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[戈林-戈尔茨综合征(基底细胞痣综合征)的亲缘关系研究]

[Kinship studies in Gorlin-Goltz syndrome (basal cell nevus syndrome)].

作者信息

Brumme S, Löwicke G

机构信息

Klinik und Poliklinik für Chirurgische Stomatologie, Karl-Marx-Universität Leipzig.

出版信息

Dermatol Monatsschr. 1990;176(8):487-92.

PMID:2227061
Abstract

By means of an investigation on 7 patients from 3 families views are expressed on the problems of the basal cell-naevus-syndrome. The typical clinical signs appear preferentially and as first-symptoms in the jaw-bone as solitary and later developing multiple cysts. Because these first symptoms of the syndrome cause secondary, dentitio tarda (belated dentition) and displacement of the teeth, the dentist or the maxillo-facial-surgeon has a special responsibility for early diagnosis of this disease. Reference is made to the necessity of interdisciplinary cooperation and the ambulatory-care of the patients in the sense of a family registration and observation.

摘要

通过对来自3个家庭的7名患者进行调查,对基底细胞痣综合征的相关问题发表了看法。典型的临床症状优先且最早出现在颌骨,表现为孤立的囊肿,随后发展为多个囊肿。由于该综合征的这些首发症状会导致继发性的出牙延迟和牙齿移位,因此牙医或颌面外科医生对这种疾病的早期诊断负有特殊责任。文中提到了跨学科合作的必要性以及在家庭登记和观察意义上对患者的门诊护理。

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