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戈林-高茨综合征——幼儿罕见病例实体。

Gorlin-Goltz Syndrome - A Rare Case Entity in Young Child.

机构信息

Department of Oral and Maxillofacial Surgery, Awadh Dental College, Jamshedpur, Jharkhand, India.

Department of Oral and Maxillofacial Pathology and Microbiology, Awadh Dental College, Jamshedpur, Jharkhand, India.

出版信息

Prague Med Rep. 2024;125(1):69-78. doi: 10.14712/23362936.2024.7.

DOI:10.14712/23362936.2024.7
PMID:38470440
Abstract

Gorlin-Goltz syndrome (GGS) is an infrequent multisystemic disease with an autosomal dominant trait, which depicted presence of numerous basal cell carcinoma in conjunction with multiorgan abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the keratocystic odontogenic tumour are usually one of the first manifestations of the syndrome. This article includes a case report of the GGS with regard to its history, incidence, etiology, features, investigations, diagnostic criteria, keratocystic odontogenic tumour and treatment modalities.

摘要

戈尔丁-高茨综合征(GGS)是一种罕见的多系统疾病,具有常染色体显性遗传特征,表现为大量基底细胞癌与多器官异常并存。这种综合征可以通过牙医在生命的第一个十年中进行常规放射学检查早期诊断,因为角化囊性牙源性肿瘤通常是该综合征的最初表现之一。本文报告了一例 GGS 病例,包括其病史、发病率、病因、特征、检查、诊断标准、角化囊性牙源性肿瘤和治疗方式。

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