Bone Abstracts

Objective: Muscular dystrophy is characterized by lower skeletal muscle quality, lower muscle strength and physical performance. The aim of the study was to assess regional muscle density and its correlation with regional muscle area in Duchenne muscular dystrophy (DMD) subjects and able bodied controls.Method: Skeletal muscle pQCT (peripheral quantitative computed tomography) scans at the non-dominant forearm were performed in patients with muscle dystr...

Objectives: Chronic non-bacterial osteomyelitis (CNO) is an inflammatory, non-bacterial disorder of the skeletal system of yet unknown etiology (ORPHA 324964). CNO predominantly affects the metaphyses of long bones, but lesions can occur at any sites of the skeleton. Patients present with local bone pain and inflammation and - to our experience - often suffer from functional impairment with significant disabilities of daily life. The objective of this study was to assess physi...

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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Purpose: Multipotent stromal cells (MSC) have been considered promising for the regenerative strategies of articular cartilage. However, the MSC chondrogenic differentiation can ultimately lead to the formation of hypertrophic chondrocytes responsible for the calcification of cartilage. To prevent this MSC-dependent production of a calcified matrix in articular site, MSC hypertrophic differentiation has to be carefully controlled. Given that articular cartilage is avascular, w...

Objectives: To study acceptance and the effects on bone during a 6-month whole body vibration (WBV) therapy in severely disabled children.Methods: Nineteen patients, age 5–16 years, with severe motor disabilities completed the 6-month WBV therapy standing on a self-controlled dynamic platform with vibration, jumps and rotation. The WBV was performed twice per week at 40–42 Hz. Bone mass and biochemical markers were measured at start and after 6...

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Hypoxia and the hypoxia-inducible factor 1alpha (Hif1alpha) are known to play critical physiological functions in endochondral bone development. However, their role in abnormal cartilage formation (chondrodysplasia) is unknown. Our goal was to test the possibility that altered oxygen homeostasis, which would result in abnormal Hif1alpha expression and activity, could lead to chondrodysplasia. This was done using matrix metalloproteinase (MMP) 9 and 13 deficient mice, which pre...

Introduction: Langerhans cell histiocytosis (LCH) is a monoclonal disorder characterized by proliferation and accumulation of atypical Langerhans cells. Bone involvement is particularly destructive and to date, no standard of care exists. Bisphosphonates are osteoclast inhibitors that could target the multinucleated giant cells within the LCH lesions and might be used to alleviate bone pain and the progression of disease.Objective: To evaluate the effica...

Background: X-linked hypophosphatemic rickets (XLHR) represents the most common form of hypophosphatemia.Aim: The aim of this prospective study was to describe and analyze the incidence of cranial and cervico-occipital junction (COJ) anomalies in a series of children with XLHR.Patients and methods: Seventeen children (13 girls, 4 boys, mean age 7.3 years) followed for XLHR at the French national reference center for rare diseases o...