title: Bone mineralization in polyostotic fibrous dysplasia: histomorphometric analysis.
abstract: Fibrous dysplasia (FD) of bone can be complicated by renal phosphate wasting. The effect of hypophosphatemia on normal and dysplastic bone of FD patients has not been well characterized. In this study, we compared serum phosphorus (sPi) levels to histomorphometric findings in 27 iliac bone samples from 23 children and adolescents (aged 4.2-16.4 years) with polyostotic FD. The samples were separated into two groups, based on the presence (n = 10) or absence (n = 17) of a dysplastic lesion within the specimen. Histomorphometric results were compared with those from 18 age-matched control subjects without metabolic bone disease. In dysplastic lesions, trabeculae were clearly thinner and increased in number. Osteoid indices, osteoblast surface per bone surface, and mineralization lag time were elevated in dysplastic areas, but there was no detectable effect of sPi concentrations on these indices. In nondysplastic bone tissue, low sPi levels were associated with mildly increased osteoid thickness and prolonged mineralization lag time. None of the 13 patients in whom hand X-rays were available at the time of biopsy had radiological signs of rickets. In conclusion, low sPi can cause a mild systemic mineralization defect in FD, but the more severe mineralization defect seen in dysplastic lesions is independent of sPi levels. It is debatable whether the mild systemic mineralization defect warrants treatment with oral phosphorus supplementation if signs of rickets are absent.
authors.affiliation: Genetics Unit, Shriners Hospital for Children and McGill University, Montréal, Québec, Canada.
authors.forename: Leonieke
authors.initials: L
authors.lastname: Terpstra
authors.forename: Frank
authors.initials: F
authors.lastname: Rauch
authors.forename: Horacio
authors.initials: H
authors.lastname: Plotkin
authors.forename: Rose
authors.initials: R
authors.lastname: Travers
authors.forename: Francis H
authors.initials: FH
authors.lastname: Glorieux
mesh_terms: D000293:Adolescent; D001706:Biopsy; D001842:Bone and Bones / Q000033:anatomy & histology / Q000473:pathology; D002113:Calcification, Physiologic; D002118:Calcium / Q000097:blood; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D006225:Hand / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D010281:Parathyroid Hormone / Q000097:blood; D010758:Phosphorus / Q000097:blood; D011859:Radiography
chemical_list: D010281:Parathyroid Hormone; D010758:Phosphorus; D002118:Calcium