Searchable abstracts of presentations at key conferences on calcified tissues

ba0004p153 | (1) | ICCBH2015

In-vivo high-resolution peripheral quantitative computer tomography assessment of skeletal microstructure in children with osteogenesis imperfecta

Digby Maria , Dimitri Paul , Arundel Paul , Bishop Nick , Paggiosi Margaret , Offiah Amaka

Objectives: The clinical assessment of bone mass in children with osteogenesis imperfecta (OI) is normally assessed by DXA. Further information about bone microarchitecture is obtained from bone biopsy which is invasive and requires anaesthetic. High-resolution peripheral quantitative computer tomography (HRpQCT) is an in-vivo imaging modality capable of assessing skeletal microstructure and integrity to a resolution of 80 μm. To date, no HRpQCT studies have incl...

ba0004p92 | (1) | ICCBH2015

Risedronate in the treatment of children with osteogenesis imperfecta: retrospective review of practice and outcomes at a large paediatric metabolic bone unit

Lawrence Neil , Bishop Nick , Arundel Paul

Objectives and method: We retrospectively reviewed records of current patients with osteogenesis imperfecta (OI) to identify those on risedronate, doses used and effects of treatment. BMD changes over time were calculated as difference between measurement before risedronate and at one year (including measurements −3–0 m and 10–14 m after starting risedronate respectively).Results: 219/296 (74%) patients with OI had received bisphosphonate...

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...

ba0002p118 | (1) | ICCBH2013

Variation in response to vitamin D therapy in a series of consecutive children referred to a paediatric bone disease service

Arshad Fawaz , Hinton Sally , Bishop Nick , Arundel Paul

Objectives: Guidelines for treatment of vitamin D deficiency (VDD) vary. We aimed to review the range of treatment regimens for VDD used locally and variation in responses.Methods: We conducted a retrospective review of the records of consecutive patients referred to a Children’s Bone Disease Service with a putative diagnosis of VDD over a 14-month period. Data collected includes vitamin D type used, dose and duration of treatment, and pre- and post...

ba0002p121 | (1) | ICCBH2013

Tibial metaphyseal shape varies between children according to history of fracture

Arundel Paul , Hangartner Thomas , Short David , Holden Ben , Bishop Nick

Background: Measurement and interpretation of metaphyseal shape in children is difficult. We aim to develop relevant assessment methods using commonly available spiral computed tomography (CT) scanning.Methods: We analysed 12 pairs of age and pubertal stage matched subjects from a larger group of children recruited into a non-interventional case–control study in which all had suffered trauma; half had sustained a fracture. Each subject underwent ant...

ba0002p166 | (1) | ICCBH2013

Severe metabolic bone disease of prematurity following continuous veno–veno haemofiltration

Elder Charlotte , Arundel Paul , Perring Jeff , Bishop Nick

A first twin born at 28 weeks gestation weighing 630 g underwent an end-to-end anastamosis for colonic stricture on day 92 of life. He collapsed with severe Escherichia coli sepsis post-operatively and became anuric. Veno–veno haemofiltration (CVVH) was instituted as a life-preserving measure, continuing for 3 days.On day 119, osteopaenia and rachitic changes were noted on a chest X-ray. Review of his prior biochemistry showed a precipitous...

ba0004p88 | (1) | ICCBH2015

Vitamin D and bone health: A practical clinical guideline for management in children and young people in the UK

Thurston Anne , Bowring Claire , Shaw Nick , Arundel Paul

Background: There is currently considerable clinical and academic interest in vitamin D in children and young people. This partly relates to recognition of a resurgence of symptomatic vitamin D deficiency with reports of children presenting with rickets or hypocalcaemic symptoms. An additional development has been the recognition that vitamin D may have a physiological extraskeletal role beyond its traditional function as a key regulator of calcium and bone metabolism.<p c...

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...

ba0006p177 | (1) | ICCBH2017

The elbow in type V osteogenesis imperfecta: is early functional loss related to radiographic findings?

Hill Claire , Offiah Amaka , Bishop Nick , Arundel Paul

Objectives: Type V osteogenesis imperfecta (OI) results in abnormal modelling of the ulna, dislocation of the radial head and interosseous membrane calcification (IOM). Individuals develop reduced functional ability as a consequence of reduced range of movement (ROM) including elbow flexion and/or supination, which may be intrinsic or secondary to the radiographic findings. We describe the evolution of radiographic and functional parameters in a cohort seen in our centre.<...

ba0007p10 | (1) | ICCBH2019

Radiographic evidence of zoledronic acid given during pregnancy – a case report

Peacock Amanda , Offiah AC , Balasubramanian Meena , Bishop Nick , Arundel Paul

Background: A 3.2 year old boy was reviewed following 2 low trauma femoral fractures. He had been born at 31 weeks gestation, his mother having been diagnosed with metastatic breast cancer at 12 weeks gestation. She received chemotherapy at 20 weeks gestation and was given intravenous zoledronic acid (ZA) during the second trimester for hypercalcaemia.Presenting problem: At 2.7 years, he fell on a tarmacked surface sustaining a diaphyseal fracture of his...