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SYNDROMES (DOMINANT INHERITANCE (Crouzon syndrome (Premature cranial…
SYNDROMES
DOMINANT INHERITANCE
Nasofrontal dysplasia
Incomplete formation nose tip, V-shaped skull, cleft nose/lip
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Cleidocraneal dysostosis
Delayed closure anterior fontanella, braquicephalic
Class III occlusion, delayed eruption, supernumerary teeth
Otomandibular dysostosis
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Gondenhar syndrome: hearing loss, heart, vertebra
Apert syndrome
Premature fusion coronal & sagittal, heart, mental, syndactyly
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Pierre-Robin syndomre
Triad: micrognathia, cleft palate, retroglossoptosis
Crouzon syndrome
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Hypertelorism, exophtalmos, nasomaxilar retrusion
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Van der Woude syndrome
Cleft lip/cleft palate, dental agenesis
Acondroplasia
Dwarfism, alteration cartilage
Big skull, frontal & occipital protuberances, short nasal bones
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RECESSIVE INHERITANCE
Hypophosphatasia
Enzymatic deficiency, irregular ossification cartilage
Treatment: phosphorous, vit D, parahormone
Clinical presentation
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Childhood - dental anomalies, Rickets
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AUTOSOMAL
Down syndrome
Trisomy chromosome 21, mental, heart, perio
Braqui, flat occipital, microcephalia, brushfield spots
Delayed eruption, agenesis, microdontia, hypocalcification enamel, taurodism