Background Osteogenesis imperfecta is caused by a genetic defect resulting in an abnormal
type I collagen bone matrix which typically results in multiple fractures with little or no …

The extracellular matrix (ECM) of bone and dentin contains several non-collagenous
proteins. One category of non-collagenous protein is termed the SIBLING (S mall I ntegrin-B …

As one of the most important natural materials, cortical bone is a composite material
comprising assemblies of tropocollagen molecules and nanoscale hydroxyapatite mineral …

Although disorders of the skeleton are individually rare, they are of clinical relevance
because of their overall frequency. Many attempts have been made in the past to identify …

Infantile cortical hyperostosis (Caffey disease) is characterized by spontaneous episodes of
subperiosteal new bone formation along 1 or more bones commencing within the first 5 …

The multiscale hierarchical structure of bone is naturally optimized to resist fractures. In
osteogenesis imperfecta, or brittle bone disease, genetic mutations affect the quality and/or …

Osteogenesis imperfecta is an inherited disorder characterized by increased bone fragility,
fractures, and osteoporosis, and most cases are caused by mutations affecting the type I …

Disorders of Connective Tissue Page 1 CHAPTER 45 Disorders of Connective Tissue NP
Burrows1 & CR Lovell2 1Department of Dermatology, Addenbrooke’s Hospital, Cambridge …

Abstract Background/Aims: To systematically assess contemporary knowledge regarding the
effectiveness and safety of bisphosphonates (BPs) in children with osteogenesis imperfecta …

Following on from the highly successful first edition, published in 2006, the second edition of
Basic Orthopaedic Sciences has been fully updated and revised, with every chapter …