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Introduction Osteogenesis imperfecta is an inherited condition also known as brittle bone disease, which results from an abnormality in connective

tissue called type I collagen. Type I collagen fibers are found in bones, tendons, the skin, and in the eyes. This deficiency arises from an amino acid substitution of glycine to bulkier amino acids in the collagen triple helix structure. The larger amino acid side-chains create steric hindrance that creates a bulge in the collagen complex, which in turn influences both the molecular nanomechanics as well as the interaction between molecules, which are both compromised. As a result, the body may respond by hydrolyzing the improper collagen structure. If the body does not destroy the improper collagen, the relationship between the collagen fibrils and hydroxyapatite crystals to form bone is altered, causing brittleness There are different types of osteogenesis imperfect; type I, type I I, type I I I and type IV. Type I is the mildest and most common form of OI (about 50% of individuals) whites of the eyes (sclera) are blue; bone fractures more common during infancy and decrease after puberty; easy bruising; high incidence of hearing loss. Type II is the most severe form bone fractures are present before birth, often in the skull, long bones, or vertebrae; whites of the eyes are dark blue or gray; bones are malformed; lungs are underdeveloped. Babies born with Type II often die within weeks of birth due to heart and breathing problems. Type III - 50% of individuals have bone fractures before birth, the rest have bone fractures shortly after birth; shortening of limbs and deformities; facial abnormality; children and adults have short stature. Type IV - Bone fractures begin in infancy; children have difficulty growing. Osteogenesis imperfecta is a rare disease that occurs in one per 30,000 to 70,000 live births, depending on the type, and in the Philippines 2,587 in 86,241,6972, As we said that Osteogenesis imperfecta is a rare disease, and sadly it is a lifetime and untreatable disease. The only treatment for OI is reducing the number of fracture by teaching the parents about proper positioning and handling of the children, also the supportive shoes with arch supports are important, and leg braces may be helpful for the child learning to walk. Physical therapy can help keep joints mobile and strengthen muscles, but still further research study is being done in discovering drugs that will help strengthen the bone.

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