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Brittle bone disease or osteogenes is imperfecta an inherited
disorder of the connective tissue resulting in the formation
of bones which are unusually fragile and brittle and
which break very easily.
If present at birth, the baby has multiple
fractures, is usually deformed and this severe form is
called osteogenesis imperfecta cognita. A generally less serious
form may develop later when a baby reaches the toddler
stage, and this is called osteogenesis imperfecta tarda.
This
form may improve as the child grows older. Other associated
symptoms may occur including transparent teeth, unusually
mobile joints and dwarfism. There is little effective treatment.
Osteoporosis affects approximately 25 million Americans and causes nearly 1.5 million fractures each year, according to a recent National Institutes of Health report.
People with kidney disease and those on long-term medication, including transplant recipients, are at increased risk of developing the disease. These people often develop osteoporosis as a result of medications or a disease process rather than menopause or through the normal aging process.
In extreme cases, a newborn baby has multiple fractures that have occurred in the womb and many infants die shortly following birth.
What treatment can be given in Brittle bone disease?
The mainstay of treatment is competent orthopaedic care at the time of fractures, to ensure that each fracture heals in a good position. Patients should be mobilised as early as possible to minimise the loss of bone due to immobilisation.
In some circumstances ‘rodding’ operations, in which fixed or telescopic metal rods are inserted into the shafts of bones, are very helpful, particularly in children with very frequent fractures or appreciable deformity.
Competent occupational therapy help may be invaluable in ensuring that parents are given good advice in handling of a young child, in prescribing the most appropriate seating or wheelchairs, in advising on adaptations to the home and on practical ways of ensuring a good education.
There is no drug treatment of osteogenesis imperfecta itself. Trials of growth hormone have been disappointing. Trials of various bisphosphonate drugs are in progress and have given encouraging results in some patients with the more severe types of OI. The management of older women with OI is discussed next.
Bone disorder drugs are medicines used to treat this diseases that weaken the bones.
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