@entity461 (1-34) replacement therapy in a XXXX with @entity261 caused by a sporadic @entity460 receptor mutation.

@entity1 @entity8 @entity174 @entity11604 @entity261 @entity421 @entity74 @entity460 @entity5200 @entity461 @entity1760
@entity74 ( @entity74 ) is an inherited form of @entity261 caused by activating mutations in the @entity460 -sensing receptor (CaR). Treatment with @entity461 (1-34) may be superior to conventional therapy but is contraindicated in @entity1 , and long-term effects on the skeleton are unknown. The @entity1 is a 20-yr-old female with @entity74 treated with @entity461 continuously since 6 yr and 2 mo of age. A bone biopsy was obtained for histomorphometry and quantitative backscattered electron imaging (qBEI). Her data were compared with one age-, sex-, and length of @entity261 -matched control not on @entity461 and two sex-matched @entity74 controls before and after 1 yr of @entity461 . The @entity1 's growth was normal. @entity5200 and hypermagnesuria persisted despite normal or subnormal serum @entity460 and @entity1760 levels. @entity11604 , without evidence of @entity8 , developed by 19 yr of age. Cancellous bone volume was dramatically elevated in the @entity1 and in @entity74 controls after 1 yr of @entity461 . BMD distribution (BMDD) by qBEI of the @entity1 and @entity74 controls was strikingly shifted toward lower mineralization compared with the non- @entity74 control. Moreover, the @entity74 controls exhibited a further reduction in mineralization after 1 yr of @entity461 . These findings imply a role for CaR in bone matrix mineralization. There were no @entity174 or @entity421 . In conclusion, long-term @entity461 replacement in a @entity1 with @entity74 was not unsafe, increased bone mass without negatively impacting mineralization, and improved serum mineral control but did not prevent @entity11604 . Additionally, this may be the first evidence of a role for CaR in @entity1 bone.
