Searchable abstracts of presentations at key conferences on calcified tissues

ba0004p98 | (1) | ICCBH2015

Clinical predictors of low bone mineral density in children with juvenile idiopathic arthritis

Kostik Mikhail , Larionova Valentina , Scheplyagina Larisa

Objectives: Juvenile idiopathic arthritis (JIA) – is a chronic inflammatory disease of joints which can affect optimal linear growth, risk of fractures and bone quality during the childhood and adult life. The aim of our study was to detect the simple clinical and laboratorial markers which can predict the realization of low bone mineral density (LBMD <−2 S.D.) in children with JIA.Methods: 198 children (82 boys and 116 girl...

ba0004p154 | (1) | ICCBH2015

A longitudinal, prospective, long-term registry of patients with hypophosphatasia

Kishnani Priya , Langman Craig , Linglart Agnes , Mornet Etienne , Ozono Keiichi , Rockman-Greenberg Cheryl , Seefried Lothar , Bedrosian Camille , Fujita Kenji , Cole Alex , Hogler Wolfgang

Objective: Hypophosphatasia (HPP) is a rare, inherited metabolic disease characterized by bone mineralization defects and osteomalacia, as well as systemic manifestations, including seizures, respiratory insufficiency, muscle weakness, nephrocalcinosis, and pain. The biochemical hallmark of HPP is low serum alkaline phosphatase, resulting from loss-of-function mutations in the gene encoding tissue non-specific alkaline phosphatase. HPP presents a broad spectrum of disease seve...

ba0004p188 | (1) | ICCBH2015

Fractures, bone mass and geometry in black and white South African children: The Birth to Twenty cohort

Thandrayen Kebashni , Schoenbuchner Simon , Ward Kate , Micklesfield Lisa , Norris Shane , Prentice Ann , Pettifor John

The prevalence of fractures in white children in South Africa is double that of black children (1). White males who fractured were shown to be more physically (2). The aim of this study was to compare the bone mass and geometry measures using peripheral quantitative computed tomography (pQCT) and dual energy x-ray absorptiometry (DXA) in black and white children with and without a history of fracture to determine the risk factors for fractures.Lifetime f...

ba0005cabs.oc2.4 | Oral Communications | ECTS2016

Peripheral tumour re-growth following combination therapy – role of the bone microenvironment

Haider Marie-Therese , Ottewell Penelope D. , Brown Nicola J. , Lefley Diane V. , Holen Ingunn

Background: Cancer patients often receive a combination of drugs that target both the microenvironment and the tumour cells. However, the role of the bone microenvironment (BME) in mediating peripheral breast cancer growth remains poorly understood. This is the first study to determine whether reduced subcutaneous tumour growth following combination therapy is due to direct interactions between the drugs and tumour cells or through zoledronic acid induced alterations to the BM...

ba0005p130 | Cancer and bone: basic, translational and clinical | ECTS2016

Uptake of different nitrogen containing bisphosphonate formulations by breast cancer cells

Zlatev Hristo , Auriola Seppo , Monkkonen Jukka , Maatta Jorma

Nitrogen-containing bisphosphonates (N-BPs) are used to treat osteolytic bone metastases. N-BPs have been previously shown to enter macrophages via macropinocytosis, but the mechanisms how they are taken up by breast cancer cells are so far incompletely known. In breast cancer primary tumours N-BPs have been shown, by other researchers, to be bound to micro-calcifications present in the tumour stroma. In our study we have characterized how different N-BP formulations, free, ca...

ba0005p439 | Other diseases of bone and mineral metabolism | ECTS2016

Quality of life and psysical activicy in patients with non-surgical hypoparathyroidism and pseudohypoparathyroidism

Underbjerg Line , Sikjaer Tanja , Rejnmark Lars

Background: Non-surgical hypoparathyroidism (NS-HypoPT) and pseudohypoparathyroidism (Ps-HypoPT) are both rare diseases, with a prevalence of 2/100.000 and 1/100.000, respectively.Studies on Quality of Life, QoL, are sparse, but studies have shown an increased risk of fatigue and depression.Methods: Using the Danish version of SF36v2 and WHO-5 Well Being Index, we investigated the physical and mental QoL among patients with NS-Hypo...

ba0006oc12 | (1) | ICCBH2017

Fracture incident rate and growth in a nationwide cohort of boys with Duchenne Muscular Dystrophy

Joseph S , Bushby K , Guglieri M , Horrocks I , Straub V , Ahmed SF , Wong SC , Northstar Clinical Network SC

Background: Fracture incidence rate and growth according to different glucocorticoid (GC) regimen in Duchenne Muscular Dystrophy (DMD) is currently unknown.Objective: To determine the extent of skeletal morbidity and the influence of GC regimen on fracture incidence rate and growth in a contemporary cohort of DMD in the UK.Method: Clinical details of 832 boys with DMD in the North Star database (2006–2015) from 23 centres were...

ba0006oc18 | (1) | ICCBH2017

Stimulation of angiogenesis and osteogenesis by enhanced preosteoclast platelet derived growth factor type BB attenuates glucocorticoid-induced osteoporosis in growing mice

Yang Ping , Wang Yan , Xia Zhuying , Cao Xu , Crane Janet

Survival of chronic diseases in childhood is often achieved utilizing glucocorticoids. However, the survival comes at a cost to the growing skeleton, resulting in impairment in the acquisition of peak bone mass and is the major etiology of secondary osteoporosis in children. We recently found that preosteoclasts secrete platelet derived growth factor type BB (PDGF-BB) to promote angiogenesis and osteogenesis during both modeling and remodeling. As glucocorticoid therapy affect...

ba0006p199 | (1) | ICCBH2017

Validation of questionnaire for measurement of sunlight exposure in children from Pune, India

Mandlik Rubina , Ekbote Veena , Kajale Neha , Patwardhan Vivek , Vispute Smruti , Kirtikar Utkarshini , Jaiswal Anjali , Ladkat Dipali , Palande Sonal , Deshpande Pranati , Michael Natasha , Deshpande Varsha , Vartak Varsha , Chiplonkar Shashi , Khadilkar Vaman , Padidela Raja , Mughal Zulf , Khadilkar Anuradha

Objective: Although there is adequate sunlight throughout the year, low serum 25OHD concentrations are being increasingly reported among Indian children (Gupta, 2014). Thus, quantifying individual sunlight exposure may be an important step in understanding hypovitaminosis D in sun-rich geographies. The objectives of our study were to quantify the sunlight exposure of school-children using a questionnaire and to use polysulphone film badges to validate the questionnaire adminis...

ba0007oc6 | (1) | ICCBH2019

Anthropometric characteristics of pediatric patients with hypophosphatasia: data from the Global Hypophosphatasia Patient Registry

Hogler Wolfgang , Linglart Agnes , Petryk Anna , Kishnani Priya , Seefried Lothar , Fang Shona , Rockman-Greenberg Cheryl , Ozono Keiichi , Martos-Moreno Gabriel Angel

Objectives: Limited data exist on growth parameters in children with hypophosphatasia (HPP), a rare metabolic disease characterized by impaired bone mineralization. We aimed to describe growth characteristics in untreated children with HPP enrolled in the Global HPP Patient Registry.Methods: Children (<18 years old) with a diagnosis of HPP who were not receiving enzyme replacement therapy with asfotase alfa at the time of evaluation were identified f...