呈现,管理,和巨型花红细胞瘤患者的结果
Bahar Bahrani Fard1, Nasrin Nikravangolsefid2, Justin W Maroun3
1Division of Endocrinology, Metabolism, and Nutrition, Mayo Clinic, Rochester, MN 55905, USA.
The Journal of clinical endocrinology and metabolism
|September 4, 2024
概括
巨型染细胞瘤 (PHEOs) 呈现出更严重的症状和过多的甲基荷胺. 这些大型瘤与术后并发症的增加以及转移和死亡的显著风险有关.
科学领域:
- 内分泌学 在内分泌学.
- 手术瘤学手术瘤学
- 在瘤学瘤学.
背景情况:
- 巨型血红细胞瘤 (PHEOs),定义为≥10厘米的瘤,很少见,对其特征的数据有限.
- 了解巨型和非巨型PHEO之间的差异对于最佳的患者管理至关重要.
研究的目的:
- 为了比较巨型PHEO与非巨型PHEO的临床表现,管理策略和患者结果.
- 确定与管理巨型PHEO相关的特定挑战和风险.
主要方法:
- 在2000年至2023年期间进行了一项回顾性队列研究.
- 患有巨型PHEO的患者与随机选择的非巨型PHEO患者队列进行了比较 (1:6比率).
- 评估的结果包括术后并发症,转移性疾病发展和死亡率.
主要成果:
- 巨型PHEO (n=31,中位尺寸12厘米) 与非巨型PHEO (n=186,中位尺寸4厘米) 相比,与多种catecholamine过量的症状 (76%vs30%) 和更高的术后并发症率 (31%vs10%) 相关.
- 巨型PHEO患者 (26%与3%相比) 中使用甲氨酸的频率更高.
- 在3年的中位数随访期间,45%的巨型PHEO患者发生了转移,疾病特异性死亡率为7%与非巨型PHEO组的0%相比.
结论:
- 巨型PHEO存在,临床严重性更大,甲基荷兰胺过量更高,外科手术期间风险增加.
- 巨型PHEO患者的转移率高 (45%),需要对长期的随访和监测保持警.
相关概念视频
Adrenal Gland Disorders
1.4K
Adrenal gland disorders manifest when the production of adrenal hormones deviates from the norm, resulting in either excessive or insufficient concentrations.
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
1.4K
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
147
Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
147
Hypoglycemia and Glucagon
217
Without prolonged fasting, healthy individuals maintain blood glucose levels above 3.5 mM due to a well-adapted neuroendocrine counterregulatory system that effectively prevents acute hypoglycemia, a potentially life-threatening condition. The primary clinical scenarios for hypoglycemia encompass diabetes treatment, inappropriate production of endogenous insulin or insulin-like substances by tumors, and the use of glucose-lowering agents in non-diabetic individuals. Notably, hypoglycemia in the...
217
Transducer Mechanism: Enzyme-Linked Receptors
2.4K
Enzyme-linked receptors are cell-surface receptors acting as an enzyme or associating with an enzyme intracellularly. They make excellent drug targets. Drugs can bind to the extracellular ligand-binding domain or directly affect their enzymatic domain and alter their activity.
Major types that are helpful drug targets include:
Major types that are helpful drug targets include:
2.4K
Adrenergic Antagonists: Pharmacological Actions of β-Receptor Blockers
544
β-receptor blockers significantly impact the cardiovascular system by counteracting catecholamine-induced sympathetic responses. These medications decrease heart rate, contractility, and cardiac output, potentially leading to cardiac depression, life-threatening bradycardia, and death. Therapeutically, β-blockers function as mild antihypertensives and are utilized in treating angina pectoris and cardiac arrhythmias. However, nonselective β-blockers inhibit β2-receptors in...
544
Adrenergic Agonists: Therapeutic Uses
752
Adrenergic agonists have diverse therapeutic uses across various medical conditions and emergencies.
Emergency and Intensive Care Unit (ICU) applications: Pressor agents increase blood pressure, heart rate, and contractility in shock and organ failure situations. Dopamine can induce vasodilation and stimulate adrenoceptors. Endogenous catecholamines are effective in treating cardiogenic shock. α2-agonists like clonidine can reverse anesthesia-induced hypertension.
Allergies and...
Emergency and Intensive Care Unit (ICU) applications: Pressor agents increase blood pressure, heart rate, and contractility in shock and organ failure situations. Dopamine can induce vasodilation and stimulate adrenoceptors. Endogenous catecholamines are effective in treating cardiogenic shock. α2-agonists like clonidine can reverse anesthesia-induced hypertension.
Allergies and...
752


