TY - CHAP
T1 - Fibrous Dysplasia
AU - Bianco, Paolo
AU - Wientroub, Shlomo
N1 - Funding Information:
We are indebted to Dr Mara Riminucci (Sapienza University of Rome, Italy) for extensive discussions, critical reading of the manuscript, and liberal sharing of data and observations. We acknowledge insightful discussions with Professor Alan Boyde (University College London), who also generously provided the photographs in Figure 22.11 . His contributions and those of Drs Sergei Kuznetsov, Larry Fisher, Michael T. Collins, and Kenn Holmbeck (NIDCR, NIH NIDCR, NIH) and of Dr Alessandro Corsi, Stefania Piersanti, Isabella Saggio (Sapienza University of Rome) to work mentioned in this chapter are also acknowledged. Published and unpublished personal investigations mentioned in this chapter were supported in part by Telethon Grants E.519 and E.1029, GGP04263 and GGP09227 to PB.
PY - 2012
Y1 - 2012
N2 - Recognition of fibrous dysplasia (FD) as a distinct skeletal disease is commonly attributed to the description of an osteitis fibrosa disseminata occurring in conjunction with various endocrinopathies and skin pigmentation. The bone that is turned over more rapidly in FD is abnormal, qualitatively and quantitatively, in many other critical features ranging from primary modeling to chemical composition, which more directly translate into clinically adverse effects. FD is a disease of excess; abnormal, and imperfect bone growth. The disease produces excess bone growth by causing a localized increase in bone tissue within bone (or local bone mass). The disease causes abnormal bone growth because bone formation does not adhere to the architectural design of the affected, growing bone segments. The territorial definition of cortical bone, cancellous bone, and marrow space is lost, and bone is formed with haphazard trabecular architecture and an irregular internal structure and is mechanically unsound. The disease causes imperfect bone growth because the matrix deposited has an abnormal chemical composition, an abnormal "tricotage," and an abnormal mineral content.
AB - Recognition of fibrous dysplasia (FD) as a distinct skeletal disease is commonly attributed to the description of an osteitis fibrosa disseminata occurring in conjunction with various endocrinopathies and skin pigmentation. The bone that is turned over more rapidly in FD is abnormal, qualitatively and quantitatively, in many other critical features ranging from primary modeling to chemical composition, which more directly translate into clinically adverse effects. FD is a disease of excess; abnormal, and imperfect bone growth. The disease produces excess bone growth by causing a localized increase in bone tissue within bone (or local bone mass). The disease causes abnormal bone growth because bone formation does not adhere to the architectural design of the affected, growing bone segments. The territorial definition of cortical bone, cancellous bone, and marrow space is lost, and bone is formed with haphazard trabecular architecture and an irregular internal structure and is mechanically unsound. The disease causes imperfect bone growth because the matrix deposited has an abnormal chemical composition, an abnormal "tricotage," and an abnormal mineral content.
UR - http://www.scopus.com/inward/record.url?scp=84882900197&partnerID=8YFLogxK
U2 - 10.1016/B978-0-12-382040-2.10022-X
DO - 10.1016/B978-0-12-382040-2.10022-X
M3 - ???researchoutput.researchoutputtypes.contributiontobookanthology.chapter???
AN - SCOPUS:84882900197
SN - 9780123820402
SP - 589
EP - 624
BT - Pediatric Bone
PB - Elsevier Inc.
ER -