循环系统精品教学（首都医科大学）继发性高血压.pptVIP

嗜铬细胞瘤 高血压:持续性、阵发性、正常 三联征:头痛、心悸、多汗 体位性低血压 难治性高血压 家族史： MEN 嗜铬细胞瘤 10%双侧 10% 肾上腺外 10% 恶性 10% 家族性 10% 儿童 10% 血压正常 酪氨酸 多巴 DA NE E 酪氨酸羟化酶 多巴脱羧酶 苯乙醇胺-N-甲基转移酶（PNMT） 多巴胺－β羟化酶 儿茶酚胺的合成 嗜铬细胞瘤 定性诊断： 尿/血浆儿茶酚胺 (NE, E, DA) 儿茶酚胺代谢产物 (VMA,MN, NMN) 血浆嗜铬粒蛋白 A (chromogranin A) 嗜铬细胞瘤 形态学:CT MRI 形态与功能:131I- metaiodobenzylguanidine (MIBG) 奥曲肽显像 A large left adrenal mass was found by CT scan (Figure 2), Abdominal CT scan demonstrating a 5×6×7-cm left adrenal mass (arrow). which enhanced on iodine 131meta-iodobenzylguanidine (MIBG) scan without evidence of metastatic disease (Figure 3). Figure 3. MIBG scan demonstrating a region of increased activity in the left suprarenal region consistent with pheochromocytoma (arrow). Blue arrow points to residual normal medulla.  ? Yellow arrows point to golden yellow adrenal cortex.  After 2 weeks of medical stabilization, a laparoscopic left adrenalectomy was performed, and histological examination confirmed the diagnosis of pheochromocytoma (Figure 4). Adrenergic blockade was discontinued postoperatively. The patient’s vital signs remained normal and he was discharged in good condition. Figure 4. Histological confirmation of pheochromocytoma. Hematoxylin-and-eosin stain demonstrates clusters of tumor cells with abundant amphophilic cytoplasm and prominent nucleoli. Tumor cells stained positively for multiple neuroendocrine markers, including chromogranin, synaptophysin, neuronspecific enolase and S100. 嗜铬细胞瘤 药物治疗 ? - adrenergic blockade phentolamine ? 1 + ? 2 phenoxybenzamine ? 1 ? 2 prazosin ? 1 urapidil ? 1 ? 2 ? - adrenergic blockade