Bone Abstracts

see P475....

Hypophosphatasia (HPP) is the rare inherited metabolic disease caused by loss-of-function mutations in the tissue-nonspecific alkaline phosphatase (TNSALP) gene. TNSALP deficiency leads to extracellular excess of inorganic pyrophosphate, a bone mineralization inhibitor. Here, we report the validity and reproducibility of a novel scale to quantify HPP-specific radiographic changes in pediatric patients.The Radiographic Global Impression of Change (RGI-C) ...

Hyperthyroidism in mice is associated with a low bone mass, an increased bone turnover and high serum levels of sclerostin, a potent Wnt inhibitor. Here, we explored the effects of either reducing bone turnover with bisphosphonates or increasing bone formation with neutralizing sclerostin antibodies (Scl-Ab) on bone mass and estimated strength in hyperthyroid mice.Twelve-week-old C57BL/6 male mice were rendered hyperthyroid by adding L-thy...

Cysteine-rich with EGF like domains 2 (Creld2) has recently been identified as an endoplasmic reticulum (ER) stress inducible gene in the context of skeletal dysplasia caused by mutant protein accumulating in the ER eliciting an unfolded protein response (UPR). Creld2 was originally implicated in ER stress following the treatment of Neuro2α cells with thapsigargin. Furthermore, the promoter of Creld2 contains an ER stress activating transcription factor ...

Objectives: Evaluate nutritional status influence upon bone mineral density (BMD) of children with CP diagnosis after reconstructive hip joint surgery.Methods: Eighteen children with CP diagnosis with III-V level Gross Motor Function Classification System took part in the research. All patients received reconstructive medical treatment in child traumatic-orthopedic unit of health center after reconstructive hip joint surgery. Anthropometric measurement c...

Presenting problem: Severe forms of juvenile Paget’s Syndrome (JPD) result in extreme bone turnover, necessitating long-term treatment with anti-resorptive drugs to control for bone pain and modeling of bones.Objective: To report clinical and biochemical effects of intravenous treatment with Pamidronate in two children with severe forms of JPD over a time period of 3 and 9 years, respectively. Treatment was commenced at 12 months and 3 years of age,...

Objectives: Optic neuropathy (ON) is a critical complication of fibrous dysplasia (FD). Early surgical decompression can prevent blindness; however, prophylactic intervention may cause blindness from intra-operative nerve damage. There is therefore a critical need to develop diagnostic tests for accurate and early detection of ON in patients with FD. Currently used methodologies, including radiographic evaluation of optic canal size and optic nerve length, are suboptimal and c...

Despite widely accepted classical mechanical theory of buttresses as a load bearing areas in the midfacial skeleton, the data related to its microstructural adaptation on functional demands are scarce. In this study we investigated microstructural features of the different regions in the facial bones in relation to the occlusal load dissipation using a combination of finite element analysis (FEA) and micro-CT analysis (μCT). The FEA was performed on the model of the dry h...

Introduction: Using the autograft bones in treatment orthopaedic diseases and trauma is common. However, cases of the large bone fragments autoplasty in acute trauma is rare. We have studied the case of succesfull autoplasty of the large tibial fragment in severe road accident.Materials and methods: Child O., born in 2000, was delivered to the emergency room of Mogilev City Hospital after road accident (he was hit by a car while crossing the street). His...

Bone remodeling is the key process in bone structural reorganization, and its alterations lead to changes in bone mechanical strength. Since osteons reflect different bone remodeling patterns, we hypothesize that the femoral cortex of females with miscellaneous age, disease, and treatment conditions will display distinct osteonal morphology and osteocyte lacunar numbers along with different mechanical properties.The specimens used in this study were coll...