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Majorana A, Bardellini E, Brunelli PC, Lacaita M, Cazzolla AP, Favia G. Dentinogenesis imperfecta in children with osteogenesis imperfecta: a clinical and ultrastructural study. Int J Paediatr Dent. 2010; 20:112-118
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A review of osteogenesis imperfecta in relation to orthodontic treatment

From Volume 6, Issue 1, January 2013 | Pages 21-24

Authors

Anna Cala

BDS

Department of Orthodontics, Kettering General Hospital, Kettering, UK

Articles by Anna Cala

Julian O'Neill

MOrth, BDS, FFD RCS RCS(Eng)

Department of Orthodontics, Kettering General Hospital, Kettering, UK

Articles by Julian O'Neill

Abstract

This case report describes a 13-year-old patient suffering from osteogenesis imperfecta (OI) and hypodontia. Different issues related to clinical presentation and orthodontic treatment of patients with OI are discussed in this report.

Clinical Relevance: Dental and orthodontic features associated with OI and their impact on treatment are described in this report. It is important to know these features as patients presenting with OI may require orthodontic treatment.

Article

Osteogenesis imperfecta (OI) known as ‘brittle bone disease’ is defined as a group of heritable connective tissue disorders, caused by a defect in the synthesis of type I collagen. The most common causes of this condition are mutations of the genes COL1A1 and COL1A2 that encode chains of type I collagen.1 Because of the variety in clinical presentation and the emergence of new genetic mutations, the exact incidence of OI is unknown. However, an incidence rate of 1:5000 to 1:20,000 live births was reported by different authors.2,3

Patients with OI may show a plethora of signs and symptoms related to the defective collagen present in different structures of the body, including bone, sclera, skin, ligaments, blood platelets, auditory ossicles, inner ear and teeth. Thus, the signs and symptoms include:

OI is classified on the basis of clinical, genetic and radiographic features.1 Sillence et al18 divided OI into four types. Since then further types have been added:

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