Searchable abstracts of presentations at key conferences on calcified tissues

ba0005p394 | Osteoporosis: treatment | ECTS2016

Inhibitory effects of nanopartical calcium administration on dexamethasone-induced osteoporosis development in axial skeleton of pigs

Tymczyna Barbara , Tatara Marcin , Tymczyna-Sobotka Monika , Krupski Witold , Szabelska Anna

The aim of the study was to evaluate effects of 6-month-long administration with nanopartical calcium on morphological and densitometric properties of axial skeleton in pigs subjected to osteoporosis induction with dexamethasone. Male newborn piglets (N=28) were divided after birth into four equal groups (control group receiving placebo, Dex group receiving dexamethasone, NanoCa group receiving orally nanopartical calcium and NanoCa/Dex group) receiving simultaneously...

ba0005p485 | Preclinical and ex vivo imaging | ECTS2016

Sex-related differences of morphological and densitometric properties of mandible in silver foxes (Vulpes vulpes)

Luszczewska-Sierakowska Iwona , Tatara Marcin , Krupski Witold , Charuta Anna , Tymczyna Barbara , Furmanek Miroslaw , Maciejewski Ryszard

The aim of this study was to determine morphological and densitometric parameters of mandible obtained from male and female silver foxes (Vulpes vulpes). The study was performed on 1-year-old males (n=7) and females (n=8). Mandible was isolated and subjected to morphological and densitometric analysis using quantitative computed tomography (QCT) method and Somatom Emotion Siemens apparatus (Siemens, Erlangen, Germany) equipped with Somaris/5 VB10B so...

ba0006p047 | (1) | ICCBH2017

Vitamin D status before and during treatment for childhood cancer

Latoch Eryk , Muszynska-Roslan Katarzyna , Osinska Milena , Pazik Anna , Krawczuk-Rybak Maryna

Recent studies suggest the link between low vitamin D levels and the prevalence of cardiovascular disease, diabetes, hypertension and a number of different types of cancer. Nowadays, vitamin D deficiency is recognized as a pandemic health problem and pediatric cancer patients may be even at higher risk than the healthy children. Children suffered from malignancy are especially exposed to its deficiency, because of the potential impact of the disease and its treatment. However,...

ba0006p116 | (1) | ICCBH2017

Classical SATB2-associated syndrome with severe osteoporosis, recurrent bony fractures and tibial bowing

Jacobs Benjamin , Rose Anna M. , Zulf Mughal M. , Keen Richard , Wakeling Emma L.

Background: Mutations in SATB2 have been described in association with a unique phenotype known as SATB2-associated syndrome (SAS). This condition is characterised by severe intellectual disability affecting speech development, behaviour, facial features and dental anomalies. Skeletal features and osteoporosis have been reported in older individuals (aged 15–36), in association with point mutations. We report a 24-year-old man with a SATB2 misse...

ba0006lb10 | (1) | ICCBH2017

Effects of long-term sedentary behaviour on the cortical bone mass and distribution during growth: The HAPPY bone study

Duckham Rachel L , Rantalainen Timo , Rodda Christine , Timperio Anna , Hawley Nicola , Hesketh Kylie

Introduction: Whilst it is well-established that sedentary behaviour may increase the risk of paediatric obesity, and potentially result in early onset cardio-metabolic diseases such as type 2 diabetes and cardiovascular disease, there is less consensus about the potential detrimental effects that long-term sedentary behaviour may have on bone health during childhood.Purpose: To determine if long-term sedentary behaviour affects accrual of bone mass, str...

ba0007p30 | (1) | ICCBH2019

Bone mass, sclerostin and body composition in women with anorexia nervosa: a 3-year follow-up after weight gain therapy

Svedlund Anna , Tubic Bojan , Pettersson Cecilia , Elfvin Anders , Ellegard Lars , Magnusson Per , Swolin-Eide Diana

Objectives: Patients with anorexia nervosa (AN) are at high risk of reduced bone mass. The aim of this intervention study was to investigate the long-term effects on bone and body composition three years after intense weight gain therapy.Methods: Twenty-five female AN patients, mean age 20.1 years, mean BMI of 15.5 kg/m2, were included. Twenty-two patients fulfilled the treatment for 12 weeks with a high-energy diet. Body composition and bone ...

ba0007p36 | (1) | ICCBH2019

ALPL gene mutation in a family

Vai Silvia , Broggi Francesca , Luisa Bianchi Maria , Ponti Emanuela , Mihalich Alessandra , Maria Di Blasio Anna

Introduction: The clinical diagnosis of mild forms of hypophosphatasia [HPP], a rare genetic bone disease, is often made in adulthood, on the basis of persistently low serum levels of alkaline phosphatase [ALP], often coupled with signs of poor bone/teeth mineralization.Case report: A 50-year-old male on treatment with vitamin D supplementation because of osteoporosis of lumbar spine (T-score –3.2) and femoral neck (T-score –2.4), was referred ...

ba0007p38 | (1) | ICCBH2019

Clinical features and approach to treatment in pediatric patients with McCune-Albright syndrome: monocentric experience

Makazan Nadezhda , Orlova Elizaveta , Kareva Maria , Kolodkina Anna , Kalinchenko Natalia , Petrov Michael , Zubkova Natalia , Peterkova Valentina

McCune-Albright-Syndrome (MAS) is a rare multisystem disorder presenting with polyostotic fibrous dysplasia accompanied by a list of extraskeletal features including café-aulait spots and various endocrine hyperfunctioning. There is no effective treatment for FD in MAS nowadays. Patients with MAS (n=60, 49 girls(G) and 11 boys (B) have been diagnosed and followed up during 20 years in the Institute of Pediatric Endocrinology. First clinical manifestations were pe...

ba0007p77 | (1) | ICCBH2019

Long-term efficacy profile of asfotase alfa in the treatment of patients with hypophosphatasia: a pooled analysis

Hogler Wolfgang , Rockman-Greenberg Cheryl , Petryk Anna , Zhou Shanggen , Whyte Michael P , Bishop Nick

Objectives: Asfotase alfa (AA), an enzyme replacement therapy, is the only approved medical treatment for pediatric-onset hypophosphatasia (HPP), which is caused by deficient tissue-nonspecific alkaline phosphatase activity. We detail the long-term efficacy of AA observed from the pediatric clinical trial program.Methods: Efficacy data collected to study completion were pooled from 3 open-label, multicenter investigations of children who manifested HPP s...

ba0007p214 | (1) | ICCBH2019

Assessment of multidisciplinary care of children with osteogenesis imperfecta at The Royal Manchester Children's Hospital

Galloway Paula , Nixon Anna , Rayner Lauren , Panchbhaya Nicola , Collins Helen , Skae Mars , Mughal Zulf , Padidela Raja

Dedicated occupational therapy and physiotherapy service for children and families with Osteogenesis Imperfecta (OI) at the Royal Manchester Children’s Hospital were set up in September 2017 to provide multidisciplinary management.Objectives: The aim is to assess if the newly established specialist paediatric occupational therapy and physiotherapy service is meeting the needs of the patients and their families.Method: A paper ...