Osteogenesis Imperfecta, Type IV

Alternative Names

  • OI, Type IV
  • OI4
  • Osteogenesis Imperfecta with Normal Sclera
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WHO-ICD-10 version:2010

Congenital malformations, deformations and chromosomal abnormalities

Congenital malformations and deformations of the musculoskeletal system

OMIM Number

166220

Mode of Inheritance

Autosomal dominant

Gene Map Locus

7q21.3,17q21.33

Description

Osteogenesis imperfecta is characterized chiefly by multiple bone fractures, usually resulting from minimal trauma. Affected individuals have blue sclerae, normal teeth, and normal or near-normal stature. Fractures are rare in the neonatal period; fracture tendency is constant from childhood to puberty, decreases thereafter, and often increases following menopause in women and after the sixth decade in men. Fractures heal rapidly with evidence of a good callus formation, and, with good orthopedic care, without deformity. Hearing loss of conductive or mixed type occurs in about 50% of families, beginning in the late teens and leading, gradually, to profound deafness, tinnitus, and vertigo by the end of the fourth to fifth decade. Additional clinical findings may be thin, easily bruised skin, moderate joint hypermobility and kyphoscoliosis, hernias, and arcus senilis. Mitral valve prolapse, aortic valvular insufficiency, and a slightly larger than normal aortic root diameter have been identified in some individuals, but it is not clear that these disorders are significantly more frequent than in the general population. [From OMIM]

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
166220.1Saudi ArabiaMaleNo Reduced bone mineral density; Bowing of ...NM_000089.4:c.1378G>AHeterozygousAutosomal, DominantMaddirevula et al. 2018
166220.2EgyptMaleYes Recurrent fractures; Triangular face; Bl...NM_000089.4:c.2873G>AHeterozygousAutosomal, DominantMaddirevula et al. 2018 De novo mutation
166220.3Saudi ArabiaMaleNo Recurrent fractures; Multiple prenatal f...NM_000089.4:c.3043G>AHeterozygousAutosomal, DominantMaddirevula et al. 2018 De novo mutation
166220.4.1Saudi ArabiaMaleYes Recurrent fractures; OsteoporosisNM_000089.4:c.821G>AHeterozygousAutosomal, DominantMaddirevula et al. 2018 Patient's parents ar...
166220.4.2Saudi ArabiaFemaleYes Recurrent fractures; OsteoporosisNM_000089.4:c.821G>AHeterozygousAutosomal, DominantMaddirevula et al. 2018 Relative of 166220.4...
166220.5Saudi ArabiaFemale Recurrent fractures; Triangular face; Ab...NM_000089.4:c.1378G>AHeterozygousAutosomal, DominantMaddirevula et al. 2018 Patient's parents ar...
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