Bone Abstracts

Aim: To evaluate the effect of bisphosphonate treatment in children with McCune Albright syndrome (MAS).

Materials and methods: Six MAS patients diagnosed between 1998–2012 with bone pain, bone fracture and bone deformity were evaluated retrospectively. Main outcome measures included disease presentation, diagnostic evaluation, management strategy, and outcome.

Results: The mean age of the patients were 16.5±8.2 years (four girls, two boys). The first signs has started at the age of 4.5±2.7 years and the patients were diagnosed at the age of 9.5±5.8 years. First patient admitted with recurrent fractures, the second and the sixth patients with deformities on the extremities, the fifth patient with bone pain, the third and fourth patients with early puberty and facial bone deformity. Bisphosphonate therapy was initiated at a dose of 20 mg/m² every 2 months. The mean serum levels of Ca, P, ALP, PTH, and osteocalcin were 9.9±0.8 mg/dl, 3.6±1 mg/dl, 368.8±175.6 IU/l, 35.3±1.6 pg/ml, 73.2±31.6 ng/ml respectively at the beginning of therapy. On the last evaluation the mean serum levels of Ca P; ALP; PTH; and osteocalcin were 9.2±0.3 mg/dl, 3.8±0.2 mg/dl, 418.3±427.9 IU/l, 47.2±22.5 pg/ml and 65.6±26.9 ng/ml retrospectively. The first patient who admitted with recurrent fractures had no fractures for four years and the dysplastic lesions did not progress. The third patient with cranial deformity the lesion progressed whereas in the other patient with cranial deformity the size of the lesion did not change.

Conclusion: As a result, although bisphosphonate therapy in fibrous dysplasia improves bone mineral density, it has no effect on regression of the lesions.

Disclosure: The authors declared no competing interests.