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Osteogenisis Imperfecta

OI is several genetically & clinically heyerogeneous  syndromes, characterized by skeletal fragility.  

 disease characterized by failure of maturation and organization of collagen fibers;

           - defect in collagen (procollagen to type I collagen sequence and abnormal cross linking) leading to decreased collagen secretion, bone fragility;

           - there may be an inability to form normal bone due to a defect in osteoblastic function

Ability to sit by 10 months of age is a good predictor of future walking ability.

Classification:

Type I: 

most common type

autosomal dominant

blue sclerae

fractures during childhood; hearing loss

Type II: 

very rare

autosomal recessive

blue scelae

lethal in perinatal period; crumpled long bones; flattened vertebrae

Type III: 

autosomal recessive

white sclerae

short stature; fractures and progressive deformity; spinal deformity

Type IV: 

   

autosomal dominant

white sclerae

skeletal fragility; no hearing loss; moderate growth failure



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