Bone Abstracts

Objectives: We evaluated the efficacy and safety of intravenous zoledronic acid (IV ZA) in children with glucocorticoid-induced osteoporosis (GIOP) through a randomized, placebo (PBO)-controlled trial.Methods: In this multi-national Phase 3 trial (NCT00799266), children 5–17 years of age with GIOP and low-trauma vertebral fractures (VF) were randomized 1:1 to IV ZA 0.05 mg/kg or IV PBO every six months for one year. Changes in lumbar spine areal bon...

Objective: We compared the efficacy and safety of burosumab, a monoclonal antibody against FGF23, to conventional therapy [oral phosphate and active vitamin D (Pi/D)] in children with X-linked hypophosphatemia (XLH).Methods: In this Phase 3 trial (NCT02915705), 61 children with XLH (1-12 years-old) were randomized 1:1 after a 7-day Pi/D washout to receive burosumab starting at 0.8 mg/kg SC Q2W or reinitiate Pi/D optimally titrated by investigators. Eligi...

Background: Osteogenesis imperfecta (OI), the commonest inherited bone fragility disorder, affects 1/15,000 live births resulting in frequent fractures and reduced mobility, with significant impact on quality of life. Early diagnosis is important, as therapeutic advances can lead to improved clinical outcomes.Methodology and results: Trio whole exome sequencing in patients with OI identified, in two patients, compound heterozygous mutations in NBAS</...

The objective of this study was to analyze measurement properties of BeamMed Omnisense quantitative ultrasound (QUS) of the radial and tibial speed of sound (SoS) for assessing bone health and screening bone fragility in youth. Bone fragility was defined as low whole body less head bone mineral density (WBLH BMD) measured by DXA (first tertile, 95% CI: −1.1– (−0.9)) and as past history of fractures evaluated by questionnaire. The study was conducted with 319 n...

Aim: To investigate the effect of psychoeducation program in families with osteogenesis imperfecta (OI).Methods: Sixteen family members of OI patients were included in the program. The research was designed as a semi structural, semi experimental pre and post test. The scales used were; Introductory Information Form, Burden Interview (BI), Coping Strategies Scale (CSS), Problem Solving Inventory (PSI), and Psychosocial Adjustment to Illness Scale (PAIS)....

Objective: Idiopathic hypercalciuria (IH) is defined as excessive 24 h urinary calcium excretion (>4 mg/kg per 24 h), that persists after correction of dietary imbalances in the absence of secondary causes. Recent studies with DXA in children with IH provide evidence of decreased areal BMD. We used peripheral quantitative computed tomography (pQCT) of the tibia, to test the hypothesis that IH results in decreases of volumetric (mg/cm3) BMD of the trabecular and/...

Hip fractures are a major cause of burden in terms of mortality, disability, and costs. In Ireland, 3000 hip fracture occur annually and is expected to increase over the coming years1. Estimated cost of hip fractures is &z.euro;14 300/admission2. Outpatient non-attendance is a source of inefficiency, wasting time, resources and lengthens waiting lists. Non attendees have a significant negative impact on productivity, their own care and resources. In 2008,...

We report a male child presenting with antenatally diagnosed bilateral talipes equinovarus, short stature, bilateral cryptorchidism and poor weight gain; born at 39 weeks gestation (birth weight 2.56 kg) to non-consanguineous Caucasian parents. Facial dysmorphism included a prominent forehead, brachycephaly, shallow orbits, a high anterior hairline, a narrow nasal bridge and small mouth with a thin upper lip. He had white sclera and was short for height (−4.4 SDS) with a...

The impact of sex and maturation on trabecular (Tb) and cortical (Ct) microarchitecture in children and young adults has not been well established. The new second-generation high-resolution peripheral quantitative CT (HR-pQCT) scanner (XCT II, Scanco Medical) incorporates three important advances to provide greater spatial resolution, direct measures of Tb thickness and spacing and measures in the Ct midshaft. The aim of this study was to identify sex and maturation effects on...

Objectives: FGF23 is expressed in clusters of osteocytes at the trabecular periphery suggesting that FGF23-expressing osteocytes are confined to specific basic multicellular units (BMUs) at the trabecular surface. Higher numbers of FGF23-expressing osteocytes are found in chronic kidney disease (CKD) patients with preserved skeletal mineralization indices. We thus combined immunohistochemistry and quantitative backscattered electron imaging (qBEI) to explore the hypothesis tha...