Searchable abstracts of presentations at key conferences on calcified tissues

ba0007lb2 | (1) | ICCBH2019

Lumbar spine quantitative computed tomography (QCT) is a better predictor of vertebral fracture in boys with Duchenne muscular dystrophy (DMD) than either DXA or peripheral QCT

Crabtree Nicola , Machin Michael , Padidela Raja , Kariki Eleni , Hughes Imelda , Shaw Nick , Mughal Zulf

Objectives: Vertebral fractures are common in boys with DMD taking daily corticosteroids. Treatment is usually initiated when vertebral fractures have been identified. However, prophylactic treatment may be possible if reliable risk factors for vertebral fracture can be identified. The aim of this work was to compare the diagnostic accuracy of three different bone strength assessment techniques in a cohort of DMD boys.Methods: Thirty-three boys with DMD ...

ba0004p174 | (1) | ICCBH2015

Case report of reversible cardiomyopathy secondary to 1 alpha hydroxylase deficiency

Cheung Moira , Braha Nirit , Beck-Nielsen Signe , Brain Caroline , Allgrove Jeremy

Background: Dilated cardiomyopathy is the leading cardiac cause of death in children. Treatment options include heart transplantation. Reversible causes are rare but hypocalcaemia secondary to vitamin D deficiency is a recognised cause and presents during infancy in at risk populations. Risk factors include babies who were breast fed, dark skinned and from mothers with vitamin D deficiency. We present an unusual case of vitamin D replete, hypocalcaemic cardiomyopathy secondary...

ba0005p389 | Osteoporosis: treatment | ECTS2016

Hypercalcemia after discontinuation of long-term denosumab treatment

Langdahl Bente , Harslof Torben , Kaal Andreas , Rejnmark Lars , Sophie Solling Anne

Purpose: Denosumab is commonly used as an anti-resorptive agent to treat osteoporosis. After discontinuation of denosumab, however, bone resorption increases again, and the bone mass gained during therapy is lost within a year.Methods: We present a case report of asymptomatic hypoparathyroid hypercalcemia in a patient who discontinued long-term treatment with denosumab.Results: A 67-year old women with osteoporosis was treated with...

ba0006p188 | (1) | ICCBH2017

Paradoxical response of serum parathyroid hormone concentration in response to vitamin D and calcium supplementation in undernourished Indian children

Mandlik Rubina , Ekbote Veena , Patwardhan Vivek , Kajale Neha , Khadilkar Vaman , Padidela Raja , Khadilkar Anuradha , Mughal Zulf

Objectives: We have previously described biochemical evidence of resistance to PTH in Indian toddlers, which reversed on calcium supplementation. We performed a post-hoc analysis on data from an RCT of vitamin D and calcium supplementation, which was designed to assess if supplementation would reduce infection rate in undernourished school children with adequate sun exposure. Specifically we investigated the effect of oral vitamin D and calcium supplementation on biochemical p...

ba0007p72 | (1) | ICCBH2019

Burosumab experience in UK X-linked hypophosphataemia children under five years old

Dharmaraj Poonam , Burren Christine , Cheung Moira S , Padidela Raja , Mughal Zulf , Shaw Nick , Saraff Vrinda , Nadar Ruchi , Randell Tabitha , Mushtaq Talat , Ramakrishnan Renuka , Sennipathan Senthil , Sakka Sophia , Bath Louise , Elleri Daniela , Davies Justin H , Barton John , Tucker Ian , Rayner Lauren , Arundel Paul , Gilbey-Cross Robyn , Tothill Alexander M , Philip James , Sawoky Nadine , Connor Paul , Mathieson Leigh

Objectives: X-linked hypophosphataemia (XLH) is a rare inherited form of osteomalacia characterised by low blood phosphate levels which lead to inadequate mineralisation of bone and rickets. Burosumab is an anti-FGF23 fully human monoclonal-antibody, and the first treatment to target the underlying pathophysiology of XLH. Real-world evidence is important in validating the findings of clinical studies. We report relevant real-world biochemical data on children under five years ...

