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[家族性发育异常痣综合征。一项家族研究]

[Dysplastic nevus syndrome of familial type. A family study].

作者信息

Grimstvedt M B

出版信息

Tidsskr Nor Laegeforen. 1992 Mar 10;112(7):869-72.

PMID:1557751
Abstract

A family with heritable occurrence of dysplastic nevus syndrome has been studied through 20 years and five generations. The intention was to study how the disease is inherited, how often malignant melanomas developed from such moles, and which factors caused this change. 59 out of 103 family members had dysplastic nevus syndrome. Occurrence was distributed equally between males and females. The moles were not present at birth, but first appeared in childhood and adolescence. The moles differ clinically from common moles by being flat, irregularly coloured, and irregularly delimited in relation to the surroundings. There are also large variations between moles in the individual. 20% of the patients developed hereditary malignant melanoma from these particular moles. Dysplastic nevus syndrome was inherited autosomally dominant and might develop into malignant melanoma many years later in life. In some cases the development was provoked by sunshine. Patients who suffer from dysplastic nevus syndrome are in danger of developing malignant melanoma and should therefore be careful of exposure to sun and should regularly examine their moles for possible changes.

摘要

一个患有发育异常痣综合征的家族已历经20年、五代人进行了研究。目的是研究该疾病如何遗传、由这类痣发展为恶性黑色素瘤的频率以及导致这种变化的因素。103名家族成员中有59人患有发育异常痣综合征。发病在男性和女性中分布均等。这些痣并非出生时就有,而是在儿童期和青春期首次出现。临床上,这些痣与普通痣不同,表现为扁平、颜色不规则且与周围界限不清。个体身上的痣之间也存在很大差异。20%的患者从这些特定的痣发展为遗传性恶性黑色素瘤。发育异常痣综合征呈常染色体显性遗传,可能在多年后发展为恶性黑色素瘤。在某些情况下,其发展是由阳光诱发的。患有发育异常痣综合征的患者有患恶性黑色素瘤的风险,因此应注意避免阳光照射,并应定期检查痣是否有可能的变化。

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