医学
嗜铬细胞瘤
副神经节瘤
低血糖
基因检测
神经内分泌肿瘤
重症监护医学
内科学
内分泌学
病理
糖尿病
作者
José Viana Lima,Cláudio E. Kater
标识
DOI:10.1590/s1677-5538.ibju.2023.0038
摘要
Pheochromocytomas/paragangliomas (PPGL) are rare, metastatic, and potentially fatal neuroendocrine tumors, often neglected because they present symptoms similar to other prevailing clinical conditions such panic syndrome, thyrotoxicosis, anxiety, hypoglycemia, etc., delaying diagnosis and treatment. The rate of diagnosis of PPGL has been increasing with the improvement in the measurement of catecholamine metabolites and the expanding availability of imaging procedures. Its essential genetic nature has been extensively investigated, comprising more than 20 genes currently related to PPGL and more new genes will probably be revealed. This overview will shed some light on the clinical, laboratory, topographical, genetic diagnosis, and management of PPGL.
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