1 40 1 Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. Citation List Month Backlog URL Address <a href="http://doi.org/10.1097/01.bpb.0000049567.52224.fa" target="_blank" rel="noreferrer">http://doi.org/10.1097/01.bpb.0000049567.52224.fa</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Modern approach to children with osteogenesis imperfecta Publisher An entity responsible for making the resource available Journal Of Pediatric Orthopaedics. Part B / European Paediatric Orthopaedic Society, Pediatric Orthopaedic Society Of North America Date A point or period of time associated with an event in the lifecycle of the resource 2003 Subject The topic of the resource Child; Female; Humans; Male; Prognosis; Treatment Outcome; Severity of Illness Index; Risk Assessment; Biopsy; Combined Modality Therapy; Preschool; infant; Needle; Clodronate; Diphosphonates/therapeutic use; Immunohistochemistry; Bone Density/physiology; Physical Therapy Modalities; Orthopedic Fixation Devices; Orthopedic Procedures/instrumentation/methods; Osteogenesis Imperfecta/pathology/therapy Creator An entity primarily responsible for making the resource Zeitlin L; Fassier F; Glorieux FH Description An account of the resource Osteogenesis Imperfecta (OI) is characterized by bone fragility. At least seven discrete types have been described ranging from mild disease to a lethal form. In a large number of cases, mutations in one of the two genes encoding type I collagen have been found. In forms recently described (types V, VI, VII), such mutations have been excluded. In two other forms, (Bruck, and osteoporosis - pseudoglioma syndromes) defects in other proteins have been characterized. In OI, bone fragility stems from: decreased bone mass, disturbed organization of bone tissue, and altered bone geometry (size and shape). Histologic studies have shown that increased bone turnover is the rule in OI bone. This justifies using bisphosphonates in order to reduce osteoclast mediated bone resorption. Initial results are encouraging. Cyclical intravenous pamidronate administration reduces bone pain and fracture incidence, and increases bone density and level of ambulation, with minimal side effects. Effects on bone include increase in size of vertebral bodies and thickening of cortical bone. These results allow for more efficacious corrective surgery using intramedullary rodding of the long bones and paravertegral instrumentation. Specific occupational and physiotherapy programs are integral parts of the treatment protocol. This multidisciplinary approach will prevail until strategies aiming at the correction of the basic defect(s) will have come to fruition. 2003 Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1097/01.bpb.0000049567.52224.fa" target="_blank" rel="noreferrer">10.1097/01.bpb.0000049567.52224.fa</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). Type The nature or genre of the resource Journal Article 2003 Backlog Biopsy Bone Density/physiology Child Clodronate Combined Modality Therapy Diphosphonates/therapeutic use Fassier F Female Glorieux FH Humans Immunohistochemistry Infant Journal Article Journal Of Pediatric Orthopaedics. Part B / European Paediatric Orthopaedic Society, Pediatric Orthopaedic Society Of North America Male Needle Orthopedic Fixation Devices Orthopedic Procedures/instrumentation/methods Osteogenesis Imperfecta/pathology/therapy Physical Therapy Modalities Preschool Prognosis Risk Assessment Severity Of Illness Index Treatment Outcome Zeitlin L