All are features of Osteogenesis imperfecta except:
Correct Answer: Cataract
Description: Ans. is 'c' i.e. Cataract Osteogenesis imperfecta (firagilitas ossium):This condition is characterised by tendency for frequent fractures because of weak and brittle bones.It results from defective collagen synthesis (type I collagen. Thus also affecting other collagen containing soft tissue such as skin, sclera, teeth, ligaments etc.inheritance is AD or AR*AR is very severe form and the child is bom with multiple fractures and diesClinical features:Blue sclera* (because of thin sclera colour of underlying choroid is visible.)Joint laxity * (b/c of weak ligaments)Hearing loss (b/c of otosclerosis)*Hernias* (because of defective skin)Brittle bones* (because of abnormal synthesis of bone matrix).The fractures unite normally but deformities secondary to malunion or joint contractures may occur.Other causes of 'tendency for easy fractures':Osteoporosis*Osteopetrosis*Osteomalacia*Polio limb
Category:
Orthopaedics
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