在使用voxelotor治疗后,独特的血球蛋白病变模式
Izmarie Poventud-Fuentes1,2, Tania Platero Portillo1, Lily Olayinka1
1Department of Pathology and Immunology, Baylor College of Medicine, Houston, TX, USA.
Annals of clinical and laboratory science
|August 25, 2023
概括
儿科状细胞病 (SCD) 患者的voxelotor治疗显示出独特的血红蛋白分离模式. 这些变化与改善的血液溶解标志物相关,这表明监测治疗反应和遵守治疗的潜力.
科学领域:
- 血液学 血液学 血液学
- 药理学 药理学是指药理学的学科.
- 儿科医学 儿科医学
背景情况:
- 沃克塞洛特被FDA批准用于状细胞疾病 (SCD),抑制HbS聚合并减少血液溶解.
- 发声机改变了血红蛋白分离模式,影响了解释.
- 评估voxelotor对儿科SCD患者的血液溶解标记的影响至关重要.
研究的目的:
- 为了评估是否 voxelotor 诱导的血红蛋白分离的变化与儿科 SCD 患者的血解标记的改善相关.
- 探索血红蛋白分成的实用性在监测voxelotor治疗.
主要方法:
- 分析了15名小儿病患者 (12-20岁) 在voxelotor.
- 在发声器启动之前和之后比较血红蛋白分离,总血红蛋白,网状细胞百分比,LDH和胆红素.
- 使用毛细血管电泳来分化血红蛋白.
主要成果:
- 在60% (9/15) 的患者中观察到血红蛋白分成形状的变化.
- 44% (4/9) 患有分化变化的患者显示总血红蛋白增加>1 g/dL.
- 观察到LDH (4/4),网细胞百分比 (6/8) 和 bilirubin (3/4) 的降低.
结论:
- 在接受voxelotor治疗的SCD患者中,血红蛋白分离分析揭示了独特的模式.
- 这些模式可以作为一种工具来评估患者对声器治疗的反应和遵守.
- 进一步的研究可以验证血红蛋白分离作为监测voxelotor治疗疗效的工具.
相关概念视频
Hemoglobin
3.7K
Hemoglobin is a globular protein made up of four subunits. Two of these subunits are alpha chains, and the other two are beta chains. Each subunit contains a molecule of heme, which has an iron atom and can bind to oxygen. When an oxygen molecule binds to one heme group, it changes the shape of hemoglobin, making it easier for the other heme groups to bind oxygen as well.
When all four heme groups are bound to oxygen, the resulting molecule is called oxyhemoglobin. As a result, arterial blood...
When all four heme groups are bound to oxygen, the resulting molecule is called oxyhemoglobin. As a result, arterial blood...
3.7K
Disorders of Erythrocytes
989
Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...
989
Blood Transfusion and Agglutination
10.9K
Blood transfusion is a therapeutic measure to restore the blood volume after extensive blood loss due to an accident or a medical procedure. Blood transfusion involves drawing a certain amount of blood from a suitable donor and infusing it into the recipient.
History
The history of blood transfusion dates back to the 17th century, when early attempts were made in animals. In 1818 James Blundell, a British doctor, performed the first successful human blood transfusion. Later in 1900, Karl...
History
The history of blood transfusion dates back to the 17th century, when early attempts were made in animals. In 1818 James Blundell, a British doctor, performed the first successful human blood transfusion. Later in 1900, Karl...
10.9K
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
201
Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
201
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
263
Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
263
Disorders of Hemostasis
989
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.
989


