Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp205 | Cell biology: osteoblasts and bone formation | ECTS2013

Thrombin receptor deficiency leads to osteopetrosis by decreasing the RANKL/OPG ratio

van der Eerden BCJ , Tudpor K , Jongwattapapisan P , Woudenberg-Vrenken TE , Bindels RJM , Hoenderop JGJ , van Leeuwen JPTM

Communication between osteoblasts and osteoclasts is crucial for bone remodeling. Thrombin and its thrombin receptor (TR; PAR-1) are expressed in osteoclasts and osteoblasts, respectively. To date, the physiological roles of thrombin and TR in bone metabolism have not been elucidated. Therefore, we fully characterized the bone phenotype of mice lacking the thrombin receptor.We performed bone microarchitectural analyses of the femurs of 10–12 week ol...

ba0002p21 | (1) | ICCBH2013

Bone disease in children with geroderma osteodysplasticum: a 25-year experience from a single tertiary centre

Gopal-Kothandapani J S , Padidela R , Clayton-Smith J , Chandler K E , Adams J E , Freemont A J , Mughal M Z

Geroderma osteodysplasticum (GO) is a rare autosomal recessive connective tissue disorder characterised by progeria like facies, wrinkled lax skin, joint hypermobility, congenital dislocation of hips and propensity to fragility fractures. In the past 25 years, five patients (three females and two males) diagnosed with GO were referred to our Paediatric metabolic bone service for assessment and management of secondary bone problems. All five children were born to consanguineous...

ba0002p97 | (1) | ICCBH2013

Vitamin D status and bone health in survivors of childhood lymphoblastic leukemia

Schundeln Michael M , Hauffa Pia K , Goretzki Sara C , Lahner Harald , Marschke Laura , Eggert Angelika , Hauffa Berthold P , Grasemann Corinna

Introduction: Lymphoblastic leukemia is the predominant form of childhood malignancies with survival rates of >80%. Late effects of cancer and treatment can affect endocrine function and may account for acute and chronic impairment of bone health.Aim and design: To assess bone health in pediatric patients after therapy for lymphoblastic leukemia we initiated a clinical trial investigating clinical and biochemical parameters of growth, puberty, bone t...

ba0005oc3.5 | Clinical trials, FGF-23 and focal osteoporosis | ECTS2016

Low serum iron is associated with high serum FGF23 in elderly men: the Swedish MrOS study

Lewerin Catharina , Ljunggren Osten , Nilsson-Ehle Herman , Karlsson Magnus K , Herlitz Hans , Lorentzon Mattias , Ohlsson Claes , Mellstrom Dan

Introduction: Fibroblast growth factor (FGF23) is a hormone derived from osteoblasts and osteocytes being involved in calcium and phosphate homeostasis, where serum iron (S-Fe) has been suggested as a potential mediator of FGF23 regulation. The aim was to determine whether iron status is a determinant of FGF23 in elderly men.Methods: The MrOS (osteoporotic fractures in men is a population based study of elderly men, in the Gothenburg part, (median age of...

ba0005p228 | Energy metabolism and bone, fat and bone | ECTS2016

Effects of roux-en-Y gastric bypass surgery on bone quality: a pilot study

Hogestol I.K. , Paschalis E.P. , Gamsjaeger S. , Hassler N. , Shabestari M.G. , Gulseth H.L. , Mala T. , Klaushofer K. , Eriksen E.F.

Roux-en-Y gastric bypass surgery (RYGBP) is one of the leading surgical treatments for morbid obesity and leads to significant long-term weight loss, diabetes remission, decreased cardiovascular events, and reduced mortality. RYGBP is, however, also implicated in increased fracture risk, mainly due to higher bone turnover rates and malabsorption. In the present study we used Raman microspectroscopic analysis to determine bone quality (an important determinant of bone strength)...

ba0005p407 | Osteoporosis: treatment | ECTS2016

Impact of 3-year vitamin D and calcium supplementation on mineral and organic matrix formation of trabecular bone in postmenopausal osteoporosis

Paschalis E P , Gamsjaeger S , Hassler N , Fahrleitner-Pammer A , Dobnig H , Stepan J J , Eriksen E F , Klaushofer K

Clinical trials involving drug therapies for postmenopausal osteoporosis typically compare effects of the active drug combined with vitamin D (vit D) and calcium (Ca) vs vit D and Ca supplementation on its own. Bone strength is estimated based on the amount of bone, frequently expressed as bone mineral density determined by dual X-ray absorptiometry, and quality of bone, hardly measured in clinical practice.The purpose of the present study was to compare...

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...

ba0006p042 | (1) | ICCBH2017

Osteogenesis imperfecta type VI presenting as suspected physical abuse -- a report of two cases

Sithambaram Sivagamy , Bishop Nick , Shankar Lata , Offiah Amaka C , Pollitt Rebecca C , Balasubramanian Meena , Saggar Anand K , Arundel Paul

Background: Osteogenesis imperfecta (OI) type VI is a rare recessive disease that may present with long bone fractures in early childhood. Bone in this condition is particularly brittle; the resulting pattern of long bone fractures and lack of distinct radiographic findings can make the diagnosis less obvious than in other types of OI. We report 2 unrelated children who presented with long bone fractures and were suspected of having suffered physical abuse with removal of pare...

ba0006p137 | (1) | ICCBH2017

Growth, bone and muscle mass are adversely affected in bone marrow transplant recipients: a body composition analysis

Doulgeraki Artemis , Vlachopapadopoulou E , Peristeri I , Paisiou A , Polizois G , Kaisari K , Monopolis I , Vessalas G , Michalacos S , Kitra V

Objectives: There are many factors leading to poor bone health and imbalanced body composition in bone marrow transplant (BMT) recipients. We aimed to report our patients’ profile and to correlate it with clinical parameters.Methods: Cross-sectional study of paediatric BMT patients. Assessment of growth (height, weight, BMI) and dual-energy X-ray absorptiometry (DXA) for evaluation of bone mineral density (BMD) and geometry, muscle and fat mass. All...