Clinical Considerations for Hutchinson-Gilford Progeria Syndrome in Dental Treatment Sung-Ying Ye Department of Prosthodontics Chi Mei Medical Center, Tainan, Taiwan Jui-Chung Chang Director, Department of Prosthodontics Chi Mei Medical Center, Tainan, Taiwan Corresponding author: Jui-Chung Chang Director, Department of Prosthodontics Chi Mei Medical Center, Tainan, Taiwan E-mail: csg129@gmail.com Tel: (06)2812811 ext. 53175 關鍵字 : 早衰症 張口受限 口顏面障礙 牙科處理 Abstract Hutchinson-Gilford Progeria syndrome HGPS is a rare genetic disorder in which the individual suffers from various afflictions similar to an aged one. HGPS individuals exhibit many dentofacial disorders. These manifestations may affect dental treatment. The purpose of this report presented a case of HGPS. Clinical considerations in dental treatment were emphasized. Key word: Hutchinson-Gilford Progeria syndrome, mouth open limitation, dentofacial disorder, dental treatment. Hutchinson-Gilford Progeria syndrome, 早 HGPS 97% 1.5 13 atherosclerosis 1 2 Journal of Prosthodontics and Implantology 31
1 2X 3X 1886 LMNA Lamin A Prelamin A LMNA Lamin A C T Progerin 3,4 1 bird face 2 34 5 6 42 bird face 32 Volume 3, Number 1, 2014
1 X 2X cephalometric radiograph mandibular ramus obtuse mandibular angle 3 4 4 5 X Journal of Prosthodontics and Implantology 33
6 7 X 5 6 7 balanced occlusion 8-12 DNA 34 Volume 3, Number 1, 2014
8 9 5 micrognathia atrophy of alveolar process palatal vault hypoplastic mandible 6 X cephalometric radiographmandibular ramus obtuse mandibular angle Journal of Prosthodontics and Implantology 35
10 11 36 Volume 3, Number 1, 2014
12 X vertical dimension anodontia 7 progeria syndrome(mim 176670) but not in Wiedemann- Rautenstrauch progeroid syndrome(mim 264090). J Hum Genet. 2003;48:271-4. 5. Saigal S, Bhargava A. Progeria: Pathogenesis and oral manifestation- A review. Kathmandu university Medical 2012;10(1):72-6. 6. Gordon LB, McCarten KM, Giobbie-Hurder A et al. Disease progression in Hutchinson-Gilford progeria syndrome: Impact on growth and development. Pediatrics 2007;120(4):824-33. 7. Maloney W. The integral role of the dentist in treating individuals with Hutchinson-Gilford progeria syndrome. WebmedCentral 2010;1:1-3. 1. Domingo DL, Trujillo MI, Council SE et al. Hutchinson-Gilford progeria syndrome: Oral and craniofacial Phenotypes. Oral Dis 2009;15(3):187-95. 2. Hennekam RC. Hutchinson-Gilford progeria syndrome: Review of the Phenotype. American J Med Genet A. 2006;140:2603-24. 3. Goldman Rd, Shumaker DK, Erdos MR et al. Accumulation of mutant lamina causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome. Proc Natl Acad Sci USA. 2004;101:8963-8. 4. Cao H, Hegele RA. LMNA is mutated in Hutchinson-Gilford Journal of Prosthodontics and Implantology 37