Searchable abstracts of presentations at key conferences on calcified tissues

ba0005p409 | Osteoporosis: treatment | ECTS2016

Atypical femur fracture in an adolescent with X-linked osteoporosis based on PLS3 mutation

van de Laarschot Denise M , Zillikens M Carola

Background: Long-term use of bisphosphonates has raised concerns about the association with Atypical Femur Fractures (AFFs) that have been reported mainly in postmenopausal women.Clinical case: An 18-year-old patient with juvenile osteoporosis based on X-linked osteoporosis due to a PLS3 mutation developed a low trauma femoral fracture after seven years of intravenous and two years of oral bisphosphonate use, fulfilling the revised ASBMR diagnostic crite...

ba0005p468 | Other diseases of bone and mineral metabolism | ECTS2016

In vitro model of antioxidant prevention of urolithiasis

Kizivat Tomislav , Smolic Martina , Bilic-Curcic Ines , Smolic Robert , Maric Ivana , Roguljic Hrvoje , Tolusic-Levak Maja , Kuna Lucija , Vcev Aleksandar , Tucak Antun

Urolithiasis is characterized by formation and retention of solid crystals within the urinary tract. There are numerous causes that may lead to urinary stone formation. However, kidney stones are mostly composed of calcium oxalate that predominantly generates free radicals that are toxic to renal tubular cells.The aim of the study is to explore the toxic effect of oxalate to renal epithelial cells and to explore possible effects of antioxidants on its in...

ba0005p470 | Other diseases of bone and mineral metabolism | ECTS2016

Dose response radiation induced bone loss: FDG PET-CT shows a threshold effect in changes in metabolism and density

Oz Orhan K , Ramezani Saleh , Cuberovic Ivana , Anderson Jon , Brown Keenan , Medin Paul

Radiosurgery of the spine is used to treat patients with spinal metastases. Recent studies have shown increased fracture risk in patients treated with high doses of irradiation. This study tested if there is a dose response relationship between prescribed radiation and induced bone loss and metabolism.Methods: Vertebral bodies C5–C7 of ~50 week old female Yucatan minipigs were irradiated at 16, 20, and 24Gy (n=5/dose). The cervical spine re...

ba0005p487 | Preclinical and ex vivo imaging | ECTS2016

The CAM assay for human bone regeneration evaluation: the potential of Laponite® clay gel for growth factor delivery ex vivo

Moreno-Jimenez Ines , Hulsart-Billstrom Gry , Lanham Stuart A , Kanczler Janos M , Evans Nicholas D , Oreffo Richard O C

An increasing number of biomaterials are in development, seeking to mimic the natural cascade of events during fracture repair. However, these biomaterials need to be rigorously tested prior to clinical application. In vitro testing lacks the physiological environment, while in vivo studies do not always predict the patient response. Here we hypothesize that the chorioallantoic membrane (CAM) assay can be used to culture human living bone and we aim to examin...

ba0006p209 | (1) | ICCBH2017

Intensive therapy – a week of multi-disciplinary intervention at Sheffield Children's Hospital: An example of goal setting and positive outcomes

Knowles Elizabeth

Patients with moderate to severe osteogenesis imperfecta commonly have functional difficulties following fractures or surgery, which generally result in long periods of immobility. Patients quickly lose range of movement, muscle strength and conditioning, which impacts on daily living activities, mobility, self-confidence and motivation. It is natural for patients to become more dependent on their carers, some not returning to their previous level of independence, nor achievin...

ba0006lb15 | (1) | ICCBH2017

Consensus paper – Physiotherapy in children with OI

Semler Oliver , Mueller Brigitte , Mekking Dagmar

Physiotherapy is one of the most important therapeutic approaches in Osteogenesis imperfecta (OI) besides medical and surgical treatment. At the moment there are no guidelines and no consensus about appropriate physiotherapeutic concepts for children with OI. In each country different preferences regarding the therapeutic approaches (neuro developmental techniques, active and passive training, treadmill training, pool therapy etc) are used. There are hardly any scientific rese...

ba0006lb21 | (1) | ICCBH2017

Morbid obesity and respiratory failure in a child with pseudohypoparathyroidism type 1A

Gal Moran , Sarouk Ifat , Levy-Shraga Yael

Background: Pseudohypoparathyroidism type 1A (PHP1A) is a rare genetic disorder caused by mutations in the gene GNAS. It is characterized by multi-hormone resistance, obesity, cognitive impairment and the Albright hereditary osteodystrophy phenotype. A recent study found a 4.4-fold increase risk of sleep apnea in children with PHP1A compared with similarly obese children.Objective: To describe a case of morbid obesity and respiratory failure in ...

ba0007p69 | (1) | ICCBH2019

Experience of implementation and monitoring of burosumab treatment in a multi-disciplinary setting

Marr Caroline , Greenacre Gemma , Arundel Paul

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...

ba0007p200 | (1) | ICCBH2019

Whole body vibration training for children and adolescents with congenital myopathy

Hutchence Meghan , Pacey Verity , North Kathryn , Clarke Nigel , Rose Kristy , Biggin Andrew , Briody Julie , Munns Craig

Objectives: To evaluate the effect of 24 weeks of weight bearing vibration therapy (WBVT) on muscle strength, motor function and bone health in children with congenital myopathies.Methods: A prospective pilot study incorporating a six month observational period followed by 6 months home-based WBVT (Galileo® Pro) was undertaken. Ambulant children with congenital myopathies aged 4–16 years were eligible for inclusion. Participants were assessed a...

ba0001pp427 | Osteoporosis: treatment | ECTS2013

Risk factors for the development of vertebral fractures after percutaneous vertebroplasty

Martinez-Ferrer Angels , Blasco Jordi , Gifre Laia , Monegal Ana , Guanabens Nuria , Peris Pilar

We recently observed an increased risk for vertebral fractures (VF) in a randomized controlled trial comparing the analgesic effect of vertebroplasty (VP) vs conservative treatment (CT) in symptomatic VF. The aim of the present study was to evaluate the risk factors related to the development of VF after VP in these patients.Methods and results: We evaluated risk factors including age, gender, bone mineral density, the number, type and severity of verteb...