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黏多糖贮积症Ⅳ型。病例报告及临床发现综述。

Morquio's syndrome. A case report and review of clinical findings.

作者信息

Fitzgerald J, Verveniotis S J

出版信息

N Y State Dent J. 1998 Oct;64(8):48-50.

PMID:9828617
Abstract

Morquio's syndrome is a genetic disorder caused by the lack of the enzyme responsible for the breakdown of the mucopolysaccharide keratin sulfate. The resulting buildup of keratin sulfate causes a specific pattern of abnormalities, including short stature, spinal deformity, cardiac abnormalities, corneal opacities, deafness and dental abnormalities. The specific dental findings in the patient described here are as follows: the posterior teeth were tapered and had pointed. cusps; the maxillary anterior teeth were spaced and flared, and on radiographs, the enamel was less than 25 percent of normal thickness but of normal radiodensity. Despite these findings, the patient exhibited a low rate of caries. This paper is a review of medical and dental findings in the Morquio's patient and how this may affect dental management.

摘要

莫尔基奥综合征是一种遗传性疾病,由负责分解黏多糖硫酸角质素的酶缺乏所致。硫酸角质素的蓄积导致了一系列特定的异常表现,包括身材矮小、脊柱畸形、心脏异常、角膜混浊、耳聋和牙齿异常。此处描述的患者的具体牙齿表现如下:后牙呈锥形且有尖锐的牙尖;上颌前牙间隙增宽且向外展开,X线片显示牙釉质厚度不足正常的25%,但放射密度正常。尽管有这些表现,但该患者龋齿发生率较低。本文综述了莫尔基奥综合征患者的医学和牙齿表现以及这些表现可能如何影响牙科治疗。

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