Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp284 | Muscle, physical activity and bone | ECTS2013

Raising CK: what it means according to different clinical landscape?

Juan Jose Scali , Visentini Susana , Berruezo Ramiro , Sevilla Daniel , Pacheco Gonzalo , Garcia Rodrigo

Introduction: The creatine kinase (CK) is a dimeric enzyme, involved in energetical metabolism. It is present in many tissues, but higher concentration in skeletal and cardiac muscle.Therefore, conditions that involve muscle tissue may increase this serum enzyme. Such enzyme elevation is usually observed in inflammatory myopathies and others autoimmune diseases.Sometimes some elevation in CK is not fully understood out off these co...

ba0003oc5.1 | Important pathways in bone biology and cancer | ECTS2014

In vivo efficacy of a pharmacological inhibitor of TNAP to prevent arterial calcification and its associated cardiac hypertrophy and mortality

Sheen Campbell , Chhea Thangchanthida , Pinkerton Antony , Jackson Michael , Millan Jose Luis

Medial vascular calcification (MVC) is a pathological condition common to a variety of diseases, including chronic kidney disease, diabetes, obesity, generalized arterial calcification of infancy, arterial calcification due to deficiency of CD73, and Keutel syndrome. These diseases share the common feature of tissue-nonspecific alkaline phosphatase (TNAP) upregulation in the vasculature. We developed a mouse model that overexpresses human TNAP in vascular smooth muscle cells, ...

ba0004p116 | (1) | ICCBH2015

Osteopetrosis with uncommon final height: can only local IGF1 hold normal growth during pubertal spurt?

Milani Soraya Sader , Paula Francisco Jose , Liberatore Jr Raphael , Martinelli Jr Carlos Eduardo

Background: Final stature is a result of concomitant growth hormone (GH)-IGF1 axis integrity, a healthy environment and genetic background. Occasionally, growth occurs despite remarkable impairment in hormone profile. Presenting problem: An uncommon case of longitudinal growth in a boy with very low IGF1 concentrations and clinical diagnosis of osteopetrosis. Clinical management: We report on the case of a 19 year-old male patient with the diagnosis of osteopetrosis who had a ...

ba0005oc2.4 | Bone mass and bone strength Wnt signalling | ECTS2016

Up-regulation of Wnt antagonists contributes to the attenuated response of bone formation to repeat doses of sclerostin antibody in a mouse model

Holdsworth Gill , Greenslade Kevin , Stencel Zofia , Jose Joby , Kirby Hishani , Moore Adrian , Robinson Martyn , Ke David

Loss of the gene encoding the secreted Wnt antagonist sclerostin results in increased bone mass in humans and mice. Administration of antibodies to sclerostin (Scl-Ab) has been shown to increase bone mineral density (BMD) by increasing bone formation and decreasing bone resorption, in both animal studies and human clinical trials. In these studies, the magnitude and rate of increase in bone formation markers, and the rate of increase of BMD, diminishes upon repeat dosing with ...

ba0007p109 | (1) | ICCBH2019

Fracture prevalence in children 0–19 years-old in Mexico: A 10-year cross-sectional analysis

Clark Patricia , Barbato Annarella , Guagnelli Miguel Angel , Rascon Jose Alberto , Denova Edgar , Borja Victor Hugo

Fracture prevalence in children appears to change in recent years due to variations in physical activity and enforcement of laws that protect children within motor vehicles. However, in Mexico such variation has not been explored so far.Objective: To analyze fracture prevalence in the paediatric population of Mexico to detect patterns of change in time, between genres and among different diagnoses.Methods: We analyzed data from the...

ba0001pp482 | Other diseases of bone and mineral metabolism | ECTS2013

Zoledronate efficacy and safety in active Paget's disease: long-term follow-up and retreatment in clinical practice

Vieira-Sousa Elsa , Rodrigues Ana , Caetano-Lopes Joana , Capela Susana , Ramos Filipa , Figueira Ricardo , Joaquim Polido-Pereira1 , Ponte Cristina , Raquel Campanilho-Marques1 , Barros Rita , Romeu Jose Carlos , da Silva Jose Alberto Pereira

