Searchable abstracts of presentations at key conferences on calcified tissues

ba0001pp235 | Cell biology: osteoclasts and bone resorption | ECTS2013

Differential effects of nitrogen-containing bisphosphonates on human PBMCs and MUTZ-3 cells

Kwaasi Aaron , Mabilleau Guillaume , Dunford James , Ebetino Frank , Zarei Ali , Pazianas Michael , Sabokbar Afsie , Russell Graham

Introduction: Nitrogen-containing bisphosphonates (N-BPs) can inhibit the differentiation and function of osteoclasts derived from Peripheral Blood Mononuclear cells (PBMCs) in a dose-dependent manner. MUTZ-3 cells are a potentially useful human cell line for studying osteoclast differentiation. The aim of this study was to elucidate the action of N-BPs on MUTZ-3 cells.Methods: Human PBMCs and MUTZ-3 cells were cultured in α-MEM supplemented with he...

ba0003pp407 | Paediatric bone disease | ECTS2014

Glucocorticoid-treated boys with Duchenne muscular dystrophy DMD and osteoporosis have higher bone matrix mineralization before and after i.v. bisphosphonate therapy

Roschger Paul , Misof Barbara M. , Klaushofer Klaus , Rauch Frank , Ward Leanne M

Duchenne muscular dystrophy (DMD) causes progressive muscle weakness and loss of ambulation. While glucocorticoid (GC) therapy improves motor function, many boys sustain fractures due to osteoporosis. Recently, i.v. bisphosphonate (i.v.BP) therapy has shown promise in the treatment of DMD-related osteoporosis. At the same time, bone histomorphometry revealed lowered bone volume and significant reductions in bone formation pre-i.v.BP treatment, and a further drop after 2 years&...

ba0004p160 | (1) | ICCBH2015

Prevalence of muscle deficits in survivors of childhood acute lymphoblastic leukemia

Veilleux Louis-Nicolas , Rauch Frank , Curnier Daniel , Krajinovic Maja , Laverdiere Caroline , Sinnett Daniel , Alos Nathalie

Overall cure rates for childhood acute lymphoblastic leukemia (ALL) have improved allowing the cure of over 85% of patients. At least 70% of survivors of childhood ALL cancers have substantial morbidities as a result of their treatment. There is evidence that survivors of ALL have increased rates of long-term skeletal muscle dysfunction and weakness. Our objective was to determine the percentage of patients with muscle dysfunctions (muscle force, power, endurance, and % of fit...

ba0004p179 | (1) | ICCBH2015

An atypical case of bone fragility and dysmorphism with an unusual and novel de novo COL1A1 mutation

Skae Mars , Rauch Frank , Mughal Zulf , Sims Jo , Davis Naomi , Scott B , Arundel Paul , Hobson Emma

We report a male child presenting with antenatally diagnosed bilateral talipes equinovarus, short stature, bilateral cryptorchidism and poor weight gain; born at 39 weeks gestation (birth weight 2.56 kg) to non-consanguineous Caucasian parents. Facial dysmorphism included a prominent forehead, brachycephaly, shallow orbits, a high anterior hairline, a narrow nasal bridge and small mouth with a thin upper lip. He had white sclera and was short for height (−4.4 SDS) with a...

ba0005p35 | Bone biomechanics and quality | ECTS2016

Bone material properties as measured by Reference Point Indentation are low in subjects with acromegaly

Malgo Frank , Hamdy Neveen A T , Pereira Alberto M , Biermasz Nienke R , Appelman-Dijkstra Natasha M

Introduction: Acromegaly is a rare disease caused by excess growth hormone (GH) production by an adenoma of the anterior pituitary gland. The skeletal complications of GH and IGF-1 excess include increased bone turnover, increased cortical bone mass and deteriorated microarchitecture of trabecular bone, associated with a high risk of vertebral fractures in the presence of a relatively normal Bone Mineral Density (BMD). There are no data on bone material properties in patients ...

ba0007oc3 | (1) | ICCBH2019

Osteocyte lacunae characteristics in healthy children

Blouin Stephane , Hartmann Markus A , Klaushofer Klaus , Glorieux Francis H. , Rauch Frank , Zwerina Jochen , Roschger Paul

Objectives: Osteocytes play a major role in bone metabolism as mechanosensors, key regulators of osteoblast and osteoclast activity and of the mineral homeostasis. Therefore the assessment of osteocytes characteristics is important to understand bone pathology. We propose to study indirectly the osteocytes by performing quantitative backscattered electron imaging to quantify the sectioned osteocyte lacunae density and size in 2D on bone samples.Methods: ...

ba0007p150 | (1) | ICCBH2019

Osteogenesis imperfecta: skeletal outcomes after bisphosphonates discontinuation at final height

Robinson Marie-Eve , Trejo Pamela , Palomo Telma , Glorieux Francis H , Rauch Frank

Objectives: Intravenous cyclical bisphosphonates are widely used to treat children with moderate to severe osteogenesis imperfecta (OI). They increase bone mineral density (BMD), diminish fracture rates and improve mobility. Bisphosphonates are often discontinued when growth is completed. We aimed to determine if the skeletal gains achieved with bisphosphonates persist after their discontinuation in patients with OI.Methods: We assessed patients with OI ...

ba0003pp292 | Osteoporosis: pathophysiology and epidemiology | ECTS2014

CXCL8 and CCL20 enhance osteoblast-mediated osteoclastogenesis

Pathak Janak L , Bakker Astrid D , Verschueren Patrick , Lems Willem F , Luyten Frank P , Klein-Nulend Jenneke , Bravenboer Nathalie

Osteoporosis is common in rheumatoid arthritis (RA). Since osteoblasts express receptors for CXCL8 and CCL20, which are produced by inflammatory cells around the inflamed joints in RA, we hypothesized that CXCL8 and CCL20 contribute to osteoporosis in RA by affecting osteoblast proliferation, differentiation, and osteoblast-osteoclast communication.Primary human osteoblasts were cultured±CXCL8 (2–200 pg/ml) and CCL20 (5–500 pg/ml) for 14 d...

ba0005p43 | Bone development/growth and fracture repair | ECTS2016

Serum of patients with active rheumatoid arthritis inhibits differentiation of osteochondrogenic precursor cells

Pathak Janak L , Verschueren Patrick , Lems Willem F , Bravenboer Nathalie , Klein-Nulend Jenneke , Bakker Astrid D , Luyten Frank P

Delayed fracture healing is frequently experienced in patients with systemic inflammation such as during rheumatoid arthritis (RA). The reasons for this are diverse, but could also be caused by inflammatory cytokines and/or growth factors in serum from patients with active disease. We hypothesized that serum from patients with active RA contains circulating inflammatory factors that inhibit differentiation of osteochondrogenic precursors.Serum was obtain...