鎖骨顱骨發育不良(Cleidocranial dysplasia, CCD)病患於口腔表徵與合併矯正治療之文獻回顧

A Systemic Review of Oral Phenomenon and Orthodontic Treatment in Patients with Cleidocranial Dysplasia

藺國豪(Kuo-Hao Lin) ； 况守信(Shouh-Sin Kuang) ； 吳政憲(Cheng-Hsien Wu) ； 吳姿瑩(Tzu-Ying Wu)

《中華民國齒顎矯正學雜誌》 22卷1期 (2010/03) Pp. 14-20

https://doi.org/10.30036/TJO.201003.0002

摘要

鎖骨顱骨發育不良為一自體顯性遺傳疾病，在目前基因遺傳的研究方面，此疾病之變異基因位在6p21之RUNX2^2。臨床方面會造成全身多部分骨骼成骨不全，在病患的口腔方面，常見恆齒齒列延遲或異位萌發、多發性贅生齒、乳牙齒列牙根的延遲吸收、恆齒及贅生齒之牙根彎曲，及合併有明顯的咬合關係不良(malocclusion)。病患往往因口腔問題前來求診時，進而被診斷出患有此種罕見疾病。本文以文獻回顧的方式整理此遺傳疾病之臨床表徵與致病機轉。

關鍵字

鎖骨顱骨發育不良 ； 口腔表徵

並列摘要

Cleidocranio-dysplasia is an autosomal dominant disease. RUNX2 (CBFA1) is the only gene known to be associated with CCD. The clinical characteristic of skeletal dysplasia including delayed or ectopic eruption of permanent teeth, multiple supernumanary teeth, delayed resorption of primary root, dilacerations of permanent and supernumanary teeth, malocclusion with skeletal discrepancy. Diagnosis of CCD in dental clinic due to oral phenomenens happened usually. This paper would systemically reviewed the genetic and pathological mechanism of CCD.

並列關鍵字

Oral Phenomenon ； Cleidocranial dysplasia