Dental Anomalies Associated with Craniometaphyseal Dysplasia

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چکیده

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منابع مشابه

Craniometaphyseal dysplasia.

Craniometaphyseal dysplasia is a rare congenital anomaly involving the modelling of the metaphyses and characterized by pronounced splaying of the metaphyseal ends of the long bones, enormous thickening of the base of the skull, and absence of pneumatization of the paranasal sinuses and mastoid region. Pyle (I93i) reported the first case and the disease is sometimes named after him. Ellis (1934...

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Craniometaphyseal Dysplasia

Craniometaphyseal dysplasia is a rare bone disorder of unknown etiology characterized by overgrowth of the skull base or craniofacial bones and abnormal remodeling of the metaphyses of the long bones. We present a sporadic case of craniometaphyseal dysplasia associated with facial paralysis observed in a 4-year-old female, which emphasizes the importance of the early detection of accompanying l...

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Dental anomalies in a child with craniometaphysial dysplasia.

Craniometaphysial dysplasia (CMD) is a rare disorder that mainly affects craniofacial bones. It is caused by mutations within a region of human homolog (Ankh) of the mouse progressive ankylosis (Ank) gene. ANK, together with other factors, regulates intracellular and extracellular levels of pyrophosphate/inorganic phosphate critical for maintaining mineral homeostasis. The systemic manifestatio...

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Case Report Craniofacial surgery for craniometaphyseal dysplasia

Craniometaphyseal dysplasia (CMD) is a rare congenital bone dysplasia with abnormal bony overgrowth leading to characteristic facial features and cranial nerve compression. We present a 10-year-old child with bony swelling at the nasal root since birth along with decreased hearing in both ears. She had normal developmental milestones and intelligence. On examination, she had bossing of forehead...

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Complex dental anomalies in a belatedly diagnosed cleidocranial dysplasia patient

Cleidocranial dysplasia (CCD) is a rare congenital disorder, typically characterized by persistently open skull sutures, aplastic or hypoplastic clavicles, and supernumerary teeth. Mutations in the gene encoding the runt-related transcription factor 2 (RUNX2) protein are responsible for approximately two thirds of CCD patients. We report a 20-year-old CCD patient presenting not only with typica...

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ژورنال

عنوان ژورنال: Journal of Dental Research

سال: 2014

ISSN: 0022-0345,1544-0591

DOI: 10.1177/0022034514529304