How to detect pheochromocytomas?--the diagnostic relevance of plasma free metanephrines.
How to detect pheochromocytomas?--the diagnostic relevance of plasma free metanephrines.
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如何检测嗜铬细胞瘤？——血浆游离变肾上腺素的诊断意义。
DOI：
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发表时间：
2002
影响因子：
2.6
通讯作者：
M. Roden
中科院分区：
文献类型：
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作者：
M. Roden
嗜铬细胞瘤是一种嗜铬细胞瘤，大多起源于肾上腺髓质，是一种由于儿茶酚胺(去甲肾上腺素和/或肾上腺素)过度产生而导致高血压的罕见原因。超过10%发生在多发性内分泌肿瘤II型、von Hippel-Lindau病、神经纤维瘤病I型和家族性颈动脉体瘤的家族中。由于大约一半的患者没有或仅有发作性高血压，诊断必须进行详细的临床评估和敏感的生化测试，这依赖于儿茶酚胺产生增加的检测。常用的检测方法，如测定血浆和尿液中的游离儿茶酚胺或其代谢物香草扁桃酸和尿液中总的甲肾上腺素(=游离+结合的去甲肾上腺素和甲肾上腺素)，会受到外界因素的干扰，有时临床敏感性和/或特异性较低。最近的技术进步现在使我们能够测量血浆中游离(非结合)的甲肾上腺素，从而将临床灵敏度和特异度提高到接近100%。血浆游离间肾上腺素对嗜铬细胞瘤的检测具有以下优点：(1)不依赖于体位改变、运动或术中应激引起的儿茶酚胺分泌的短期变化；(2)有关儿茶酚胺产生长期增加的信息；(3)与肿瘤质量密切相关；(4)药物的轻微干扰。这种方法不需要耗时的标准化采血程序，这是测定游离儿茶酚胺的先决条件。总而言之，建议使用血浆游离偏肾上腺素作为检测嗜铬细胞瘤的一线生化试验。
Pheochromocytomas are chromaffin cell tumors, mostly originating from the adrenal medulla, and represent a rare cause of hypertensiondue to excessive production of catecholamines (norepinephrine and/or epinephrine). More than 10% occur in families with multiple endocrine neoplasia type II, von Hippel-Lindau disease, neurofibromatosis type I, and familial carotid body tumors. Since approximately half of the afflicted patients present without or with only episodic hypertension, detailed clinical evaluation and sensitive biochemical tests are mandatory for the diagnosis, which relies on the detection of increased catecholamine production. Commonly employed tests such as the measurement of free catecholamines in plasma and urine or of their metabolites, vanillylmandelic acid and total metanephrines (= free + conjugated normetanephrine and metanephrine) in urine, suffer from interference from external factors and sometimes low clinical sensitivity and/or specificity. Recent technical advances now allow us to measure plasma free (unconjugated) metanephrines, thus increasing clinical sensitivity and specificity to close to 100%. Plasma free metanephrines offer the following advantages for the detection of pheochromocytomas: (i) independence of short-term changes in catecholamine secretion which may result from change of posture, exercise or intraoperative stress, (ii) information on long-term increase of catecholamine production, (iii) tight correlation with tumor mass, and (iv) only minor interference from drugs. This method does not need time-consuming standardized procedures for blood sampling, which are a prerequisite for the determination of free catecholamines. In conclusion, it is therefore recommended to use plasma free metanephrines--after meticulous clinical screening--as the first-line biochemical test for detecting pheochromocytomas.