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Brittle Bone Disease

Brittle Bone Disease Definition

Osteogenesis imperfecta (OI, or sometimes known as Brittle Bone Disease) is a genetic bone disorder. People with this disease are born without the proper protein (collagen), or the ability to make it, usually because of a deficiency of Type-I collagen. People with OI either have lesser collagen than normal or the quality is poorer than normal. As collagen is an important protein in bone structure, this impairment can result to those with the condition to have weak or fragile bones. As a genetic disorder, brittle bone disease is an autosomal dominant defect. Most people with OI inherit it from a parent but it can also be an individual (as in de novo or "sporadic") mutation.

Brittle Bone Disease Prevalence

Frequency is approximately the same across the different groups, but for unknown reasons the Shona and Ndebele tribes of Zimbabwe seem to have a higher proportion of Type III to Type I than other groups. A similar pattern, however, was found in some segments of the Nigerian and South African population. In these varied cases the total number of OIs of all four types was basically similiar to any other ethnicity and race.

Brittle Bone Disease Treatment

As of now, there is no cure for OI. Treatments are aimed at increasing the patient's overall bone strength to prevent fracture and maintain mobility. There have been many clinical trials done with the drug, Fosamax, a drug used to treat females experiencing brittleness of bones due to osteoporosis. More success was evident in the pill form versus the IV form, but success was still seen. The FDA will not approve Fosamax as a treatment for OI because the long term effects of the drug have not been studied, although it is often used in Preteens, instead of Pamidronate. Bone infections are treated as and when they appear with the appropriate antibiotics and antiseptics. Surgery can also be used to help patients with OI. Metal rods can be surgically inserted in the long bones to help improve their strength. The placement of stainless steel rods into the intramedullary canals of the long bones helped to stabilize and strengthen them. The treatment proves extremely useful in the rehabilitation and prevention of fractures. Spinal fusion can also be done to correct scoliosis, although the inherent bone fragility makes this operation more complex in OI patients. Surgery for basilar impressions can be carried out if pressure being exerted on the spinal cord and brain stem leads to neurological problems.

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