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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。

相似文献

1
Carney's complex of primary pigmented nodular adrenocortical disease and pigmentous and myxomatous lesions.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。
Surg Gynecol Obstet. 1989 May;168(5):413-6.
2
Association of Fallot Tetralogy with Carney's complex.法洛四联症与卡尼综合征的关联。
Ir Med J. 2001 Nov-Dec;94(10):305-7.
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[Heart-skin syndrome and cardiac myxoma: presentation of a case and clinical experience of 17 years].[心脏-皮肤综合征与心脏黏液瘤:1例报告及17年临床经验]
Can J Surg. 1992 Feb;35(1):79-83.
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[Familial cases of cutaneous myxomas and spotty pigmentation (Carney's complex)].[皮肤黏液瘤和斑点状色素沉着的家族性病例(卡尼综合征)]
Nihon Hifuka Gakkai Zasshi. 1990 Sep;100(10):1047-52.
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A Chinese lady with Carney's complex.一位患有卡尼综合征的中国女性。
Chin Med J (Engl). 1994 Feb;107(2):95-8.
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[Association of cutaneous myxoma, recurrent cardiac myxoma and Cushing's syndrome (Carney's complex). Description of a case and review of the literature].[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]
Arch Mal Coeur Vaiss. 1990 Jan;83(1):121-4.
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[The myxoma syndrome: "cardiac myxoma, cutaneous pigmented lesions and peripheral and endocrine neoplasms". Apropos 2 cases].[黏液瘤综合征:“心脏黏液瘤、皮肤色素沉着病变及周围和内分泌肿瘤”。附2例报告]
Rev Esp Cardiol. 1994 Feb;47(2):113-5.
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Familial Cushing's syndrome due to primary pigmented nodular adrenocortical disease. Reinvestigation 50 years later.原发性色素沉着性结节性肾上腺皮质病所致的家族性库欣综合征。50年后的再次研究。
N Engl J Med. 1989 Dec 14;321(24):1659-64. doi: 10.1056/NEJM198912143212407.
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Testicular tumors in Carney's complex.卡尼综合征中的睾丸肿瘤。
J Urol. 2002 Mar;167(3):1299-302.
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[Carney's complex (myxomas, pigmented spots and endocrine hyperactivity). Apropos of a partial form in monozygotic twins].[卡尼综合征(黏液瘤、色素沉着斑和内分泌功能亢进)。关于一对单卵双胞胎中的部分病例]
Ann Pathol. 1988;8(3):239-43.

引用本文的文献

1
Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2.卡尼综合征,一种家族性多发性肿瘤和雀斑综合征。对11个家系的分析及与2号染色体短臂的连锁关系。
J Clin Invest. 1996 Feb 1;97(3):699-705. doi: 10.1172/JCI118467.