Bone Abstracts

Osteonecrosis of the jaw (ONJ) has been recently described after i.v. administration of amino-bisphosphonates and – less frequently – in association with the use of oral bisphosphonates. Bisphosphonate-related osteonecrosis of the jaw (BRONJ) may affect mandible bone (65%), maxilla bone (26%) and rarely (9%) both sites simultaneously. Although causality may never be proven, emerging experimental data have established a strong association between monthly i.v. bisphosp...

Objective: In 2018 we started treating children with X-linked hypophosphataemic rickets (XLH) with burosumab, initially as part of an industry-sponsored early access program. We present what we did, the barriers to implementation and broader lessons for the introduction of treatments for rare bone diseases in the future.Method: Firstly, we identified suitable patients under the age of 18 years with XLH. For convenience we arranged dedicated clinics in wh...

A hallmark of Multiple Myeloma is skeletal colonization resulting in painful osteolytic lesions. Matrix metalloproteinase-13 (MMP-13) is a type-1 collagenase largely expressed by mesenchymal stromal cell (MSCs) in the bone tissue. In agreement, immunohistochemical staining of human myeloma biopsies demonstrated the localization of MMP-13 to the stromal compartment with expression in a subset of myeloma cells. This observation was further supported by studies showing that co-cu...

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...

The aim of management is to normalize serum calcium and reduce PTH levels, leading to improvement in any associated symptoms. Parathyroidectomy (PTx) is the only curative treatment and should be recommended for PHPT patients with symptomatic disease, but also considered in asymptomatic patients who meet surgical criteria defined by international guidelines. Studies of the natural history of asymptomatic PHPT indicate that in the absence of PTx some patients show stability in s...

We have previously shown that female neuronal nitric oxide synthase knockout (nNOS KO) mice have increased trabecular bone volume. However, this study was performed in mice at 10 weeks of age only. To investigate whether the high bone volume is maintained during ageing, we compared 3-month- and 12-month-old wild type (WT) and nNOS KO mice using μCT. The tibias from 8 WT and 8 nNOS KO mice at each age were dissected, fixed for 24 h in buffered formalin, stored in 70% ethan...

The microenvironment of osteolytic metastases includes carcinoma cells derived from the primary lesion as well as bone-forming and bone-resorbing cells, namely osteoblasts (OB) and osteoclasts (OC). At this site, both the high glycolysis of cancer cells, and the bone resorption process result in a very acidic milieu. This, in turn, induces the surrounding stroma and OB to secrete pro-inflammatory cytokines and growth factors that promote tumorigenesis and cancer-associated ost...

An increasing amount of scientific evidence suggests that western diet is a risk factor for osteopenia and osteoporosis. Indeed, metabolic acidosis, occurring after high levels of protein intake, may adversely impact on the skeleton by disrupting calcium metabolism, and leads to a decrease of bone mineral density (BMD). Currently, the prevention and treatment of osteopenia and osteoporosis are mainly based on lifestyle modifications (i.e. exercise, quitting smoking and ensurin...

Objectives: From late 2015 a new protocol for zoledronic acid was adopted in our centre. This led to many children changing from pamidronate (PAM) to zoledronic acid (ZA) treatment. In a minority of cases the children and/or their families felt strongly that they wanted to change back to PAM. We present the characteristics of that minority and how bone turnover markers (BTMs) and bone mineral densities (BMD) changed whilst on ZA.Method: From Nov 2016 to ...

Bone metastasis (BM) is a dismal complication of cancer, occurring frequently in patients with advanced breast carcinoma. During metastatic progression, carcinoma cells harness osteoclast (OC) activity, promoting osteolysis. To adapt to hypoxia and/or to support proliferation, carcinoma cells adopt primarily glycolysis for energy production, therefore releasing lactic acid in the microenvironment through monocarboxylate transporter 4 (MCT4). Stressed by tumor cells, osteoblast...