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原发性色素沉着性结节性肾上腺皮质疾病与色素性及黏液瘤性病变的卡尼综合征。

Carney's complex of primary pigmented nodular adrenocortical disease and pigmentous and myxomatous lesions.

作者信息

Cheung P S, Thompson N W

机构信息

Division of Endocrine Surgery, University of Michigan Medical Center, Ann Arbor.

出版信息

Surg Gynecol Obstet. 1989 May;168(5):413-6.

PMID:2711295
Abstract

A complex of primary pigmented nodular adrenocortical hyperplasia associated with myxomatous masses and pigmented lesions of the skin was recently described by Carney. Herein, we describe three patients with Carney's complex seen at our institution. Two patients presented with Cushing's syndrome and one patient with atrial myxoma. The diagnosis was revealed in all three because of an awareness of the disease in association with primary adrenocortical hyperplasia. Because Cushing's syndrome and atrial myxomas are life-threatening components of the disease and can occur metachronously, an early diagnosis in combination with the appropriate treatment is mandatory, and follow-up measures should be taken to define the natural course of events in the disease.

摘要

卡尼最近描述了一种与皮肤黏液瘤性肿块和色素沉着病变相关的原发性色素沉着性结节性肾上腺皮质增生复合体。在此,我们描述了在我们机构就诊的3例卡尼综合征患者。2例患者表现为库欣综合征,1例患者表现为心房黏液瘤。由于对该疾病与原发性肾上腺皮质增生相关的认识,这3例患者均得以确诊。由于库欣综合征和心房黏液瘤是该疾病危及生命的组成部分,且可异时发生,因此必须尽早诊断并采取适当治疗措施,同时应采取随访措施以明确疾病的自然病程。

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