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[嗜铬细胞瘤。其诊断与治疗特点]

[Pheochromocytoma. Its diagnostic and therapeutic characteristics].

作者信息

Lopes H F, Silva H B, Bortolloto L A, Frimm C de C, Bellotti G M, Pileggi F, Jatene A D

机构信息

Instituto do Coração do Hospital das Clínicas, FMUSP.

出版信息

Arq Bras Cardiol. 1992 Nov;59(5):395-400.

PMID:1340739
Abstract

Pheochromocytoma is a cause of hypertension that frequently can be cured by surgery. The aim of this paper, based on 5 cases of pheochromocytoma, is to relate our experience in diagnosis and treatment in this pathology. In four of 5 patients with pheochromocytoma we observed unusual characteristics of the disease. Association with neurofibromatosis in one case, with rheumatic mitral regurgitation in another; and in a third case the tumor was malignant. One patient had catecholamine-mediated electrocardiographic changes which disappeared with treatment. Since symptoms of adrenergic hyperactivity were present in all cases, the rise in the levels of vanilmandelic acid and urinary metanephrines were useful in confirming the diagnosis. Computed tomography and I-131 meta-benzylguanidine for radioisotopic imaging, displayed not only all tumoral masses but also bone metastases in the malignant case. During the follow-up period, from the sixth month to the fourth year after surgery, four patients were asymptomatic, and have normal urinary catecholamine metabolite levels. The patient with a malignant form of pheochromocytoma continued to show elevated catecholamines release and remained hypertensive in spite of adrenal mass resection.

摘要

嗜铬细胞瘤是高血压的一个病因,通常可通过手术治愈。本文基于5例嗜铬细胞瘤病例,旨在分享我们在该疾病诊断和治疗方面的经验。在5例嗜铬细胞瘤患者中,有4例呈现出该疾病的异常特征。1例与神经纤维瘤病相关,另1例与风湿性二尖瓣反流相关;第3例肿瘤为恶性。1例患者出现儿茶酚胺介导的心电图改变,治疗后消失。由于所有病例均存在肾上腺素能亢进症状,香草扁桃酸和尿甲氧基肾上腺素水平升高有助于确诊。计算机断层扫描和用于放射性同位素成像的碘-131间碘苄胍,不仅显示了所有肿瘤肿块,还显示了恶性病例中的骨转移。在随访期间,从术后第6个月至第4年,4例患者无症状,尿儿茶酚胺代谢产物水平正常。恶性嗜铬细胞瘤患者尽管切除了肾上腺肿块,但仍持续显示儿茶酚胺释放增加且血压持续升高。

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