Searchable abstracts of presentations at key conferences on calcified tissues

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 ...

ba0006p059 | (1) | ICCBH2017

Retrospective evaluation of serum alkaline phosphatases (ALP) in Italian children referred to a tertiary children's hospital

Stagi Stefano , Rubino Chiara , Petrolini Chiara , Sandini Elena , Maggioli Chiara , Schiatti Roberto , de Martino Maurizio

Objectives: To evaluate the frequency of low serum alkaline phosphatase (ALP) activities in patients referred to a tertiary children’s hospital. Another item was to explore potentially missed diagnoses and to evaluate the role of laboratory screening for hypophosphatasia.Study design: A retrospective evaluation over an 6-year period (between December 2010 and December 2016) carried out to identify children and adolescents, referred to Anna Meyer Chi...

ba0001pp123 | Calciotropic and phosphotropic hormones and mineral metabolism | ECTS2013

Vitamin D supplementation during lactation: effect on maternal and offspring's vitamin D status and bone mass–double-blind randomized control trial

Czech-Kowalska Justyna , Jaworski Maciej , Latka-Grot Julita , Bulsiewicz Dorota , Pludowski Pawel , Chazan Bogdan , Pawlus Beata , Wygledowska Grazyna , Zochowska Anna , Kornacka Maria K , Kryskiewicz Edyta , Karczmarewicz Elzbieta , Dobrzanska Anna

Introduction: Optimal vitamin D intake for lactating women remains controversial. We hypothesized that 1200 IU/day (vs 400 IU/day) of vitamin D during breastfeeding will enhance maternal vitamin D status and bone mass.Methods: Healthy mothers after term, singleton delivery were randomized to receive vitamin D3: 1200 IU/day (800+400 IU/day from multivitamins) or 400 IU/day (placebo+400 IU/day from multivitamins) during lactation. Serum 25-hydroxyvitamin D...

ba0005p108 | Cancer and bone: basic, translational and clinical | ECTS2016

Clinical and experimental evidence suggest a protective effect of Paget’s disease of bone against skeletal metastasization from solid tumors

Merlotti Daniela , Rucci Nadia , Rendina Domenico , Bianciardi Simone , Evangelista Isabella Anna , Ucci Argia , Rotatori Stefano , Sebastiani Guido , Dotta Francesco , Cenci Simone , Strazzullo Pasquale , Nuti Ranuccio , Teti Anna , Gennari Luigi

Paget’s disease of bone (PDB) is a common disorder of bone metabolism characterized by focal areas of excessive and rapid bone resorption and formation, leading to bone pain, deformity and fractures. Despite the well documented increase in the risk of primary bone tumors due to neoplastic degeneration of pagetic tissue, a large retrospective analysis suggested that patients with prostate cancer and PDB have delayed time to bone metastases and improved overall survival tha...

ba0001pp80 | Bone development/growth and fracture repair | ECTS2013

Bone metabolism is influenced by serum 25-hydroxyvitamin D in healthy children

Czekuc-Kryskiewicz Edyta , Karczmarewicz Elzbieta , Jaworski Maciej , Czech-Kowalska Justyna , Gorska Anna , Konstantynowicz Jerzy , Pludowski Pawel , Piskorski Jaroslaw , Lorenc Roman

Introduction: Serum 25(OH)D concentrations for optimal bone metabolism in children is unknown. Only few data exist describing the effects of increasing serum 25(OH)D on bone metabolism markers.Aim: The aim of the study was to explore the association between serum 25(OH)D and bone metabolism markers in children.Patients and methods: Serum levels of bone formation (OC, P1NP) and bone resorption (CTx) markers (Cobas e411, Roche Diagno...

ba0001pp319 | Osteoporosis: evaluation and imaging | ECTS2013

Prevalence of FRAX clinical risk factors: dietary calcium intake habits and osteoporosis screening in Greek women

Bakides Sofoclis , Sakellariadis George , Alevizou Stavroula , Koussi Kleoniki , Rapti Anna , Tsiverdis Panayiotis , Kavvadia Konstantina , Drivas Kyriakos , Ververeli Charilila-Loukia

Introduction: Osteoporosis-related fractures can cause substantial disability and increase health care costs, and mortality. There are many difficulties to access Greek women residing in remote villages and perform the FRAX tool for osteoporosis evaluation, especially, after the global economy crisis.Purpose: To estimate the prevalence of FRAX clinical risk factors, calcium intake habits and perform osteoporosis screening in 275 postmenopausal Greek wome...

ba0001pp475 | Other diseases of bone and mineral metabolism | ECTS2013

Multidisciplinary studies of ancient calcified tissues: renal stones from mummies

Capulli Mattia , Arrizza Lorenzo , Rucci Nadia , Piperni Sara Gemini , Quaresima Raimondo , Giuffra Valentina , Fornaciari Gino , Teti Anna , Ventura Luca

The renal stones found in the mummies of Pandolfo III Malatesta, Lord of Fano (1370–1427) and an anonymous nobleman from Popoli (XVIII century) were investigated using different techniques. Both specimens were examined with binocular stereomicroscopy (BSM) and scanning electron microscopy (SEM), also with energy dispersive X-ray analysis (EDX). Multiple tiny fragments from surface and inner portions were submitted to X-ray diffraction (XRD) analysis. Subsequently, the cal...

ba0001pp476 | Other diseases of bone and mineral metabolism | ECTS2013

multidisciplinary studies of ancient calcified tissues II: contents from Egyptian canopic jars

Rucci Nadia , Arrizza Lorenzo , Capulli Mattia , Maurizi Antonio , Mercurio Cinzia , Guidotti Maria Cristina , Fornaciari Gino , Teti Anna , Ventura Luca

Ancient Egyptians were well known for the art of embalming, performed in the belief that preservation of the body was essential for survival in the afterlife. During the mummification process, internal organs were removed and some of them (lungs, stomach, liver, and intestines) washed, dehydrated with natron, perfumed, and stored in so-called canopic jars, buried with the mummy. Each jar had established contents and its own protective deity. To date, a limited number of studie...