5193
Mennat Allah Ismail Mehrez
Craniofacial & OroDental Evaluation of Some Progeria and Pseudoprogeria Syndromes
Craniofacial, OroDental , Progeria
The purpose of this descriptive study was to evaluate the craniofacial and orodental manifestations in some progeria and pseudoprogeria syndromes both clinically and radiographically and highlight the differences between each group. The study included 16 cases. The ages ranged from 1y8/12 to 30 years of age. The cases comprised 8 syndromes under two groups; A. progeria and B. pseudoprogeria. Progeria syndromes included 2 subgroups; 1.A) progeria laminopathies and 2.A) progeroids due to DNA repair defects.Progeria laminopathies (group 1.A) included 3 syndromes; Atypical Hutchinson Gilford progeria (2 siblings; cases 1 & 2), Atypical Werner (case 3) and mandibulacral dysplasia (2 siblings; cases 4 & 5). Progeroid syndromes due to defective DNA repair (group 2.A) included 3 syndromes as well; Kindler syndrome (2 siblings; cases 6 & 7), Cockyne syndrome (cases 8 to12) and xeroderma pigmentosum (case 13). The pseudoprogeria syndromes (group B) included 2 syndromes; pycnodysostosis 14 & 15) and cleidocranial dysplasia (case 16).
2015
Ph.d
Al-Azhar
Dental Medicine