Bone Abstracts

Objectives: Neurofibromatosis 1 (NF1) is one of the most common autosomal dominant diseases. Skeletal involvement such as short stature, kyphoscoliosis, tibial bowing and pseudarthrosis are common osseous manifestations of NF1. Moreover, there is a growing evidence that reduced bone mineral density (BMD), is a common feature of NF1 subjects. The aim of the study was to evaluate the usefulness of Bone Mineral Density and Quantitative Ultrasound (QUS) at phalanxes in the assessm...

Background and aim: Multifaceted risk factors impair bone mass in childhood cancer survivors. Aims of the study were to evaluate bone mass and it’s determinant and fracture prevalence in CBCS 2 (G+2) or 5 (G+5) years after off therapy (OT).Methods: Seventy-three (G+2) and 87 (G+5) CBCS were evaluated at 12.9±4.2 and 14.9±4.4 yrs, respectively. Diagnoses were: astrocytic (G+2:n=25, G+5:n=24), embryonal (G+2:n=28, ...

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...

Objective: Existing case series examining outcomes following spine surgery for patients with skeletal dysplasias demonstrate high rates of complications. Yet, these earlier studies are from a single institution, contain surgeries performed almost 50 years ago, and only include patients with a diagnosis of achondroplasia. The objective is to determine contemporary outcomes after spinal surgery in patients with skeletal dypslasias, focusing on complications and revisions. We pre...

Osteogenesis imperfecta is a disorder characterized by bone fragility. Current management includes the usage of bisphosphonates to improve bone stock and manage pain. Outcomes in adults have mainly been reported in terms of presentation compared to genotype, most probably due to the heterogeneity of the disease. The head and neck resection of the femur, or Girdlestone procedure, was a common procedure to treat infections of the hip but it has fallen out of favor due to the adv...

Introduction: Epithelioid hemangioendothelioma (HEE) is a tumor of vascular origin, infrequent in the pediatric age and even more infrequent at intestinal level. To our knowledge, there are no previous reports of pediatric patients with malignant humoral hypercalcemia associated with this tumor. Humoral mechanism is seen more often in lung, uterine cérvix, skin and esophagus tumors. The presence of hypercalcemia appears to be an ominous prognostic sign.<p class="abste...

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Osteoporotic fractures are very common and represent an enormous unmet medical need. Our group has previously reported that addition of rTNF to the fracture site promotes fracture healing in C57/BL6 mice (Glass et al. PNAS 2011). Using a murine fracture model of endochondral healing, we observed that local addition of rTNF only accelerates fracture repair if administered within the first 24 h following injury. The optimal therapeutic dose is 1 ng. TNF is firs...

Objective: Texture information of the subchondral bone area (SBA) of 2D radiographs represents a promising possibility for evaluating the state of osteoarthritis (OA). However such features are likely to vary within the SBA and therefore the selection of the region of interest (ROI) plays a crucial role. Thus, a feature selection algorithm (FSA) is being applied in order to determine ROIs that enable an optimum discrimination between patients with and without OA.<p class="...

Osteosarcoma is the most frequent primary bone tumor that develops mainly in young adults. The survival rate at 5 years is below 30% for patients with poor response to treatment or with metastasis.The histones modifications are of critical importance in maintaining the transcription program of both normal and tumor cells. The bromodomain and extra-terminal domain (BET) protein family is an important class of ‘histone reading protein’ capable to...