Bone Abstracts

Background: Elevated plasma homocysteine levels are a risk factor for osteoporotic fractures. Supplementation with vitamin B12/folic acid lowers homocysteine levels. This study aimed to determine whether vitamin B12/folic acid supplementation reduces osteoporotic fracture incidence in hyperhomocysteinemic elderly.Methods: B-PROOF is a double-blind, randomized, placebo-controlled trial including 2 919 participants aged ≧65 years with elevated homocyste...

Objectives: Osteoporosis is a frequent cause of morbidity in boys with glucocorticoid (GC)-treated Duchenne Muscular Dystrophy (DMD). We sought to determine the frequency and characteristics of the two most debilitating types of fractures, vertebral and long bone (VF and LBF), in pediatric DMD.Methods: This was a prospective, bi-centre, single-visit observational study in boys 4 to 17 years of age with genetically-confirmed DMD. The bone health assessmen...

It has suggested that BGP is inversely related to body fat. BGP appears to regulate energetic metabolism through the insulin receptor signaling pathway in the osteoblast, which affects bone resorption and BGP activity. Fat tissue is an endocrine organ that secretes different hormones and growth factors; several of them affect bone remodeling and insulin secretion. The present report comparatively studied total BGP levels, glucose homeostasis and body fat mass in spontaneous ob...

Objectives: Marfan syndrome (MFS) is a connective disorder caused by mutations in FBN1 gene which encodes the extracellular matrix protein fibrillin 1. Pathogenesis relies on a dysregulation of activated TGF-β. Cardiovascular, ocular and skeletal systems are involved with a variable expressivity. Findings evolve with age, making the diagnosis in children more difficult. Skeletal involvement includes disproportionate long bone overgrowth, scoliosis, and chest deformity. Al...

Background: The tyrosine kinase inhibitor (TKI) imatinib is applied as front-line treatment in adult and pediatric patients with chronic myeloid leukemia (CML) in order to selectively inhibit the causative oncogenic BCR-ABL1 tyrosine kinase. However, TKIs exhibit off-target effects on further kinases involved in the regulation of bone metabolism. As consequence, pediatric patients display longitudinal growth retardation while on imatinib treatment. As CML is a rare disease in ...

Objectives: Within the National Health Service there is a recognised need for supporting young peoples transition into adult services1. Focus groups and forums have historically been used to identify how to achieve ‘good’ transition between services, from the perspective of patients, carers and professionals. A recent article recommended an individualised programme supporting transition for young people with osteogenesis imperfecta (OI)2. The ai...

Objective: Evaluate the clinical characteristics of all Latin American pediatric patients with Gaucher disease type 1 (GD1) enrolled in the ICGG Gaucher Registry at baseline and investigate long-term outcomes and clinical benefit of prolonged imiglucerase/alglucerase therapy in patients with manifestations of GD1 at baseline.Methods: All Latin American patients with GD1 in the ICGG Gaucher Registry (NCT00358943) who were <18 years of age at the start...

Objective: Low dietary calcium intakes and increasing adiposity in Indian adolescents may increase their risk of hypertension (Sanwalka et al., Asia Pac J Clin Nutr. 2010, Khadilkar et al., Int J Pediatr Obes. 2010). Therefore, the aim of the present study was to explore association of dietary calcium intakes and body composition with blood pressure in 10–14 year old Indian adolescents.Method: 370 apparently healt...

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...

Objectives: Young male jockeys undertake calorie restriction and high volumes of physical activity during periods of musculoskeletal growth and development. Previous research shows that jockeys have compromised bone health1–4 and display disordered eating5–7. Restricted intakes of calcium and vitamin D, together with excessive amounts of exercise, increase the risk of osteoporosis in males8. The aim of this study was to establish whet...