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[恶性嗜铬细胞瘤与冯雷克林霍增氏病相关。附病例报告。新方法在嗜铬细胞瘤诊断中的价值]

[Malignant pheochromocytoma associated with Recklinghausen's disease. Apropos of a case. Value of new methods in the diagnosis of pheochromocytoma].

作者信息

Tison E, Marchand X, Kacet S, Proye C, Lekieffre J

出版信息

Ann Cardiol Angeiol (Paris). 1986 Nov;35(9):551-5.

PMID:3101574
Abstract

After having reported the case of a pheochromocytoma associated to Recklinghausen's disease, the authors define the best criteria of detection and localization of the pheochromocytoma and study its association to phacomatoses. Headaches, bouts of tachycardia and excessive inappropriate diuresis are the most evocative clinical signs of a pheochromocytoma. The different hormones and their urinary metabolites must be titrated separately and repeatedly. Two other examinations, scanner and scintigraphy with MIBG, visualize quite reliably the tumor foci. Calcium inhibitors are quite effective in sudden blood pressure rises. The association described here, may be explained by the fact that the two pathologies belong to the group of neurocristopathies.

摘要

在报告了一例与冯雷克林霍增氏病相关的嗜铬细胞瘤病例后,作者确定了嗜铬细胞瘤检测和定位的最佳标准,并研究了其与错构瘤病的关联。头痛、心动过速发作和过度不适当的利尿是嗜铬细胞瘤最具提示性的临床症状。必须分别且反复地测定不同的激素及其尿液代谢产物。另外两项检查,即扫描和用间碘苄胍进行的闪烁扫描,能相当可靠地显示肿瘤病灶。钙抑制剂对突然的血压升高相当有效。此处描述的这种关联,可能是因为这两种病症都属于神经嵴病这一类别。

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