Bone Abstracts

Intravenous (IV) Pamidronate (PAM) has been used in the treatment of Osteogenesis Imperfecta (OI) and is known to increase bone mineral density (BMD) and reduce the incidence of fractures. However an attractive alternative is the more potent IV Zoledronic acid (ZOL).Objectives: To determine the clinical efficacy of IV PAM vs ZOL in children with mild to moderate OI and compare the cost benefits of the two drugs.Methods: A retrospec...

Introduction: Although previously babies with genetic type II Osteogenesis Imperfecta (OI) would not have expected to survive, they are now surviving beyond the neonatal period. We describe two such children who have survived beyond infancy.Aim & methods: To identify differences in motor developmental progress between a typical severe (type III) OI child vs two Type II OI children and suggest possible causes. Medical, nursing and therapy (physiothera...

Objectives: To ascertain if parents and consultants at secondary care level hospitals felt there were areas that the Clinical Nurse Specialists (CNS), working with children and families with Osteogenesis Imperfecta (OI) could improve upon within their service, and to gain feedback on the current service provided.Methods: A SurveyMonkey© questionnaire was created through the audit team at Great Ormond Street Hospital and the Paediatric Ost...

Background: DMD is a progressive neuromuscular disease often treated with oral glucocorticoids (GC) to prolong ambulation and maintain cardiovascular function. However, the osteotoxic GC effects often result in a skeleton prone to fractures. DMD boys presenting with fractures are treated with bisphosphonates (BP) but evidence of beneficial effects is limited. The aim of this work was to review the use of intravenous BPs in a group of severely affected boys with fractures.<...

Background: Osteoporosis in children with osteogenesis imperfecta (OI) type 1 and acute lymphoblastic leukaemia (ALL) is characterised by high bone turnover. However the ability of spontaneous healing and reshaping of bone is retained in ALL even in the absence of bisphosphonate (BP) therapy, but impaired in OI.Aim: To compare the response to BP therapy in children with ALL and OI.Methods: Retrospective case note review of children...