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[皮肤黏液瘤、复发性心脏黏液瘤与库欣综合征(卡尼综合征)的关联。1例病例描述及文献复习]

[Association of cutaneous myxoma, recurrent cardiac myxoma and Cushing's syndrome (Carney's complex). Description of a case and review of the literature].

作者信息

Champagnac D, Gayet C, Mornex R, Termet H, Pierrard B, Milon H

机构信息

Service de cardiologie, hôpital de la Croix-Rousse, Lyon.

出版信息

Arch Mal Coeur Vaiss. 1990 Jan;83(1):121-4.

PMID:2106300
Abstract

The authors report the case of a rare clinical syndrome which has recently been called Carney's complex or "myxomas, spotty pigmentation and endocrine overactivity". Three components of this complex are described: cutaneous myxomas, Cushing's syndrome of unpredictable evolution treated by bilateral adrenalectomy for multiple adrenal adenoma and left atrial myxoma which recurred twice. The authors review the literature and discuss the practical implications of this new syndrome which may be familial. The role of echocardiography, the key investigation for the detection of the myxoma and follow-up of these patients who have a high risk of recurrence, is underlined.

摘要

作者报告了一例罕见的临床综合征病例,该综合征最近被称为卡尼综合征或“黏液瘤、斑点状色素沉着和内分泌功能亢进”。描述了该综合征的三个组成部分:皮肤黏液瘤、因多发性肾上腺腺瘤接受双侧肾上腺切除术治疗的演变不可预测的库欣综合征以及复发两次的左心房黏液瘤。作者回顾了文献并讨论了这种可能为家族性的新综合征的实际意义。强调了超声心动图的作用,它是检测黏液瘤以及对这些复发风险高的患者进行随访的关键检查。

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