Searchable abstracts of presentations at key conferences on calcified tissues

ba0006p141 | (1) | ICCBH2017

Unique correlation pattern between cortical trabecular bone qualities and standard dynamometer handgrip strength in girls with adolescent idiopathic scoliosis (AIS)?

Tam Elisa M S , Cheuk Ka-Yee , Hung Vivian W Y , Yu Fiona W P , Ng Bobby K W , Guo X Edward , Cheng Jack C Y , Lam Tsz-Ping

Objective: Grip strength is a marker of muscle mass which can optimize bone strength during puberty. While previous studies have shown AIS girls had poor bone qualities and mechanical properties when compared with non-AIS girls, the correlation between bone qualities and handgrip strength in AIS remains undefined. This study aimed to investigate the correlation between handgrip strength and bone qualities including volumetric bone mineral density (vBMD), bone geometry, trabecu...

ba0002p87 | (1) | ICCBH2013

Serum homocysteine levels in children and adolescents with impaired bone health

Kutilek Stepan , Skalova Sylva , Rehackova Petra

Background: Association between high serum homocysteine (S-Hcy) levels and low bone mineral density (BMD) and increased fracture risk in postmenopausal women has been repeatedly documented. There are scarce data concerning S-Hcy and bone health in children and adolescents.Patients and methods: We assessed S-Hcy levels in 37 children and adolescents (22 boys and 15 girls; mean age 13.9±3.5 years) with prevalent low-energy trauma fractures (mean 3.3&#...

ba0004p38 | (1) | ICCBH2015

Serum 25-vitamin D level is lower in African American compared to Caucasian children

Srivastava Tarak , Sebestyen Judith , Alon Uri

Background and Objective: Serum 25-Vitamin D level (S-VitD) was recently reported to be significantly lower in African American (AA) adults compared with their Caucasian (CS) counterparts, despite having a higher bone mineral density, due to lower serum vitamin D-binding protein (NEJM 2013; 369: 1991). The objective of this study was to examine if this observation holds true in Hispanic and AA children.Material and methods: The database of clinical labor...

ba0003pp297 | Osteoporosis: treatment | ECTS2014

Contribution of circulating sclerostin and estradiol for inadequate response to bisphosphonate therapy in women with postmenopausal osteoporosis

Munoz-Torres M , Diez-Perez A , Olmos J M , Nogues X , Sosa M , Diaz-Curiel M , Perez-Castrillon J L , Perez-Cano R , Torrijos A , Jodar E , Rio L Del , Caeiro-Rey J R , Rubio V Avila , Garcia-Martin A , Reyes-Garcia R , Garcia-Fontana B , Gonzalez-Macias J , Morales-Santana S

Bisphosphonate treatment reduces fracture risk in women with postmenopausal osteoporosis. However, some patients have an inadequate response to treatment. Estradiol and sclerostin play an important role in bone metabolism. Sclerostin is an endogenous inhibitor of osteoblastic activity and estrogen deficiency increases osteoclast activity and bone resorption.We examined the influence of both measures on fracture incidence in postmenopausal osteoporosis in...

ba0007p46 | (1) | ICCBH2019

Vertebral fractures are more prevalent than long bone fractures in boys with glucocorticoid-treated Duchenne Muscular Dystrophy: Results of a prospective observational study

Jackowski Stefan A , McAdam Laura C , Ma Jinhui , McMillan Hugh J , Konji Victor N , Matzinger Mary Ann , Shenouda Nazih , Jaremko Jacob L , Koujok Khaldoun , Khan Nasrin , Khatchadourian Karine , Robinson Marie-Eve , MacLeay Lynn , Saleh David S , Siminoski Kerry , Rauch Frank , Ward Leanne M

Objectives: Osteoporosis is a frequent cause of morbidity in boys with glucocorticoid (GC)-treated Duchenne Muscular Dystrophy (DMD). We sought to determine the frequency and characteristics of the two most debilitating types of fractures, vertebral and long bone (VF and LBF), in pediatric DMD.Methods: This was a prospective, bi-centre, single-visit observational study in boys 4 to 17 years of age with genetically-confirmed DMD. The bone health assessmen...

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...

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...

ba0006p010 | (1) | ICCBH2017

Transient hyperphosphatasemia in a child with nephrolithiasis And history of severe prematurity

Kutilek Stepan , Formanova Daniela , Senkerik Marian , Skalova Sylva , Markova Daniela , Langer Jan

Background: Transient hyperphosphatasemia of infancy and early childhood (THI) is a benign, usually accidentally detected condition characterized by transiently increased activity of serum alkaline phosphatase (S-ALP), its bone or liver isoform, in children under five years of age, without signs of metabolic bone disease or hepatopathy. When encountered in a child with either chronic bone, liver or kidney disease, THI might concern the physician. We present a patient with urol...

ba0006p196 | (1) | ICCBH2017

A qualitative enquiry examining the lived experience of mothers who have children with osteogenesis imperfecta

Marr Caroline

Osteogeneses Imperfecta (OI) is a skeletal dysplasia which affects collagen biosynthesis. As with other chronic conditions it is recognised that the heterogeneity of perspectives between parents and health care professionals (HCP) can alter the course of a child’s health outcomes irrespective of the child’s disease. This qualitative study was employed to explore a mother’s lived experience of having a child with OI.Objectives: To gain impr...