Bone Abstracts

The aim of our study was to evaluate the anthropometric characteristics in postmenopausal women depending on appendicular skeletal mass.Materials and methods: We’ve examined 8882 women aged 20–89 years (mean age – 56.7±0.14 years; mean height – 162.5±0.07 cm; mean weight – 73.5±0.16 kg), among them anthropometric measures were performed in 79 postmenopausal women aged 40–82 years (mean age – 63.53±1....

Objectives: Hypophosphatasia (HPP) is a rare metabolic disease caused by loss-of-function mutation(s) in the gene encoding tissue-nonspecific alkaline phosphatase (TNSALP). HPP in infants is characterized by poor skeletal mineralization, respiratory compromise, and a high risk of mortality. We previously reported improved mineralization and respiratory function in 15 patients enrolled in this second study of asfotase alfa, a bone-targeted recombinant human TNSALP, in infants a...

Background: Hypophosphatasia (HPP) is a rare inborn error of metabolism caused by mutations in the gene encoding tissue-nonspecific alkaline phosphatase gene (TNSALP), leading to low alkaline phosphatase (ALP) activity. At least 6 clinical forms of HPP have been reported. Certain benign or asymptomatic presentations of HPP in older children may remain undiagnosed, in contrast to severe perinatal and infantile types. The underlying reason of this diagnostic inconsisten...

We recruited 60 healthy volunteers ages 5 to 21 to perform scan-rescan precision tests on the XtremeCT II. Participants were positioned in a carbon fiber immobilization cast. iPad-based video content was used to facilitate motion-free compliance. Distal radius and tibia scans were acquired starting 2 mm proximal to the proximal margin of the growth plate or growth plate remnant. Diaphyseal radius and tibia scans were centered at an offset from the same landmark, corresponding ...

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...

Objectives: Asfotase alfa (AA), an enzyme replacement therapy, is the only approved treatment for pediatric-onset hypophosphatasia (HPP). We evaluated the safety profile of AA from the clinical trial program spanning pediatric and adult patients.Methods: Safety data were pooled from 4 open-label, multicenter studies in children aged ≤3 years (study 002/003 [NCT00744042/NCT01205152]; n=11) and ≤5 years (study 010-10 [NCT01176266]; <em...

Bone turnover markers (BTMs) are particularly useful in the early monitoring of the effectiveness of anabolic and antiresorptive therapy in osteoporosis and may help in the assessment of treatment compliance. How and when BTM levels change under antiresorptive or anabolic drugs is a key factor in assessing response to therapy. Thus, changes in BTM levels depend on the dose of the drug and the route of administration, and particularly on the mechanism of action. In this sense, ...

Collagen XV is a secreted proteoglycan localized in the outermost layer of the basement membrane and in the fibrillar matrix. Previously, the collagen XV gene (COL15A1) has been linked to osteogenic differentiation, being identified mainly in mature osteoblasts forming new bone tissue or lining bone trabeculae. Our previous data on collagen XV knockout fetuses reports subtle skeletal changes. The aim of this study was to analyse skeletal changes in adult mice lacking collagen ...

Osteoarthritis (OA) and osteoporosis (OP) are two diseases characterized by the alteration of bone quality, that affect mainly elderly people reducing their quality of life. Although an inverse relationship between has been shown by some studies, other reports supported the co-existence of these pathologies. In this study we combined clinical and structural features to clarify the relationship between OA and OP.Among all the patients who underwent a tota...

Purpose: The purpose of this study was to scan femoral heads from cadaveric donors and investigate the microarchitecture within each femoral head comprehensively.Material and methods: Ten proximal femora was harvested from eight human cadaveric donors and these specimens were scanned using micro-computed tomography. Reconstructed batches of images were aligned along the main trabecular direction (MTD). The upper hemisphere of each femoral head was includ...