Searchable abstracts of presentations at key conferences on calcified tissues

ba0005p486 | Preclinical and ex vivo imaging | ECTS2016

Age-related changes in 3D bone microstructure are more pronounced in the sub-endplate region than in the central region of human vertebral bodies

Thomsen Jesper Skovhus , Ebbesen Ebbe Nils , Bruel Annemarie

The vertebral body microstructure of young individuals appears to be divided into three equally high horizontal regions: two adjacent to the endplates and one in the centre of the vertebral body. With age this subdivision of the vertebral microstructure seems to vanish. The aim of the study was to investigate the differences in the age-related changes in vertebral microstructure in the two regions.Vertebral (L2) bone specimens from 41 women and 39 men ag...

ba0006is15 | (1) (1) | ICCBH2017

Bone in chronic kidney diseases: a systemic problem

Langman Craig B

Chronic kidney disease (CKD) is defined according to the presence of kidney damage and level of kidney function – irrespective of the type of kidney disease (diagnosis). Among individuals with chronic kidney disease, the stages are defined based on the level of glomerular filtration rate. From infancy through young adulthood, the major causes of CKD arise from congenital abnormalities of the kidneys and urinary tract (CAKUT), acquired or congenital forms of nephrotic synd...

ba0006p036 | (1) | ICCBH2017

Decreased incidence of fracture after IV bisphosphonates in girls with Rett syndrome and severe bone fragility

Lambert Anne-Sophie , Rothenbuhler Anya , Charles Perrine , Celestin Elisabeth , Bahi-Buisson Nadia , Linglart Agnes

Background: Rett Syndrome (RS) is a disabling condition due to mutations in MECP2. Girls affected with RS are at risk of developing osteoporosis and fractures at a young age because of their lack of mobility and through a direct effect of MECP2 on bone mineralization. In these girls, bone fragility inflicts pain and may seriously impair the quality of life.Objective: To retrospectively assess the effect of IV bisphosphonates on fracture, bone mineral den...

ba0006p205 | (1) | ICCBH2017

Osteopathologies and endocrine late effects in a cohort of 102 juvenile survivors of brain tumors

Schundeln Michael , Fritzemeier Sebastian , Hauffa Pia , Bauer Jens , Hauffa Berthold , Grasemann Corinna

Objectives: Endocrine late effects, including osteopathologies, following diagnosis and treatment of childhood malignancies are studied in adult survivors with alarming results. However, in pediatric patients with brain tumors the risk to develop endocrine late effects is high even during childhood and adolescence.Aim and design: To investigate osteopathologies and endocrine function in juvenile survivors of pediatric brain tumors we conducted a cross-se...

ba0007p60 | (1) | ICCBH2019

FGF23-expressing osteocytes are confined to bone packets that completed primary mineralization in patients with chronic kidney disease on dialysis (CKD5D)

Fratzl-Zelman Nadja , Pereira Renata C , Roschger Paul , Salusky Isidro B , Klaushofer Klaus , Wesseling-Perry Katherine

Objectives: FGF23 is expressed in clusters of osteocytes at the trabecular periphery suggesting that FGF23-expressing osteocytes are confined to specific basic multicellular units (BMUs) at the trabecular surface. Higher numbers of FGF23-expressing osteocytes are found in chronic kidney disease (CKD) patients with preserved skeletal mineralization indices. We thus combined immunohistochemistry and quantitative backscattered electron imaging (qBEI) to explore the hypothesis tha...

ba0007p191 | (1) | ICCBH2019

Supporting the emotional well-being of children living with osteogenesis imperfecta; an upstream health promotion initiative

Seasman Ali

Background: Sheffield Children’s NHS Foundation Trust offers a highly specialised metabolic bone service for children and young people living with Osteogenesis Imperfecta (OI). OI is a chronic health condition causing bone fragility, bone pain, bone deformity, frequent fractures and variable physical limitations with wide reaching consequences on both children and families; the potential to experience elevated psychological distress is common.Presen...

ba0007p216 | (1) | ICCBH2019

Dual diagnosis of autism and osteogenesis imperfecta: Case examples to illustrate the implications of dual diagnosis for enhanced outcomes for child and family

Jones Rebecca , Seasman Alison , Marr Caroline , Bishop Nicholas , Arundel Paul , Balasubramanian Meena , Team Metabolic Bone

Background: A minority of children with Osteogenesis Imperfecta (OI) seen within the Sheffield National Severe, Complex and Atypical Service (SCAOI) were also identified as showing symptoms consistent with an Autism Spectrum Disorder (ASD) (Balasubramanian et al. 2018). Diagnosis of ASD in conjunction with OI may be delayed due to presenting problems being inappropriately attributed to OI resulting in specialised ASD input not being received by children.<p class="...

ba0001mtp14.1 | (1) | ECTS2013

CRC Grants

Camilo Joana

Turn your challenges into opportunities through EU funding for research and innovation.The European Union encompasses several funding mechanisms to support research and innovation (R&I). One of its main instruments is Seventh Framework programme (FP7), established for the period 2007–2013, which is now approaching its end.The successor EU instrument, called Horizon 2020 (H2020), is currently under ...

ba0001pp306 | Muscle, physical activity and bone | ECTS2013

Increased activity associated with exercise does not rescue aged bone's impaired response to local mechanical loading

Meakin Lee , Udeh Chinedu , Sugiyama Toshihiro , Galea Gabriel , Lanyon Lance , Price Joanna

Bones’ fracture resistance is achieved in vivo by adaptation to habitual loading. Aged bone can adapt to exercise1 but in female rodents ageing impairs the adaptive response to artificial loading2,3. This inconsistency led us to investigate whether treadmill exercise, sufficiently mild to not itself stimulate new bone formation, could rescue aged bone’s diminished response to artificial loading.Young adult 17-week...

ba0002is7 | (1) (1) | ICCBH2013

Osteogenesis imperfecta

Pals Gerard

Osteogenesis imperfecta (OI) is a genetic disorder, leading to fragility of the bones. The clinical variability is extreme, ranging from relatively mild to perinatally lethal. Secondary features such as short stature, blue sclerae, dentinogenesis imperfecta and hearing loss may also exist in affected individuals. OI is most often caused by mutations in the collagen type I genes COL1A1 and COL1A2, that show a dominant mode of inheritance. The least severe OI c...