Searchable abstracts of presentations at key conferences on calcified tissues

Bone Abstracts

Published by BioScientifica

Page 17 of about 205 results

ba0002p191 | (1) | ICCBH2013

Long-term follow-up in Stuve–Wiedemann syndrome: a case report

Sabrina Buonuomo Paola , Macchiaiolo Marina , Cambiaso Paola , Capolino Rossella , Digilio Maria Cristina , Andrea Bartuli

Background: Stuve–Wiedemann syndrome (SWS, OMIM 601559) is a severe autosomal recessive condition characterized by bowing of the lower limbs with cortical thickening, wide metaphyses, abnormal trabecular pattern and camptodactyly. Additional features include dysautonomia symptoms with temperature instability, respiratory distress and sucking/swallowing difficulties in the first months of life.Most SWS cases do not survive beyond the first y...
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ba0003pp145 | Cell biology: osteoblasts and bone formation | ECTS2014

Effects of strontium ranelate on the bone-like mineralized matrix produced in osteoblast cell cultures

Querido William , Campos Andrea , Ferreira Erlon Martins , Gil Rosane San , Rossi Alexandre , Farina Marcos

Strontium ranelate is a promising drug used in the treatment of osteoporosis. This drug has a unique dual effect on bone turnover, simultaneously increasing bone formation by osteoblasts (anabolic effect) and decreasing bone resorption by osteoclasts (antiresorptive effect). The goal of this study was to evaluate: i) anabolic effects of strontium ranelate on the formation of bone-like mineralized matrix in osteoblast cell cultures and ii) changes the drug could cause on the ma...
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ba0004p73 | (1) | ICCBH2015

Long-term outcomes of surgical treatment for craniofacial fibrous dysplasia

Boyce Alison , Burke Andrea , Peck Carolee Cutler , Dufresne Craig , Collins Michael

Fibrous dysplasia (FD) is a benign skeletal disease caused by somatic activating mutations of Gsα leading to formation of expansile fibroosseous lesions. These may occur in isolation or in association with McCune-Albright syndrome (MAS), characterized by skin pigmentation and hyperfunctioning endocrinopathies, including growth hormone (GH) excess. FD in the craniofacial skeleton may result in significant morbidity including facial asymmetry, vision and hearing ...
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ba0004p158 | (1) | ICCBH2015

Extreme, biomechanically-explained remodelling of biological femoral reconstructions in pediatric oncology

Taddei Fulvia , Valente Giordano , Piroddi Sabina , Schileo Enrico , Pitto Lorenzo , Roncari Andrea , Leardini Alberto , Manfrini Marco

Introduction: Vascularised fibula autograft combined with a massive bone allograft (Capanna 2007) is used in skeletal reconstructions in children. If vascularisation is successful a clear remodelling of the reconstruction can be observed. This study aims to define a protocol to characterise bone’s structural evolution in skeletal reconstructions through a computer-aided analysis, and attempts a biomechanical interpretation of the observed phenomena through a multiscale mo...
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ba0006p039 | (1) | ICCBH2017

Cystinosin deficiency affects bone phenotype

Battafarano Giulia , Rossi Michela , Di Giovamberardino Gianna , Pastore Anna , Taranta Anna , Del Fattore Andrea

Objective: Cystinosis is a rare lysosomal storage disorder caused by loss-of-function mutations of the CTNS gene, encoding for cystinosin symporter that mediates cysteine efflux from lysosome. ~95% of cystinotic patients display nephropathic Fanconi’s syndrome, short stature, osteopenia and rickets. In this study we evaluated whether the absence of cystinosin primarily affects bone remodeling activity.Methods: We analyzed bone phen...
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ba0006pl1 | (1) | ICCBH2017

Poster List

Poster No. Category Title Author P002 Bone development A 3-year longitudinal study of skeletal effects and growth in children after kidney transplantation <a href="ba...
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ba0002p133 | (1) | ICCBH2013

Linear growth over 2 years of velaglucerase alfa therapy in children with type 1 Gaucher disease previously treated with imiglucerase

Zimran Ari , Hughes Derralynn , Elstein Deborah , Smith Laurie , Harmatz Paul , Rhead William , Giraldo Pilar , Mendelsohn Nancy , Park Chan-Hoo , Zahrieh David , Crombez Eric

Objectives: As children with confirmed type 1 Gaucher disease (inherited metabolic disorder) may have linear growth retardation, we evaluated linear growth over 2 years in children enrolled in the interventional study TKT034, in which patients receiving imiglucerase enzyme replacement therapy were switched to velaglucerase alfa.Methods: Trial TKT034 enrolled patients who were ≥2 years of age with type 1 Gaucher disease and stable clinical parameter...
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ba0004is26 | (1) | ICCBH2015

Duchenne and cerebral palsy

Munns Craig

Childhood and adolescence are critical periods for optimizing bone growth and mineral accrual. Bone strength is determined by bone size, geometry, quality, and mass – variables that are influenced by genetic factors, physical activity, nutrition, and pubertal hormones. Duchenne muscular dystrophy (DMD) and cerebral palsy (CP) are two chronic medical conditions of childhood associated with reduced mobility and increased rate of pathological fracture....
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ba0004p136 | (1) | ICCBH2015

A modified performance-oriented mobility assessment tool for assessing clinically relevant gait impairments and change in children with hypophosphatasia: development and validation

Phillips Dawn , Griffin Donna , Przybylski Tracy , Morrison Erica , Reeves Amy , Vallee Marc , Fujita Kenji , Madson Katherine , Whyte Michael

Objective: Mobility is an important endpoint in patient care with implications for activities of daily living, community participation, and quality of life. We adapted the performance-oriented mobility assessment (POMA-G),1 a widely used and validated clinical gait assessment tool for adults, to use in children with hypophosphatasia (HPP). HPP is the rare metabolic disease caused by loss-of-function mutation(s) in the gene encoding tissue non-specific alkaline phosp...
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ba0006p071 | (1) | ICCBH2017

In search of hypophosphatasia: a need to establish normative data for low alkaline phosphatase in pediatric population

Abramowicz Pawel , Konstantynowicz Jerzy , Zelazowska-Rutkowska Beata , Cylwik Bogdan

Background: Hypophosphatasia (HPP) is a rare inborn error of metabolism caused by mutations in the gene encoding tissue-nonspecific alkaline phosphatase gene (TNSALP), leading to low alkaline phosphatase (ALP) activity. At least 6 clinical forms of HPP have been reported. Certain benign or asymptomatic presentations of HPP in older children may remain undiagnosed, in contrast to severe perinatal and infantile types. The underlying reason of this diagnostic inconsisten...
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Bone Abstracts
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