親子にみられた鎖骨頭蓋骨異骨症

概要

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Two cases of cleidocranial dysostosis found in two generations are presented. The propositus was a 21-year-old female who presented a chief complaint of malocclusion. On the examination of our patient's family pedigree, her father and brother had similar physical findings as she. This syndrome shows the clinical characteristics such as, short statures, brachycephalic skull, broad base of nose, ocular hypertelorism, high arched palate, reversed bites, failure eruption of permanent teeth, and delay in deciduous dentition. The characteristic roentgenological findings of this syndrome are delayed closure of fontanels and sutures, numerous wormian bone formation, underdeveloped accessory sinus, supernumerary teeth, rounded mandibular angle, aplastic clavicle bilaterally, and absence of ossification of public bones. The propositus and her father who is 53-year-old revealed the above clinical and roentgenological characteristic findings. Cleidocranial dysostosis is transmitted as autosomal dominant trait. In our cases, it seemed that genetic factor was transmitted to a daughter through her father.
九州歯科学会の論文
1983-06-25