Searchable abstracts of presentations at key conferences on calcified tissues

ba0003pp382 | Other diseases of bone and mineral metabolism | ECTS2014

Osteogenic differentiation of fibroblast derived from patients with fybrodisplasia ossificans progressiva

Bravenboer Nathalie , Micha Dimitra , van Essen Huib , Netelenbos Coen , Eekhoff Marelise , Pals Gerard

Fibrodysplasia ossificans progressiva (FOP) is a rare, extremely disabling genetic disorder characterized by progressive heterotopic ossification preceded by episodic inflammatory soft tissue swellings (flare ups) leading to early death. There is no proven effective treatment yet. We aimed to develop an in vitro system to investigate the working mechanism of flare ups induced ossification.Skin biopsies were obtained from four patients with FOP. ...

ba0004oc17 | (1) | ICCBH2015

Inhibition of TGFβ signalling delays ossification in patients with fibrodysplasia ossificans progressiva

Micha Dimitra , Voermans Elise , Huib van Essen , Drukker Jesse , Netelenbos Coen , Eekhoff Marelise , Bravenboer Nathalie

Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disorder characterized by progressive heterotopic ossification. FOP patients only present great toe malformations at birth. However, as they grow older they develop soft tissue lumps as a result of flare-ups causing the irreversible replacement of skeletal muscle tissue with bone tissue leading to cumulative physical immobility. Classical FOP patients possess a mutation (c.617G>A; R206H) in the activin recept...

ba0005cabs.oc2.1 | Oral Communications | ECTS2016

Prostate cancer microRNAs in extracellular vesicles stimulate osteoclastogenesis

Bijnsdorp Irene , Mulder Jorn , Geldof Albert , Bakker Astrid , de Vries Teun , van Moorselaar Jeroen

Prostate cancer (PCa) is the most common cancer in males. When patients develop metastasis, no curative therapy is available. To find new therapeutic options, it is crucial to understand how PCa cells induce metastasis. Recently, it was shown that PCa cells secrete small extracellular vesicles (EVs) that can be found in the circulation and in bones. Uptake of EVs by other cells may change their behaviour. We previously identified three miRNAs that were uniquely upregulated in ...

ba0005p9 | Arthritis and other joint diseases: translational and clinical | ECTS2016

Apolipoprotein E aggravates inflammation and bone destruction in murine antigen – induced arthritis

Ascone Giuliana , Ceglie Irene Di , de Munter Wouter , Walgreen Birgitte , Sloetjes Annet , van Lent Peter

Background and Objectives: Rheumatoid arthritis (RA) is a chronic disease characterized by severe bone destruction which has been associated with altered lipid metabolism. Apolipoprotein E (Apo E) is a lipoprotein mainly produced by macrophages. ApoE has been described as crucial in lipid metabolism but also as an important anti-inflammatory mediator regulating innate immunity and bone turnover. In the present study we investigated the role of Apo E in bone destruction during ...

ba0005p318 | Osteoporosis: evaluation and imaging | ECTS2016

Circulating microRNA in metabolic bone diseases-osteoporosis

Mandourah Abdullah , Hof Rob Van' T , Ranganath Lakshminarayan , Barraclough Roger , Barraclough Dong

Osteoporosis is the commonest worldwide age-related bone disease. It is clinically symptomless until the first fracture happens. Circulating microRNAs can be used as novel biomarkers to assess health status and progression of complex diseases. A recent review highlighted the involvement of microRNAs in the control of bone formation and remodeling. Most of these studies have been done on animal models, but few on human blood samples.This research aims to ...

ba0005p463 | Other diseases of bone and mineral metabolism | ECTS2016

Change in bone mineral density with high-dose prednisone in patients with rheumatoid arthritis

Rasch Linda , van Tuyl Lilian , Kremer Martijn , Bultink Irene , Boers Maarten , Lems Willem

Background: Recently, we showed that treatment with COBRA-light therapy including prednisone with initially 30 mg/day was as effective as the original COBRA scheme, with initially 60 mg/day (Ter Wee, ARD 2015), in the treatment of rheumatoid arthritis (RA). Since high-dose glucocorticoids are associated with bone loss, we investigated the differences in bone mineral density (BMD) after 1 year of treatment in both arms.Objective: To determine whether ther...

ba0005p464 | Other diseases of bone and mineral metabolism | ECTS2016

The course and management of craniofacial fibrous dysplasia: a case series

Majoor Bas , Appelman-Dijkstra Natasha , Bruggemann Jens , van de Sande Michiel , Dijkstra Sander , Hamdy Neveen

Introduction: Cranialfacial fibrous dysplasia (CFD) presents with pain, facial asymmetry and/or neurological complications. It has been suggested that patients with CFD respond favourably to treatment with bisphosphonates, by a decrease in pain and arrest of progression. Therefore, we performed a retrospective study of 56 patients with CFD in our center.Methods: We assessed clinical characteristics and disease course. Furthermore, clinical and biochemica...

ba0007p166 | (1) | ICCBH2019

The ketogenic diet and bone density: a retrospective longitudinal cohort study

Draaisma Jos , Hampsink Brieke , Willemsen Michel , Linders Miel , van Houdt Nicole

Background: The Ketogenic Diet Treatment (KDT) is a well-established intervention for intractable childhood epilepsy and the first choice treatment for Gluccose-1-transporter-deficiency-syndrome and Pyruvate-dehydrogenase-complex deficiency. During long-term follow up of children treated with KDT an increased incidence of bone fractures has been found. However, the exact contribution of KDT to a decreased BMD remains unclear. Prophylactic supplementation with calcium and vitam...

ba0002oc17 | Diagnostics | ICCBH2013

Determinants of bone mineral density in long-term adult survivors of childhood cancer

Klap B C , te Winkel M L , den Hoed M , van Waas M , Neggers S J C M M , Boot A M , Pieters R , Pluijm S M F , van den Heuvel-Eibrink M M

Objectives: Osteopenia is a complication of childhood cancer treatment but it is unknown to which extend this occurs in adult survivors, and which subgroups are at risk. We examined bone mineral density (BMD) and assed the relative importance of potential determinants of low BMD in very long-term adult survivors of childhood cancer.Methods: The single-center cohort study included 410 adult CCSs (median age at diagnosis: 6.6 years (range: 0–17 years)...

ba0004p193 | (1) | ICCBH2015

Aggravated bone density decline following symptomatic osteonecrosis in children with acute lymphoblastic leukemia

den Hoed MAH , SMF Pluijm , de Groot-Kruseman HA , Fiocco M , Hoogerbrugge P , JA Leeuw , MCA Bruin , van der Sluis IM , Bresters D , MH Lequin , JC Roos , AJP Veerman , Pieters R , van den Heuvel-Eibrink MM

Objectives: Osteonecrosis (ON) and decline of bone mineral density (BMD) are serious side effects during and after treatment of childhood acute lymphoblastic leukemia (ALL). It is unknown whether ON and low BMD co-occur in the same patients, and whether these two osteogenic side-effects can mutually influence each other’s development.Methods: BMD and the incidence of symptomatic ON were prospectively assessed in a national cohort of 466 patients wit...