Anthropometry of patients with osteogenesis imperfecta

Citation
Am. Lund et al., Anthropometry of patients with osteogenesis imperfecta, ARCH DIS CH, 80(6), 1999, pp. 524-528
Citations number
24
Categorie Soggetti
Pediatrics,"Medical Research General Topics
Journal title
ARCHIVES OF DISEASE IN CHILDHOOD
ISSN journal
00039888 → ACNP
Volume
80
Issue
6
Year of publication
1999
Pages
524 - 528
Database
ISI
SICI code
0003-9888(199906)80:6<524:AOPWOI>2.0.ZU;2-P
Abstract
standing height Standing height, sitting height, armspan, subischial leg le ngth, head circumference, and growth hormone-insulin-like growth factor I ( IGF-T) axis were determined in 86 patients with osteogenesis imperfecta, Th e aim of this study was to determine standing height and body proportions a nd their variability among osteogenesis imperfecta types and collagen defec ts. Mean standing height was reduced in all groups of patients, to the grea test extent and variability in osteogenesis imperfecta type III/IV and in t hose with qualitative collagen defects. The mean standing height of patient s with osteogenesis imperfecta was lower than that of their unaffected firs t degree family members. Truncal height of patients with osteogenesis imper fecta was reduced; head size was increased, and this was more pronounced in patients with osteogenesis imperfecta type and qualitative collagen defect s than in patients with osteogenesis imperfecta type I and quantitative col lagen defects, Mean concentrations of IGF-I and IGF binding protein 3 (IGFB P-3) were low, but most values were within age specific referencevalues, Th e reduction of appears to correlate with osteogenesis imperfecta type and t he type of collagen defect. A relatively short trunk is typical and head ci rcumference and body length are disproportionate.