Searchable abstracts of presentations at key conferences on calcified tissues

ba0002oc22 | Miscellaneous | ICCBH2013

Prolidase deficient mice are osteoporotic in early life

Foster Sarah , Grabowski Peter , Gallagher Orla , Besio Roberta , Rossi Antonio , Bishop Nick , Forlino Antonella

Background: Proline and hydroxyproline account for ~25% of aminoacids in collagen., Prolidase (peptidase D (EC 3.4.14.9)), cleaves iminodipeptides with a C-terminal proline or hydroxyproline, playing a major role in collagen catabolism. Mice with prolidase deficiency (PD) present with varied phenotypes including reduced size compared to wild-type littermates. We measured structural and mechanical properties of bones in PD mice.Methods: Whole femurs from ...

ba0006pl1 | (1) | ICCBH2017

Poster List

Poster No. Category Title Author P002 Bone development A 3-year longitudinal study of skeletal effects and growth in children after kidney transplantation <a href="ba...

ba0004p152 | (1) | ICCBH2015

Syphilitic bone disease: a case report

Sukthankar Shaila , Sukthankar Ashish , Wright Neville , Foster Anne , Mughal Zulf

Background: Syphilis remains a great imitator of myriad of clinical diagnosis. Congenital syphilis, though still uncommon, is on the rise once again. Recognition of syphilitic bone disease and its potential impact on growth is important for long term prognosis. We describe our experience of managing a child with congenital syphilis and multisite osteolytic lesions.Presenting problem: A 6 week-old girl presented with a swollen and painful elbow with reduc...

ba0006p088 | (1) | ICCBH2017

Normative data for lateral distal femur bone mineral density in children from 3 to 18 years of age using Lunar Prodigy absorptiometry

Fiscaletti Melissa , Rauch Frank , Foster Bethany , Alos Nathalie

Objectives: Children with compromised weight bearing and limited mobility are particularly at risk for fractures with minimal trauma. Unfortunately, they can also present several obstacles that can impede proper dual X-ray absorptiometry (DXA) scan assessment of whole body or spine bone mineral density (BMD). Since the distal femur is the most common site of fracture in those patients, the lateral distal femur (LDF) represents the site that best addresses the challenges of BMD...

ba0006p175 | (1) | ICCBH2017

Management of Gorham disease in the cervicothoracic spine with mobile gravity traction and Sirolimus

Foster Paul , Mughal M. Zulf , Leong Julian , Jacobs Benjamin

Background: Gorham Disease is a rare condition characterised by massive osteolysis. The pathophysiology is related to angio/lymphatic proliferation within bone. No genetic transmission has been identified and onset occurs in patients of all ages. Surgical fixation of the spine may be unsuccessful due to progressive osteolysis of bone surrounding the metalwork, or of the bone graft.Presenting problem: An 11 year-old boy presented with a 2 year history of ...

ba0004p178 | (1) | ICCBH2015

The precision of partial image analysis of trabecular bone microarchitecture by high-resolution magnetic resonance imaging in people with childhood-onset bone abnormalities

Chen Suet Ching , Abdalrahaman Naiemh , McComb Christie , Foster John , Ahmed Syed Faisal

Background and objective: High-resolution magnetic resonance imaging (hrMRI) can assess trabecular bone microarchitecture but the number of image slices required for reliable assessment is unclear.Methods: MRI was performed just below the growth plate of the proximal tibia from 20 healthy controls (all female; median age 21 years (range 18–35) and 10 cases (three mals: seven females; median age 19.5 years (range 16–48) with known bone abnormali...

ba0007oc20 | (1) | ICCBH2019

Identifying the role of NBAS in bone fragility using zebrafish and exploring therapeutic targets to reverse NBAS activity

Balasubramanian Meena , Baxendale Sarah , Roehl Henry

Background: We discovered that variants in NBAS (Neuroblastoma Amplified Sequence Gene) known to be associated with acute liver failure are also responsible for skeletal abnormalities. This work was published as a novel cause of bone fragility [Balasubramanian et al., 2017]. NBAS role in bone fragility provides an opportunity to use tractable animal research to advance understanding of mechanism and identify potential new treatments. This would be beneficial to patien...

ba0007oc8 | (1) | ICCBH2019

Bisphosphonate improves hip range of motion and pain but not femoral head sphericity: A multicentre, randomized clinical trial of children with Perthes disease

Jamil Kamal , Zacharin Margaret , Foster Bruce , Donald Geoffrey , Hassall Timothy , Siafarikas Aris , Johnson Michael , Tham Elanie , Whitehead Colin , Gebski Val , Barnes Liz , Cowell Chris , Little David , Munns Craig

Introduction: Perthes disease (PD), idiopathic femoral head avascular necrosis, often results in deformity. The underlying cause is unclear and long-term function is directly related to the roundness of femoral head. Current treatment include mechanical treatments and various surgical procedures, which are therapeutic but can’t prevent collapse. A multicentre, prospective, randomised controlled trial of 12 months zoledronic acid (ZA) in children with PD was conducted. We ...

ba0004p112 | (1) | ICCBH2015

Type V osteogenesis imperfecta: confirmation of highly characteristic radiographic findings in early infancy

Arundel Paul , Offiah Amaka , Bishop Nick , Ehtisham Sarah

Type V OI is characterised by interosseous membrane calcification and hyperplastic callus formation, but the infantile phenotype is less well recognised. In 2012 Arundel et al. described distinctive radiographic changes in an infant with type V OI. We report two further male infants (with genetic confirmation of type V OI) confirming the highly characteristic and consistent radiographic appearances that should aid early diagnosis.Case 1 – P...

ba0004p170 | (1) | ICCBH2015

Reduced spinal volumetric trabecular bone mineral density in adolescent girls with anorexia nervosa

Ehtisham Sarah , Whittaker Jane , Adams Judith , Mughal Zulf

Background: Anorexia nervosa (AN) presenting in childhood can have devastating implications for growth, puberty, menstruation and bone health. It may lead to altered bone structure and inadequate acquisition of bone mass with increased risk of fracture.Objective: To describe growth, pubertal and bone mineral density data in a cohort of adolescents with AN managed in a tertiary metabolic bone service.Methods: 62 adolescent females w...