Abstract:
BACKGROUND:Fibrodysplasia ossificans progressiva (FOP) is a catastrophic genetic disorder of progressive heterotopic ossification (HO). Assessment of functional mobility in FOP will be essential to support clinical trials of investigational agents. RESULTS:Of necessity, we developed a simple, rapidly-administered, cumulative analogue joint involvement scale (CAJIS) for FOP based on assessments in 144 individuals worldwide with classic FOP. CONCLUSIONS:CAJIS scores correlated with patient age, activities of daily living, and ambulatory function with excellent inter-rater variability. We show here that the CAJIS score provides an accurate and reproducible snapshot of total body and regional mobility burden in FOP.
journal_name
Bonejournal_title
Boneauthors
Kaplan FS,Al Mukaddam M,Pignolo RJdoi
10.1016/j.bone.2017.04.015subject
Has Abstractpub_date
2017-08-01 00:00:00pages
123-128eissn
8756-3282issn
1873-2763pii
S8756-3282(17)30157-6journal_volume
101pub_type
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