ba0007p73 | (1) | ICCBH2019

Burosumab initiation in a UK X-linked hypophosphataemia cohort: real-world use resonates with research evidence

Dharmaraj Poonam , Burren Christine , Cheung Moira S , Padidela Raja , Mughal Zulf , Shaw Nick , Saraff Vrinda , Nadar Ruchi , Randell Tabitha , Mushtaq Talat , Ramakrishnan Renuka , Sennipathan Senthil , Sakka Sophia , Bath Louise , Elleri Daniela , Davies Justin H , Barton John , Tucker Ian , Rayner Lauren , Arundel Paul , Gilbey-Cross Robyn , Tothill Alexander M , Philip James , Sawoky Nadine , Connor Paul , Mathieson Leigh

Objectives: X-linked hypophosphataemia (XLH) is a rare inherited form of osteomalacia characterised by low blood phosphate levels which lead to inadequate mineralisation of bone resulting in rickets, skeletal abnormalities, physical impairment, weakness, and pain. Burosumab is an anti-FGF23 fully human monoclonal-antibody, and the first treatment to target the underlying pathophysiology of XLH. Real-world evidence is important in validating the findings of clinical studies. We...

ba0002p171 | (1) | ICCBH2013

Vitamin D levels and signs of metabolic bone disease in adolescents with idiopathic scoliosis

Adodra Annika , Kouklinos Andreas , Julies Priscilla , Shaw Mathew , Jacobs Benjamin

Objective: Biochemical assessment of teenage patients with adolescent idiopathic scoliosis (AIS).Methods: Blood sampling from 120 patients with AIS aged 12–17 years. The patients all had significant scoliosis and blood samples were taken a few days or weeks before they underwent scoliosis surgery. Serum 25-hydroxy-vitamin D (25-OHD), calcium, phosphate, alkaline phosphatase and parathyroid hormone (PTH) were measured. Patients with a 25-OHD level be...

ba0003pp232 | Osteoporosis: evaluation and imaging | ECTS2014

Serum creatinine profiles in hip fracture patients, an observational study

Frankland Stuart , Gupta Abhaya

Introduction: Kidney dysfunction is common in the elderly and has been proven to have an association with increased hip fracture incidence. Acute dysfunction may also delay surgery. Serum creatinine, a product of metabolised muscle tissue is a marker used to determine changes in renal function.Objective: To assess the renal function of hip fracture patients throughout their hospital stay using serum creatinine as the marker of renal function.<p class...

ba0006p011 | (1) | ICCBH2017

Neonatal hypocalcemia – transient neonatal pseudohypoparathyroidism

Kutilek Stepan , Vracovska Martina , Pikner Richard , Fejfarkova Zlatka

Background: Neonatal hypocalcemia is defined as S-Ca <2 mmol/l in fullterm newborns and <1.75 mmol/l in preterm newborns. Neonatal hypocalcemia is either early onset (<3 days of age) or late onset (>3 days of age). Newborns with hypocalcemia are often asymptomatic, but mays present with hypotonia, apnea, poor feeding, jitteriness, seizures, cardiac failure. Signs of hypocalcemia rarely occur unless S-Ca drops below 1.75 mmol/l.Case presen...

ba0004p181 | (1) | ICCBH2015

Short stature in osteogenesis imperfecta: consider alternative diagnoses

Steele Caroline , Sultan Wajid , Bishop Nick , Ehtisham Sarah

Background: A 15 year old boy with antenatally diagnosed osteogenesis imperfecta (OI) was reviewed following concerns about poor linear growth, lack of pubertal development and persistent deciduous teeth. He had a history of crush fractures of L1–L2 and previous intra-medullary rodding of his right femur and had been on oral bisphosphonates (Risedronate 70 mg once weekly). He was significantly short (height SDS −4.9), substantially below his genetic potential (targe...