Searchable abstracts of presentations at key conferences on calcified tissues

ba0002lb1 | (1) | ICCBH2013

Influence of age and gender on spine bone density and TBS microarchitectural texture parameters in infants

Winzenrieth Renaud , Cormier Catherine , DiGregorio Silvana , Rio Luis Del

Children bone knowledge is relatively sparse. This is especially true for infant and for bone microarchitecture data. We have investigated, in this study, the age-related modifications of spine microarchitectural texture, as assessed by TBS, on male and female infants during their two first years of life.The study group was composed of 143 and 109 healthy female and male infants aged between 0 and 2 years. Height and weight Z-scores were signifi...

ba0004p155 | (1) | ICCBH2015

Hydroxylase (CYP27B1) deficiency presenting with marked hypotonia, growth failure, hypoventilation, pulmonary hypertension and a renal proximal tubulopathy

Barton John , Hayes Wesley , Burren Christine

Background: 1α-hydroxylase is a mitochondrial P450 enzyme critical to the synthesis of active calcitriol from the pro-hormone 25(OH) D. Multiple different mutations in the CYP27B1 gene have been identified that abolish or reduce 1α-hydroxylase enzymatic activity resulting in vitamin D dependent rickets type 1. Children with 1α-hydroxylase deficiency present with a clinical picture of joint pain and deformity, hypotonia, muscle weakness, growth failure and someti...

ba0006p162 | (1) | ICCBH2017

Nutritional rickets presenting to secondary care in children (<16 years) -- A UK surveillance study

Julies Priscilla , Pall Karina , Lynn Richard , Calder Alistair , Mughal Zulf , Shaw Nicholas , McDonnell Ciara , McDevitt Helen , Blair Mitchell

Objectives: Rickets is a disease of growing children with potentially serious short and long-term complications. The United Kingdom (UK) national incidence of Nutritional Rickets(NR) is unknown and thought to be increasing. This study aims to describe the incidence, presentation and clinical management of children with NR in the UK and Republic of Ireland.Methods: Data is being collected prospectively monthly between March 2015 and March 2017 from 3500 p...

ba0006is10biog | (1) (1) | ICCBH2017

Arterial calcification syndromes: causes and treatments

Rutsch Frank

Biographical DetailsFrank RutschFrank Rutsch is a consultant and Associate Professor in Pediatrics at Münster University Children’s Hospital, Münster, Germany. He graduated from Münster University Medical School in 1992 and took part in the Pediatric residency program in Dresden University and Dortmund Municipal Hospital, Germany...

ba0004is8biog | (1) (1) | ICCBH2015

Early-onset osteoporosis

Makitie Outi

Biographical DetailsDr. Outi Mäkitie received her MD and PhD from the University of Helsinki, Finland where she also completed training in Pediatrics and in Pediatric Endocrinology. After a 3-year post-doctoral clinical and research fellowship at The Hospital for Sick Children in Toronto, Canada, she returned to Finland and served as Head of the Metabolic Bone Clinic, Children’s...

ba0005p75 | Bone development/growth and fracture repair | ECTS2016

Increased periosteal expansion, Osterix expression and osteogenic potential upon bone injury during perturbed PI3K signaling

Walia Bhavita , Scanlon Vanessa , Yu Jungeun , Maye Peter , Drissi Hicham , Sanjay Archana

Periosteum contains mesenchymal progenitors and is essential for fracture healing. Signaling mechanisms governing periosteal reaction to injury remain largely unidentified. We previously investigated how PI3 kinase signaling affects the skeletal system using CblYF/YF knock-in (YF) mice wherein PI3K signaling is perturbed by abolition of interaction between Cbl, an E3 ubiquitin-ligase/adaptor protein, and p85 subunit of PI3K. YF mice displayed increased bone volume u...

ba0004is14biog | (1) (1) | ICCBH2015

Somatic mosaic skeletal overgrowth disorders

Warman Matthew L

Biographical DetailsDr Matthew L Warman is the Harriet M Peabody Professor of Orthopaedic Surgery and Genetics at Harvard Medical School. He attended college at Brown University and Medical School at Cornell University. While in medical school, he performed research with Dr Adele Boskey at The Hospital for Special Surgery. After medical school he trained in Pediatrics at the Children&#146...

ba0005p205 | Cell biology: osteoclasts and bone resorption | ECTS2016

Better understanding the potency and cytotoxicity of different bisphosphonates on murine osteoclast formation and activity: implications for its better clinical use in treatment cancers

Razai Mohammad , Orriss Isabel , Arnett Timothy

Bisphosphonates are widely used drugs in the fight against osteoclast-mediated bone loss, including osteoporosis and Paget’s disease of bone. The first generation of these potent drugs such as clodronate, a non nitrogen-containing bisphosphonate, has been shown to inhibit osteoclast formation and osteoclastic bone resorption both in vitro and in vivo as well as inducing apoptotic cell death. Recent interest has centred on the effects of more potent nitro...

ba0001pp174 | Cell biology: osteoblasts and bone formation | ECTS2013

Elevated levels of serotonin decrease bone volume by direct effects on bone turnover in rats

Erjavec Igor , Bordukalo-Niksic Tatjana , Brkljacic Jelena , Pauk Martina , Grgurevic Lovorka , Thompson David D , Paralkar Vishwas M , Cicin-Sain Lipa , Vukicevic Slobodan , Mokrovic Gordana , Kesic Maja , Grcevic Danka

Elevated levels of circulating serotonin have been reported to decrease bone mineral density1. Conversely, reduced serotonin (5HT) in mice lacking TPH1, the rate limiting enzyme for 5HT synthesis, was reported to be anabolic to the skeleton with high osteoblastic activity2. However, in other studies TPH1 deletion led to either an initial increase in BMD due to inhibition of osteoclastic bone resorption3, or had no bone effect4</su...

ba0001pp428 | Osteoporosis: treatment | ECTS2013

Persistence with different anti-osteoporosis medications: a population-based cohort study.

Pages-Castella Aina , Carbonell-Abella Cristina , Nogues Xavier , Javaid M Kassim , Arden Nigel K , Cooper Cirus , Diez-Perez Adolfo , Prieto-Alhambra Daniel

Objective: Several reports suggest very low persistence with oral bisphosphonates, but there is a scarcity of data on persistence with other anti-osteoporosis medications. We therefore compared rates of early discontinuation (in the first year of therapy) between all available outpatient anti-osteoporosis drugs in Catalonia, Spain.Study design: population-based retrospective cohort study.Participants and source of data: The data in...