Searchable abstracts of presentations at key conferences on calcified tissues

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...

ba0004p84 | (1) | ICCBH2015

Different causes of infantile hypercalcemia

Kutilek Stepan , Plasilova Ivana , Gut Josef , Konrad Martin , Schlingman Karl P , Machata-Hanulikova Kristyna , Teterova-Cerna Hana

Background: Hypercalcemia in childhood is rare, can be of various origin and might go unrecognized until severe signs appear. We demonstrate hypercalcemia of different causes in two infants.Case no 1.: 5-month old girl with failure to thrive, constipation, muscle hypotonia, dehydration, where total calcaemia peaked to 4.25 mmol/l. Low calcium diet, application of glucocorticoids and furosemide resulted in a drop in calcaemia to values below 3 mmol/l and ...

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...

ba0006oc24 | (1) | ICCBH2017

KRN23 effects on phosphate and vitamin D dysregulation in children <5 years old with X-Linked Hypophosphatemia (XLH)

Imel Erik , Carpenter Thomas , Gottesman Gary S , Martin Javier San , Mao Meng , Skrinar Alison , Whyte Michael P

Objectives: XLH features renal phosphate (Pi) wasting, hypophosphatemia, rickets, and skeletal deformities from elevated circulating levels of fibroblast growth factor 23 (FGF23). KRN23, an investigational fully human monoclonal antibody, binds FGF23 and inhibits its action. Our Phase 2 study of KRN23 in XLH children (ages 5–12 years) is demonstrating improvements in serum Pi and rickets. Here we present our Phase 2 trial evaluating the efficacy and safety of KRN23 in you...

ba0006p037 | (1) | ICCBH2017

Early fragility fractures in Zellweger syndrome spectrum – peroxisome dysfunction affecting osteogenesis?

Nicholls Rachel , Pierre Germaine , Chronopoulou Effie , Smithson Sarah F , Offiah Amaka C , Barton John S , Burren Christine P

Background: Peroxisomal Biogenesis Disorders (PBD) is a group of rare metabolic diseases in which peroxisomal function is disrupted. PBD encompasses Zellweger Syndrome Spectrum (ZSS) disorders, which range in severity from classical ZS with severe neurological impairment and markedly reduced life expectancy to Refsum Disease presenting later in childhood. Recent fragility fractures in our ZSS patients in very early childhood prompted case series review.P...

ba0001pp425 | Osteoporosis: treatment | ECTS2013

Evaluation with densitometry of patients with breast cancer and low bone mineral density after 2 years of treatment

Gil Sonia Munoz , Dolera Tomas Mut , Lopez Belen C Garrido , Maicas M D Torregrosa , Sarrio R Girones , Tendero P Lopez , Armario M D Garcia , Mira Pascual Munoz

Aim: Evaluate the differences with densitometry after 2-year treatment in patients with breast cancer and LBMD.Materials and methods: A 2 year duration longitudinal study was done in patients diagnosed with breast cancer sent to the Rheumatology Osteoporosis Unit in Hospital d’Ontinyent, who required supplements of calcium and vitamin D+bisphosphonates after a risk fracture study. Socio-demographic data, breast tumor characteristics, risk f...

ba0002p185 | (1) | ICCBH2013

An unusual presentation of progressive osseous heteroplasia in a 7-year-old female child

Schrander D E , Welting T J , Schrander J J P , van Rhijn L W , Korver-Keularts I , Schrander-Stumpel C T R M

Background: Progressive osseous heteroplasia (POH) (OMIM 166350) is a rare autosomal dominant condition, characterized by heterotopic ossification of the skin, subcutaneous fat and deep connective tissue. This condition is distinct from Albright’s hereditary osteodystrophy or Mccune–Albright syndrome (AHO) (OMIM 103580) and fibrodysplasia ossificans progressiva (FOP) (OMIM 135100).Presenting problem: We present an unu...