BENEFICIAL EFFECT OF BISPHOSPHONATE DURING 5 YEARS OF TREATMENT OF SEVERE OSTEOGENESIS IMPERFECTA

We report results of 2-5 y treatment with intravenous disodium pamidro nate (APD) in three girls with severe osteogenesis imperfecta (OI). Tr eatment was given as monthly infusions. Additional oral 1,25-dihydroxy -cholecalciferol was given to compensate for a transient decrease in s erum calcium levels. During treatment, DEXA measurements showed a grad ual increase in bone density in all patients. All parameters analysed in serum (ALP, osteocalcin, PICP, ICTP) and in urine (deoxypyridinolin e and pyridinoline) showed a decreased bone turnover. The two younger patients reported a major improvement in well-being, pain and activiti es of daily life. The effect on the older patient was less pronounced. No negative side effects in clinical or laboratory variables were obs erved. This study indicates that APD is of value in the symptomatic tr eatment of children with severe OI.