Bisphosphonates help children with osteogenesis imperfecta

Children who suffer from a bone-thinning disease may benefit from treatment with an intravenous drug used to strengthen the bone.

According to a new study out of Case Western Reserve University in Cleveland, bisphosphonates improve bone mineral density scores and physical abilities in children with osteogenesis imperfecta.

OI is a genetic disease characterized by brittle bones. In addition to being at high risk for fractures, children with the condition often suffer from short stature, scoliosis, progressive long bone deformity, hearing loss, and abnormal dentition. There is no cure for the disease and treatment options have been limited. Recent studies, however, have suggested a role for bisphosphonates, a class of drugs commonly used to treat osteoporosis in older individuals.

These researchers tested the treatment in six children between 22 months old and 14 years. All received the drug pamidronate intravenously over three consecutive days about every four months for at least two years. The average annual BMD increase seen in the children while they were on the therapy was 48 percent, and all the patients demonstrated functional improvement in mobility. Patients also reported decreased bone pain and parents reported the quality of their children’s’ lives improved.

While all of the children experienced new fractures during the study, the researchers note many occurred as the children engaged in increased physical activity. The two oldest children progressed from being wheelchair bound to being completely ambulatory while receiving the therapy.

The investigators believe these results support earlier findings suggesting a role for intravenous bisphosphonates in children with OI.

SOURCE: Pediatrics, 2003;111:573-578