title: Treatment considerations in hutchinson-gilford progeria syndrome: a case report.
abstract: Hutchinson-Guilford progeria syndrome is an extremely rare condition classified as one of the premature ageing syndromes. This case presents a 16-year-old Israeli female patient, suffering from a variant of Hutchinson-Guilford progeria with a history of treatment with oral biphosphnates. The patient presented with typical cranial and facial features of the syndrome including delayed teeth eruption and root development probably due to insufficient jaw growth and severs retrognatic position of the maxilla and mandible. Orthodontic treatment considerations are described along with those required in light of the previous treatment by oral biphosphonates.All primary teeth were extracted in three appointments while creating as minimal trauma as possible to the surrounding tissue and alveolar bone. For now, the patient refuses to begin the orthodontic treatment course. There are no limitations to conduct any dental procedures in progeria patients, however, extreme caution must be exercised during oral surgery due to the inelasticity of tissues and dermal atrophy. Orthodontic procedure commencement should be early enough to manage the delayed development and eruption of teeth. Patients taking oral biphosphonates should be advised of this potential complication. If orthodontic treatment is considered appropriate, plans should be assessed and modified to include compromises.
authors.affiliation: Orthodontic and Craniofacial Department, Graduate School of Dentistry, Rambam Health Care Campus, Bruce Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel.
authors.forename: H
authors.initials: H
authors.lastname: Hazan-Molina
authors.affiliation: Orthodontic and Craniofacial Department, Graduate School of Dentistry, Rambam Health Care Campus, Bruce Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel.
authors.forename: D
authors.initials: D
authors.lastname: Aizenbud
authors.forename: Aizenbud D
authors.initials: AD
authors.lastname: Dror
mesh_terms: D000293:Adolescent; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D002508:Cephalometry / Q000379:methods; D004164:Diphosphonates / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007093:Imidazoles / Q000627:therapeutic use; D008310:Malocclusion / Q000628:therapy*; D009805:Odontogenesis / Q000502:physiology; D010347:Patient Care Planning; D011371:Progeria / Q000150:complications*; D063173:Retrognathia / Q000628:therapy; D014078:Tooth Eruption / Q000502:physiology; D014081:Tooth Extraction / Q000379:methods; D014092:Tooth Root / Q000254:growth & development; D014094:Tooth, Deciduous / Q000503:physiopathology / Q000601:surgery; D000077211:Zoledronic Acid
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D007093:Imidazoles; D000077211:Zoledronic Acid
keywords: Biphosphonates; Hutchinson-Guilford progeria; Orthodontics