BACKGROUND: Progeria is a rare segmental premature aging disease with 
significant skeletal abnormalities. Defining the full scope of radiologic 
abnormalities requires examination of a large proportion of the world's progeria 
population (estimated at 1 in 4 million). There has been no comprehensive 
prospective study describing the skeletal abnormalities associated with 
progeria.
OBJECTIVE: To define characteristic radiographic features of this syndrome.
MATERIALS AND METHODS: Thirty-nine children with classic progeria, ages 2-17 
years, from 29 countries were studied at a single site. Comprehensive 
radiographic imaging studies were performed.
RESULTS: Sample included 23 girls and 16 boys-the largest number of patients 
with progeria evaluated prospectively to date. Eight new and two little known 
progeria-associated radiologic findings were identified (frequencies of 3-36%). 
Additionally, 23 commonly reported findings were evaluated. Of these, 2 were not 
encountered and 21 were present and ranked according to their frequency. Nine 
abnormalities were associated with increasing patient age (P = 0.02-0.0001).
CONCLUSION: This study considerably expands the radiographic morphological 
spectrum of progeria. A better understanding of the radiologic abnormalities 
associated with progeria and improved understanding of the biology of progerin 
(the molecule responsible for this disease), will improve our ability to treat 
the spectrum of bony abnormalities.