Osteogenesis Imperfecta

Osteogenesis imperfecta

Question. Write a note on osteogenesis imperfecta.

Answer.

Osteogenesis Imperfecta

It is also called brittle bone or lobstein osteogenesis or fragility ossium or osteoarthritis.

This is an autosomal dominant condition affecting bone formation.

It presents a hereditary autosomal dominant trait.

osteogenesis imperfecta

“Role of collagen defects in osteogenesis imperfecta”

Types Of Osteogenesis Imperfecta.

• Congenital type
• Lobstein type.

“Genetic testing for osteogenesis imperfecta”

Clinical Features Of Osteogenesis Imperfecta.

• It usually occur in infants.
• There is extreme fragility or porosities of bone with proneness of fracture.
• There is occurrence of pale blue sclera which is thin and pigmented choroids shows through and produces blue color.
• There is deafness due to osteosclerosis, laxity of ligaments and peculiar shape of skull.
• Increase tendency for capillary bleeding.

“Genetic mutations linked to osteogenesis imperfecta”

Oral Manifestations Of Osteogenesis Imperfecta.

• Osteogenic imperfecta is associated with dentinogenesis imperfecta.
• There is hypoplasia of teeth.
• Deciduous teeth are poorly calcified and semi-translucent or waxy.
• Teeth appear as faintly dirty pink, half normal size, with globular crown and relative short roots, in proportion to other dimensions.

“Tests for diagnosing osteogenesis imperfecta”

Histopathological Features Of Osteogenesis Imperfecta.

• Osteoblastic activity appears as retarded and imperfect.
• Failure of fetus to be transformed with mature collagen.
• The trabeculae of cancellous bone are delicate and often show fracture.

brittle bone disease

“Emerging treatments for osteogenesis imperfecta”

Treatment Of Osteogenesis Imperfecta.

No known treatment.