Intravenous @entity5520 Therapy of Young XXXX With Osteogenesis Imperfecta: Skeletal Findings During Follow Up Throughout the Growing Years.

@entity1 @entity5279 @entity171 @entity174 @entity3814 @entity1635 @entity5520 @entity4381 @entity6926
UNASSIGNED: Cyclical intravenous @entity5520 therapy is widely used to treat @entity1 with @entity6926 ( @entity6926 ), but little is known about long-term treatment outcomes. We therefore reviewed 37 @entity1 with @entity6926 ( @entity6926 type I, n   =   1; @entity6926 type III, n   =   14; and @entity6926 type IV, n   =   22) who started intravenous @entity5520 therapy before 5 years of age (median 2.2 years; range, 0.1 to 4.8 years), and who had a subsequent follow-up period of at least 10 years (median 14.8 years; range, 10.7 to 18.2 years), during which they had received intravenous @entity5520 treatment ( @entity5279 or @entity4381 ) for at least 6 years. During the observation period, the mean lumbar spine areal bone mineral density Z-score increased from -6.6 (SD 3.1) to -3.0 (SD 1.8), and weight Z-score increased from -2.3 (SD 1.5) to -1.7 (SD 1.7) (p   <   0.001 and p   =   0.008). At the time of the last assessment, @entity1 with @entity6926 type IV had significantly higher height Z-scores than a control group of @entity1 matched for age, gender, and @entity6926 type who had not received @entity171 . @entity1 had a median of six femur @entity174 (range, 0 to 18) and five tibia @entity174 (range, 0 to 17) during the follow-up period. At baseline, 35% of vertebra were affected by @entity3814 , whereas only 6% of vertebra appeared compressed at the last evaluation (p   <   0.001), indicating vertebral reshaping during growth. Spinal fusion surgery was performed in 16 @entity1 (43%). Among the 21 @entity1 who did not have spinal fusion surgery, 13 had @entity1635 with a curvature ranging from 10 to 56 degrees. In conclusion, long-term intravenous @entity5520 therapy was associated with higher Z-scores for lumbar spine areal bone mineral density and vertebral reshaping, but long-bone @entity174 rates were still high and the majority of @entity1 developed @entity1635 .    2015 American Society for Bone and Mineral Research.
