小板缺血
Yanbin Wei1, Chuyan Chen1, Peng Li1
1Department of Gastroenterology, Beijing Digestive Disease Center, Beijing Key Laboratory for Precancerous Lesion of Digestive Disease, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
The American journal of case reports
|June 10, 2025
概括
这份病例报告详细介绍了在一个遗传性出血长管病患者的严重胃肠道出血的管理,该患者的血小板数量非常低. 它建议停止使用质子抑制剂,并在高风险患者中使用输血小板输血与互白素-11.
科学领域:
- 内部医学 内部医学
- 胃肠病学 胃肠病学
- 血液学 血液学 血液学
背景情况:
- 遗传性出血性长膜症 (HHT) 是一种罕见的遗传疾病,导致异常的血管形成.
- 患者经常经历复发性表和胃肠道出血 (GIB).
- 在HHT患者中治疗严重的血小板狭窄症的GIB并未得到充分的记录.
研究的目的:
- 在HHT患者中报告严重GIB病例,患者患有严重的血小板缺血.
- 讨论这种罕见的临床场景的管理策略.
- 提供对HHT患者血小板缺血的潜在原因和治疗方法的见解.
主要方法:
- 一个66岁的女性HHT患者的病例报告,患有复发性表和急性GIB.
- 用阿贡血凝固的胃镜检查活跃的出血斑点.
- 支持性治疗包括血小板监测和输血.
主要成果:
- 这位患者出现了严重的GIB,血小板数低至1×109/L.
- 用阿贡血凝固治疗血管切开斑块的内镜治疗成功控制了活跃出血.
- 患者在治疗和出院后表现出良好的预后.
结论:
- 这一案例突出了罕见的HHT,GIB和严重的血小板缺血的同时发生,可能是PPI引起的.
- 建议立即停止违规PPI的使用.
- 对于高风险的GIB患者,建议进行血小板输血和复合人间白血素-11;如果PPI治疗是必不可少的,切换PPI并进行密切监测是可行的策略.
相关概念视频
Esophageal Varices-II: Clinical Features and Management
57
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...
57
Disorders of Hemostasis
758
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.
758
Esophageal Varices-I: Introduction
95
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...
95
Anticoagulant Drugs: Low-Molecular-Weight Heparins
654
Hemostasis is a crucial process that prevents excessive blood loss from damaged blood vessels. It involves various mechanisms such as vasoconstriction, platelet adhesion and activation, and fibrin formation. The importance of each mechanism depends on the type of vessel injury. In contrast, thrombosis is the abnormal formation of a blood clot within the blood vessels, leading to potential complications if the clot obstructs blood flow. Thrombosis can be caused by increased coagulability of the...
654
Peptic Ulcer Disease I: Introduction
161
Peptic Ulcer Disease (PUD) is characterized by mucosal excavation in the esophagus, stomach, pylorus, or duodenum. It can manifest as acute or chronic based on the extent and duration of mucosal involvement.
An acute ulcer, marked by superficial erosion and minimal inflammation, swiftly resolves upon identifying and addressing the underlying cause. In contrast, a chronic ulcer persists, potentially eroding through the muscular wall and forming fibrous tissue.
Peptic ulcers can also be...
An acute ulcer, marked by superficial erosion and minimal inflammation, swiftly resolves upon identifying and addressing the underlying cause. In contrast, a chronic ulcer persists, potentially eroding through the muscular wall and forming fibrous tissue.
Peptic ulcers can also be...
161
Introduction to Hemostasis
6.8K
Hemostasis is a complex physiological process that prevents excessive bleeding when a blood vessel is injured. It's crucial for maintaining the integrity of the circulatory system, as it ensures that our blood remains fluid while still within the vascular network and yet clots to prevent blood loss upon vessel injury.
The three phases of hemostasis involve many clotting factors present in plasma and several substances released by platelets and injured tissue cells. It is a fast, localized,...
The three phases of hemostasis involve many clotting factors present in plasma and several substances released by platelets and injured tissue cells. It is a fast, localized,...
6.8K