Background: Zoledronate, a third generation bisphosphonate, has showed high efficacy in the inhibition of bone resorption. The objective of this observational study was to assess short and long-term efficacy and safety of zoledronate in the treatment of active Paget’s disease (PD).Methods: Patients with active PD treated with zoledronate 5 mg were consecutively recruited. Clinical and laboratory parameters were determined before, at 3 and every 6 mo...

ba0001oc5.5 | Treatment of osteoporosis | ECTS2013

Bone anabolic efficacy and safety of ba058, a novel analog of hPTHrP: 12-month extension data from a phase 2 clinical trial in postmenopausal women with osteoporosis

Hattersley Gary , Bilezikian John , Guerriero Jonathan , Kumar Prasanna , Zanchetta Jose , Lyttle C Richard , O'Dea Louis Saint L

A randomized, placebo-controlled phase 2 study evaluated the safety and efficacy of BA058, an analog of hPTHrP(1–34), in postmenopausal women with severe osteoporosis. 221 patients were randomized to received BA058 20, 40, and 80 μg, placebo or teriparatide (TP) 20 μg, by daily s.c. injection. 184 (83%) patients completed an initial 24 weeks of treatment. The mean percent change in lumbar spine BMD at 24 weeks was 1.6% with placebo, 6.7% with BA058 80 μg, a...

ba0001oc6.2 | Mineralisation and energy metabolism | ECTS2013

Deficiency of the bone mineralisation inhibitor NPP1 protects against obesity and diabetes

Huesa Carmen , Morton Nicholas M , Ferron Mathieu , Karsenty Gerard , Millan Jose Luis , Ahmed Faisal , Farquharson Colin , MacRae Vicky E

Bone has recently emerged as a novel endocrine organ regulating glucose metabolism. Ectonucleotide pyrophosphatase/phosphodiesterase-1 (NPP1) controls bone mineralisation by generating the mineralisation inhibitor pyrophosphate. In clinical studies increased activity of NPP1 has been found in patients with insulin resistance, and it has been shown to directly inhibit the insulin receptor. We hypothesised that mice lacking NPP1 (Enpp1−/−) would exhibit im...

ba0001pp337 | Osteoporosis: evaluation and imaging | ECTS2013

Dietary calcium and vitamin D intake and serum 25-hydroxyvitamin D and parathyroid hormone levels in healthy elderly Spanish men: the relationship with calcaneal and phalangeal quantitative ultrasound and phalangeal dual energy X-ray absorptiometry

Leal Alejo , Luz Canal-Macias Maria , Fernando Calderon-Garcia Julian , Roncero-Martin Raul , Rodriguez-Dominguez Trinidad , Moran Jose M

Purpose: To evaluate whether calcium and vitamin D intake is associated with 25-hydroxyvitamin D (25(OH)D) and parathyroid hormone (PTH) serum concentrations or is associated with either the phalangeal dual energy X-ray absorptiometry (pDXA) or the quantitative bone ultrasound (QUS) at the phalanges and the calcaneus in independent elderly men from southwestern Spain.Methods: Serum PTH and 25(OH)D were measured in 199 healthy elderly men (mean age: 73.31...

ba0002p181 | (1) | ICCBH2013

Long-term imiglucerase/alglucerase treatment in Latin American children with type 1 Gaucher disease: lessons from the International Collaborative Gaucher Group (ICGG) Gaucher Registry

Camelo Jr Jose Simon , Cabello Juan Francisco , Drelichman Guillermo G , Kerstenetzky Marcelo M , Sarmiento Isabel C , Linares Adriana

Objective: Evaluate the clinical characteristics of all Latin American pediatric patients with Gaucher disease type 1 (GD1) enrolled in the ICGG Gaucher Registry at baseline and investigate long-term outcomes and clinical benefit of prolonged imiglucerase/alglucerase therapy in patients with manifestations of GD1 at baseline.Methods: All Latin American patients with GD1 in the ICGG Gaucher Registry (NCT00358943) who were <18 years of age at the start...