The craniocervical junction following successful XXXX for @entity12751 ( @entity12751 ).

@entity1 @entity8 @entity66 @entity24179 @entity5684 @entity2132 @entity12751 @entity1613 @entity204
@entity12751 ( @entity12751 ) is a progressive @entity66 caused by @entity12751 . First choice of treatment in @entity12751 @entity1 is @entity8 ( @entity8 ). The effect of @entity8 has been shown to have limited influence on skeletal manifestations by poor penetration of musculoskeletal tissues by the enzyme derived from donor leucocytes. Aim of this study was to investigate the effect of @entity8 on the craniocervical junction (CCJ) in @entity12751 @entity1 . We analysed retrospectively sequential magnetic resonance imaging (MRI) scans of 30 @entity1 with @entity12751 treated by @entity8 since 1982 at the Royal Manchester @entity1 's Hospital, UK, in order to determine whether the @entity1 suffer from @entity24179 . Results were compared with biochemical and clinical characteristics: Enzyme activity (EA), chimerism, urinary glycosaminoglycan (GAG) excretion and neurological status. Investigations were part of standard clinical procedures. Results are descriptive in presentation. In 26/30 @entity1 a @entity204 was feasible. The majority showed a normal dens length and an increase with age. Only 3/26 revealed a @entity24179 . One of them had only partial donor engraftment (DE) with reduced EA, one of them suffered from @entity2132 ( @entity2132 ). One @entity1 with only partial DE and reduced EA presented with @entity1613 until preadolescence but normalized later on. There may be a trend towards lower EA and the occurrence of DH in transplanted @entity5684 @entity1 - perhaps the dosage of enzyme plays a role in the correction of skeletal complications in this @entity1 group. @entity8 @entity1 with incomplete DE and therefore lower EAs may require special attention and care.
