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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.
AuthorsH Hazan-Molina, D Aizenbud, Aizenbud D Dror
JournalThe Journal of clinical pediatric dentistry (J Clin Pediatr Dent) Vol. 39 Issue 2 Pg. 172-8 ( 2015) ISSN: 1053-4628 [Print] United States
PMID25823488 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Bone Density Conservation Agents
  • Diphosphonates
  • Imidazoles
  • Zoledronic Acid
Topics
  • Adolescent
  • Bone Density Conservation Agents (therapeutic use)
  • Cephalometry (methods)
  • Diphosphonates (therapeutic use)
  • Female
  • Humans
  • Imidazoles (therapeutic use)
  • Malocclusion (therapy)
  • Odontogenesis (physiology)
  • Patient Care Planning
  • Progeria (complications)
  • Retrognathia (therapy)
  • Tooth Eruption (physiology)
  • Tooth Extraction (methods)
  • Tooth Root (growth & development)
  • Tooth, Deciduous (physiopathology, surgery)
  • Zoledronic Acid

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