Bone Abstracts

Background and Objectives: The relations among cortical volumetric bone mineral density (CortBMD) and comprehensive measures of mineral metabolism have not been addressed in chronic kidney disease (CKD). The aim was to identify determinants of CortBMD in childhood CKD. A secondary objective was to assess if CortBMD was associated with subsequent fracture.Design/participants: This prospective cohort study in children, adolescents and young adults (ages 5&...

Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. Data from adult ADPKD population show increased fibroblast growth factor 23 (FGF23) levels while circulating Klotho levels decrease, with a low TmP/GFR even in patients with normal renal function. Moreover, in ADPKD animal models, cyst lining renal cells were demonstrated to produce FGF23, although the animals displayed FGF23 resistance. No data are available in a paediatr...

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...

Type 2 diabetes and osteoporosis often occur together in postmenopausal women. Sitagliptin is a new drug used in the therapy of type 2 diabetes; it affects incretin system as a result of inhibition of dipeptidyl peptidase-4. So far, the impact of the drug on bone remodeling processes is unknown. The aim of this study was to investigate the effect of sitagliptin on bone growth and remodeling in non-ovariectomized and ovariectomized rats.The experiments we...

Introduction: Serum 25(OH)D concentrations for optimal bone metabolism in children is unknown. Only few data exist describing the effects of increasing serum 25(OH)D on bone metabolism markers.Aim: The aim of the study was to explore the association between serum 25(OH)D and bone metabolism markers in children.Patients and methods: Serum levels of bone formation (OC, P1NP) and bone resorption (CTx) markers (Cobas e411, Roche Diagno...

Objectives: Vitamin D intake at a dose 400 IU/d is recommended for breastfed infants and is considered as sufficient. Vitamin D status is connected with bone mass and fat mass. We aimed to assess, if 400 IU/d has the same efficiency and impact on body composition in infants with different vitamin D status at birth (<20 ng/ml vs ≧20 ng/ml).Methods: 148 breastfed infants were supplemented with 400 IU/d of cholecalciferol up to 6 months of age. S...

Introduction: The body composition at term equivalent age of infants born preterm is different than that of infants born at term.Aim: The aim of the study was to compare the body composition in preterm and full-term infants at age 3 and 4 years.Patients and methods: Total body bone mineral content (TBBMC, g), density (TBBMD, g/cm2), and body composition (fat mass – FM, g; lean body mass – LBM, g) were measured ...

Proteoglycans is a component of extracellular matrix, forming a mesh around structural proteins including collagen and elastin, and providing elasticity to tissue structure. Proteoglycans is formed by the attachment of glycosaminoglycans to a core protein, a process that requires three enzymes: galactosyltransferase-1, galactosyltransferase-2 and glucoronosyltransferase-1, encoded by B4GALT7, B3GALT6 and B3GAT3 respectively. B3GALT6 mutation...

Background: X linked hypophosphataemic rickets is the commonest cause of renal phosphate wasting, however sporadic cases may warrant additional investigations to exclude less common causes, as exemplified by our case.Presenting problem: A 3 year 7 month boy was referred for assessment and ongoing management of rickets and short stature (height less than 1st %.) He originally presented with leg bowing and waddling gait from the age of 12 months...

Objective: Over the last decade, there has been increasing scientific interest in vitamin D, and it is now advised that all pregnant women and infants should receive vitamin D supplementation. Despite of this, it is recognized that knowledge of vitamin D in the general public is limited. The internet is now an important source of health care information and analysis of internet search activity rates can provide information on disease epidemiology, health related behaviors and ...